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A 2-day-old girl was evaluated for a decreased red reflex in the right eye. She was born at term by spontaneous vaginal delivery to a 21-year-old woman who had an uncomplicated pregnancy. There was no family ocular history. On initial examination, pupils were equal, round, and reactive bilaterally with no afferent pupillary defect; however, a right afferent pupillary defect was noted on subsequent examinations. A decreased red reflex was noted in the right eye. Slit lamp examination of the right eye revealed a posterior lenticular membrane with a small area of blood adherent to the lens capsule. The left eye was unremarkable. Dilated funduscopic examination of the right eye showed dense intravitreal hemorrhage and a limited view of the retina. The left eye was remarkable for a small temporal intraretinal hemorrhage but no other peripheral retinal abnormalities such as avascularity, neovascularization, or exudates.\nAt 3 weeks old, the patient underwent examination under anesthesia which revealed a retrolental collection of dehemoglobinized and fresh blood in the right eye. Corneal diameters were symmetric, 10 mm in both eyes. Dilated fundoscopic examination of both eyes was unchanged. B-scan ultrasonography of the right eye confirmed a mobile vitreous hemorrhage with no obvious stalk and a few areas of high internal reflectivity corresponding with a possible retinal detachment; however, no masses were identified (Fig. ). Given the lack of peripheral retinal abnormalities seen on fundoscopic examination in the unaffected left eye, further testing such as fluorescein angiography was not performed. After the examination under anesthesia, lensectomy and vitrectomy were performed in the right eye clearing the vitreous hemorrhage. There was a 360° tractional detachment of the retina in a funnel configuration surrounding a hypoplastic optic nerve. The visible areas of retina were atrophic and showed no retinal vessels or recognizable retinal structures, and repair was not deemed possible. No further intervention was performed.\nOne month later, the infant returned with an inflamed right eye. On examination, her right eye intraocular pressure was 24 mm Hg with a 1-mm layered hyphema. She was managed with prednisolone, atropine, latanoprost, and timolol. At subsequent visits, her right eye was noted to have no light perception, persistently elevated eye pressures to as high as 46 mm Hg, a chronic hyphema, and corneal blood staining. The mother declined further surgical intervention but agreed to continued observation. Four months later, B-scan ultrasonography of the right eye showed a 4.1 × 4.7 mm, well-demarcated, round mass over the optic nerve with high internal reflectivity and without any frank calcifications (Fig. ). The mass was thought to represent residual retinal dysplasia, but retinoblastoma could not be ruled out. CT and MRI imaging showed a well-demarcated spherical mass over the right optic nerve head. Retinoblastoma could not be ruled out. Enucleation of the right eye was recommended; however, the patient's mother declined surgery.\nWhen the patient was 4 years old, her right eye became more red and painful. External examination of the right eye was notable for ptosis, band keratopathy, and chronic hyphema covering 50% of the anterior chamber. The mother agreed to proceed with right eye enucleation with dermis fat graft and temporary tarsorrhaphy. Surgery proceeded without complication. Postoperatively, the patient healed well and was fitted for a prosthetic eye. The patient was never found to have any other medical conditions and met all her developmental milestones.\nPathology revealed a completely detached, folded retina with atrophy and gliosis (Fig. ). The anterior chamber angle was closed as a result of iris neovascularization and peripheral anterior synechia. Additionally, there was posterior synechiae with seclusio pupillae (Fig. ). There was fibrous metaplasia with focal areas of ossification of the retinal pigment epithelium present. The retina showed prominent abnormal intraretinal vascular proliferations in the folded detached retina (Fig. ). The atrophic retina showed areas of infolding and dysplasia in the area of detachment (Fig. ). Additionally, a preretinal fibrovascular membrane was also present (Fig. ). Remnants of lens capsule and cataractous lens were present. There was no evidence of persistent fetal vasculature or a posterior lenticular capsular plaque. The optic nerve showed atrophic changes secondary to marked retinal atrophy and gliosis. There were no subretinal exudates seen. The overall findings of congenital retinal detachment with a retinal fold were consistent with FEVR. |
The patient was a 65-year-old man with a prior history of repeated plastic surgery for scar contracture of the hands and fingers, ablation surgery for idiopathic ventricular tachycardia, and diabetes. There was no family history of no consanguineous marriage or EB.\nHe had experienced recurrent blistering of the skin that was readily caused by an external force since the time shortly after birth, which had been treated symptomatically. He was diagnosed with EB during a genetic consultation that he had received before getting married at the age of 28 years. Subsequently, he was diagnosed with RDEB at the age of 38 years. Application of a strong external force to the skin results in blister formation as early as 15 min. In June 2012, he presented to a nearby hospital with epigastric pain, where he was diagnosed with cholelithiasis and cancer in the transverse colon and was referred to our hospital. In September 2012, transverse colectomy and cholecystectomy were performed via laparotomy, followed by an uneventful postoperative course. In April 2013, a liver metastasis (S2) was detected. The lesion was a solitary tumor measuring ≤2 cm and was treated by radiofrequency ablation (RFA) in June 2013, again followed by an uneventful postoperative course. In September 2015, a recurrent tumor was detected at the site of RFA, with suspected invasion into the diaphragm. He was then admitted to our hospital for curative open surgery. On admission, although no active blistering was noted, pigmentation and scars due to recurrent blistering were noted especially in the extremities and back. Most fingers in both hands were club-shaped, with a few intact fingers. Blood test showed a mild increase in glucose to 123 mg/dl and increases in tumor markers, including mean levels of CEA and CA19-9 of 25.0 (0–5) ng/ml and 62.1 (0–37) U/ml, respectively.\nAbdominal CT/MRI revealed a 3.5 cm metastatic liver carcinoma with diaphragmatic invasion in the lateral segment of the liver. In January 8, 2016, open partial hepatectomy of the lateral segment with combined diaphragmatic resection was performed.\nThe patient was asked to climb on the operating table on his own to minimize application of an external force to the skin. Epidural anesthesia was achieved by just one injection of 5 ml of 0.5% procaine into the epidural space. For endotracheal intubation, due to a difficulty in manually fixing a mask and lifting the lower jaw, the patient was asked to open his mouth and intubation was performed while the patient was conscious using intravenous injection of 1% propofol and intratracheal spraying of 1% xylocaine, under bronchoscopic guidance using a McGRATH™ MAC video laryngoscope (Covidien). Isodine disinfectant was used for skin disinfection of the surgical site, as the patient was tolerant of chemical stimulations. A skin incision was made sharply with a scalpel, with particular care taken to avoid contact of a steel instrument with the skin. Partial hepatectomy of the lateral segment with combined diaphragmatic resection was performed. The diaphragmatic defect was closed with a 2-0 nonabsorbable suture while the lung was compressed, without chest tube placement. A 19 Fr closed low-pressure continuous-suction drain was placed on the liver resection surface. A block catheter was also placed on the bilateral rectus sheaths in case of postoperative wound pain. The wound was closed by two-layer suturing with a 0 monofilament absorbable suture for the peritoneal muscle layer and a 4-0 monofilament absorbable suture for dermal closure. The wound was covered with a Mepilex® Border Ag dressing (Mölnlycke Health Care). The drain was fixed with a needle and a suture and then with a Mepitac® tape (Mölnlycke Health Care). The operative and anesthetic times were 346 and 457 min, respectively.\nThe patient was discharged from the hospital on day 9. He had an uneventful postoperative course with no abnormality of the wound in postoperative outpatient examination. |
A 43-year-old female presented to the emergency department with a nearly full body rash, including the vaginal introitus and perianal skin. The patient described the rash as “blistered and flaky.” She also had peripheral edema, glossitis, cheilitis, and alopecia described as “my hair has been falling out in chunks.” She reported a loss of appetite from the glossitis along with increasing diarrhea and abdominal pain. She further stated that she had recent onset of decreased focus and memory. The patient presented two weeks prior with a similar rash that had been less widespread. She reported that the rash started on her feet and mouth before spreading to her knees and was accompanied by lower extremity swelling, cheilitis, and glossitis. At that time, she was presumed to have scabies and also tested positive for Staphylococcus in a skin sample. She was treated with vancomycin before leaving the hospital against medical advice prior to the completion of workup and treatment. She now returned, two weeks later, with an acute worsening of the rash accompanied by worsening memory and concentration difficulties.\nThe patient had a lifetime history of multiple allergies, food intolerances/sensitivities, asthma, abdominal pain and diarrhea, and rash due to certain foods including dairy products. She stated she had been diagnosed with a liver disease several months before the admission and that she had been taking more than 4000 mg Acetaminophen per day in the months leading up to the diagnosis. It was also noted in several past medical records that this patient had a lengthy history of alcohol abuse. She was also admitted due to anxiety, depression, insomnia and a history of bulimia. She was admitted for an unknown amount of time seven years ago with her first attack of pancreatitis; approximately four years later, she was admitted again for several weeks with an attack of pancreatitis, during which she developed erythema, swelling, and blistering of the dorsal aspects of her feet. Other possible admissions for pancreatitis at regional hospitals were surmised from her history but were unable to be verified. She stated that she had been adherent to a vegan diet for the last 17 years to avoid rashes and diarrhea that accompanied several types of food and in the last few years, she had been unable to afford or acquire good vitamin supplementation. The patient had a history of non-compliance with her pancreatic enzyme replacement regimen due to socioeconomic factors.\nOn initial workup, the patient had a generalized maculopapular rash with a scale that blanched by pressure. She stated that the rash started on her dorsal feet and mouth, and then spread up her legs, genitals and perirectal areas, and distal upper extremities. The rash spared the palms, soles, and face with the exception of glossitis and cheilitis, which were present. Notable labs obtained during the time as an inpatient can be seen in Table .\nA biopsy of an area of rash from the patient’s left leg was performed and analysis revealed the following: epidermal ulcer, spongiosis, dermal fibrosis and mixed inflammation including many dermal eosinophils. Immunoglobulin G (IgG): negative, Immunoglobulin M (IgM): negative, Immunoglobulin A (IgA): negative, C3: negative, fibrinogen: negative.\nClinical course\nThe patient was treated for Mycoplasma pneumoniae with antibiotics and breathing treatments. Pancreatic enzymes were provided and steroid cream was given for symptomatic treatment of the rash. Dermatology was consulted, who ultimately determined that the skin findings represented zinc deficiency dermatitis and recommended oral zinc therapy. The rash demonstrated significant improvement over the next two days and was mostly clear by the third day of zinc treatment. The patient also reported improvements in her confusion and could remember details of her past medical history that she previously was unable to provide. Her hospital course was complicated by hemolysis of unknown etiology. This was possibly related to extrapulmonary Mycoplasma pneumoniae, which was treated with infusion of packed red blood cells and prednisone. Prior to discharge, the patient provided informed consent for the case write-up on her condition and clinical course. |
A 57-year-old female presented with vision loss in the left eye during the restoration of consciousness after endoscopic DCR surgery for the left eye. In this case, the DCR surgery was performed under general anesthesia. Notably, 2 ml of 1% lidocaine with 1:100,000 epinephrine was injected into the axilla of the middle turbinate and the frontal process of the maxilla using a dental syringe. In this case, the neurosurgical patties soaked in 2 ml of 1:1000 epinephrine were inserted between the inferior turbinate and the nasal septum and in the middle meatus to achieve topical decongestion. In the process of making mucosal flap and incision, the patient had a higher bleeding tendency than was noted with other patients, and a suction diathermy was used meticulously for the incidence of hemostasis. For this reason, it did not lead to a major bleeding in this case.\nThe patient’s medical history was notable for thrombocytopenia and MHA. Upon review, the patient denied temporal headache, pain, or flashes. When tested, the patient’s best-corrected visual acuity (BCVA) was 20/20 in the right eye and light perception in the left eye. Her intraocular pressure (IOP) was 14 mmHg in the right eye and 16 mmHg in the left eye. Her visual field test result was normal for the right eye. However, the test could not be conducted for the left eye due to the incidence of poor vision. When tested with the swinging flashlight maneuver, a relative afferent pupillary defect was found in the left eye of the patient. Her extraocular movements were noted as being full and painless. However, mild periorbital bruising and swelling were detected in the left eye. Additionally, there was mild maxillary sinusitis noted as well. However, it was shown there was no underlying disease in the other sinuses. On the funduscopic examination, there were no obvious abnormal findings in the macula of either eye. The use of a fluorescent angiography did not reveal leakage or a filling defect at the disc. The baseline testing included blood tests to evaluate syphilis, systemic lupus erythematosus, and neuromyelitis optica. Her erythrocyte sedimentation rate and C-reactive protein results were noted as normal. Her pre-operative platelet count was 61 × 103/mm3. A chest x-ray was performed to evaluate sarcoidosis. She was transfused with six units of platelets preoperatively, which increased her platelet count to 123 × 103/mm3. No other cause of optic neuropathy was found in this evaluation.\nThe pattern visual evoked potential revealed delayed P100 latency (Fig. ). Her electroretinogram showed normal electrical activity in the retina. The magnetic resonance imaging (MRI) of the orbit revealed a focal hyperintensity within the intra-orbital segment of the left optic nerve on the T2-weighted image (T2-WI) and flair image. At evaluation, the MRI showed an enhancement on the T1 post-contrast imaging (Fig. ). It did not show any demyelinating disease in the brain. The patient was diagnosed with left optic neuropathy and treated with 1 g/day of intravenous methylprednisolone for 3 days, followed by 1 mg/kg/day of oral prednisone with subsequent dose tapering. It is noted that the patient’s BCVA improved to 20/30 after the treatment. Although her vision improved, she was left with a visual field defect in the left eye. |
A 73-year-old woman was admitted to the hospital after sudden unconsciousness and vomiting without a preceding trauma. The patient was able to open her eyes and bend her limbs, but only after painful stimuli. The patient’s Glasgow coma score was 8 and a head computed tomography (CT) scan showed intraventricular hemorrhage (). An external ventricular drain was placed immediately and then the patient was transferred to the Intensive Care Unit (ICU). Five days later, the patient regained consciousness and became cooperative. At this time, the neurological examination failed to show any strength deficits. The endotracheal tube was removed and the patient was transferred to the general ward for enhanced recovery. Two days later, a head CT scan revealed the elimination of the intraventricular hemorrhage, resulting in the external ventricular drainage tube being removed.\nHowever, 3 days later, the patient presented acute respiratory failure with shortness of breath. The patient’s oxygen saturation was approximately 75% and her temperature was 38.5°C. In addition, the patient’s blood pressure was 99/60 mmHg with a heart rate of 110 bpm. Arterial blood gas analysis showed that the PO2 was 59 mmHg and the PCO2 was 49 mmHg. An emergent head CT scan excluded intracranial re-hemorrhage (). A pulmonary artery embolism was also excluded by pulmonary CT angiography (CTA). Despite an attempt of non-invasive ventilation, the patient deteriorated and required intubation with mechanical ventilation. During the following days, the patient was conscious, but with a Glasgow coma score of 4 + T + 6 and bilateral limb weakness (muscle strength was 1/5 the strength in the upper and lower extremities). Tendon reflexes were absent. The neck muscle strength and sensation in the limbs were normal. Eye movements and corneal reflexes were intact. Serum sodium was within normal limits, but the serum potassium was low (2.65 mmol/L). After the correction of hypokalemia, the patient’s bilateral limb strength worsened (muscle strength was 0/5, the strength in the upper and lower extremities). A lumbar puncture was then performed because of the worsening clinical condition. Examination of the cerebrospinal fluid (CSF) showed albuminocytologic dissociation with a cell count of 8 × 106 cells/L (normal range is below 8 × 106 cells/L) and a total protein level of 107.3 mg/dl (normal range is 8–43 mg/dl). An extensive series of serum antibodies against ganglioside were negative, including GQ1b, GT1b, GD1b, GD1a, GM1, GM2, and GM3. The result of a creatinine kinase test was within normal limits (48 U/L; normal range is below 145 U/L). Unfortunately, the electromyogram failed because of substantial logistical difficulties. In addition, the relatives of the patient denied any history of recent viral illnesses and any other previous diseases.\nThe patient was treated with intravenous immunoglobulin (0.4 g/kg/day) for 5 days. Despite therapeutic intervention, the limb weakness persisted and the patient required continuous ventilator assistance beyond the 5 days of immunoglobulin treatment. Subsequently, a tracheotomy was performed. One month after the operation for the intraventricular hemorrhage, a head CT scan revealed a significant decrease of the intraventricular hemorrhage (). A head CTA, which was performed to identify the cause of the cerebral hemorrhage, only showed cerebral arteriosclerosis (). Five days later, the patient was transferred to the local hospital for further rehabilitation therapy. Unfortunately, the patient passed away after the 1 month follow-up. |
A 44-year-old woman visited the Department of Conservative Dentistry because of tooth avulsion of the left maxillary central incisors after an injury caused by falling down one day before visiting the dental office. The patient brought the avulsed tooth wrapped in tissue paper. The tooth was totally dry. Clinical and radiographic examination revealed that tooth #21 was avulsed, and a blood clot was found in the alveolar socket (). Debridement of the contaminated root surfaces, endodontic treatment, and resin-wire splint were performed. At the 8-week recall, mobility of tooth #21 had increased and periapical radiographs revealed that there was an obvious radiolucent band around the root surface (). It was diagnosed as a failure of replantation on #21. The patient was referred to the Department of Prosthodontics for further treatment. However, the patient did not show up for 10 months for personal reasons.\nOne year after the trauma, the patient came to our department again for checkups. The patient was free of symptoms, and #21 was retained by a lingual fixed retainer, which was made at a local clinic. Clinical examination showed a gingival recession of about 4 mm on #21 and full probing depth around #21. Additionally, a black triangle was formed between #11 and #21 (). The patient complained about the conventional treatment options and refused both conventional fixed partial prosthesis and implant restoration. In case of treatment with conventional fixed partial prosthesis, excessive preparation of the adjacent tooth was necessary for crowding relief, and additional endodontic treatment was required. She refused this option because of the tooth preparation of the adjacent teeth. When we considered an implant, additional bone graft was necessary due to the loss of the buccal plate in the extraction socket area. In addition, she was anxious about implant treatment due to past experience during implant surgery on the left mandibular second molar. Also, due to crowding on the left maxillary lateral incisor, implant placement was particularly difficult. After discussing all treatment options with the patient, the FRC fixed partial prosthesis was chosen. An impression was made with alginate for the FRC fixed partial prosthesis fabrication. It was possible to avoid occlusal stress on restoration because the avulsed tooth was out of contact ().\nAfter local anesthetizing the area, the tooth was extracted. A piece of sterile gauze was gently packed into the extraction site to prevent bleeding. After hemostasis, the prefabricated temporary composite crown was held in position attached to the adjacent teeth with direct composite, and then, the interproximal gingival contour was reformed ().\nThirty minutes before the patient's next visit, a composite pontic was fabricated using nanofilled composite (Filtek Z350 XT, 3M ESPE, St Paul, MN, USA) to enhance the cohesion between the lab-fabricated pontic and the intraoral direct-filled composite. The fiber framework (FibreKor, Jeneric/Pentron Inc., Wallingford, CA, USA) was cut to the appropriate length and adapted to the abutment teeth and the pontic area. The framework was covered with a body layer of the pontic and light polymerized. The FRC framework was located in the palatal 1/3 area of #11 and #21, and in the buccal 1/3 area of #21 pontic ().\nWhen the patient arrived, the abutment teeth were anesthetized and the temporary composite crown was removed (). The abutment teeth were prepared using tapered diamond burs (835.31.009, Brasseler, Savannah, GA, USA). The preparations consisted of removing approximately 1.0 mm of the lingual surface of the abutment teeth to ensure adequate space for the placement of the fibers and composite resin. All margins were in enamel (). After the preparation, a 37% phosphoric acid gel (ETCH-37, Bisco Inc., Schaumburg, IL, USA) was applied to the enamel margin for 20 seconds. The etchant was thoroughly rinsed off and gently dried. Clearfil SE bond (Kuraray Medical Inc., Tokyo, Japan) was applied following the manufacturer's instructions, and a thin layer of flowable composite resin was placed on the prepared surfaces of the abutment teeth. The prefabricated FRC fixed partial prosthesis () was inserted in the cavity. Fiber was pressed into the unpolymerized flowable composite resin (Aelite Flow, Bisco Inc.) using a hand instrument and cured for 20 seconds. The exposed fiber surfaces on the abutment teeth were covered with Filtek Z350 XT and cured for 40 seconds (). Composite was added to the pontic in order to obtain a more natural shape and shade of the final restoration and to reinforce the connector area of the interproximal surface. Occlusal adjustment was made using articulating paper and diamond finishing burs, and surfaces were polished with Soflex (3M ESPE) ().\nAt the 1-year recall, the patient stated that she was satisfied with the appearance and function of the prosthesis. The pontic area and resin-bonded margins were clinically sound, and the abutment teeth were intact (). The pontic was in good condition, and the desired shade of the teeth had been maintained. |
A 74-year-old man, in 2013, asymptomatic right ICA stenosis was found and followed up, but in June 2017, progressive stenosis, transient left limb numbness, and an abnormal right visual field appeared. He had a previous history of brachytherapy and tumor excision for oral floor cancer in 2011 and 2014. He also had dyslipidemia, hyperuricemia, and smoking history. He was given aspirin 100 mg and bezafibrate 400 mg daily before and after the surgery. Magnetic resonance imaging (MRI) showed an old deep white matter infarct, but no new lesion was noted, and the right ICA-middle cerebral artery (MCA) was poorly visualized on magnetic resonance angiography (MRA). Single-photon emission computed tomography (SPECT) showed no apparent decrease in blood flow. Cerebrovascular angiograms showed that the right CCA was occluded immediately after its origin and blood was supplied from the right vertebral artery to the ICA through the external carotid artery (ECA) [].\nConsidering the symptomatic CCA occlusion presenting with transient ischemic attack, CEA was selected as a treatment that could achieve revascularization and plaque removal at the same time. In addition, from the results of cerebral angiography, the occluded site extended to the CCA origin, and the proximal end of the thrombus was secured using a partial sternotomy. Under general anesthesia with transnasal intubation, surgery was started under intraoperative electroencephalogram monitoring. The skin incision along the front edge of the sternocleidomastoid muscle (SCM) was extended to the front chest, and the sternum was cut into an L shape from the median cervical incision to the right second intercostal space, which exposed the CCA origin. The treatment of the precordial region was performed jointly with a cardiovascular surgeon. The proximal end of the plaque was confirmed directly under intraoperative echo and indocyanine green (ICG) video-angiography and blood flow from the ECA to the ICA through the bifurcation was confirmed on ICG video-angiography. The ICA, ECA, and CCA were clamped at sites where no blood clots had occurred, an arteriotomy was placed from the CCA to the ICA, and a shunt tube was inserted. When the lumen was exposed, a long plaque from the beginning of the CCA to the ICA was observed, which was excised in one mass. A hemashield was used to repair the CCA-ICA because primary suture was considered to have a risk of postoperative stenosis. Blood flow was confirmed by Doppler echo and the sternum was fixed with a wire [].\nOn magnetic resonance diffusion-weighted imaging after surgery, no new infarction appeared, and visualization of the main artery was improved on MRA. Computed tomography angiography confirmed good patency of the CCA. Pathological findings showed fibrous thickening and calcification of the blood vessel wall near the carotid bulb, and the lumen was narrowed. The elastic fibers in the vascular lumen were torn and ulceration was observed. Red thrombus was observed near the bifurcation, but on the central side, white thrombus rich in fiber formation was observed [].\nThere were no complications associated with the sternal incision and the patient was discharged on postoperative day (POD) 11 with a modified Rankin Scale (mRS) score of 0. There was no new cerebral infarction during the 18 months of follow-up after surgery and no CCA restenosis was observed. |
A 24-year-old female patient was referred to the Department of Periodontics, K.S.R Institute of Dental Sciences with the chief complaint of growth in the gums of right upper front teeth for the past 2 months. She was 10 days old of post-partum phase when she visited the Department of Periodontics. Past dental history revealed of similar growth in the same region which was excised in a private clinic during the 7th month of pregnancy. No history of radiographs taken in the same region. Within a week, same type of growth reappeared and gradually started increasing in size. As the lesion re-appeared, patient was apprehensive that it could be a carcinogenic tumor which was interfering with speech and mastication. She wanted immediate attention toward the lesion and treatment for the same.\nOn extra oral examination, no gross abnormalities were detected. On intra oral examination, diffused gingival growth was noticed between the 12 and 13 region. On the labial aspect, it measures l cm × 2 cm in diameter, red, sessile, smooth and shiny, soft in consistency and bled on slight provocation []. On the palatal aspect, the lesion was similar, but the size is somewhat more pronounced when compared with labial aspect [] Due to the increase in size of the tumor, subgingival local irritants were found more around the involved teeth due to the improper plaque control measures. On periodontal examination, periodontal pocket depths of 7 mm, clinical attachment loss of 5 mm, with Millers Class I gingival recession was associated with the involved 12 and 13 teeth region. Based on the clinical findings, the lesion was provisionally diagnosed as a pregnancy tumor with localized periodontitis and the differential diagnosis was made as peripheral giant cell granuloma, peripheral ossifying fibroma with localized periodontitis.\nRoutine blood investigation was advised and values were found to be within normal limits. Full mouth intraoral radiographic investigations revealed horizontal type of bone loss extending up to the middle third of 12 and 13 teeth region only [].\nPatient was reassured with proper periodontal treatment plan. Scaling and root planning was carried out under phase I therapy and was treated accordingly with plaque control measures. Although plaque control measures improved the periodontal status dramatically, the lesion appeared static. A complete surgical excision was planned.\nUnder local anesthesia, excisional biopsy procedure was performed using electrocautery thereby minimizing the pre-operative bleeding for proper manipulation of the tissues in elevating the flap []. Full thickness mucoperiosteal flap reflected involving 11-13 regions. Root planning and flap curettage was done and irrigated with saline. Flap repositioned with figure of eight sutures and non-eugenol periodontal dressings placed. Post-operative instructions with medications were given. Excised specimen was sent for histological examination.\nHistopathologic examination of the excised specimen confirmed our clinical diagnosis; the specimen stained with hematoxylin and eosin showed partial covering by parakeratotic stratified squamous epithelium of variable thickness which showed areas of ulceration and fibrinopurulent membrane. Under fibrous connective tissue stroma, numerous small and larger endothelium lined channels are formed that are engorged with red blood cells. A mixed population of inflammatory cell infiltrates comprising of neutrophils, plasma cells and lymphocyte is evident. The microscopic appearance was compatible with clinical picture and final diagnosis of pregnancy tumor was made [].\nOne week post-operative healing showed some marginal inflammation with receded gingival margin. The patient was comfortable and the healing was uneventful. Sutures were removed and proper maintenance program was initiated. During recall visits of 3 and 6 months, the surgical site appeared normal without recurrence of the lesion []. |
39-year-old gentleman who noticed progressive difficulty in walking along with tingling sensation in all four limbs since September 2002. He was diagnosed to be suffering from spastic quadriparesis due to cervical spondylotic myelopathy secondary to prolapsed intervertebral disc at C5/6 and C6/7 levels and was operated in July 2003 in another hospital. Through a left anterior approach, C6 corpectomy, C5/6 and 6/7 discectomy and autogenous bone grafting was done along with stabilization using stainless steel plate/screws [Figure , ]. He made good postoperative recovery with improvement in gait, paresthesia, and grip strength. Radiograph done in the immediate postoperative period was showing a stable graft implant construct in proper alignment. In the postoperative period, around 2 weeks, he noticed a discharging sinus from the operation site, which healed completely with antibiotics at around 4 weeks (records of culture/sensitivity of that hospital were not available, but as the sinus had healed well, we presumed the organism had responded to the antibiotic prescribed). As there was clear evidence of postoperative infection at the surgical site, further investigation with a sinogram and computed tomography (CT) scan at this point with proper recording of the investigations could have yielded much better insight about the progression of the disease. This remains a limitation of this study.\nHe developed neck pain along with weakness of all four limbs and incomplete bladder evacuation after 3 months of surgery. His quadriparesis progressed to a completely bedridden state before admission to this hospital. Examination showed diffuse tenderness around the neck and diminished neck movements. Neurologically, he had spasticity with grade 2/5 power in all four limbs. Both the upper and lower limb reflexes were exaggerated with bilaterally extensor plantar and positive Hoffman sign. There was sensory diminution to pinprick C5 downward and he had to be catheterized for urinary incontinence.\nThe total and differential white cell count, random blood sugar, blood urea, serum creatinine, alkaline phosphatase, and serum electrophoresis were within normal limits, except for a very high erythrocyte sedimentation rate (ESR) of 102 mm/h and C-reactive protein (CRP) of 12.1 mg/L. The enzyme-linked immunosorbent assay (ELISA) for human immunodeficiency virus (HIV), Hepatitis B Antigen HBsAg, and hepatitis C virus (HCV), were negative. The radiograph on admission showed C5–C7 instrumented fusion and collapse of C3 and C4 with wedging of the vertebral bodies. C3/4 and C4/5 disc space was diminished. Marked kyphosis with apex at C3/4 and mild forward subluxation of C2 on C3 was also noted []. As the implant was of stainless steel, further assessment with magnetic resonance imaging (MRI) could not be done. CT myelogram was done through lumbar puncture with Iohexol. CT myelogram showed inability of the dye to move upward. The plate and screws were in good position without loosening and bone graft at C6 corpectomy site was well incorporated. Localized kyphosis and soft tissue thickening in front of C3 and C4 were noted [].\nHe was initially put on skull traction for 2 days and repeat surgery was done in December 2003. The patient was examined by an ENT surgeon and normal functioning of vocal cords and intactness of the left recurrent laryngeal nerve was confirmed. Right sided approach was used to reach the cervical spine. Implant removal was done and there was solid fusion demonstrated from C5 to C7. Thickened soft tissues anterior to C3–C4 vertebral body were removed and sent for biopsy. Aspirate from the region was sent for aerobes, anaerobes, tuberculosis, and fungal culture. Destroyed C3 and C4 bodies were removed and bone grafting was done along with titanium plate screw fixation from C2 to C5 []. The bone graft was taken from right anterior iliac crest. He made an uneventful recovery. At the time of discharge, his neurological status had improved from ASIA “C” to ASIA “D”. His grip strength had improved. He was mobilized on a Sternal Occipital Mandibular Immobilization (SOMI) type of brace.\nThe culture report showed growth of Pseudomonas aeruginosa and according to the sensitivity, gentamycin 80 mg BD was given intravenously for 3 weeks as inpatient and then he was switched over to ciprofloxacin 500 mg BD orally for another 3 weeks. Histology ascertained pyogenic osteomyelitis. He was discharged after 3 weeks of IV antibiotics when his consecutive CRP levels (done at weekly intervals postoperatively) started showing a decreasing trend. His CRP level at discharge was 1.2 mg/L. At 6 weeks followup, his neurological status improved to ASIA “E”. SOMI brace was discontinued after 6 weeks and hard collar was given for another 6 weeks. Full activity was allowed at 6 months.\nThe patient was followed up at yearly interval 6th month followup. At his latest followup, (December 2008), his motor power was normal, and he was doing his routine activities satisfactorily. He was back to work. Anteroposterior and lateral flexion extension radiographs were done at followups to check for loosening of implant, signs of recurrence of infection, and adjacent level changes after the long fusion. Most recent imaging confirms adequate decompression of the spinal cord and good fusion from C2 to C7 []. X-rays did not show any instability at C1/C2 at the last followup. |
An 18-year-old previously healthy male presented to the clinic with the complaints of sore throat and generalized malaise. The patient denied any history of alcohol or drug use. He denied any history of recurrent infections. The patient had no history of bleeding diathesis. His platelet count was normal. He had a negative rapid monospot and rapid strep antigen test. He recovered from acute illness in 3 days.\nHe noticed dysphagia and paresthesias of both lower extremities and weakness in all extremities (lower > upper, proximal > distal) one day after recovering from sore throat. On neurological examination, patient had bilateral facial nerve palsy and bulbar palsy. Motor strength was grade 1 in lower extremities and grade 2 in upper extremities. Muscle tone of the limbs was decreased. Deep tendon reflexes on the upper limbs were diminished and absent on the lower limbs.\nBlood chemistry was normal, except for total bilirubin of 2.3 mg/dL, alanine transferase and aspartate transferase levels of 87 IU/L and 67 IU/L respectively, indicating hepatitis, which may be due to acute CMV infection. Test for human immunodeficiency virus was negative. His serology was positive for CMV immunoglobulin M (IgM) and Immunoglobulin G (IgG) antibodies. CMV polymerase chain reaction (PCR) was positive with <2000 copies of deoxyribonucleic acid (DNA). Cerebrospinal fluid analysis was done, and it was positive for CMV IgG antibody and increased amount of proteins. He was treated with ganciclovir for acute CMV viremia and received three doses of intravenous immunoglobulin without much response. Patient had diffuse decrease in sensory and motor amplitudes, low normal conduction velocities, prolonged distal motor latencies in the left upper limb, and absent or prolonged F-wave responses on nerve conduction studies with severely decreased recruitment throughout, and a few positive sharp waves and fibrillation potentials on needle electromyography. These findings were most consistent with an acute diffuse neuropathy, most likely acute inflammatory demyelinating polyradiculopathy in early stage.\nDay 4: Patient developed acute respiratory failure and was placed on mechanical ventilator.\nDay 12: He demonstrated a slow and gradual improvement in his strength mostly in upper extremities after starting plasma exchange daily for 5 days.\nDay 15: He was discharged to a rehabilitation facility. He had bifacial weakness; motor strength was 2/5 in lower and 3/5 in upper extremities. Sensations were intact.\nDay 16: He developed acute onset of abdominal pain in left upper quadrant with high grade fever (103.4 F) and was admitted to the hospital. He was found to have dislodged gastrostomy tube, which was replaced by the interventional radiology. After that, the patient had an exploratory laparotomy. He was started on broad-spectrum antibiotics and culture was sent which grew coagulase negative staphylococcus. The patient continued to have high-grade fevers (102.9 F) despite exploratory laparotomy and aggressive wash out. Repeat CMV PCR was negative. Serum IgG ganglioside GQ1b antibody was <1:100 titers.\nDay 21: Repeat computed tomography (CT) abdomen showed modest sized splenic infarct [Figures and ] and prominent colitis. Patient had a small hypodense focus in the periphery of the spleen on the follow-up CT abdomen on day 25 and day 32, which was consistent with evolution of splenic infarct.\nDay 42: CT abdomen on the day of discharge showed decrease in the size of splenic infarct. Patient's condition was improved gradually and ultimately he was discharged to a rehabilitation facility. |
A 76-year-old woman diagnosed with an aortic arch aneurysm was admitted to our hospital for worsening dyspnea on exertion. The patient presented without hoarseness or symptoms of airway obstruction. The dyspnea had started 2 weeks before admission and gradually worsened until provoked by light exercise. The patient was referred to our department for treatment. Upon admission, the physical examination showed a blood pressure of 95/70 mmHg, heart rate of 100 beats/min, and oxygen saturation of 95% as measured by pulse oximetry. After light exercise, her oxygen saturation decreased to 90%. Laboratory tests showed increased liver transaminase (64 U/L and 100 U/L of aspartate aminotransferase and alanine aminotransferase, respectively) and brain natriuretic peptide concentrations (1967 pg/mL). A computed tomography (CT) scan showed that a large 70-mm diameter saccular aortic arch aneurysm had substantially compressed the periphery of the pulmonary artery and that a large thrombus had protruded into the main trunk of the pulmonary artery. No other CT findings of chronic pulmonary thromboembolism, including abrupt narrowing, obstruction, or calcified thrombi, were found in the pulmonary artery branches (). Echocardiography showed preserved left ventricular ejection fraction of 61.5%; decreased left ventricular dimension of 26.6 and 18.2 mm in diastole and systole, respectively; profound pulmonary hypertension characterized by a right ventricular systolic pressure of 95.9 mmHg; moderate to severe tricuspid regurgitation; and pulmonary stenosis indicated by a peak velocity of 3.84 m/s and a peak pressure gradient of 59.2 mmHg. Echocardiography did not show intracardiac or extracardiac left-to-right shunt flow. The patient was preoperatively diagnosed with a large saccular aortic arch aneurysm that caused the pulmonary stenosis. An urgent aortic repair was scheduled on day 6 of hospitalization.\nDuring anesthetic induction, the patient’s systolic blood pressure was temporally decreased to 40 mmHg. During the surgery, transesophageal echocardiography showed no intracardiac or extracardiac left-to-right shunt flow. A median sternotomy was performed, and a serous pericardial effusion was found in the pericardial space. The ascending aorta was intact on inspection. A cardiopulmonary bypass was established with an ascending aorta cannulation, bicaval venous cannulations, and placement of a left ventricular vent via the right superior pulmonary vein. The patient’s body temperature was cooled down to a tympanic temperature of 26°C, and the cardiopulmonary bypass was arrested. The ascending aorta was incised, and a selective cerebral perfusion via the three supra-arch branches was initiated; the cerebral circulatory arrest time was 5 min. Aortic arch opening revealed a thrombus filling the entrance of the saccular aneurysm at the lesser curvature of the aortic arch. First, the aortic wall was trimmed at the distal portion of the thoracic aorta over the entrance of the saccular aneurysm, and a four-branched 26-mm vascular prosthesis (Japan Lifeline Co., Ltd., Tokyo, Japan) was then anastomosed to the distal thoracic aorta. After the distal anastomosis, the lower body perfusion was resumed via a side branch of the prosthesis; lower body ischemia time was 59 min. After removing the thrombus from the aneurysm, a 3-cm diameter foramen was detected at the bottom of the aneurysmal sac in contact with the lumen in the main trunk of the pulmonary artery (). No obvious pus or tumor was present inside or surrounding the aneurysmal cavity. She was diagnosed with ductus arteriosus aneurysm. The pulmonary stenosis was considered to have been caused by not only the external compression but also the thrombus protrusion into the pulmonary artery. The foramen in the anterior wall of the pulmonary artery was closed using a vascular patch () and proximal anastomosis was performed. Following tricuspid annuloplasty, the vascular prosthesis was declamped; the myocardial ischemia time was 170 min. The three supra-arch vessels were reconstructed. The duration for cardiopulmonary bypass, selective cerebral perfusion, and surgery were 274, 199, and 440 min, respectively.\nThe postoperative CT scan showed disappearance of the pulmonary artery stenosis and successful placement of the three-branched vascular prosthesis (). Echocardiography confirmed a 33-mmHg decrease in right ventricular systolic pressure and reduction in trivial tricuspid regurgitation. The preoperative dyspnea experienced by the patient on exertion was relieved. The pathological examination showed that the thrombus protruding into the pulmonary artery was composed of thrombotic materials alone without any vascular wall components. Postoperatively, the patient experienced a parietal lobe stroke. However, she gradually recovered after rehabilitation and was discharged on postoperative day 61. |
A 10-year-old girl presented with a 2-day history of constant right-sided flank pain with intermittent episodes of increased intensity. Movement and laying supine exacerbated the pain, whereas some relief was found with paracetamol. There was no trauma to the area, but the patient reported playing netball shortly before the pain started. There was no associated nausea, vomiting, or change in bowel or urinary habit. There was no fever and no symptoms of cough or breathing difficulties.\nThe patient had no significant medical history and immunisations were up to date. On examination, she was apyrexial with a heart rate of 110 beats per minute, oxygen saturations of 99% on room air and respiratory rate of 22 breaths per minute. She was warm and well perfused with a central capillary refill time of <2 seconds. She appeared distressed when laying down reporting worsening pain; however, the abdomen was soft and non-tender to palpation. Systemic examination was otherwise unremarkable.\nUrine analysis showed no evidence of infection and blood tests revealed a C-reactive protein (CRP) of 23 mg/L and a normal full blood count with white cells of 13.4 × 109 cells/L. Liver function tests, urea and electrolytes, and venous blood gas were also normal.\nWith normal observations, examination and investigation results and a good response to analgesia in the department, the patient was discharged with a planned review.\nOn re-assessment the following day, she continued to complain of significant pain on the right side of the abdomen with similar features on examination. Owing to the persistence of symptoms, chest radiography (Fig. ) and abdominal ultrasound were performed. Both were reported as normal. With no surgical or medical cause of the pain identified, it was deemed musculoskeletal in origin, associated with playing netball. She was discharged with advice to take regular analgesia and to return if symptoms were not settling.\nThe patient represented the following day looking unwell. She appeared very pale with dry lips and walked slowly, hunched over. She was tachycardic at 130 beats per minute and tachypnoeic with a respiratory rate of 32 breaths per minute. Oxygen saturations, temperature, and blood pressure were normal at 98%, 36.80C, and 119/72 mmHg respectively. She stood with her right hip and knee slightly flexed, with her trunk deviated to the left giving her a marked scoliosis. Musculoskeletal examination of the spine, hips, and knees was unremarkable, but it was noted that abdominal pain worsened on flexion of the hip against resistance. Cardiovascular and respiratory examinations were normal. She was unable to lie supine because of severe pain and was examined in the semirecumbent position. Active distension of the abdomen and coughing further exacerbated the pain and she was found to have guarding with rebound tenderness in the right flank and hypochondrium. An intravenous cannula was inserted and intravenous fluids, paracetamol, and ibuprofen were commenced while awaiting a surgical review for her acute abdomen. Concurrent orthopedic opinion was also sought because of the new-onset scoliosis.\nAfter treatment the patients’ pain was significantly reduced, pallor had resolved and her observations normalised. Abdominal examination at the time of surgical review was normal, but because of repeated presentations with abdominal pain and a rising CRP (93 mg/L) and white cell count (13.9 × 109 cells/L) the patient was admitted and a computed tomography (CT) scan of the abdomen and pelvis was arranged. Orthopedic review did not yield any acute orthopedic concerns regarding the scoliosis and they agreed with the plan for abdominal imaging. The CT scan failed to reveal an abdominal cause for her pain, but to our surprise showed an organizing right lower lobe pneumonia with a small effusion (Figs. and ).\nThe patient was started on intravenous antibiotics for the thus far silent pneumonia. Despite treatment during the next few days, the pneumonia progressed and she developed a large parapneumonic effusion (Figs. and ). On the 6th day of hospital admission, she deteriorated rapidly with signs of sepsis and respiratory distress. This was accompanied by a further rise in CRP (351 mg/L) and white cell count (17.3 × 109 cells/L). She was stabilized and then transferred to a tertiary hospital for further management.\nAt the tertiary center, a chest drain was inserted and the patient was treated with intrapleural urokinase therapy and intravenous antibiotics. She was discharged after a 2-week stay.\nWith the complete resolution of the pneumonia and scoliosis, the patient made a graduated return to school and at 4 months post discharge was almost back to full-time attendance. |
The patient was an 80-year-old male who had been followed up for hypertension and dyslipidemia. His height was 167 cm, his weight was 69.7 kg, and he had a surgical history of appendectomy for appendicitis. Anemia was observed in the conjunctiva. The abdomen was flat and soft without tenderness. There was a surgical scar from the appendectomy. No superficial lymph node was palpated.\nIn the blood test, the Hb level was 10.2, suggesting anemia. AMY at 66 IU/L was within the reference range, and no abnormality in other blood biochemistry tests was observed. Tumor marker levels were CEA 1.9 ng/mL and CA19–9 13.6 U/mL, both within their reference ranges. A pedunculated gastric tumor was detected in the upper gastric body with upper gastrointestinal endoscopy (Fig. ). Reddish changes, which may indicate epithelial tumors, were detected on the apex of the tumor. Ulcer formation was observed, and invasion to the submucosal tissues could not be ruled out. There was no obvious bleeding detected in this examination. A biopsy was performed, and histopathological malignancy was not revealed. A kind of gastric gland cell hyperplasia was diagnosed, probably because it seemed that no significant atypia was found in the specimen.\nOn abdominal/pelvic computed tomography (Fig. ), a neoplastic lesion of 5 cm in diameter was observed in the upper gastric body. As it was located on the pyloric side of the upper gastric body, pyloric obstruction was also suspected. There was no finding suggesting a pancreatic tumor, lymphadenopathy, or metastatic lesions. No ascites was observed.\nA surgical procedure was arranged for the following reasons: bleeding from the tumor was suspected as a cause of anemia, ball valve syndrome had been induced by the tumor, the possibility of malignancy could not be ruled out, and the risk of incomplete resection or bleeding with endoscopic resection was considered high.\nLaparoscopic endoscopic cooperative surgery was performed. A tumor incarcerated from the greater curvature of the upper gastric body to the duodenum was guided into the gastric cavity under endoscopic observation. Full-thickness resection was performed while maintaining an approximately 10-mm margin. The resected site was closed with layer-to-layer sutures.\nMacroscopically, an elastic-hard grayish-white solid neoplastic 3.5 × 3.3 × 2.5-cm lesion protruding inside the gastric cavity was observed. Some parts were edematous on the sectional surface, and no necrosis or bleeding was observed (Figs. , ).\nWhole-section slides from the resected tissues were made. Histologically, the lesion consisted of a remarkable proliferation of several types of epithelial cells in the mucosa and submucosa. The border of the lesion was very irregular in the mucosa or the superficial layer of the lesion; however, it was clear in the submucosa or middle and deep inside layer of the lesion with partial fibrosis and a capsule. The propria muscularis was not involved. Proliferating epithelial cells showed basically acinar-glandular, trabecular and solid patterns (Fig. ). These patterns were seen separately in some parts and intermingled with one another in other parts. Small amounts of stromal mucus deposition were found in some parts of the superficial layer of the lesion. Regarding the general tendency, the solid pattern was found mainly in the mucosa or the superficial layer of the lesion, while the acinar-glandular and trabecular patterns were observed in the submucosa or deep inside of the lesion. Since the morphological atypia of these proliferative epithelial cells was not significant and these cells resembled pancreatic acinar cells well, the possibility of a special type of ectopic pancreas was considered first. However, normal pancreatic cells (e.g., acinar, ductal, islet cells) that support a diagnosis of heterotopic pancreas were not observed at all.\nThe cells that proliferated as acinar-glandular and trabecular patterns (Fig. a and b) contained eccentric nuclei, which appeared to be slightly enlarged, presenting conspicuous nucleoli, as well as eosinophilic granules inside the cells. The cells that proliferated as the solid pattern (Fig. c) had oval nuclei, presenting salt and pepper-like chromatin that was distributed heterogeneously and slightly eosinophilic granules inside the cells. Aggregations/mixtures of small cells and large cells were also observed on an image (Fig. d). Although significant atypical cells were rare for each type, the characteristics of the nuclei and nucleoli of the cells mentioned above and irregularity of the border of the lesion in the mucosa or the superficial layer led us to the idea that the lesion may be acinar cell carcinoma with some neuroendocrine features.\nTo confirm this idea, immunostaining was performed using various pancreas-related markers or neuroendocrine markers. Immunostaining tests revealed that most of the lesion was strongly positive for BCL-10 antibody (Fig. a) and trypsin but negative or weak in some small parts of the superficial layer of the lesion. In addition, strongly positive results were obtained for chromogranin A, synaptophysin, and CD56 neuroendocrine markers, especially in the superficial layer of the lesion, in which the negative or weak parts for BCL-10 and trypsin were almost positive. Regarding neuroendocrine reactivity in this case, chromogranin A was most remarkable among the three neuroendocrine markers, suggesting that it accounted for 40% of the lesion (Fig. b). Concerning the mitotic rate and Ki-67 index of the lesion, the proportions of acinar-glandular and trabecular cells proliferating mainly in the submucosa or middle and inside of the lesion were < 2 and < 3%, respectively, while those of solid cells proliferating mostly in the mucosa or superficial layer of the lesion were mainly 2–5 and 3–20%, respectively. Moreover, in some small parts of the layer, the proportions were > 20 and 20–40%. Epithelial markers such as CK7, CK20, CAM5.2 and AE1/AE3 were positive in the entire lesion as well as in the existing gastric mucosal epithelium. CK19, which is a pancreatic ductal marker, showed positivity in some parts of the superficial layer of the lesion and the existing gastric mucosa but negativity in the middle or deep inside of the lesion.\nAdditionally, the tests for pancreatic and digestive tract-related hormones (e.g., insulin and gastrin) were negative. No intranuclear positive finding was observed with beta-catenin either. The immunostaining tests are presented in Table .\nThe nearest resected margin from the lesion was 0.5 cm and free from the tumor. No fundic gland was observed in the existing gastric mucosa. The mucosa consisted of crypt epithelium with intestinal metaplasia and hyperplasia, and no obvious malignancy was detected.\nRegarding the tumor tissues presenting acinar-glandular and trabecular patterns, a diagnosis of pancreatic-type acinar cell carcinoma of the stomach was made according to the characteristic findings, such as clear and swollen nucleoli and the results of immunostaining tests. Most of the tumor tissue that presented a solid pattern showed neuroendocrine tumor (NET) G2 characteristics as well as acinar cell carcinoma. However, some parts of that area showed the characteristics of NETG3 without those of ACC.\nConsidering that each component accounted for more than 30% and that both of them had been mixed and shifted with each other, a diagnosis of pancreatic-type mixed acinar neuroendocrine carcinoma (pancreatic-type MiNEN) of the stomach (gastric MiNEN) was made.\nPostoperative course: No perioperative complications were observed; the patient started consuming meals on day 3 after the operation, and on day 13, he was discharged from the hospital. In the blood test, the Hb level had increased to 14.1, suggesting that his anemia had been resolved. Unfortunately, the patient died of brainstem hemorrhage 4 months after the operation. No autopsy was performed. |
A 68-year-old male patient was referred to our department from neurosurgery due to the occurrence of diplopia 10 days after a head surgery that was performed following a pedestrian traffic accident. On the day of the initial trauma, the patient was admitted to the intensive care unit after neurosurgical evaluation, because of a compound comminuted depressed fracture of the right temporal bone. In the initial ophthalmologic examination, there were no ocular symptoms. On day 4 after trauma, an open reduction and internal fixation were performed on the temporal bone fracture by the neurosurgeon. On day 2 after neurosurgery, the patient complained of diplopia and orbital computed tomography (CT) revealed bilateral orbital superior wall fractures. In contrast to the fact that a herniation of the brain parenchyma was unclear on the initial facial CT scan (Fig. a), the fracture fragment and the brain parenchyma were downwardly moved into the orbit, observed on CT scans taken when diplopia occurred (Fig. b). Upon physical examination at the time of admission to the department of oral and maxillofacial surgery, right eye movement limitation and right eye protrusion were observed (Fig. a, c). The surgical plan was to reconstruct the bilateral medial orbital wall using a titanium mesh via coronal approach. For better fitness of the titanium mesh, the mesh was contoured preoperatively on a model of the patient’s skull that included the orbital wall defect. The defect on the model was restored using a plate wax (Fig. ); following pre-operative manipulations, the mesh was sterilized.\nThe reconstruction of the orbital wall was performed 1 month after trauma. A bicoronal approach was attempted in order to easily access the tissue on the right orbital wall. Due to severe tissue adhesion, a craniotomy was performed on the frontal bone to approach the anterior cranial base, even though this is a more invasive approach. Despite utilizing this approach through the anterior cranial base, sufficient tissue dissection was not achieved due to severe adhesion. We thus decided to remove the superior orbital rim in order to secure the operating field, which was successful. Before the osteotomy of the superior orbital rim, a miniplate for fixation of the bony fragment was prepared to reposition the fragment in its original position. After osteotomy, strong adhesions between the brain parenchyma and orbital contents were found (Fig. a). Further forcible dissection of the adherent tissue was expected to cause damage to the meninges and parenchyma, so after a neurosurgery consultation, neurosurgical procedures were performed in order to dissect the adherent tissue, remove the fractured fragment and necrotic brain tissue, and repair the damaged meninges (Fig. b). After the adhered tissue was dissected, the superior orbital wall was reconstructed with a pre-prepared titanium mesh, and the superior orbital rim bone fragment was placed in the original position with a miniplate (Fig. c).\nIn the left superior orbital wall fracture where the tissue adhesion was not severe, tissue dissection was completed without an osteotomy of the superior orbital wall rim. The titanium mesh was placed and fixed through conventional methods. After fixation of the fracture fragment, which had been obtained from the craniotomy site, the surgical site was closed.\nWe could confirm the improvement of the exophthalmos immediately following surgery (Fig. d) as the CT scan taken immediately after the operation showed that the brain parenchyma that had been protruding into the orbit returned to its normal position (Fig. c). Postoperative diplopia and exophthalmos were improved, and the patient was discharged without complications. At 3 months after surgery, further improvements in diplopia and exophthalmos with no limitation of ocular motility were found (Fig. b, d). |
A 19-year-old male presented with gross total painless hematuria of a 5 days duration. There was no past family history of cancer. General physical examination and systemic examination were normal. Blood workup showed anemia. Renal function and liver functions were within normal limits. An ultrasound showed a polypoidal mass attached to the anterior wall of the bladder of 7 × 5 cm in size, which was further confirmed by a contrast enhanced CT scan (CECT) of abdomen. There was no evidence of lymph node or visceral metastasis. Transurethral resection of the bladder mass was performed. The upper gastrointestinal tract (GI) and lower GI endoscopy was within normal limits. A bone scan did not show any skeletal metastasis. Thereafter, the patient underwent robot assisted partial cystectomy and bilateral lymph node dissection till aortic bifurcation. The histopathology was suggestive of a high-grade urothelial carcinoma with six out of seven nodes showing metastasis. Post operatively, the patient developed fever and intestinal obstruction, initially managed conservatively, however, the patient did not show improvement. A repeat CECT abdomen was done which showed soft tissue lesions in both lungs, with pleural effusion, multiple liver lesions and ascites, suggestive of disseminated metastasis. The patient's general condition deteriorated, and he subsequently succumbed to his disease. An overview of the medical disease history is illustrated in a timeline (). Photomicrographs of the tumor from the urinary bladder showed a high-grade urothelial carcinoma with plenty of large pleomorphic cells and infiltrating the detrusor muscle ().\nWhole-exome sequencing (WES) analysis of the paired tumor-normal sample from the patient was performed. A detailed description of the sequencing methods is provided in the . WES data analysis revealed 558 exonic somatic mutations, of which 360 missense, 26 nonsense, 30 frameshift deletions/ insertions and, also 10 splice site mutations were annotated (). Thirty mutations are reported in COSMIC database () including in genes, such as TP53, ABL1, ARID5B, and P2RX7 (). In addition, using Cancer Genome Interpreter (), we predicted eight potential driver mutations among all the somatic mutations detected in this rare tumor. These predicted driver mutations including loss-of-function mutations in TP53, RB1, MED23, CTNND1 and activating mutations in NSD1and MED17 (). The TP53 p.V157L a known oncogenic mutation was identified as a recurrent hotspot in various cancer types (). RB1 is involved in the regulation of the cell cycle checkpoint and DNA damage response. The RB1 c.1498+1G>T alteration is likely oncogenic. Mutations in RB1 is associated with poor overall survival in patients with urothelial carcinoma (). Domain structures of these genes highlighting the predicted deleterious mutations were generated using MutationMapper ().\nGiven that the above predicted driver mutations are in the genes that are limited to already known/predicted cancer driver genes, we carried out a network analysis of 347 genes that harbor a missense mutation using the STRING database. An analysis of the enriched interaction network was performed against the whole genome genes and the enrichment of ion channel pathways was identified (). Ion channels play a pivotal role in regulating self-sufficiency in growth, insensitivity to anti-growth signals, evasion of apoptosis, limitless replication potential, sustained angiogenesis, tissue invasion and metastasis (, ). We identified somatic alterations in 22 genes involved in the ion channels. shows the list of seventeen missense and one frameshift insertion somatic mutations in genes involved in the ion channels. The human genome encodes approximately 328 ion channel genes (). Mutated genes in this patient belong to 11 groups of ion channels (). We generated the ion channels interaction network of 141 genes () comprising of 11 groups using STRING database. Interaction network shows the highly connected network of voltage-gated calcium, cation channels, voltage-gated potassium and voltage-gated sodium channels (). Domain structures of nine genes highlighting the predicted deleterious somatic mutations are shown in . |
A 19-year-old male patient had presented to our outpatient department with pain in the right hip and thigh which was of gradual onset, increasing in intensity over a period of 12 months which had worsened over the last three to four months before presenting to us. The clinical examination of the patient had revealed dysmorphic facies with multiple hyperpigmented macules with irregular margins (café-au-lait spots) noticed over the posterior aspect of the trunk (Figures , ).\nTenderness was noted over the hip joint and around the thigh and range of motion at the left hip was restricted with flexion of 0 to 100 degrees, abduction of 0 to 30 degrees, adduction of 0 to 20 degrees, internal rotation of 0 to 20 degrees and external rotation of 0 to 40 degrees. There was a shortening of the right lower limb by 3 cm. Plain radiographs of the hip showed a varus deformity of the proximal femur with the neck-shaft angle of 88.0 degrees and loss of trabecular pattern with multiple radiolucent ground-glass opacities in the proximal femur were suggestive of fibrous dysplasia (Figure ).\nThe appendicular skeleton and skull were screened for the presence of the asymptomatic lesions which showed a lesion of the left humerus. The clinicoradiological features were suggestive of McCune-Albright Syndrome with polyostotic fibrous dysplasia. Baseline blood investigations performed were within the normal range. The serum alkaline phosphatase was raised at 252 IU/L (50-136 IU/L). We ran an endocrine profile, including thyroid and parathyroid function, adrenal function, serum prolactin, and sex hormone levels. The thyroid profile was suggestive of hyperthyroidism. The patient was advised surgical correction of the deformity and fixation.\nThe plan for surgery involved preparing the paper templates of the deformity by tracing it onto a sheet of white paper from the radiographic films. A lateral closing wedge osteotomy was planned. The patient was put in the supine position on a fracture table. A lateral incision of appropriate length centred over the greater trochanter was used to access the proximal femur. The osteotomy was performed as planned at the level of the lesser trochanter. The lesions were completely curetted and the tissue samples were collected for histopathological examination (Figure ). The bone defects so created were filled with morselised femoral head allografts harvested from the bone bank (Figure ).\nA fibular strut allograft was also incorporated into the lesion. The osteotomy was stabilised with a dynamic hip screw. The final neck-shaft angle achieved was 133 degrees. The biopsy showed curvilinear trabeculae of woven bone in the fibrous background with no osteoblastic rimming. There were spindle cells present in the stroma which were cytologically bland (Figures , ).\nThe patient was allowed to ambulate with protected weight-bearing after eight weeks. The patient was followed up in the outpatient department at regular intervals of six weeks, three months, six months and one year. At six months follow-up, there were signs of bony union and graft integration on radiographs. Functional performance improved with time and the patient could perform his regular activities by 6 months after surgery. At 14 months follow-up, the range of motion showed improvement with painless passive flexion of 0 to 130 degrees, abduction of 0 to 40 degrees, adduction of 0 to 30 degrees and internal rotation of 0 to 30 degrees. The patient remained functionally active without any clinical or radiological recurrence (Figure ). |
A 62 year old gentleman came to the surgical out patient with complaints of abdominal discomfort, occasional left sided abdominal pain for the past 15 days. He had decreased appetite, projectile vomiting, no dyspepsia, no history of fever and bowel habits were normal. On examination, a large mass of 10 × 8 cm was palpated in the left hypochondrium, left lumbar, and umblical region. It was mobile, not moving with respiration and firm in consistency. On palpation of neck, the thyroid gland was found to be enlarged with palpable right lobe. Upper and lower gastrointestinal endoscopy was normal.\nContrast enhanced computed tomography (cect) abdomen showed large lobulated, heterogenously enhancing mass with internal necrosis and calcifications in the left hypochondrium in the region of distal body and tail of pancreas (). There were no other foci of metastasis in abdomen or chest. Chest X-ray of patient was normal. Ultrasound of neck revealed a suspicious nodule in right lobe of thyroid measuring 1*1 cm with no nodal enlargement. Pre-operative ultrasound guided biopsy showed features suggested of poorly differentiated malignancy (that was negative for gastro intestinal stromal tumor markers). His CEA and Ca 19-9 were normal. Fine needle aspiration cytology of thyroid nodule was done under image guidance which was suggestive of papillary carcinoma thyroid.\nAs image guided biopsy of abdominal tumour could not be done patient was planned for laparotomy. At laparotomy, patient was found to have a bilobed tumor arising from the lesser sac adherent to the pancreas and abutting the stomach, transverse colon, and left adrenal and splenic hilum (). The tumor was resected en bloc (distal pancreatectomy and splenectomy). The post operative period for the patient was uneventful. Oral feed started on 3rdpost operative day drain tube was removed on 5thpost operative day. Patient was discharged on the 12thpost operative day.\nThe histopathology showed the presence of very irregularly shaped cells with nuclei that exhibited a “wavy, buckled appearance”. Multiple sections studied showed pancreatic tissue with adjacent fairly circumscribed neoplasm composed of spindle cells arranged in sheets and fascicles. Pancreatic margin appeared free from tumour. There are areas showing alveolar and glandular patterns.There are areas showing hyalinization, necrosis and fibrosis. Tissue organization shows great variability, with hypocellular myxoid areas and areas of major cellularity diagnostic of Malignant peripheral nerve sheath tumour (MPNST). The histologic changes of MPNST include spindle cells with comma-shaped nuclei, tactoid bodies, nuclear palisading, hyaline bands, and schwannoma-like and curlicue foci ().The tumour showed focal S100 positivity,was strongly positive for ki67, was positive for vimentin ( and 5) and negative for cytokeratin,synaptophysin and all GIST markers(CD 117,DOG 1,PDGF) We re-examined the patient thoroughly and there were no cutaneous markers for neurofibromatosis and examination of the eye was normal. Patient was reviewed after a period of 2 months. Positron emission tomography () was done which revealed para aortic nodes and 2 mesentric nodes. It also revealed a metabolically active nodule in thyroid with cervical nodal metastasis. Patient was taken up for total thyroidectomy with functional neck dissection. Histopathology confirmed papillary carcinoma of thyroid with positive lymph nodes. Patient was advised to undergo a radioactive iodine scan which showed 0.3% uptake. Hence patient was started on chemotherapy for para aortic nodes with a regimen of paclitaxel, adriamycin, ifosumide and mesma. Patient has completed 3 cycles till date and is on regular follow up. |
An 18-year-old woman was admitted to our hospital for further management of a genital tract anomaly. Two months earlier, an MRI had been performed for dysmenorrhoea lasting for a year. The MRI revealed 2 separated uterine bodies and cervices. The anatomy of the vagina was difficult to view. Left-sided haematocolpos/haematometrocolpos was noted (66 × 43 × 67 mm). Both ovaries were noted to have small follicles; the dimensions of the right ovary were 31 × 18 × 25 mm, and those of the left ovary were 20 × 34 × 21 mm ().\nThe patient attained menarche at 12 years of age. After menarche the patient experienced slightly heavy and painful regular menstruation every 28 days that lasted for 7 days each.\nMenstrual history the year before the investigations revealed successively increased pain with each cycle, reaching a peak at the 3rd or 4th cycle, during which she experienced very heavy and painful menstruation. A brief spontaneous relief of symptoms was followed by successively increased pain in the manner described before. The patient was not sexually active at the time of the investigation. She denied chronic diseases, medication intake, or any surgical operations.\nA 6-cm lump above the anterior vaginal wall on the left side was felt on bimanual examination. Ultrasound examination revealed the lump to be a cystic lesion with heterogenic echogenicity of the fluid content (dimensions 60 × 50 mm). Diagnostic hysteroscopy under general anaesthesia was considered.\nHysteroscopic examination revealed normal vaginal walls. A small cervix was observed to the right side of the vaginal vault. A hysteroscope was introduced through the cervical canal and reached the uterine cavity. The uterine cavity was lined with normally shaped endometrium and uterine orifice of the Fallopian tube, shown on the right side. Ultrasound-guided aspiration of the fluid in the lump was performed. The fluid had chocolate-like colour and consistency as that of haematoma. The lump’s interior was rinsed with normal saline. Ultrasound examination revealed 2 uterine cavities – the right one was filled with fluid and the left one remained empty. Both cavities had normal endometrial lining. Further extension of the surgery was halted due to bladder injury risk and performance of the procedure in the hysteroscopic unit. The patient was advised to undergo the second step of surgery in the main operating theatre.\nTwo months later the patient was followed up and had no complaints of painful menstruations. Ultrasound examination showed a recurrent haematoma at the same location. Additionally, the second step surgery was postponed due to relief of symptoms. Eight months later, incremental dysmenorrhoea was experienced, and the patient was hospitalized for further management by laparohysteroscopy.\nLaparoscopic surgery confirmed 2 separated uterine bodies, which were fused at the level of internal cervical orifices. Both uterine bodies had regular outlines and were mobile. Each Fallopian tube was attached typically to its corresponding uterine body. Both adnexa were without macroscopic changes (). Then a hysteroscope was introduced through the vagina, and the right-sided component of the uterus was visualized. The total length of the right cervix and uterus was 6 cm. The hysteroscope was introduced through the incision made for the previous procedure to a pouch filled with haematoma. The haematoma was evacuated and the hysteroscope revealed a small uterine cervix. The hysteroscope was introduced further to the left-side uterine cavity and showed the uterine orifice of the left Fallopian tube. The total length of the left-sided uterine body and left cervix was 8 cm. Excision of the partial vaginal septum was performed. The septum was the wall of the blinded space where the haematoma collected. The edges of the wound were sutured by continuous sutures. Bilateral tubal patency was confirmed with methylene blue. The postoperative period was uncomplicated.\nAbdominal ultrasound was performed to identify other possible anomalies. Renal agenesis was diagnosed on the same side of the vaginal septum. The right kidney was typically located with normal echostructure and a total length of 14 cm. Diagnosis of OHVIRA syndrome was established depending on the characteristic congenital anomalies. At the 3-month postoperative follow-up of the patient, no abnormal mass was felt or observed by ultrasound and the patient did not complain of any recurrence of symptoms. |
Eight years ago, a 31-year-old male patient presented with symptoms of dull back pain, insidious in onset, felt in the lower lumbar and gluteal region, accompanied by morning stiffness which initially improved with activity. Within a few months, the problems became persistent and bilateral. With the passage of time, he experienced neck pain and stiffness with restricted mobility. He was diagnosed with having AS based on available criteria. Then, after 8 years, at the age of 39 years, he experienced weakness of all four limbs which started gradually and was progressive for the past 1 year. It started with the right upper limb followed by involvement of the right lower limb. Both left upper and lower limb were also involved sequentially within a few months with progressive wasting and atrophy of muscles incapacitating him to perform his daily works. There was also a history of slurring of speech and occasional choking along with flickering movement of the muscle. Bladder and bowel functions were normal. The patient is nondiabetic but hypertensive. There was no history of chronic obstructive airway disease, tuberculosis, or trauma. There was no history of fever, abdominal pain, convulsion, skin changes, diminished vision, ptosis, redness of the eye, or any episode of bloody diarrhea. He denied any substance or illicit drugs abuse. Family history was insignificant.\nOn clinical examination, general survey was unremarkable. Higher function and cranial nerve examinations were normal. The neck was stiff with restricted movements in all directions. Other signs of meningeal irritations were absent. The cranium was normal, but the spine was curved with concavity anteriorly. In motor system, there was gross atrophy of muscles of the shoulder and pelvic girdles and distal limb muscles without any sensory loss. There was gross atrophy of the tongue, and fasciculation was also seen. The tone was increased. Power was 3/5 in proximal group of muscles in all four limbs with severe weakness in distal muscles with bilateral claw hands []. Deep tendon reflexes in both the upper and lower limb were exaggerated bilaterally. Plantar reflex was extensor bilaterally. Glabellar tap and pout reflexes were present. Cerebellar signs were absent.\nMovements were restricted in all directions in cervical, dorsal, and lumbar spines. Modified Schober test was positive. Small joints of hands were swollen without any signs of inflammation. There was clawing of fingers and toes. No extraarticular manifestation was present.\nInvestigations of routine blood were not remarkable apart from high erythrocyte sedimentation rate of 72 mm. Liver function and lipid profiles were normal. C-reactive protein was positive. Rheumatoid factor and anti-cyclic citrullinated peptide both were negative. Urine and stool analysis were normal. Both antithyroglobulin and anti-microsomal antibodies along with thyroid profiles were in normal range. Analysis of human leukocyte antigen-B27 (HLA-B27) was negative. Electrocardiography showed normal sinus rhythm. Echocardiogram was normal. Electromyography and nerve conduction velocity study of all four limbs revealed chronic diffuse alternating hemiplegia of childhood disease with secondary motor axonopathy. Dual-energy X-ray absorptiometry scan of hip and left forearm showed osteopenia (T score is 1.3 and 1.2, respectively) and lumbosacral spine was normal (T score 0.3).\nChest X-ray showed increased reticular markings. X-ray of the cervical and dorsolumbar spine revealed gross ligamentous calcification of both anterior and intraspinal ligaments along with the loss of cervical and lumbar lordosis []. Shoulder and bilateral knee joint X-rays both were normal. Hip X-ray showed left hip arthritis. Juxtaarticular osteopenia was seen in X-ray hand and wrist. X-ray of SI joint revealed mild sclerosis bilaterally. Magnetic resonance imaging of the spine revealed ankylosis of lower thoracolumbar vertebra with calcified intervertebral discs. Discs were fused in all direction with height reduction. No evidence of cord compression and significant compromise of neural foramina were present. Based on the clinical presentations, examinations, and radiological features, this patient was diagnosed with a case of seronegative spondyloarthropathy, i.e., AS and amyotrophic lateral sclerosis.\nHe was put on methotrexate 10 mg once a week with folic acid and calcium supplementation. He was already on levothyroxine 50 μg once a day plus antihypertensive in the form of amlodipine and losartan combination. At his last turn up in outpatient, bath ankylosing spondylitis disease activity index score remains the same. |
A 73-year-old nulligravid woman presented to our emergency room with severe abdominal pain. The patient recurrently experienced mild abdominal pain for the past week. She was examined at a nearby hospital 5 days before where plain CT scan and blood exams were performed. Her primary diagnosis was traction pain caused by a large uterine leiomyoma. Since her symptoms subsided with pain control medications, she was discharged from the hospital after 4 days of observation. However, she was referred to our facility the next day with complaints of a worsening abdominal pain. The patient had a medical history of an untreated large uterine leiomyoma diagnosed 25 years ago. She had stopped visiting her gynecologist after menopause at the age of 55. Although she felt a growing mass in her abdomen, it had been asymptomatic until the current episode, except for the sudden appearance of an umbilical hernia a few years ago. On admission, the patient was generally stable with normal vital signs. The abdomen was soft with no rebound tenderness or rigidity; however, a large mass in the left lower quadrant and an umbilical hernia were noted. Pain was localized over the abdominal mass, which was approximately the size of a second-trimester pregnancy and was easily movable by palpation. No vaginal bleeding was observed. Ultrasound imaging showed an abdominal mass > 10 cm in size and free fluid in the abdominal cavity. Blood exams () indicated signs of inflammation with elevated white blood cell (WBC) count (21500/μL) and C-reactive protein (CRP) levels (13.6 mg/dL), in addition to low hemoglobin levels (10.6 g/dL). Biochemistry tests showed elevated muscle enzyme levels, lactate dehydrogenase (LDH; 503 IU/L), and creatinine kinase (CPK; 601 IU/L). However, other parameters were within normal limits. Compared with the blood exams performed 5 days before, WBC, CRP, LDH, and CPK levels were significantly elevated. The level of hemoglobin had slightly decreased and mild coagulopathy was observed; however, it did not meet the diagnostic criteria for disseminated intravascular coagulation (DIC).\nThe patient underwent a plain CT scan, which revealed a large uterine leiomyoma with calcification measuring 15 × 13 × 9 cm (). Compared to the CT scan taken 5 days before, the leiomyoma that was previously 14 × 9 × 7 cm had slightly enlarged and deviated anteriorly, from the pouch of Douglas to the abdominal cavity. Diffuse high-density area was observed in the uterine body with an increasing amount of abdominal free fluid, indicating hemorrhagic tissue necrosis. Given these findings, contrast enhanced CT scan was additionally performed for a detailed evaluation. Enhanced CT images showed an elongated cervix, twisted at the uterine body and cervical junction (). The enhancement effect was diminished above the twisted cervix, including the whole uterine body and the leiomyoma. Our preoperative diagnosis was uterine torsion. The decision to perform an emergency surgery was made on the day of admission. Upon entry to the peritoneal cavity, 270 mL of hemorrhagic fluid was extracted. The uterus with a large leiomyoma was rotated 540 degrees counterclockwise at the level of the junction between the cervix and uterine body (Figures and ). Both fallopian tubes and the uterine body were discolored and necrotic, indicating total tissue ischemia. The untwisted cervix was found to be atrophic and unusually elongated. Total hysterectomy, bilateral salpingo-oophorectomy, and umbilical hernia repair surgery were performed. The patient remained stable without blood transfusion, and her postoperative course was uneventful. Elevated plasma LDH levels and signs of inflammation improved on postoperative day 1. She was discharged on postoperative day 7 and has been followed up in our clinic. No postoperative complications are found so far. The final pathologic finding of the specimen was uterine interstitial leiomyoma with diffuse calcification and hemorrhagic necrosis, weighing 1360 g (Figures and ). Both ovaries, fallopian tubes, and uterine body were necrotic, suggesting total tissue infarction due to the axial rotation of the uterus. No signs of malignancy were observed. |
A previously healthy 33 year old woman was admitted to the acute medical care unit with a two day history of feeling lethargic and unwell. She was a non smoker and did not drink alcohol. She had developed severe continuous pain in the right upper limb with no specific aggravating or relieving factors and associated with severe weakness. She had had three episodes of vomiting and diarrhoea and had been treated by her general practitioner with paracetamol for flu-like symptoms for three days. On the morning of the day of admission she had noticed that her lips and tongue were swollen and she felt short of breath. She was given intramuscular adrenaline by the ambulance staff for a threatened airway and was transferred to the acute medical unit for further evaluation and management.\nOn admission she had pulse rate of 110/min, respiratory rate of 22/min and was febrile with a temperature of 38.9 deg c. She was agitated, restless and incontinent. Three hours later she started to develop swelling of the face. The facial skin was intact and there was no erythema, rash or blisters. Examination of the oral cavity revealed an oedematous tongue, pharynx and buccal mucosa with no detectable septic focus. There was no neck stiffness, no localising neurological signs and her GCS was 14. There was no local rise of temperature and soft tissue palpation of the head and neck was normal with no localised tenderness and no palpable lymph nodes or surgical emphysema. Air entry was equal on both sides but there were diffusely scattered rhonchi. Cardiovascular system examination was normal. Abdominal examination revealed a localised tenderness in the left iliac fossa. Pelvic and perineal examination by the gynaecologist was normal. Right upper limb examination was negative for any soft tissue or skeletal injuries. There were no signs of cellulitis, erythema or blisters. Palpation of the soft tissues was non tender, with no signs of grimacing or withdrawl and consistency was soft with no signs of compartment syndrome. Active movements at shoulder, elbow and wrist joints were within normal range and painless. All peripheral pulsations were normal with satisfactory capillary circulation.\nThe laboratory parameters following admission have been summarised in table . The C -reactive protein [CRP], urea, creatinine and leucocyte count were elevated. Arterial blood gas analysis was consistent with metabolic acidosis. Chest X-ray and CT scan of the abdomen were normal. Blood sample for Polymerised Chain Reaction [PCR] test for meningococcal antigen was negative. Lumbar puncture examination was normal. Synacthen stimulation test revealed a high baseline reading which was suggestive of maximal stimulation. A provisional diagnosis of meningococcal septicaemia was made and on microbiological advice treatment was commenced with a third generation cephalosporin and teicoplanin by intravenous route. Vital parameters were supported with intravenous fluids and oxygen therapy by mask. Intake output chart was strictly maintained.\nEight hours later her GCS dropped to 7 and in view of her threatened airway, she was transferred to the intensive care unit. She was further resuscitated, intubated, and ventilated. 'Sepsis care bundle' was commenced [] which included optimisation of arterial pressure, haematocrit, central venous pressure and mixed venous saturation [], low dose hydrocortisone administration following the synacthen test [], intensive insulin therapy [], and low tidal volume ventilation[]. Renal replacement therapy by veno-venous filtration was begun because of the worsening acidosis. Recombinant human protein C was not given, as the criteria for its administration were not met [].\nOver the next 18 hours a non-blanching erythematous rash appeared over the right upper limb, which progressed rapidly to a blister over the medial aspect of the arm with gross swelling. There was no local rise of temperature and the consistency was soft with satisfactory capillary circulation [Fig ]. A diagnosis of necrotising fasciitis was considered. X-ray of the arm revealed no bony injury and no gas was evident in the subcutaneous tissues. The patient was hypotensive, clinically unstable and required constant monitoring and support. MRI was not available over the weekend and it was decided to proceed further on clinical assessment alone. Emergency surgery including the option of an amputation or a disarticulation was considered.\nOn microbiological advice, the antibiotics were changed to imipenem to cover both staphylococcal and streptococcal infection and clindamycin for its improved soft tissue penetration and ability to halt toxin production. Repeat blood samples revealed an elevated white cell count and CRP and normal coagulation parameters. Creatinine kinase was high and urine was negative for myoglobin. Initial blood cultures were negative and a repeat blood culture at 48 hours following admission was positive for alpha haemolytic (viridans) streptococcus mitis. The sepsis care bundle was continued, and the clinical and biochemical picture were reviewed frequently by senior orthopaedic, and intensive care staff.\nThe patient improved within the next 20 hours and she was weaned from renal replacement therapy, ventilation and was extubated. She was transferred to the ward after 24 hours for further management. Oral clindamycin was continued for a further 2 weeks until her blood parameters were optimised. The skin lesions disappeared over the next three weeks with full recovery of her upper limb function [Fig ]. |
A 23 year old male was transferred to the ICU one month after a fall from height and head injury. On admission the patient was in a persistant vegetative state with clinical evidence of dehydration. The patient was on 5 litres O2 per minute via trachestomy and arterial blood gases were within normal limits. Clinical examination revealed evidence of dehydration. Chest X-ray showed no abnormalities as shown in figure .\nTo correct dehydration and monitor volume status, a central venous line was inserted. Right subclavian approach was used. Four attempts to cannulate the subclavian vein were performed by a skilled intensivist using an 18 gauge needle but were all unsuccessful. During these attempts there was neither arterial puncture nor air aspiration. After getting little backflow of blood which was dark and nonpulsatile, ensuring a venous position of the needle, An 0.035" guidewire was then introduced afterwhich a dilator was advanced for 5 cm without apparent resitance. This was followed by sliding of a 7 french catheter over the guidewire, and the wire was then withdrawn without any resistance. Despite the smooth insertion of the catheter, there was a negative aspiration of blood which we attributed to severe dehydration. Central venous pressure was measured after insertion and was -1 mmHg so one liter of colloid was infused followed by 1 liter of crystalloids over a period of 12 hours.\nSeveral hours later, the patient started experiencing severe respiratory distress with rise in the central venous pressure from -1 mmHg to 16mmHg. Clinical examination revealed a respiratory rate of 40/minute and absent air entry on the right hemithorax as well as dullness to percussion. An arterial blood gas revealed hypoxia and combined metabolic and respiratory acidosis with Po2 of 49 mmHg, oxygen saturation 79%, pco2 54.9, PH 7.11 and HCo3 17.2. The patient started to develop severe bradycardia and hypotension necessitating atropine and mechanical ventilation. Bedside anteroposterior chest radiograph was performed as shown in figure panel B, which revealed the presence of massive right side pleural effusion and mediastinal shift to the left. The presence of pleural effusion was confirmed by syringe aspiration of clear fluid. A 28- French intercostal tube connected to underwater seal was then inserted followed by gradual withdrawal of the pleural fluid which was continuously clear till 3 liters were withdrawn over several hours. This was the same amount of fluid given to the patient through the subclavian catheter for correction of dehydration. At that time aspiration from the central venous catheter revealed the same clear fluid as that was drawn from the intercostal tube raising the suspicion of the intrapleural position of the venous catheter. The catheter was then removed from the subclavian vein and reinserted in the left internal jugular vein through an anterior approach. After withdrawal of the pleural fluid, marked relief of the respiratory distress and improvement of the oxygenation ensued.\n3 weeks later the patient was readmitted to the ICU for increasing difficulty in breathing. On examination, the patient was tachypnic with diminished air entry over the right lung and dullness to percussion and a purulent fluid coming from an opening in his right chest wall as shown in figure . Diagnostic aspiration of the pleural fluid revealed frank pus. Analysis of the pleural fluid revealed low glucose and PH and culture revealed methicillin resistant staphylococcus aureus. An intercostals tube was placed for drainage and the patient was started on vancomycin therapy. |
A 58-year-old Malay woman had ulcerative colitis that was diagnosed 21 years ago when she presented with recurrent diarrhea. Five years ago, she started to develop peripheral polyarthritis. There were no other extra-articular manifestations such as uveitis or pyoderma gangrenosum. She had multiple relapses of colitis with polyarthritis and therefore had received multiple courses of tapering-dose oral prednisolone with maintenance of 1 g of mesalazine three times a day and subsequently 100 mg of azathioprine once daily. Finally, two years later, with the above treatment, the primary disease managed to be brought to remission.\nIn spite of the clinical and histological remission of the IBD, the left knee arthritis seems to be persistent, but other peripheral joints mentioned above were in remission without any permanent deformity. A clinical assessment revealed that the left knee was swollen, warm, and tender. The inflammatory marker C-reactive protein was increased to 1.14 mg/dL (normal is less than 0.5 mg/dL). A knee aspirate revealed clear synovial fluid and did not yield any growth. She received two courses of 500 mg of intravenous methylprednisolone daily for three days followed by a tapering dose of oral prednisolone for one year for active and persistent left knee arthritis, but the effects were temporary. Mesalazine was changed to 1 g of sulfasalazine twice a day, and the azathioprine dose was subsequently increased to 150 mg once daily. Unfortunately, she developed leukopenia; therefore, the azathioprine dose had to be reduced back to 100 mg daily. Despite aggressive treatment with multiple courses of high-dose systemic and local steroid with the maximum tolerated doses of sulfasalazine and azathioprine, she still had recurrent left knee effusion and synovitis. Indeed, the disease had caused significant pain and restriction of mobility. Also, after almost two years of corticosteroid therapy, our patient has endured significant iatrogenic morbidity, including Cushingoid habitus, impaired fasting glucose, hypertension, and bilateral cataract with increased intraocular pressure.\nIn view of persistent unbearable pain and swelling of the left knee with severe secondary osteoarthritis, a total left knee replacement was done in the same year. During the operation, there was active synovitis of the left knee, and a histopathological examination of the synovial tissue revealed 'rice bodies', which are fibrocollagenous connective tissue stroma admixed with fibrinous exudates and abundant foci of vascular proliferation, suggestive of inflammatory arthritis (Figure ). One month after the operation, the left knee effusion recurred and there was persistent elevation of the inflammatory markers. Subsequently, a left knee arthroscopic debridement and near-total synovectomy were performed five years ago. Ten milligrams of oral methotrexate weekly was added one month after the operation, on top of 1 g of sulfasalazine twice a day, 10 mg of prednisolone once daily, and 100 mg of azathioprine once daily. Unfortunately, despite the above treatment for four months, our patient continued to show poor response and persistent recurrence of knee effusion. Subsequently, 25 mg of subcutaneous etanercept twice weekly, together with the other DMARDs and azathioprine, was initiated after three months.\nEventually, the joint showed resolution of knee joint swelling and synovitis at the fifth week of treatment, visual analogue scale score improved from 70 to 20 mm, and the C-reactive protein level decreased to 0.8 mg/dL. After six months of treatment with etanercept, azathioprine was stopped and sulfasalazine was changed to 1 g of mesalazine three times a day as our patient had a mild flare of ulcerative colitis with community-acquired pneumonia. The steroid was also tapered off after six months of treatment.\nTwenty-five milligrams of subcutaneous etanercept biweekly and 10 mg of methotrexate weekly were continued, and after 12 months of follow-up, the colitis or arthritis was in remission and no serious side effects were noted. |
An 8-year old girl presented with a history of left-sided submandibular swelling of 1-week duration. There was no reported fever, loss of weight or appetite, or any other generalized symptoms. There was no throat pain or difficulty in swallowing. She was started on broad-spectrum antibiotic treatment with no improvement, and the swelling increased in size. Computed tomography (CT) scan was done outside our institution and showed a large cystic mass lesion in the submandibular area. She had an open drainage procedure as an outpatient on the assumption of an abscess. The family was told that the drainage was successful and no further information was given to the family regarding microbial culture results.\nShe then presented to our outpatient clinic for further investigation since the mass did not change in size. On examination, she had a large submandibular mass measuring 5 cm × 5 cm which was slightly tender on palpation; she had no evidence of fever or tachycardia.\nExamination of the throat revealed normal tonsils and no lesions could be found in the oral cavity. No abnormalities could be found in the nasopharynx or hypopharyngeal area.\nLaboratory examination showed leukocytosis with a white blood cell count of 24,000 with 70% neutrophils, 20% lymphocytes, and 7% monocytes. The C-reactive protein was mildly elevated at 83 mg/L. Levels of serum lactate dehydrogenase and uric acid were normal.\nNeck ultrasound and CT scan [] showed a necrotic mass lesion in the left submandibular region, measuring 5.6 cm × 5.2 cm × 5.2 cm, suggestive of an abscess with associated adjacent reactive appearing lymph nodes.\nThe patient was admitted and was started on intravenous clindamycin and oral augmentin and dexamethasone. The next day, she underwent an open drainage on the assumption of an organizing abscess. During the procedure and on opening the mass, we found an inflamed firm tissue surrounding the wall of a cystic cavity which was attached to the submandibular gland. The salivary gland appeared normal in size but with extensive adhesions to the cystic wall mass. The cystic cavity revealed a serous fluid with no obvious pus. The tissue was found to have extensive adhesions where dissection was difficult. However, multiple representative biopsies were taken on an attempt for a complete excision including the removal of the attached submandibular gland. The tissue was sent for histopathological examination and culture. She was discharged on oral antibiotics and advised to come back after 1 week as an outpatient follow-up.\nHowever, she was readmitted 3 days later with fever and serous discharge from the surgical wound. Laboratory parameters on the second admission revealed leukocytosis and increased inflammatory parameters. By ultrasound examination, there was a mass lesion at the left submandibular location measuring 4 cm × 3 cm × 5 cm with complex mixed echogenicity.\nComputerized axial tomography scan revealed a large submandibular collection and nearby multiple enlarged small lymph nodes. The right submandibular gland appeared normal. Chest X-ray was within normal limits.\nBasic immune workup for immunodeficiency was negative. The immune status screening showed mumps viral IgM, which was reported as positive IgM but with low levels of detection, Epstein–Bar virus (EBV) IgG was positive, and quantitative EBV polymerase chain reaction analysis showed 6500 copies/ml. Cytomegalovirus IgG was positive, but IgM was negative. Toxoplasma IgG and IgM were both negative. C3 and C4 serum levels were elevated. Bartonella antibodies were also negative. She was restarted on intravenous antibiotics, ceftriaxone, and clindamycin but developed allergy to the latter; therefore, it was replaced by augmentin.\nThe histological examination revealed intensely inflamed soft-tissue fragments with mixed inflammatory cell infiltrate composed of neutrophils, plasma cells, lymphocytes, and histiocytes. However, occasional large atypical cells were seen scattered in between these tissue fragments. These atypical cells were large, polygonal, and epithelioid with abundant cytoplasm and large nucleus. The cells contained large and prominent eosinophilic nucleoli in addition to occasional mitoses []. Immunohistochemical stains revealed immunoreactivity of these large cells for pan-cytokeratin (AE1/3) but negativity for CD68 and most lymphoid markers (CD3, CD5, CD20, CD8, CD23, and CD45) except strong membranous staining for CD4 []. Two different clones of CD4 were tested; the first clone is SP35, Rabbit monoclonal antibody, Ventana Medical Systems, Inc. Tucson, AZ, USA, while the second clone is 4B12 from Dako, Santa Clara, CA, USA. Both clones showed immunoreactivity; stronger on the Ventana clone. All positive and negative controls were appropriate.\nViral in situ hybridization staining for EBV by the EBER method showed strong nuclear staining [ inset]. A diagnosis of EBV-positive lymphoepithelial-like carcinoma (LELC) in the submandibular region was rendered.\nBased on the final pathologic diagnosis, the family was advised to have examination of the child under general anesthesia for endoscopy and biopsy from the nasopharynx, but they refused.\nBased on multidisciplinary team's discussion, it was recommended to have multimodality oncological treatment which is not available at our institution. Therefore, the patient was referred to the American University of Beirut Medical Center where advanced care for such cases would be available.\nPretreatment positron emission tomography scan showed large left retromandibular and parapharyngeal mass 7 cm × 6 cm with Standardized Uptake Value of 17 causing mass effect on the oropharynx with deviation from midline to the right, with ipsilateral cervical lymphadenopathy []. The decision was made to treat this patient as a case of nasopharyngeal carcinoma (NPC) Stage IIB. She received induction chemotherapy (cisplatin 80 mg/m2/dose and 5-fluorouracil 1 g/m2/day × 4 days, both repeated every 3 weeks for a total of 3 cycles) as per the Children's Oncology Group protocol ARAR0331. She demonstrated a very good response after 3 cycles, with significant decrease in the size of the flourodeoxyglucose-avid conglomerate of soft-tissue masses/lymph nodes in the left submandibular region to 2.5 cm × 2 cm and resolution of their activity.\nShe then received concurrent chemoradiation with cisplatin 100 mg/m2 every 3 weeks where two cycles were given during radiation therapy. The radiation dose was 45 Gy in 25 fractions to neck lymph nodes, followed by a boost of 16.2 Gy in 9 fractions to the tumor volume. The total tumor dose was 61.2 Gy in 34 fractions where the treatment was delivered using intensity modulated radiotherapy. At the end of treatment, imaging showed further decrease in the residual lesions []. |
A 78-year-old Japanese woman (gravida 10, para 2, abortion 8) had a 10-year history of uterine prolapse but she had no gynecological examination. She had no smoking history and her body mass index was 19.7. She visited our hospital because of a 1-month history of bleeding from an ulcerated lesion on the surface of the prolapsed uterus. Upon examination, the uterine prolapse appeared as complete eversion of the posterior vaginal fornix and was manually irreducible and classified as stage IV according to the Pelvic Organ Prolapse Quantification System []. On the surface of the completely prolapsed uterus, an ulcerated lesion of 3 cm in diameter was observed and a deep-seated induration was felt. A histological biopsy of a mass in the vaginal wall led to a diagnosis of keratinizing squamous cell carcinoma. Magnetic resonance imaging revealed the mass with a depth of 1.6 cm, but the endometrial cavity was not depicted (Fig. ). The mass was located approximately 3 cm from the cervical os with no sign of invasion to adjacent tissue. According to the International Federation of Gynecology and Obstetrics (FIGO) staging system, the tumor was vaginal cancer staged as I, and a fluorodeoxyglucose positron emission tomography/CT revealed no metastases to the lymph nodes or other organs. Although the diagnosis of this case was vaginal cancer, the main tumor was located in the uterine cervix because of the completely prolapsed uterus. Therefore, we considered that it is practical to develop the treatment plan according to the uterine cervical cancer. Because of the patient’s advanced age and the presence of arrhythmia (paroxysmal supraventricular tachycardia and paroxysmal atrial fibrillation), she was ineligible for a combination of surgery under general anesthesia and chemotherapy; therefore, RT alone was planned. If EBRT had been administered, radiation dermatitis of the genitalia would have been a concern because the prolapsed uterus would be irradiated. Neither a probe nor a tandem could be inserted because of severe intrauterine adhesions due to her history of multiple abortion procedures, also known as Asherman syndrome [, ]. The cervical canal was extremely narrowing due to advanced age. Thus, intracavitary BT (ICBT) could not be administered. In addition, the complete uterine prolapse inhibited fixation of an ovoid tandem to the vaginal fornices. Thus, ISBT was planned, in which plastic needles would be directly inserted into the margin of the ulcerated lesion on the surface of the completely prolapsed uterus under direct visual observation. In the BT treatment room, four plastic BT needles (LLA150-K; Eckert & Ziegler BEBIG, Berlin, Germany) were inserted into the margin of the ulcerated lesion to a depth of up to 3 cm under local anesthesia to sufficiently cover the lesion, the depth of which was 1.6 cm as previously measured by magnetic resonance imaging (Fig. ). Under only local anesthesia, direct insertion of the plastic BT needles caused very little pain. Next, a dummy source for X-ray imaging (LLH02–21 to 24) was inserted into the lumen of each of the four plastic BT needles, and X-ray imaging was performed. The patient was then transferred to the CT room. CT was performed with 2-mm slices, confirming that the BT sources had been inserted around the lesion (Fig. ). At our facility, HDR ISBT is administered using a 60Co remote afterloading system (RALS) (MultiSource; Eckert & Ziegler BEBIG). Thus, these CT scans were uploaded to the RALS treatment planning system (HDR plus; Eckert & Ziegler BEBIG). According to the guidelines established by the Groupe Europeen de Curietherapie and European Society for Radiotherapy and Oncology [, ], the gross tumor volume (GTV) and organs at risk (OARs) (i.e., the rectum and bladder) were contoured. The high-risk clinical target volume (HR-CTV) including the whole cervix could not be contoured because the endometrial cavity was not depicted and the whole extent of the cervix was not defined. The GTV was 19.4 cc, and treatment was planned with a dose of ≥6 Gy prescribed as D90 of the GTV (minimum dose delivered to 90% of the GTV) (Fig. ). On another day, the plastic BT needles and dummy sources were reinserted, and X-ray imaging was performed, followed by CT. A similar treatment plan was developed. At our facility, X-ray films alone are used to check the location of radiation sources for BT and develop treatment plans. In this case, X-ray films were obtained after insertion of each plastic BT needle and the dummy sources, and two treatment plans were developed (Table ). We selected the plan with the dummy sources placed closer to the lesion, according to which HDR ISBT was administered at 6 Gy per fraction twice weekly for a total of eight times. When the location of the dummy sources deviated from the planned sites, the plastic BT needles were reinserted to ensure administration based on one of the treatment plans. The total accumulation dose of HDR ISBT was estimated to be 65.0 Gy as D90 of the GTV (equivalent dose in 2-Gy fractions [EQD2]). One month after the completion of RT, the histological biopsy revealed remaining squamous cell carcinoma. Therefore, we performed additional HDR ISBT at 6 Gy per fraction twice weekly for a total of six times until the ulcerated lesion became soft and flat. The final total accumulation dose of HDR ISBT was estimated to be 113.8 Gy as D90 of the GTV (EQD2), 20.7 Gy as D2cm3 of the rectum (EQD2), 36.8 Gy as D2cm3 of the bladder (EQD2) with α/β = 10 for the GTV and α/β = 3 for the OARs (Table ). Because of the complete uterine prolapse, the GTV was remote from the rectum and bladder; therefore, the exposure doses to the OARs were extremely low. The only observed acute complication was grade 2 dermatitis in the genitalia (National Cancer Institute Common Terminology Criteria for Adverse Events of 4.03 []). One month after the completion of additional HDR ISBT, no residual squamous cell carcinoma was detected in the tissue biopsy. Two months after additional HDR ISBT, as late toxicities, local ulcer lesion with fibrositis was seen in the labium majus and tumor bed in the vaginal wall, and debridement was performed. Three months after additional HDR ISBT, no recurrence or rectal and urinary toxicity had occurred. |
A 37-year-old Caucasian woman who was 35 weeks pregnant with a history of 2 previous spontaneous abortions experienced a sudden, spontaneous, and very heavy epistaxis from the left nostril. Conducted to the emergency department of the local hospital, she presented hemodynamic instability and fetal monitorization compatible with severe fetal distress. She underwent an emergency cesarean section preceded by a focal convulsion upon the induction of anesthesia (attributed to induction of anesthesia in the context of severe hypovolemic anemic shock leading to cerebral hypoperfusion). The epistaxis continued and she received 5 units of red blood cells and 1 unit of fresh frozen plasma over 2 days. Because of failure to control the nasal bleeding with a local hemostatic balloon, she was transferred to a tertiary hospital for angiography with eventual embolization. During this period, she did not present any changes in her mental state or hemodynamic stability, and the local hemostatic balloon in the left nostril was kept in place.\nThe first angiography was performed using 50mL of low osmolality and nonionic organic contrast media administered in the internal and external carotid arteries. There was an abnormal vascular blush in the left nasal hemifossa dependent on branches of the sphenopalatine artery, and embolization was performed. However, the epistaxis returned shortly after, and a second angiography was performed 2 hours later, excluding the presence of any remaining vascular blush and confirming the overall success of the prior embolization. A total of 90mL of nonionic isosmolar contrast was used in both angiographies. Local tamponing was kept in the left nostril.\nImmediately after the second angiography, during the postanaesthetic recovery, the patient started to be disoriented, with sleepy speech but no formal visual complaints or motor deficits. Within 2 hours of the second procedure, she remained alert but cried continuously, developed hyperventilation, was not able to follow orders, directed her gaze but could not fix it and presented repetitive speech without intelligibility or the ability to make sentences. She was hemodynamically stable, afebrile and without any meningeal signs. The patient was transferred to the intensive care unit (ICU) due to her clinical evolution and the eventual need for airway protection in the context of an acute confusional state of unknown cause.\nGiven the patient’s agitation and noncooperation, it was not possible to properly evaluate her language and content of thought or humor, and it was not possible at that time to exclude the possibility of aphasia. A formal psychiatric evaluation, performed within a few hours of presentation, ruled out any major psychiatric illness, as well as the more common peripartum psychosis (PPP). There were no motor deficits, cranial nerve changes, or other focal signs suggesting extensive focal neurological injury. Given the acute onset, brain magnetic resonance imaging (MRI) was performed to evaluate possible complications of angiography, and a lumbar puncture was performed to exclude an infectious or inflammatory disease of the central nervous system. An electroencephalogram was also performed.\nMagnetic resonance imaging revealed a small focus of hypersignal on the diffusion weighted imaging at the level of the left corona radiata white matter (), with hyposignal on the apparent diffusion coefficient map, with a discrete hypersignal in the fluid-attenuated inversion recovery. There was also smooth swelling of the posterior temporo-occipito-parietal cortex bilaterally, with a discrete hypersignal on the fluid-attenuated inversion recovery () but without restricted diffusion, suggesting vasogenic edema. Her cerebrospinal fluid was crystal clear, with a normal opening pressure, and presented four cells, a glucose of 72mg/dL, proteins 53mg/dL and a lactate dehydrogenase (LDH) < 30UI/L. Polymerase chain reaction was negative for an extensive panel of neurotropic viruses; all bacterial cultures were negative. There were no electrolytic disturbances, and systemic tests for autoimmunity were negative.\nAn electroencephalogram showed predominant theta activity in the posterior projection, beta activity in the anterior and theta-delta activity infrequent in the frontotemporal region, and a left predominance, sometimes with a rhythmic morphology followed by base suppression. The traces revealed diffuse slowing of base electrogenesis to a moderate degree. There was fronto-temporal slow activity of the left hemisphere associated with a scarce paroxysmal component but it was mild and noncontinuous.\nSedoanalgesia and mechanical ventilation were maintained for 2 days, being withdrawn only after stabilization of the clinical picture and reviewing the results of the MRI and electroencephalogram to exclude further brain damage that could need a different approach.\nExtubated, she maintained a very poor vocabulary (with a maximum of 40 spoken words) but with no focal deficits. In less than 24 hours, a progressive improvement of her speech and reasoning content was observed, without other changes in the examination. Her renal function was normal throughout.\nA second MRI was performed 48 hours after the first, showing the presence of a few bilateral upper cortical/subcortical millimetric restricted diffusion lesions, probably related to the previous angiography. There was a complete resolution of her posterior cortical edema (). The rest of her brain parenchyma did not presented other relevant changes.\nClinically, the patient progressed very well, with quick recovery of speech and all neurological changes. Tamponing was withdrawn on the third day, and the patient was discharged home 5 days after the C-section. |
This is a case of a 54-year-old divorced Caucasian male who has no prior psychiatric history or hospitalization. He was the lead singer of a local rock band. The patient has a 20-year history of cocaine abuse. He routinely uses cocaine prior to his stage performances. He reportedly binged on cocaine following a concert performance in a downtown bar. He snorted more cocaine than usual and immediately developed a severe headache and lower extremity numbness. He was rushed to the local emergency room, and an MRI of the brain without contrast was done. The MRI of the brain without contrast showed two small foci of increased signal intensity within the subcortical white matter of the left frontal lobe (Figure ).\nIt also showed the finding of bilateral foci of infarct involving the right side of the pons (Figure ). Both figures are consistent with an embolic phenomenon.\nThe patient has not complained of any somatic issues related to his cocaine use. The patient reported some depressive and anxiety symptoms as a result of the stroke but he remained optimistic about his recovery. He denied any vegetative depressive symptoms or suicidal ideations. He was initially seen by a nurse practitioner at the emergency room who diagnosed him with an adjustment disorder. After stabilization in the medical floor and a two-week stay at a rehabilitation program, the patient was sent home with good family support. Over the course of two weeks, the patient noted significant crying spells most of the day, nearly every day. He consistently denied that he was depressed and suicidal. His family became concerned and sent him to his primary care physician. The physician believed that he was suffering from depression because of his dramatic clinical presentation. The patient was eventually referred to the local mental health center by his primary care physician because of uncontrollable crying spells. He was not subjectively depressed but objectively tearful with a flat affect. He also complained of sleeping difficulties with ruminative worries about his situation. He denied any suicidal thoughts.\nThe patient was started on Remeron (mirtazapine), 15 mg at bedtime. He also engaged in weekly psychotherapy sessions. Over the next two months, the patient noted improvements in his sleeping patterns and appetite. The crying spells persisted. The patient was observed to be tearful while at the waiting area, during the psychiatric evaluation, and after his treatment appointment. His family reports that he cries every day for no apparent reason. Despite reassurances that he was not depressed, the family was convinced that his emotional state was getting worse. The patient was eventually diagnosed with pseudobulbar affect (PBA) because of his repeated outburst of involuntary crying. The crying was occurring even though there was no sad event that triggered those emotions. These episodes were persistent and had occurred in different situations or settings. He was referred to a local neurologist who confirmed the PBA. Eventually, he was managed with dextromethorphan hydrobromide and quinidine sulfate (DM/Q), 20 mg/10 mg capsules twice a day, in addition to his mirtazapine. The patient's crying spells improved significantly after the DM/Q was started. He tolerated it very well with no complaints of any side effects.\nA year later, the patient had multiple tragedies in his family. His father, with whom he was very close with, suddenly and unexpectedly died. He also had an argument with his daughter, who later refused to talk to him. He was overwhelmed with financial problems. Because of these, the patient became more depressed and the crying spells recurred. Despite his medication compliance with DM/Q and mirtazapine, he noted worsening depression and occasional suicidal thoughts. He reported symptoms of sad mood, anhedonia, fatigue, excessive sleeping with early morning awakenings, increased appetite and weight gain, psychomotor retardation, and feelings of helplessness and worthlessness. He was having thoughts of shooting himself, even though he does not own a gun. This time, he was subjectively complaining of being "down in the dumps." He was seen in the emergency room for a crisis evaluation and referred back to the mental health center. He was reevaluated and his mirtazapine was switched to Viibryd (vilazodone) because of weight gain concerns. He also attended twice a week psychotherapy sessions. A month later, with these interventions, the patient's depressive symptoms, including the crying spells, had improved. He continues to receive his DM/Q and vilazodone and weekly psychotherapy sessions with no exacerbations of any mood symptoms. |
A 48-year-old man with ESRD secondary to diabetic nephropathy was admitted for initiation of renal replacement therapy. He had undergone 36 cycles (36 hours of 1 L/h cycle) of acute PD, through a temporary PD catheter inserted through the infra-umbilical approach, at another center 10 days prior and presented to us for maintenance dialysis. After discussing the options, he was started on hemodialysis through a temporary catheter and was planned to start on CAPD. He had a history of diabetic retinopathy and neuropathy. He did not have any history or symptoms suggestive of neurogenic bladder.\nThe patient underwent 3 sessions of hemodialysis and was planned for CAPD catheter insertion with vancomycin prophylaxis. As per protocol, he was advised to void his bladder completely and to pass stools before insertion. An infraumbilical midline incision was made under local anesthesia and IV sedation, an 18-G introducer needle was inserted through the rectus sheath until it reached the peritoneal cavity, and 1.5 L of peritoneal dialysate was instilled intraperitoneally. After instillation of fluid, a guidewire was passed through the needle, with the tip directed towards the suprapubic region. The needle was removed, the tract was dilated using a 16 Fr dilator followed by insertion of a 16 Fr peel away sheath/dilator. The guidewire was removed followed by the dilator, and Tenckhoff swan-neck double-cuff straight catheter was inserted through the peel away sheath. The sheath was peeled away, and a subcutaneous tunnel was created with the exit site facing down and out towards the left iliac fossa. The deep cuff was positioned over the peritoneum, and the superficial cuff was placed in the tunnel. The incision site was sutured in layers. Postprocedure, the inflow and outflow were found to flow without resistance, and the catheter was brought out through a left-sided tunnel. A 500-mL flush was given, and good inflow and outflow were noted. The outflow was initially blood-mixed, followed by drainage of clear fluid. There were no complications during the immediate postoperative period. With a plain abdominal radiograph, the position of the catheter tip was confirmed to be in pelvis (). Ultrasonography was not used during the procedure due to logistic reasons.\nOn the 3rd day after insertion, after instillation of 500 mL of PD fluid, the patient complained of urinary urgency with an increase in urinary volume to 700 – 800 mL/day from his baseline of 300 – 400 mL/day. Also, there was decrease in the outflow volume with drainage of only 100 mL following instillation of 500 mL of PD fluid. With the increase in the inflow volume, there was a significant increase in his urine volume. Urine analysis was done to look for glycosuria, which showed a 3(+) on dipstick, from his baseline glycosuria of 1(+). He was asked to void after instilling 500 mL PD fluid through the PD catheter, and urinary creatinine testing was done, which was found to be absent. A possibility of peritoneovesical fistula was considered, and a cystogram was planned. The instilled fluid presumably entered predominantly into the urinary bladder through the side holes and led us to presume that the PD catheter could be in the preperitoneal location. Urine culture and PD fluid cultures were sent, which revealed growth of enterococcus (> 105 colony forming units/mL), and he was treated with vancomycin intraperitoneally (IP) and broad-spectrum antibiotics intravenously.\nThe cystogram revealed a smooth-walled bladder, with no intraperitoneal leak of the contrast, and the catheter abutting the bladder wall (). A plain abdominal computed tomography (CT) was performed to confirm the position (). The catheter was seen to be piercing the anterior abdominal wall and entering the bladder anteriorly, perforating and coursing through the bladder before exiting through the posterior wall, with the tip in the rectovesical fossa. PD was discontinued, and he was continued on hemodialysis through a temporary femoral access catheter. After discussion with the urologists, it was planned to remove the catheter, followed by reinsertion at a later date, with conservative management for the bladder perforation. With cessation of PD, there was decrease in urine output to ~ 50 – 100 mL/day, and a repeat urine routine performed 3 days later showed reduction in pyuria (from plenty of white blood cells/high power field to 6 – 8 WBC/HPF).\nThe options were discussed with the patient, and the need for a vascular access was explained. However, due to logistic reasons, an attempt was planned towards catheter salvage. He was planned for catheter repositioning using fluoroscopic guidance under the cover of intravenous and intraperitoneal broad-spectrum antibiotics.\nUsing fluoroscopy with contrast (urograffin), first the bladder perforation was confirmed. The infraumbilical incision was re-explored under local anesthesia, while leaving the tunnel in situ. The deep cuff was identified, and the PD catheter was pulled back by ~ 6 cm (as measured by the prior abdominal CT image). Contrast was injected through the catheter, and it was confirmed to be intraperitoneal with no filling up of the urinary bladder. Using a guide wire, the catheter was redirected towards the right iliac fossa, and the position confirmed. On subsequent contrast injection through the PD catheter, the intraperitoneal nature and absence of bladder filling was confirmed. The bladder was later filled up with contrast, and there was no presence of any leaks (). The initial bladder perforation could have been an intraperitoneal perforation since pulling back and re-positioning the catheter, without any new entry into the peritoneum, confirmed it to be in the peritoneal cavity.\nThe patient was continued on IV antibiotics for 5 days, IP ceftazidime for a week, and IP vancomycin for 21 days, and was continued on hemodialysis. IP vancomycin was given in a single dwell (1,000 mL) for 6 hours every day. One week post repositioning, he was started on low-volume CAPD and discharged, and a repeat urine and peritoneal fluid culture were sterile. He was continued on Foley’s catheter for 4 weeks, with a residual urine output of ~ 200 – 300 mL/day. At 9-month follow-up, the patient is comfortable with CAPD 3 exchanges/day with good ultrafiltration (~ 1.4 L/day), and no change in urinary volume, with no further episodes of peritonitis. |
The patient is a 73-year-old male who was transferred from an outside hospital for repair of a right acetabulum fracture involving the femoral head after falling approximately 8-feet from a ladder while painting his house. Past medical history was significant for hyperlipidemia and osteoarthritis. Baseline metabolic equivalents were greater than four. Aside from a cataract extraction, the patient had no other operations. He denied any allergies. Prior to presentation, the patient was on Aspirin 81 mg daily for cardiovascular disease prevention and Atorvastatin 20 mg daily for hyperlipidemia. Computed tomography (CT) without contrast showed an acute, comminuted, and displaced fracture of the right acetabulum involving both posterior and anterior acetabular walls. The right femoral head was superiorly and laterally displaced with impaction fracture to its inferior and medial aspects. There were bone fragments within the right gluteus musculature, and the high attenuation in tissue density represented blood product within. Prior to surgery, the orthopedic team made an effort to reduce the patient's right acetabulum fracture with tibial traction pin under conscious sedation with midazolam, fentanyl, and ketamine in the emergency department. During the closed reduction, the patient experienced a brief period of respiratory depression. For approximately two minutes, his oxygen saturation was 85-86%, which improved to 95% with Narcan reversal. The emergency department record also noted that the patient had premature ventricular contractions on the electrocardiogram (EKG) at this time. After the closed reduction, the orthopedic team opted to monitor the patient on continuous telemetry for 24 hours and take the patient for open reduction and internal fixation of the right acetabulum the next day. He was not on prophylactic anticoagulation for this preoperative period.\nPrior to entering the operating room, the patient's vital signs were stable: blood pressure 120/64, heart rate 73 beats per minute, respiratory rate 18 per minute, oxygen saturation 95%, and temperature 98.6 Fahrenheit. Neurological exam was significant for limited right knee flexion (<30 degrees). Otherwise, overall sensation was intact and right deep tendon reflex was intact. Hemoglobin was 11.6, down from 13.4 on admission. Coagulation tests showed elevated prothrombin time at 12.8, partial thromboplastin time 26.7, and international normalized ratio 1.20. In the OR, the patient was induced with standard dosing of midazolam, fentanyl, lidocaine, propofol, and succinylcholine. Phenylephrine was given preemptively on induction to avoid hypotension. Intubation was uncomplicated. Patient had two 20g peripheral intravenous catheter in place, and a left radial arterial line was placed after intubation. Despite premedication with phenylephrine, the patient became hypotensive with systolic blood pressure (SBP) in the 80s, median arterial pressure < 65. Boluses of ephedrine and phenylephrine were given, and a phenylephrine infusion was started thirty minutes after induction. The patient initially responded appropriately to treatment (SBP > 100). Due to concern for sciatic nerve injury, intraoperative somatosensory evoked potential (SSEP) monitoring was performed, and general anesthesia was maintained with propofol at 100 mcg/kg/min and remifentanil at 0.2 mcg/kg/min, in addition to 0.5% end tidal sevoflurane.\nUpon patient positioning to the right lateral decubitus position from supine, the patient's SBP acutely declined into the 50s and the patient became hypoxic with an oxygen saturation of 88%. His hypotension was no longer responsive to phenylephrine or ephedrine. Norepinephrine and epinephrine drips were started. Also, the FiO2 was increased from 50% to 100%. There was a minimal and transient response to epinephrine administration. The patient was then returned to supine position, and he arrested shortly thereafter. Chest compressions commenced immediately upon loss of cardiac rhythm. The cardiac anesthesia team emergently responded and performed an intraoperative transesophageal echocardiography (TEE), which showed reduced left ventricular ejection fraction and reduced right ventricular systolic function. Due to unsuccessful cardiopulmonary resuscitation, the patient was placed on venoarterial ECMO via right femoral artery and vein; stable hemodynamics were achieved. A total of 8000 units of Heparin was given upon ECMO installation. At this point, the planned surgery was aborted. The patient was transported to the cardiac catheterization lab for pulmonary angiogram, cardiac catheterization, and antegrade perfusion of the right superficial femoral artery. A large thrombus in the left pulmonary artery was discovered on selective angiography (Figures –). Hypothermic protocol was initiated. Heparin therapy was also continued at this point.\nOn hospital day 5, a CT chest with contrast showed multiple bilateral pulmonary emboli and evidence of right ventricular failure. Right heart catheterization and placement of a Swan-Ganz catheter demonstrated that the patient was found to have severe pulmonary hypertension 55/30 and elevated central venous pressure (CVP) of 18. The patient underwent bilateral pulmonary artery embolectomy, exploration of right atrium with removal of clots, discontinuation of ECMO support, and repair of right femoral artery on hospital day 9. His pulmonary artery pressure and CVP improved to 31/12 and 8, respectively. One month later, the patient was discharged to a rehabilitation facility with a plan for conservative management of his fracture. |
A 4-year-old girl was admitted with a 3-year history of a cardiac murmur, and the admission weight was 17 kg. A 3/6 degree of systolic murmur at the third left intercostal space was detected by physical examination, and a perimembranous VSD of 6 mm was revealed by echocardiogram. The girl underwent VSD closure with inferior partial median sternotomy under cardiopulmonary bypass (CPB) at day 4 of hospitalization, and the VSD was closed with three interrupted horizontal mattress sutures. The operation was successful, and she was discharged at postoperative day 10.\nShe was readmitted at 20 days after the first surgery due to a high fever of 40°C at postoperative day 18 with no obvious causative factors and without characteristics of coughing, wheezing, retching, vomiting, or cardiac murmur. The girl showed a slight poor physical development and with no family history of genetic diseases and dysmorphism. She had received antibiotic therapy for 1 day before readmission at the local hospital. Laboratory examination showed that the white blood cell (WBC) count was 5.94 × 109/L, and the percentage of neutrophils was 81.8%. Blood culture showed positive for Staphylococcus aureus. The electrocardiograph showed frequently occurring ventricular premature beat, and ultrasonic cardiogram (UCG) showed a spherical cystic echo (82 × 76 mm) at the anterolateral aspect of the ascending aorta (). Then an emergent computed tomography angiography (CTA) was done which revealed an abnormal 86 × 78 mm echo located at the anterolateral aspect of the ascending aorta with a direct communication to the aorta, and the communicating hole was 8.9 mm in diameter (, ).\nAn emergency operation was performed under deep hypothermia (20°C) with CPB. In detail, the right femoral artery and vein were cannulated using 14# arterial cannula and 19# venous cannula, respectively. Then median sternotomy was immediately performed after the CPB started. We found the pseudo-aneurysm at the anterior mediastinum wrapped with amounts of inflammatory scar and thymus tissue. Because of the giant pseudo-aneurysm and severe surrounding tissue adhesions, there was no sufficient space to place the aortic cross-clamp and cardioplegic cannula needle. Then the patient was rapidly cooled to 20°C, and the heart was arrested, induced by hypothermia, while an ice hat was used to implement brain local mild hypothermia therapy. After the patient was placed in a head-down position, we incised the pseudo-aneurysm rapidly and found an ostium (8 × 9 mm in diameter) in the anterior wall of ascending aorta where the purse-string suture was made during the previous surgery. Cardioplegia solution (St. Thomas solution, 30 ml/kg) was infused directly via the coronary ostia to achieve cardiac arrest. A piece of appropriate size of aneurysmal wall was harvested and soaked in iodophor for 2 min then overlaid with Dacron patch for closing the ostium with continuous suture of 4–0 prolene (). After de-airing, the ostium was closed thoroughly, CPB was resumed, and the patient was warmed. After recovery to normal sinus rhythm, CPB was terminated, and the femoral cannulae were removed. The CPB time was 173 min, and circulatory arrest time was 15 min. After being weaned from CPB, the girl was closed up and returned to pediatric ICU for further therapy. Vancomycin and penicillin were administered intravenously for 10 days according to the results of drug sensitivity test, and then the oral antibiotics were administered until the day of discharge. The girl gradually recovered to asymptomatic condition and was discharged at 47 days after the secondary surgery. We followed up the girl for 2 years; the girl had a weight gain of 5 kg and good appetite that showed she is in good nutritional and well-developmental status. She received UCG and chest radiography examinations during the periodic outpatient reexaminations, and no abnormality was detected. The timeline of the overall therapeutic process is shown as a flow diagram in . |
A 32 year old female Cameroonian gravida 4 para 3 at 34 weeks of gestation presented to the labour and delivery unit of Mbalmayo district hospital with 8 h history of severe generalized headache, expressive aphasia and right sided paralysis in an afebrile context. This was associated with blurred vision but no convulsions. There was no epigastric pain and no difficulty breathing and no history of trauma or fall. For this current pregnancy, antenatal care (ANC) was started at 18 weeks with a booking blood pressure of 100/70 mmHg. She did four ANCs and all were uneventful. During her routine four ANCs here blood pressure was always less than 140/90 mmHg and her urine dipsticks done during the four ANCs were all negative for proteinuria. She refused neurological symptoms such as headache during pregnancy. She has a history of gestational hypertension in her third pregnancy. There was no family history of chronic hypertension, diabetes and chronic kidney diseases. On examination she was afebrile with a blood pressure of 182/126 mmHg and pulse of 112beats/minute. Neurological examination revealed Glasgow coma score of 13/15, right sided hemiparesis and expressive Broca’s aphasia, no signs of meningeal irritation. The abdomen was distended by a gravid uterus with a fundal height of 35 cm, foetus in a longitudinal lie and cephalic presentation. The cervix was long, posterior, soft and closed with a station of − 1. We had a working diagnosis of severe pre-eclampsia complicated by stroke. Shown on Table are laboratory investigations done and their results.\nAn emergency obstetric ultrasound showed a life foetus with an estimated foetal weight of 2300 g at 33 weeks of gestation. Emergency cerebral non contrast-CT scan showed a 3.2 cm hyperdense region in the left parietal lobe with surrounding hypodensity due to clot retraction as shown on Fig. . Emergency management by the obstetrician consisted of MgSO4 using the Pritchard protocol [], which consisted of 14 g loading dose then 5 g maintenance every 6 h until 24 h after caeserean section; bethamethasone 12 mg intramuscular and reduction of blood pressure with nicardipine 5 mg/h. Four hours later an emergency caesarean section was done by the obstetrician under spinal anaesthesia and it let to the extraction of a life female with APGAR 8 and 10 at the 1st and 5th minute respectively and weight 2200 g. The management after caesarean section consisted of hospitalization in the intensive care unit with nicardipine titrated in an electric syringe at 2.5 mg/hour, ceftriaxone 2 g intravenous, Paractamol 1 g 8 hourly, and ringers lactate 6 hourly for 24 h. Post-operative management was done by a multidisciplinary team including a neurologist, cardiologist, intensive care physician, obstetrician, neonatologist and physiotherapist. On postoperative day 2 she was transferred from the intensive care unit to the maternity where she spends five additional days on nicardipine slow release 50 mg 12 hourly and paracetamol 1 g 8hourly and was later release after the ten days on nicardipine 50 mg daily and daily physiotherapy. Six weeks during routine postpartum visit the blood pressure was normal and patient was no longer aphasic and shet has regained the muscle strength partially. The baby was hospitalised in the neonatal unit for 10 days and discharged alongside the mother. |
A 20-year-old female presented with dyspepsia and pain in her left, upper abdomen for the past five days. The pain was insidious in onset, originated in the left hypochondrium with radiation to the left shoulder, was constant and dull aching in nature, aggravated with fatty foods, and was relieved with analgesics and antacids. There was no history of fever, jaundice, vomiting, urinary complaints, altered bowel habits or weight loss. The menstrual history of the patient was regular. She had experienced a similar episode around three months prior, which was relieved by conservative medical therapy. She did not give any history of any medical or surgical comorbidities and had no significant drug history.\nThe general examination of the patient was essentially normal with no signs of jaundice, fever or anemia. When examining the abdomen, there was mild tenderness from deep palpation in the left hypochondrium, with normal bowel sounds. The rest of the abdominal examination was unremarkable. Examination of the cardiovascular system revealed an apex beat in the right, 5th intercostal space in the right, mid-clavicular line.\nThe patient's routine blood investigation revealed a normal complete blood count, normal liver and kidney function tests, normal thyroid function test and normal glycemic status. The urine examination also did not reveal any abnormalities. The ultrasonography of the abdomen showed as follows: 1) A gallbladder, small in size, with a thickened wall and situated on the left side. The lumen was filled with multiple, small calculi with posterior acoustic shadowing; 2) The liver situated on the left side, normal in size, with a homogenous parenchymal echo pattern. The intrahepatic biliary channels were not dilated; 3) The common bile duct was normal in size and diameter without any intraluminal lesions; 4) The spleen was normal in size and shape without any focal lesions and situated on the right side; and 5) Features were consistent with situs inversus. The electrocardiograph showed a right axis deviation while the chest x-ray (posteroanterior view) showed dextrocardia, the left hemidiaphragm slightly raised compared to the right side and a fundic gas shadow, of the stomach, on the right side, all findings consistent with situs inversus totalis (). The findings were re-confirmed with a computed tomography of the abdomen and the diagnosis was established to be a case of cholelithiasis in a patient with situs inversus totalis ( and ).\nAfter obtaining necessary anesthetic fitness, the patient was scheduled to undergo an elective laparoscopic cholecystectomy. The procedure began by adjusting the theatre equipment, including the carbon dioxide insufflator, the diathermy set and the monitor, which were placed on the left side of the patient, almost mirroring their normal positions. The patient was then positioned in the reverse Trendelenberg position after adequate anesthesia. The primary surgeon and the first assistant stood on the right side of the patient, whereas the second assistant was on the left side. A total of four ports were made - the two 10 mm ports were placed in the infraumbilical and subxiphoid regions, respectively and the two 5 mm ports were placed in the left hypochondrium in the left mid-clavicular line and in the left anterior axillary line at the level of the umbilicus ().\nThe technical challenges anticipated included creating a pneumoperitoneum from the left side, dissection of the Calot's Triangle with the right-handed surgeon using the non-dominant hand and chances of the surgeon's arms crossing during retraction of the Hartmann's pouch. The pneumoperitoneum was induced using a Veress needle through the infraumbilical incision, by the surgeon on the left side of the patient, as done conventionally. An inspection of the abdominal cavity was done and the diagnosis of situs inversus was confirmed. The camera was maneuvered from the infraumbilical port by the first assistant and the fundus of the gallbladder was retracted by the second assistant, with a toothed grasper, from the 5 mm port in the left, anterior axillary line. In order to prevent the primary surgeon from crossing arms while retracting the Hartmann's pouch from the right side of the patient, the assistant retracted it from the left side. This not only prevented the primary surgeon from crossing arms, but also enabled him to dissect the Calot's Triangle with his right hand using Maryland dissecting forceps inserted through the subxiphoid/epigastric port. The cystic duct and artery were identified and dissected free from the surrounding structures. They were clipped using titanium clips, the applicator being introduced through the 10 mm epigastric port. The gall bladder was dissected from the gall bladder fossa using a hook diathermy and was delivered outside through the epigastric port. Adequate hemostasis was ensured and the port sites were closed with non-absorbable sutures ().\nThe estimated operating time was around 70 minutes and the postoperative period was uneventful. She was discharged on the second postoperative day and the sutures were removed on the seventh postoperative day, in the outpatient department. She followed up one month after surgery and was found to have recovered well. She has not developed any postoperative complications to date. |
This 67-year-old woman presented on 3 March 2008 at 16:00 to the neurological emergency room. Earlier that day (at 14:50), her relatives had witnessed the acute onset of a fluctuating paresis of her left arm and leg. Diffusion weighted MR images, carried out at 16:30, revealed an area of restricted diffusion in the right basal ganglia and the deep periventricular white matter and an MR angiogram demonstrated complete occlusion of the right middle cerebral artery (MCA) in the M1 segment. The emergency stroke team decided to initiate intravenous thrombolysis, which started within a 2 h window (16:45). As the patient did not show any clinical improvement within the next 30 min, the attending stroke physician decided to proceed with a bridging concept and endovascular treatment was initiated, after the patient was informed of the proposed procedure. This information included a description of the procedure, the potential risks and benefits and an explanation that extensions of the procedure (eg, surgical craniectomy) or the off-label use of medical devices (eg, coronary stents, etc) might become necessary. The patient gave informed consent to the operator. The neurological examination before digital subtraction angiography (DSA) showed a severe dysarthria and a left-sided hemiplegia with facial nerve palsy (National Institutes of Health Stroke Scale (NIHSS) score of 10). The emergent angiography began 3 h and 20 min after the onset of symptoms and the first DSA run was recorded at 18:12.\nUsing a 4 F diagnostic catheter, selective injection of both vertebral arteries and internal carotid arteries (ICAs) was carried out to show the extent of leptomeningeal collaterals and to rule out distal emboli, eventually not visible on MR angiography. Complete embolic occlusion of the right MCA at the M1 segment with moderate leptomeningeal collaterals between the right anterior cerebral artery and MCA was confirmed ().\nSystemic anticoagulation with 5000 U of heparin was initiated. Via a 6 F Envoy guiding catheter (Cordis, Miami, USA) in the right ICA, a straight Rebar27 was gently advanced to the thrombus under the guidance of a SilverSpeed16 microguidewire (both ev3). Under road map, a thrombectomy brush (pCR, phenox; Bochum, Germany) was deployed in the right angular artery and subsequently slowly withdrawn under continuous aspiration. The brush was loaded with clot material but no recanalization of the target vessel was observed (TICI 1). During the catheterization, the thrombus appeared organized and firm. The chances of success of either LIF or repeated pCR passages were considered low as previous experience has shown very low recanalization rates with pCR and LIF in firm organized emboli. It appeared evident to the operator (HH) that in this situation, more interaction between clot and retrieval device was required. While contemplating the use of either a Catch or a Merci retriever (at this time the only other approved devices for mechanical thrombectomy), we recognized that neither of these tools was in stock. After balancing the potential risks and benefits to this patient and given the uncertain outcome if there was no recanalization, we decided to use a Solitaire stent in the way we would have otherwise used a Catch or a Merci device. The right angular artery was again gently catheterized with a Rebar18 catheter. After removal of the microguidewire, a self-expanding Solitaire stent (4 mm diameter, 20 mm length) was inserted under continuous slow flush with saline solution to the distal end of the microcatheter and was deployed by slowly pulling back the microcatheter. Correct deployment of the Solitaire stent over the whole length of the thrombus was observed under continuous fluoroscopy. In order to avoid mechanically induced vasospasm, 2 mg of glycerin trinitrate were injected intra-arterially. After several minutes of incubation and with the rotating hemostatic valve tightly closed, a 50 ml Luer lock syringe was used for high volume aspiration through the 6 F guiding catheter during withdrawal of the open Solitaire stent and the microcatheter into the guide catheter. Withdrawal was possible with minor effort and was observed under continuous fluoroscopy. As soon as the device became visible, the rotating hemostatic valve was quickly removed from the guiding catheter and another 50 ml of blood were aspirated. The Solitaire stent was loaded with a thrombus (). Contrast injection of the right ICA after removal of the Solitaire confirmed total recanalization of the right MCA (; TICI 3). Neither peripheral emboli nor vasospasm at the previously occluded vessel site was encountered.\nThe entire procedure (from the first to the final DSA run) lasted 59 min. The patient awoke from general anesthesia with only mild neurologic deficit (NIHSS 2). The patient was discharged to a rehabilitation center. At the 30 day follow-up, NIHSS was 0.\nThe patient was heparinized and received 100 mg of acetylsalicylic acid daily as secondary prophylaxis. ECG monitoring revealed atrial fibrillation as a potential source of cardiac embolism and hence the patient was anticoagulated with phenprocoumon. MRI examinations were refused by the severely claustrophobic patient. DSA 24 days after treatment showed a completely normal M1 segment and patent cortical branches of the entire right MCA. Follow-up CT 22 months later showed a small post-ischemic lesion of the right basal ganglia and normal cortex (). |
A 94-year-old Caucasian man presented to our hospital with extensive bleeding from his oral cavity and diffuse bruising. His medical history included severe dementia and hypertension. Our patient had a habit of repeatedly biting his tongue. This led to profuse bleeding from the dorsal surface of his tongue that was persistent despite surgical placement of sutures in the tongue and removal of his teeth. His hemostasis was previously normal and he did not take any anticoagulants or non-steroidal anti-inflammatory drugs. There was no nose bleeding, hematuria, bloody stool or accompanying hemoptysis. Our patient did not have any family history of bleeding disorders. On examination, his vital signs were stable and he was afebrile. There was profuse bleeding from the tongue with the presence of multiple clots in the oral cavity. No other bruising or active bleeding was noticed, except extensive bruising over his upper extremities and the presence of a hematoma on the left hand with active oozing. Laboratory tests revealed a white blood cell count of 9500 cells/μL with an initial hemoglobin level of 11.7 g/dL and a platelet count of 149 × 103 cells/μL. Coagulation studies revealed a normal prothrombin time and international normalized ratio, and a prolonged activated partial thromboplastin time of 73 seconds (normal: 24.8 to 36.1 seconds). The presence of an inhibitor of coagulation was diagnosed via prolonged activated partial thromboplastin time and a mixing study that did not correct with the addition of normal plasma (partial thromboplastin time (PTT) 36.4 seconds when an immediate mixing test was performed, with a ratio of patient's plasma to normal plasma of 1:1) (Figure ). Quantitative assays revealed a reduced level of factor VIII activity (1%) and the presence of factor VIII inhibitor measured at 7 Bethesda units (BU) in the serum.\nOur patient was not intubated for airway protection based on the wishes of his family. In total, two units of fresh frozen plasma and eight units of packed red blood cells were transfused and three doses (100 U/kg) of recombinant human factor VIII (Humate-P 2958 RCO) were given to our patient as initial management. Humate-P was chosen based on the lack of an alternative bypass agent such as recombinant activated FVII (rFVIIa) in the setting of acute severe bleeding. Oral prednisone therapy (60 mg/day) was given, and he also received two doses of intravenous aminocaproic acid (3 g intravenously over 1 hour followed by an infusion of 750 mg/hour for 8 hours) and topical anti-thrombolytic agents (topical thrombin cream 100 units was used once) because of ongoing and active bleeding. His hospital course was also complicated by complete heart block, which developed immediately after the infusion of the second dose of intravenous aminocaproic acid. Of note, our patient was initially admitted with a heart rate of 80 beats/minute and he had a first-degree AV block and left anterior fascicular block on his admission electrocardiogram. Our patient was not a candidate for a transvenous pacemaker secondary to his severe coagulopathy. He required use of vasopressors initially, but he subsequently remained hemodynamically stable with a heart rate of 30 beats/minute. He developed profuse melena for two weeks, most likely as a consequence of swallowing the blood coming from his tongue. His activated partial thromboplastin time (aPTT) gradually improved (38.4 seconds). Our patient's family refused further diagnostic investigation in terms of finding an underlying cause for the acquired hemophilia such as malignancy. He was discharged to a rehabilitation facility with improving symptoms, stable hemoglobin (9 g/dL) and minimized bruises after three weeks of hospitalization. A repeat test for the level of factor VIII inhibitor in serum four weeks after our patient was on steroids showed a reduction to 1BU while VIII activity had also increased (10%). Our patient was discharged on 40 mg of prednisone as immunosuppressive therapy with a treatment plan for a slow tapering of steroids as well as careful monitoring of his coagulation parameters. On follow-up six weeks after discharge, his bradycardia had reversed and his heart rate had increased to 85 beats/minute, which suggests that the initial bradycardia was likely related to the infusion of aminocaproic acid. |
A 7-year-old boy known to have homocystinuria since the age of 2 years and 10 months presented to the pediatric emergency department with abdominal pain and bloody stools. The child was born full term by cesarean section delivery with a birth weight of 2.7 kg. He was born to healthy consanguineous parents and has 5 brothers and 3 sisters. The child was growing normally except for delayed speech development. At the age of 2 years he started to have recurrent focal seizure alternating from one side to the other and associated with a disturbed level of consciousness. Electroencephalogram (EEG) showed focus of sharp waves on the right centroparietal and temporal regions. Computed tomography of the head was performed and reported as being normal. The patient was started on different antiepileptic medications which partially controlled the seizures.\nTen months later, the patient developed right-sided hemiparesis. Computed tomography of the head showed a hypodense area on the left thalamic region. Magnetic resonance imaging and magnetic resonance venography of the brain showed left thalamic hemorrhagic infarction and thrombosis of both the internal cerebral vein and vein of Galen. Following the magnetic resonance venography, the child developed deep vein thrombosis extending from the right femoral vein up to the common iliac vein. Anticoagulation therapy (heparin followed by warfarin) was started along with the antiepileptic medication. Further investigations revealed positive urine and blood for homocystinuria. Eye examination demonstrated bilateral downward lens dislocation. Sibling screenings were positive for homocystinuria for 1 brother and 1 sister. Vitamin B6, folic acid and betaine were added to the treatment and 8 weeks later warfarin was discontinued and dipyridamole was added.\nThe child improved gradually and showed signs of complete recovery. He started to walk and talk normally 8 weeks following the event. However, the child was not compliant with the treatment and did not attend the outpatient follow-up regularly. Despite the lack of compliance, the child became seizure-free and antiepileptic medication was stopped at the age of 51/2 years.\nAt the age of 7 years he had symptoms of upper respiratory tract infection with vomiting. An oral antibiotic was prescribed by his family physician. Several days later he showed up in the emergency room with crampy abdominal pain and bloody stools. Physical examination showed normal vital signs with no signs of dehydration or infections. He was admitted to the hospital for further investigations. During his stay, the child continued to have progressively worsening abdominal pain with lower GI bleeding; he was therefore transferred to the surgical side. Doppler ultrasound of the abdomen showed superior mesenteric vein and middle colic vein thrombosis. The patient underwent an emergency exploratory laparotomy which demonstrated a 120-cm ischemic black small bowel approximately 50 cm distal to the duodenojejunal junction. Resection with end-to-end anastomosis was performed and anticoagulant therapy was commenced after the procedure. A few days later the patient started to tolerate oral fluid and vitamin B6, betaine, folic acid and methionine restriction were added. He was discharged in a good general condition. Homocysteine and methionine levels on admission were 24 and 739 µmol/l, respectively, while on discharge corresponding values were 11 and 265 µmol/l (normal range: methionine 10-40 µmol/l, homocysteine not detected). The discharge medication included vitamin B6, betaine, folic acid, and warfarin. The child and the family were advised to be compliant with treatment and clinic appointments. With subsequent visits he was noted to be compliant with the medications and the follow-up as well as his affected siblings. |
A 25-year-old man presented to the ER with abdominal pain, nausea, and vomiting. He described the abdominal pain as cramping, constant, located on the left lower side of the abdomen with an intensity of 8 to 9 out of 10. He indicated that the pain had been present for 1 week and had been getting progressively worse, with no radiation to other areas of the abdomen. Associated symptoms reported were nausea and vomiting for 1 day, diarrhea that had been on and off, and fatigue for about a week. He denied any fever, chest pain, shortness of breath, constipation, dizziness, muscle weakness, or numbness.\nHis past medical history was significant for bipolar disorder and dyslipidemia. No surgical history was reported. The patient denied any allergies. He was taking gabapentin and mirtazapine. His family history is unknown, since he was adopted at the age of 5. He reported occasional alcohol and tobacco use and had used cocaine and marijuana in the past, with the last use reported to be 1 year prior to admission, since he was currently enrolled in a methadone program. He was unemployed at the time of admission. Interestingly, he reported having been admitted to a hospital 2 years prior where he was told he had some blood clots in his abdomen and that he needed to take blood thinners, an advice that he did not follow.\nOn presentation, the patient was in mild distress because of abdominal pain; the vital signs were stable, except for slight tachycardia with a heart rate of 106. No abnormal coloration was noted in the eyes or on the skin. No murmurs or additional heart sounds were noted. The lungs were clear to auscultation. The abdomen was soft and nondistended but was diffusely tender to palpation, especially in the left lower quadrant. No edema was noted in the extremities and the pulses were present. Neurological exam was intact.\nThe laboratory evaluation was basically unremarkable and revealed a WBC count of 8.89 (103/μL), Hb 15.2 g/dL, Hct 43.7%, Plt 248 (103/μL), INR within normal limits, Na 141 mEq/L, K 4.1 mEq/L, BUN 8 mg/dL, and Cr 1.2 mg/dL, Alk. Phos. 192 U/L. However, the radiological exam was significant, with an abdominal computed tomography (CT) revealing the presence of multiple nonocclusive thrombi involving the right portal vein, the splenic vein, and the left renal vein, as well as complete occlusion of the left portal vein and the superior mesenteric vein (Figures and ). Colitis involving the descending and sigmoid colon was also noted and was believed to be ischemic in nature. A Doppler ultrasound was obtained and confirmed the findings suggestive of chronic portal vein thrombosis.\nThe patient was admitted with a diagnosis of portal vein thrombosis and was started on anticoagulation with enoxaparin bridged with warfarin. A complete hypercoagulability workup was obtained, all of it coming back negative. Liver disease was also excluded, with all the liver disease workup being unremarkable as well. Patient's pain was relieved with morphine. His hospital course was uncomplicated, with significant improvement of his pain and the ability to tolerate a regular diet without any gastrointestinal symptoms, such as nausea, diarrhea, or constipation. He was extensively counseled on the need to be compliant with his anticoagulation regimen to prevent the recurrence of acute thrombosis and was uneventfully discharged with appointments to follow at the medicine and warfarin clinics. Interestingly, the patient was readmitted 2 weeks later after an INR check revealed it to be supratherapeutic at 12.8. No signs of bleeding were noted, he received a dose of Vitamin K, and his warfarin was held. After a couple of days of observation, he was discharged with instructions to follow at the warfarin clinic for readjustment of his anticoagulation regimen. |
A 68-year-old male patient was admitted to our department with a diagnosis of idiopathic dilated cardiomyopathy (normal coronary arteries) associated with left bundle branch block (QRS duration of 140 msec) and reduced ejection fraction (EF, 35%).\nIn February 2004, a biventricular defibrillator was implanted in primary prevention. The patient responded well to treatment, showing significant clinical improvement (EF 45%) without experiencing arrhythmia episodes.\nIn May 2009, the generator was replaced because of battery depletion. Six months later, displacement of the pulse generator to the subclavian vein and tunneling of the leads with subsequent pocket infection were observed. After a first attempt of percutaneous removal of the system with unsuccessful lead extraction, in February 2011 the patient underwent surgical lead removal () and associated De Vega tricuspid annuloplasty. On that occasion, we decided not to implant a left ventricular epicardial lead in order to attempt transvenous lead placement at a later stage.\nUnfortunately, the postoperative course was complicated by a “superior vena cava syndrome” with massive thrombosis of the innominate, internal jugular and subclavian veins. After a short stay in the hospital Rehabilitation Unit, the patient’s hemodynamic status deteriorated (NYHA class IV, EF 28%) and subsequent CRT was necessary. We considered the possibility of implanting a biventricular pacemaker to avoid epicardial lead placement because of the previous cardiac surgery procedure. However, phlebography showed complete occlusion of the upper extremity venous access and the femoral vein approach was used.\nFor easier lead placement and the likelihood that an optimal defibrillation vector could not be achieved at the level of the femoral vein, a biventricular pacemaker rather than a defibrillator was implanted. In addition, the patient was electrically stable and, in the past, responded well to CRT with a marked improvement in EF (> 35%).\nDuring the procedure of biventricular pacemaker implantation via the femoral approach, three femoral venous accesses were obtained. The first catheter was placed into the left ventricle. A quadripolar Josephson catheter (TORQR series) was inserted into the coronary sinus (CS).\nFirst, a peel-away delivery sheath (Attain Command 6250-MB2X, 50 cm in length) was introduced into the electrophysiology catheter. Owing to difficulties in cannulating the CS, it was replaced with a straight sheath (model 6257S, 57 cm in length), resulting in successful access to CS and good support.\nPostero-lateral tributaries were visualized with non-occlusive CS venography (). A Medtronic Attain StarFix lead (model 4195, 103 cm in length) was inserted using the standard over-the-wire technique. The Attain StarFix lead is a 5 Fr, steroid-eluting, transvenous, unipolar, left ventricular, over the wire, cardiac vein pacing lead with three deployable lobes. During the implant procedure, the lobes can be undeployed by withdrawing the push tubing. Radiopaque markers assist in full lobe deployment. A threshold voltage of 2.5 V x 1.5 msec and a sensing threshold of 12 mV with good lead impedance were obtained. No diaphragmatic stimulation was noted at maximum output (10 V).\nThe active fixation mechanism of the Attain StarFix lead contributed to the success of the procedure, enabling easy removal of the delivery system.\nThe right ventricular lead was positioned in the high right ventricular septum, and the atrial lead was implanted in the right atrial septum. These locations allowed for an easier lead placement (). Both leads had active fixation (model 4076-80, 80 cm in length), with good voltage and sensing thresholds. The leads were tunneled through the rectus abdominis fascia and connected to the pulse generator in the abdominal pocket.\nThe patient was discharged after 4 days without complications. At pre-discharge follow-up, the left ventricular device had a voltage and sensing threshold of 2.5 V x 1.5 msec and 10 mV, respectively.\nAt one-month follow-up, the electrical measurements were stable with a voltage and sensing threshold of 1.0 V x 1.0 msec and 11.2 mV, respectively. The patient showed clinical improvement, also confirmed at echocardiography (EF 40%), suggesting a positive response to CRT.\nAt 1-year follow-up, the clinical and instrumental data remained stable with marked improvement in EF (45%). |
This is a case of a 72-year-old Taiwanese man who was diagnosed as having metastatic adenocarcinoma of the lung complicated by malignant right pleural effusion 2 years ago. He initially presented with weight loss, worsening cough, and worsening exertional shortness of breath for 3 months prior to presentation. He had a past medical history of type 2 diabetes mellitus, which was well controlled on insulin. He was an ex-smoker of tobacco with a tobacco smoking history of one pack a day for 10 years but he quit smoking tobacco 20 years ago. He also had a family history of non-Hodgkin lymphoma in his brother and breast cancer in his niece. He is retired and lives with his wife.\nOn examination, he was afebrile with heart rate of 70 beats/minute and with blood pressure (BP) of 130/80 mmHg. He appeared moderately built and was not in any respiratory distress. His respiratory examination was significant for dullness over the right middle and lower chest on percussion and was associated with reduced breath sounds on auscultation. His cardiovascular, abdominal, and neurological examinations were non-contributory.\nHis initial computed tomography (CT) scans demonstrated a middle lobe mass in his right lung and right lung pleural effusion. A positron emission tomography (PET) scan showed an increased uptake in the middle lobe mass in his right lung, subcarinal lymph nodes, several bilateral subcentimeter pulmonary nodules, and diffuse osseous metastasis. There was no evidence of brain metastasis as evidenced by magnetic resonance imaging (MRI) of his brain. He then had thoracentesis and pleural biopsy with the placement of a pleural catheter. The pleural biopsy was consistent with adenocarcinoma with an acinar pattern. Immunohistochemistry of the tumor cells was positive for cytokeratin (CK) 7, thyroid transcription factor 1 (TTF-1), and negative for CK20. A fluorescence in situ hybridization (FISH) showed evidence of ALK mutation (33% of cells positive for rearrangement). His final diagnosis was stage IV ALK + adenocarcinoma of the lung with metastasis to pleura, mediastinum, and bones. Before the information of ALK positivity was obtained, he was started on combination chemotherapy consisting of carboplatin, Alimta (pemetrexed), and Avastin (bevacizumab) of which he successfully completed five cycles. He was followed up regularly in the clinic every 4 weeks. He tolerated the chemotherapy and had a good response with 30% reduction in the lung mass size. He was later started on crizotinib 250 mg twice per day and had a significant response with improved tumor burden in his metastatic sites. He followed up in the clinic every 4 weeks initially for 6 months and then every 8 weeks for 1 year. During his follow-up visits, he remained stable with no evidence of disease progression. He remained on crizotinib for over a year and tolerated it well. During one of the follow-up clinic visits at around 18 months after diagnosis, an MRI scan of his brain was arranged due to a new symptom of headache; it showed numerous brain metastases which was consistent with progression of his disease. A decision was made to stop crizotinib and to start alectinib 600 mg twice daily coupled with cranial radiation.\nWithin 5 weeks of starting alectinib, he developed ARF with his creatinine (Cr) increasing up to 8.16 mg/dL and blood urea nitrogen (BUN) to 113 mg/dl. He was anuric at presentation and his laboratory tests were consistent with hyperkalemia and acidosis with a potassium level of 7.1 mEq/L and bicarbonate (HCO3) of < 9 mmol/L. His renal workup revealed BUN/Cr ratio of 13, fractional excretion of sodium (FENa) of 16%, urine sodium of > 83 mEq/L, and urine osmolality of 334 mOsm/kg. His renal ultrasound did not show any evidence for obstruction (hydronephrosis). Table illustrates the laboratory values and Fig. illustrates the timeline of our patient’s renal functions.\nSince he was refractory to medical treatment, he required continuous venovenous hemodialysis (CVVH) due to the electrolyte imbalance. Alectinib was held on admission and dialysis was continued for 2 days until the electrolyte imbalance was corrected. Renal biopsy could not be performed as our patient refused.\nHis renal functions slowly recovered and Cr improved to 1.75 mg/dL within 2 days. He was re-challenged with alectinib at the same dose 24 hours after renal recovery. However, the medication had to be stopped again as his Cr started to worsen and rose to 3.6 mg/dL within 2 days of restarting alectinib. Currently, he is being treated with ceritinib, and his renal status has been stable with Cr levels ranging between 2 and 3 mg/dL. He also has no progression of his metastatic disease as evidenced by his recent imaging. |
A 13-year-old girl was referred to our hospital for treatment and investigations of hypertension. Eighteen months prior to her presentation, she developed headaches that slowly increased in frequency and intensity, eventually occurring daily at the time of presentation. Her other symptoms and signs included a 2-month history of lethargy and intermittent polydipsia and polyuria. There was no history of nausea, vomiting, syncope, or visual disturbance. Blood pressure measurement done by her primary care provider was 200/120.\nThe patient was in good physical health with the only past medical complaint of infrequent episodes of asthma. Her only medication was over-the-counter analgesia for headaches. The family history was significant for factor V Leiden deficiency in her father and migraines in her mother and two sisters. There were no family members diagnosed with hypertension or cardiovascular disease.\nPhysical examination on admission to hospital revealed a well-grown teenage girl. Hypertension was confirmed by blood pressure readings of 180/120 in both arms and similar readings in the legs. Pulses were well palpable in all limbs with no radio-femoral delay. Examination of the heart revealed normal heart sounds and no murmurs. There was no organomegaly or masses palpable in the abdomen. Examination of the neck revealed no bruit or thyroid gland enlargement. Fundoscopy was normal without any evidence of hypertensive changes. Neurological examination was normal.\nHypertensive target organ damage evaluation revealed normal magnetic resonance imaging (MRI) of the brain and structurally normal heart. There was normal systolic function and trivial aortic regurgitation by echocardiogram, likely due to the hypertension. Detailed ophthalmologic examination did not reveal any hypertensive changes. As previously mentioned, kidney function was normal and there was no proteinuria or hematuria present.\nBasic laboratory investigations showed normal urinalysis with no blood or protein, and normal renal (creatinine 42 μmol/L) and liver function. The biochemical profile on the first blood sample after admission was normal, apart from mild hypokalemia (3.0 mmol/L), mild metabolic alkalosis with pH 7.44, and mildly elevated serum bicarbonate (28 mmol/L). Twenty-four-hour urine collection revealed normal sodium (102 mmol) and potassium (53 mmol) excretion in 1.7 L of urine. Because of the association of long-standing severe hypertension and hypokalemia, our further work-up focused on the causes of renin-mediated hypertension.\nHormonal studies showed normal plasma and urinary catecholamine and cortisol levels, and normal thyroid function. Plasma renin (mass) and aldosterone levels were markedly elevated [1380 mU/L (normal range 3–40 U/L) and 1940 pmol/L (normal range 100–900 pmol/L), respectively].\nFurther imaging studies were aimed at localizing the source of increased renin production. Abdominal ultrasonography did not reveal any abdominal masses and showed two structurally normal kidneys, with normal renal blood flows (demonstrated by Doppler studies). Computed tomographic angiography (CTA) excluded RAS, but revealed a rounded 10 mm diameter subcapsular mass under the surface of the anterior midpole of the left kidney (Figure ), suggestive of reninoma. To confirm excessive production of renin from this tumor, renal vein renin sampling with lateralization was performed. The procedure was done under general anesthetic and ultrasonographic guidance. 4Fr catheters were inserted into the proximal vein orifices of the left and right renal vein and sampling for plasma renin mass was undertaken. Plasma renin sample from the left renal vein was significantly higher (2900 mU/L) when compared to the right renal vein (840 mU/L) and the sample from the peripheral vein (908 mU/L), with a L:R ratio of 3.45:1 (Figure ).\nThe patient remained clinically well during her stay in the hospital. Hypertension was treated with a combination of calcium channel blocker amlodipine (10 mg/day) and α-blocker prazosin (2 mg three times per day) with normalization of blood pressure (120/80) and resolution of headaches. We did not use medications targeting the renin-angiotensin-aldosterone axis [renin inhibitors, angiotensin converting enzyme inhibitors (ACEI), angiotensin receptor blockers, or aldosterone antagonists] to avoid interference with renin/aldosterone sampling. Hypokalemia was corrected with potassium supplementation.\nFollowing the confirmation of the renin-producing tumor in the left kidney, surgical resection of the tumor was performed. The patient was placed in the lateral position with left side up. A laparoscopic transperitoneal approach, with mobilization and reflection of the splenic flexure, allowed exposure of the anterior surface of the left kidney. The vessels were dissected and clamped. The tumor was removed by sharp dissection (Figure A). The parenchymal defect was closed with interrupted mattress suture and snugged with a Hem-o-lok (Weck®) to secure hemostasis (Figure B). The specimen was retrieved inside an endocatch bag as to not contaminate the abdominal wall. Warm ischemic time of the procedure was 18 min. Blood pressure immediately after the surgery was 100/50. Prazosin and potassium supplements were subsequently discontinued, and amlodipine was reduced to 5 mg per day with good control of blood pressure (120/80).\nHistological examination of the tumor revealed a well-circumscribed lesion measuring 15 mm in diameter with cystic and hemorrhagic changes. The tumor was separated from the normal kidney parenchyma by an incomplete fibrous capsule (Figure A). The tumor cells were uniform with round nuclei and eosinophilic granular cytoplasm (Figure B). All tumor cells stained positive for CD34 (Figure C) but were negative for desmin, CD31, S100, HMB-45, synaptophysin, chromogranin, and WT-1. Constellation of these histological and immunohistochemical features was consistent with the diagnosis of juxtaglomerular cell tumor (reninoma). The diagnosis was confirmed by electron microscopy revealing the presence of rhomboid dense deposits (renin crystals) in the cytoplasm of the tumor cells (Figure D). Cytogenetic examination of the tumor revealed normal female karyotype 46, XX without any chromosomal abnormalities. |
A 66-year-old woman was referred to the Oral and Maxillofacial Surgery Clinic at Hyogo College of Medicine Hospital, Japan, in September 2011, for an extraoral fistula and refractory pain of the right mandible associated with a purulent discharge and soft tissue swelling. The patient’s osteoporosis was diagnosed in 2005 and treated with 35 mg of alendronate weekly by the family doctor. The patient had a past history of severe osteoporosis, multiple vertebral fractures, and renal failure. She had taken 20 mg of prednisone for 3 months from 2005 for the treatment of IgA nephropathy.\nDental implant treatment in the maxilla and mandible was begun in June 2009 by the family dentist. Five implants (Spline Twist implant, Zimmer Dental, Carlsbad, CA) were placed at the same time in the posterior region of the mandible. The surgical procedure was uneventful, and primary stability of the implants was achieved. In September 2010, at the time of implant reopening for the second surgery, the implants had integrated and the healing abutments were connected. Provisional maxillary and left mandibular prostheses were cemented onto the abutments.\nNine months after the second surgery in June 2011, the patient started to complain of a painful cheek swelling on the right side of the mandible, associated with gingival bleeding. She was prescribed oral antibiotics by her dentist and underwent occasional antibiotic therapy thereafter.\nIn September 2011, the patient was referred to our clinic because her symptoms were getting worse. Clinical examination revealed an intraoral fistula on the lingual side of the dental implant replacing the right mandibular first molar, associated with mucosal inflammation and a purulent discharge (Fig. a, b). She also had hypoesthesia of the right lower lip. The patient underwent panoramic radiography (Fig. a) and computed tomography (CT), which showed bone resorption around the dental implant in the right mandibular first molar area and severe peri-implantitis in the right mandibular molar region. There was no obvious sequestrum separation (Fig. b, c). Under a clinical diagnosis of perimandibular inflammation and peri-implantitis, conservative treatment consisting of local irrigation and use of antibiotics was implemented. Meropenem hydrate was given initially, then changed to ampicillin/sulbactam. The inflammatory state improved, and when the symptoms subsided, treatment with clarithromycin was continued. Debridement and removal of the dental implant in the right mandibular first molar area was performed under local anesthesia. Irrigation of the site was continued as part of the treatment regimen.\nIn November 2011, after a consultation with an osteoporosis expert at the Orthopedic Medicine Clinic of our hospital, alendronate therapy was stopped and subcutaneous teriparatide therapy at a dose of 20 μg per day was started. During the course of the teriparatide therapy, the patient continued to use 0.02% benzalkonium chloride solution for local irrigation.\nIn April 2012, after 5 months of teriparatide therapy, the sequestrum separation had progressed (Fig. ), and a sequestrectomy was performed under general anesthesia (Fig. ). At 5 months after the operation, a CT scan revealed new bone formation around the bone defect in the region of the sequestrectomy, with all symptoms including bone exposure disappearing (Fig. ). The patient’s osteoporosis treatment was continued, and 16 months after the sequestrectomy, further new bone formation was observed (Fig. ). |
A 35-year-old lady presented to the craniofacial surgeons looking for a permanent fixture to the bony defect in her lower-right mandible. In 1991 she had her first operation to remove the ameloblastoma and then had it further excised 6 years later due to a reoccurrence. This resulted in an absence of teeth from the canines back and removal of bone down to the inferior alveolar nerve. The patient has since been clear of any further reoccurrences and wears lower dentures for aesthetic reasons.\nShe has no significant past medical history nor family history, nor does she have any known drug allergies. She does not smoke or drink alcohol.\nOn clinical examination, the patient experiences mild parasthesia in the distribution of her right inferior dental nerve, particularly the lateral aspect of the lower lip.\nOn examination, there is clear absence of teeth on the lower-right side from the canines back (to include the premolar, molar and wisdom teeth) and presence of healthy oral mucosa with laxity to move the submucosa medially. There is a loss of the vertical height of the posterior body of the mandible of about 50% and the vestigial remnants of the lingual plate are just palpable and visible on plain radiograph and CT. Scans show that the superior margin of the resection is well corticated under the inferior dental nerve (Fig. a and b). No functional problems exist. As a long-term solution to her defect, the patient wishes to get dental implants. The surgical approach taken, prior to the implants, shall be discussed.\nThe patient was initially given the option between a bone graft or vascularized tissue (free fibular flap) and informed of the complications—a bone graft carries a risk of rejection and vascularized tissue needs an increased amount of post-operative care to ensure that the tissue remains vascularized. The patient was not keen on a free flap and given the fact that the bony deformity was <5 cm, non-vascularized iliac bone graft was deemed to be sufficient to carry out this operation [].\nThe surgery occurred in several steps. The first step was achieving significant tissue expansion (>2 cm) using Osmed pellets, guided into the submucosa and kept over the alveolar ridge for several months (Fig. a–c).\nTwo months later, the patient underwent the main bulk of her operation. The Osmed pellets were removed from the subperiosteum (Fig. a and b) and the iliac bone graft was taken, made up of cortical and cancellous bone and placed to one side (Fig. a–c). An epidural catheter was placed inside the soft tissue of the graft site and left overnight. It had a continuous fusion of a local anaesthetic to improve post-operative pain and encourage early mobilization.\nPreviously, using CT scans, a titanium construct was made to guide the outline of the bone graft and complete the missing height and width of the mandible []. Once fitted to the patients jaw, it was packed with the bone graft and screwed down into position (Fig. ). A slight groove was made on the mandible to allow space for the inferior dental nerve to lay, so that the construct did not compact the nerve and cause functional problems.\nTo complete the surgery, the periosteum was sutured over the construct and then the buccal muscosa was sutured over again to form a double-layered closure (Fig. a–c).The purpose of this was to prevent infection and rejection, by ensuring that there was an increased distance between the foreign object (titanium construct) and the outside environment. The dental implants are scheduled to be inserted several months after this operation. |
The patient is a 47-year-old Caucasian female who presented to the Emergency Department of an academic tertiary-care hospital in the Midwestern United States with complaint of left-sided weakness of the upper and lower extremities and right gaze preference three weeks after a right pontomedullary infarct complicated by Posterior Reversible Encephalopathy Syndrome (PRES) [that initial infarct had been treated in a different state]. Imaging revealed an acute infarct in the posterior limb of the right internal capsule without hemorrhagic transformation and an acute punctate infarct in the right parietal subcortical white matter with corresponding diffusion restrictions, as well as remote evidence of subcortical chronic diffuse microhemorrhages (). The Psychiatry Consultation & Liaison service was consulted on hospital day 2 after the patient reported, “I want to strangle myself with my oxygen cord.”\nOn initial evaluation, the patient reported history of anxiety treated previously by her primary care physician (PCP). She reported she had been frustrated with her medical condition but really did not intend to harm herself. She reported fluctuating mood since her initial stroke and had “good days and bad days.” She denied prior history of inpatient or outpatient psychiatric care or prior suicide attempts. She was oriented to person and place, but not time, was able to state the days of the week forwards, but not backwards, and endorsed visual hallucinations during her hospitalization. This presentation was felt to be consistent with delirium, and she was started on quetiapine 25 mg.\nFollowing a six-day medical admission, the patient was discharged to the acute inpatient rehabilitation unit housed within the hospital. Extensive diagnostic studies did not reveal an underlying etiology for the strokes, which were thought to be due to uncontrolled hypertension.\nPsychiatry was reconsulted by the rehab physicians for management of problematic behaviors. The patient exhibited ego-dystonic behaviors for which she would later apologize including repeatedly climbing out of bed, shouting for nursing assistance without clear need for help, shoving her fist into her mouth to induce vomiting, and periodic, purposeless screaming. These behaviors were disruptive to staff and other patients on the unit. While initially conceptualized as residual hyperactive delirium, her behaviors persisted and continued testing for underlying causes of delirium including electrolyte derangement, occult infection, new or evolving cerebrovascular event, or excess medication burden which were unrevealing\nAfter 60 days of acute rehab, she had reached maximal benefit of that intervention and continued exhibiting behaviors incompatible with nursing home disposition. The patient was then transferred to the university's geriatric psychiatry inpatient unit on an involuntary mental health commitment for behavioral management.\nIneffective medication trials prior to transfer included quetiapine (25 mg at bedtime and 25 mg several times daily as needed), mirtazapine (7.5 mg at bedtime), olanzapine (initial trial of 2.5 mg at bedtime and 2.5 mg several times daily as needed and a second trial of 15 mg and 2.5 mg several times daily as needed), buspirone (15 mg TID), divalproex (initial trial of 750 mg at bedtime and a second trial of 500 mg TID with lactulose and levocarnitine for hyperammonemia), melatonin (9 mg at bedtime), propranolol (40 mg QID), trazodone (150 mg at bedtime), gabapentin (200 mg several times daily as needed), dextromethorphan (20 mg BID, given as Robitussin), and clonazepam (0.5 mg AM and 1 mg PM).\nThroughout this period, the patient remained intermittently apologetic for her behaviors. Orientation was typically attuned to person, sometimes place, and generally not to month or year. She consistently denied depressed mood, anxiety, visual hallucinations, auditory hallucinations, paranoia, suicidal ideation, or homicidal ideation. Thought process remained concrete and perseverative with limited spontaneous speech output and paucity of thought content. Language remained intact without evidence of aphasia. Recent and remote memory were difficult to assess formally due to behavioral disturbance, but she had difficulty remembering recent details of her hospital course and remote details of her life prior to moving to her current city. She required staff assistance for completion of toileting, dressing, and feeding. She had deficiencies in executing complex motor tasks, such as getting out of bed, and was frequently found diagonal in bed with a limb tossed over the side-rail. These deficiencies were in excess of the residual motor effects of her strokes and suggestive of alterations in visuospatial skills, executive function, and planning. Her aberrant vocalizations did not appear goal-oriented and were not ameliorated by staff presence. This presentation persisted and was thought to represent a new cognitive baseline meeting diagnostic criteria for major vascular neurocognitive disorder with behavioral disturbance.\nNonpharmacological strategies including music, sensory stimulation, one to one time with staff, and frequent repositioning were tried without improvement in her symptoms. Additional ineffective medication trials following transfer to inpatient psych included fluoxetine (60 mg per day), retrial of dextromethorphan with fluoxetine as an enzymatic inhibitor (again to 20 mg BID), retrial of quetiapine (up to 600 mg total per day), haloperidol (5 mg several times daily as needed IM), oxycodone (5 QID), lorazepam (up to 6 mg daily), carbamazepine (200 TID), and chlorpromazine (50 QID). Throughout these trials, the patient continued to exhibit frequent periods of severe psychomotor agitation requiring vest restraint and purposeless screaming alternating with periods of oversedation following medications. Other than providing intermittent sedation, no particular combination of medications proved effective in treating the target symptoms.\nAt this point, having exhausted all reasonable behavioral and pharmacologic options, the inpatient psychiatric team recommended ECT as a last intervention prior to pursuing a palliative approach. Medical Ethics was consulted and felt ECT to be consistent with her previously articulated beliefs and wishes.\nThe patient was formally evaluated by the ECT service and, given her incapacity to consent, a court order was obtained for the procedure. She underwent an acute course of bitemporal ECT using a MECTA Spectrum 5000Q machine. She received methohexital and succinylcholine as anesthetic and relaxant agent, respectively. A dose-titration method was used to determine stimulus intensity. She received treatments at 50% over seizure threshold with the following parameters: pulse width: 1 millisecond, frequency: 20 Hz, duration: 2 sec. Treatments were given three times per week. She was maintained on chlorpromazine (50 mg QID) and lorazepam (1 mg QID) during the treatments. Following the sixth ECT treatment, the patient rarely engaged in purposeless yelling, and remained quiet most of the day, experienced normalization of her sleep wake cycle, but still exhibited purposeless movements and psychomotor agitation requiring a vest restraint at night.\nFollowing the third week of ECT treatments, she was consistently having low scores on the Pittsburgh Agitation Scale (PAS) and had minimal requirements for as needed medications for agitation []. While she still required a vest restraint overnight, her psychomotor agitation had improved dramatically. She resumed feeding herself with her right arm and tolerated pureed foods for the first time in six months. Following an acute course of 16 treatments, ECT was tapered to twice weekly and she started sertraline 25 mg in preparation for further decrease in ECT frequency. She remained stable and was successfully discharged to a nursing home with continuation of ECT as an outpatient. Following the expiration of the original court order for ECT, outpatient ECT was discontinued and the patient's family chose to not pursue a renewal of the order for continued treatment. She received 29 treatments in total. Nursing home staff reported that her behaviors remained in control after stopping ECT and she was thereafter able to return home with her parents. |
A 19-year-old female visited our hospital with a 2 wk history of progressive pitting edema in both lower legs associated with a 2 kg weight gain. Symptom onset began shortly after intense physical exertion (dance practice). Her medical history included allergic rhinitis with sensitization to house dust mite and pollen, and she had visited the pediatric allergy clinic for about 10 years. None of her family members had a history of similar symptoms or specific diseases. Additionally, she noted mild pain in both knees and ankles, but tenderness was not remarkable on physical examination. She denied fever, dyspnea, chest pain, and urinary difficulty. She was not taking any medications. Physical examination revealed moderate bilateral pretibial pitting edema. No redness, induration, or thickened skin was observed. No contractures of joints were found, and muscle strength was normal in the upper and lower extremities.\nAt first, she visited the cardiovascular surgery clinic and was admitted for evaluation and treatment of deep vein thrombosis. However, computed tomography (CT) angiography of her lower extremities revealed no evidence of venous disturbance. Treatment with heparin and application of compression stockings did not alleviate her symptoms. Initial laboratory findings included a white blood cell count of 8,090 cells/µL with 28.9% eosinophils. Her eosinophil count was 2,340 cells/µL. Her hemoglobin level and platelet count were normal and her serum total IgE level was 786 kIU/L, but no remarkable change was observed compared to the previous year. The erythrocyte sedimentation rate and C-reactive protein level were not elevated. Serum urea nitrogen and creatinine were normal and urinalysis revealed no proteinuria or hematuria. Serum albumin was unremarkable at 4.2 g/dL. Serum protein electrophoresis results were within the normal range. Other laboratory data, including serum aminotransferase, alkaline phosphatase, cholesterol, and electrolyte levels, were normal. Further evaluations to determine the cause of her symptoms and peripheral eosinophilia were performed. There was no evidence of parasitic infection. A thyroid function test revealed normal thyroid function. The results of electrocardiogram and pulmonary function tests were normal. The patient was negative for rheumatoid factor and antinuclear antibody. An abdominal ultrasound showed no pathological changes. However, during the time of evaluation, the patient's blood eosinophil count increased rapidly to 4,880 cells/µL.\nThe patient was referred to our division of pediatric allergy considering the findings of peripheral eosinophilia and a past history of allergy, and a magnetic resonance imaging (MRI) was performed in her lower thigh. As shown in , the signal intensity of subcutaneous lesions was increased in fat-saturated T2-weighted images, suggesting inflammation of the subcutaneous fat layer and superficial fascia. Mild synovitis of both knees was also suggested. No evidence of thickening or fibrosis of the deep fascia was present. A biopsy of the right dorsal foot was performed, and the biopsy specimen included skin and underlying subcutaneous tissue. Biopsy examination revealed dermal edema and perivenular lymphoplasmacytic and eosinophilic infiltration in the subcutaneous fat tissue (). Based on the MRI and biopsy results, we diagnosed EF. We started intravenous corticosteroid therapy: dexamethasone 15 mg/day on the day of diagnosis. After 2 days of dexamethasone therapy, the symmetric pitting edema improved dramatically. The eosinophil count decreased rapidly from 4,880 to 140 cells/µL. We changed the intravenous dexamethasone to 30 mg/day of oral prednisolone, and the patient received an additional day of therapy with oral steroids. After a total of 3 days of intravenous plus oral steroid therapy, the patient recovered fully. She was discharged from the hospital without medication. Mild edematous changes in both legs developed 2 weeks later, but the patient's symptoms immediately relieved after administration of 30 mg/day prednisolone for 1day. She was monitored in an outpatient clinic for 4 months after cessation of therapy. The most recent eosinophil count was 320 cells/µL, and no relapses were observed. |
This is the case of a 13-year-old black African boy of the Bantu ethnic group, a student, who presented at the pediatric emergency room of Yaounde University Hospital Center with intense chest and vertebral pains, evolving for 48 hours before admission. A week before, in the course of a brawl his left forearm was twisted, resulting in a sharp and permanent pain in his left forearm associated with a functional impotence without any cutaneous lesions. At home, his tutor gave him paracetamol and diclofenac that were administered orally followed by a consultation the next day at a traditional healer. The traditional healer carried out scarifications on our patient’s forearm, consisting of multiple superficial incisions of the skin made by a blade, supplemented by the application on the cutaneous lesions of an ointment composed of herbs, leaves, and earth, which would be likely to contain Clostridium tetani spores. Less than 48 hours later, there was an onset of a generalized pain, predominant in our patient’s back and in his sternal region.\nThe adolescent lives with his aunt in town. There were no elements in favor of a non-accidental injury or child abuse. He had no history of chronic disease; he has never had an operation. The immunization status of the child was unknown to the next of kin.\nOn general examination on his presentation to our emergency department, he was conscious and ill-looking. His temperature ranged between 36.8 and 38.3 °C, his pulse was 88 beats per minute, his pupils were equal and reactive to light stimulus, and his blood pressure was 105/70 mmHg. He presented a trismus, spinal stiffness, a generalized contracture with abdominal rigidity, and opisthotonus. In addition, there were also spasms triggered by noise, light, and touch during care.\nThe loco-regional examination of his left upper limb revealed a balm based on herbs and black earth placed under a traditional splint. After removal of the latter, scarifications were visible with areas of cutaneous necrosis (Fig. ). The rest of the examination was otherwise normal.\nAn X-ray of his left forearm showed a slightly displaced shaft fracture of the two bones of his forearm classified Orthopaedic Trauma Association (OTA)/AO 22-A3 (Fig. ). His hematological and blood electrolytes profiles were within normal limits. No biologic test was performed for the detection of tetanus antitoxin antibodies in whole blood due to the non-availability of this test in our setting at the time.\nThe diagnosis of generalized tetanus complicating a closed fracture of the forearm with subsequent opening was made. Generalized tetanus was thus diagnosed a few hours after our patient’s admission on the basis of the clinical examination in particular, and an obvious point of entry in an individual with unknown vaccination status who clinically presented a trismus, a generalized contracture, as well as paroxysms.\nHe was subsequently admitted 6 hours later to our intensive care unit. He was placed in solitary confinement in a dimly lit room; a nasogastric tube was placed, and sedation and diazepam myorelaxation were performed. The etiological treatment consisted of an antibiotherapy based on metronidazole that was intravenously, directly, and slowly administered and an immunotherapy with equine anti-tetanus serum. Subsequent disinfection of the point of entry with resection of necrotic tissue was performed to reduce microbial growth and release of toxins.\nThe evolution was marked by a gradual deterioration in his state of consciousness, dysautonomic manifestations, particularly blood pressure surges, and tachycardia alternating with bradycardia. Moreover, the occurrence of several episodes of tonic–clonic paroxysms required additional doses of muscle relaxant and intubation. He died a few hours later in our intensive care unit, 12 hours after admission to our hospital. |
KIE was a nine-year-old female foreigner first seen by our centre at the age of 2 years for delayed speech and language development. There was an antenatal history of maternal rubella infection in the first trimester. Clinical examination of the external ear canals and tympanic membranes, then, was unremarkable. She was diagnosed with bilateral sensorineural hearing loss at a profound level for which cochlear implantation was recommended. She had right cochlear implant surgery (Advanced Bionics HiRes 90K HiFocus 1J) performed at another centre when she was 2.5 years of age. After surgery, she returned to our centre for regular audiological and auditory-verbal therapy follow-ups. As she continued medical follow-up with a local otolaryngologist from her hometown overseas, we did not possess records of her medical condition including the status of her eardrums.\nAudiologically, she was doing fine until at 3 years after implantation when she started to experience nonauditory stimulation during mapping. Whenever electrode 16 (the most proximal electrode) was stimulated, she would experience throat irritation and coughing episodes. At a subsequent review 6 months later, the nonauditory symptoms resolved, but she lost her auditory perception at electrode 16 (even at elevated M-levels of more than 400CU). Six months later, she reported pain and discomfort upon stimulation of electrodes 15 and 16. At 5 years after implantation, her score on a speech test was 80% without lip reading.\nAt 6 years after implantation, she returned for an urgent medical consult. She gave the history of suddenly experiencing severe pain and complete loss of hearing during an aural toilet procedure in the clinic the day before. Prior to that, she had been experiencing recurrent itch and discharge from the right ear for a few months but was still able to hear with the implant.\nOn examination, the tip of the electrode array could be seen in the external ear canal (EAC) (Supplementary Fig.). The eardrum appeared to be perforated with the presence of cholesteatoma debris in the middle ear and EAC. Taken together, the clinical impression, then, was that inadvertent explantation had taken place during the aural toilet procedure. The electrode array was probably exposed in the EAC secondary to the development of cholesteatoma as a complication of cochlear implantation. Indeed, X-ray and CT scan performed on the same day confirmed that explantation had taken place with the electrode array lying in the EAC (Figures and ).\nThe family was counseled accordingly. It was recommended that the cholesteatoma be eradicated and that a new device be reimplanted. Although these could be performed in 1 stage, the possibility of a 2-stage procedure was discussed. Radical mastoidectomy with overclosure of the EAC, as well as the pros and cons of mastoid cavity obliteration with abdominal fat, was also discussed. The possibility of failure to reinsert the replacement electrode array and the option of a second side cochlear implant on the opposite (left) ear were highlighted. The parents expressed preference for the avoidance of an additional abdominal wound for fat grafting and opted for a second side implant.\nReimplantation surgery was carried out 2 days later. Intraoperatively, cholesteatoma was seen in the middle ear. Embedding the electrode array, diseased tissue extended into the mastoid cavity through posterior tympanotomy (Figures and ). Histology of a sample of this tissue confirmed cholesteatoma with lamellar keratinaceous debris, strips of stratified squamous and fibrotic stroma with inflammation, and dystrophic calcification. With a canal wall down mastoidectomy, complete clearance of gross disease could be achieved. Soft tissue had formed around the cochleostomy site resulting in obstruction of the cochleostomy opening. Fortunately, following its removal with a pair of microscissors, the cochleostomy opening could be restored which permitted successful reinsertion with the same implant model.\nA blind sac was created by overclosure of EAC. The EAC skin was noted to be thinned and friable, and the nearby soft tissue observed to be scarred from the previous cochlear implant surgery. As planned, the surgery was completed without further mastoid cavity obliteration.\nThe second side cochlear implantation (Advanced Bionics HiRes 90K HiFocus Midscala) was carried out in the left ear without any problems.\nThe blind sac was initially intact but started to breakdown about 3 weeks postsurgery. As it did not heal with medical treatment, she underwent reparative surgery with abdominal fat grafting 1 month later. The blind sac was healthy since and had remained so at least 2 years after surgery.\nThe right implant was activated 2 weeks after reimplantation and the left implant 3 months after that. All the electrodes could be activated with mapping parameters on both sides falling within normal limits. The adaptation to progressive maps of increasing loudness was well tolerated with no further nonauditory stimulation. At 1 year after surgery, she achieved a 100% speech test score on her reimplanted side and 63% on the opposite side. Aided hearing thresholds of 20 dB from 250 Hz to 8000 Hz were attained on both sides. With her new implants, she experienced increased awareness of very soft sounds and was doing well in mainstream school. She had also been swimming regularly without any problems. |
The patient is a 53-year-old male with a history of NKH. His biochemical/clinical diagnosis was initially made as a toddler. This evaluation was initially prompted by a history of physical and developmental delays as well as occasional choreoathetosis. It should be noted that his sister (26 months his junior) also had similar delays prompting further study. Urine chromatography completed on his sister at 35 months of age showed an abnormally high glycine level. In addition to routine urine and hematologic studies (which were normal), a glycine loading study was completed on these patients. Pretest and posttest glycine levels were found to be elevated in CSF, urine, and serum of both patients. Glycine/serine interconversion was also impaired as well as an abnormal electroencephalographic pattern following glycine administration. Based on both biochemical and clinical phenotype at the time, the diagnosis of NKH was made. These patients fell into the attenuated classification of NKH with developmental and physical outcomes much less severe than in the classic type. Follow-up evaluations over the next 6 years showed continuation of developmental delays with a Wechsler Intelligence Scale Revised (WISC-R) of 76 at 9 years 3 months. At the time of diagnosis, the patient was given no form of treatment other than a low-protein diet. Additionally, he was not assigned regular follow-up as this condition at the time was not considered treatable, which was a recommendation carried forward for 36 years. Since diagnosis, he has had no history of seizures, but has had persistent cognitive delay. The patient has lived independently for the majority of his life and has not followed a protein restricted diet nor been on pharmacologic therapy.\nThere have been two reported episodes of metabolic decompensation over the last 2 years. Both were similar in nature with episodes of shaking, worsening ataxia, and fever. At that time, CSF studies showed elevated glycine levels. Gradual recovery occurred without pharmacologic or dietary intervention, although he reports increased tremors and decreased mobility since that time. His most recent admission was in July 2019. He was reported to have started with tremor and dystonia along with confusion and fever. He was presented with tremulousness that was thought to be seizures and required four point restraints to prevent him from falling off the bed. This progressed to needing intubation. Multifocal pneumonia was identified on chest x-ray and antibiotic treatment started. Plasma amino acids obtained during admission and prior to treatment revealed a glycine level of 482 mcmol/l (range: 129-479). Repeat plasma amino acids on treatment and nutritional repletion showed a glycine level of 444 mcmol/L. MRI and CT imaging were unremarkable. He was started on dextromethorphan at 150 mg twice daily and sodium benzoate at 5.5 g 4 times daily (which was eventually stopped in October 2019 due to concerns over worsening tremor). Extubation occurred approximately a week after admission, but worsening of cardiorespiratory status prompted reintubation and transfer to our university hospital for subspecialty care. During this hospital admission, the patient continued to have diffuse myoclonic jerks necessitating neurology consult for further treatment strategies. On EEG these were not epileptic. The patient was eventually extubated and transferred to a rehab facility a month after initial admission. Recommendations going forward were for treatment with dextromethorphan/sodium benzoate and a 40 to 50 g per day protein restriction.\nAt his 6 month follow-up in January 2020, the patient continued to have issues with cognition and recall. He also has issues with tremor, myoclonic jerks, and ataxia since discharge. He is currently on pharmacologic treatment (dextromethorphan) and trying to restrict his protein intake. His myoclonic jerks and tremors seem to be fairly well controlled on clonazepam. His plasma amino acids in January 2020 when off sodium benzoate for approximately several months was 563 mcmol/L. |
The preoperative evaluation of a 79-year-old female patient who was 83 kg with a short neck was nonspecific, other than hypertension. She was scheduled for oophorectomy. The patient was taken to the operating room without premedication and standard monitoring was applied (ECG, SpO2, and noninvasive blood pressure). Intravenous access was achieved by inserting a 20 G intracatheter. After preoxygenation, anesthetic induction was performed using lidocaine and thiopental sodium and tracheal intubation was achieved by rocuronium bromide using an appropriate size endotracheal tube. In addition to 50% O2/air, 0.125–0.25 μg·kg−1 remifentanil infusion and 0.5–1% sevoflurane were used in the maintenance of anesthesia. The right radial artery was used for invasive intra-arterial cannulation and monitoring of arterial blood pressure following anesthesia induction. Central venous catheterization was successful on the fourth attempt through the right internal jugular vein using an appropriate size central catheter (arrow multilumen central venous catheter: 7 Fr). The resident could not puncture the vein at first. Then in the second trial he had difficulty in advancement of the guide wire following a successful puncture. Then the consultant anesthetist tried. In his first attempt, the guide wire could not be advanced. Lastly he put the catheter into the internal jugular vein. We had the central catheter for a possible blood transfusion and central venous pressure monitoring intraoperatively. Blood flow was observed through all lines of the catheter without any difficulty and all lines were irrigated. Subsequently, the surgical procedure was allowed to proceed without having chest X-ray. On the other hand, we did not use ultrasound imaging for either catheterization or correction of its place. The hemodynamics of the patient deteriorated because of the hemorrhage from the surgical site and arterial blood pressure fell to 80/50 mmHg intraoperatively. The heart rate was stable, but it was decided that the lost blood volume was to be replaced. Thus the hemoglobin control resulted in 9 g·dL−1. Since the duration of the operation was extensive and acidosis developed, the patient was transferred to the intensive care unit (ICU) to be followed up. In ICU, the central venous catheter was used also for normal intravenous crystalloid and drug, antibiotic therapy. There was no problem in the speed of infusions. There were no signs of extravasation. Thus, the intensivist was not suspicious of a problem in the catheter. He did not check it with chest X-ray. The patient remained on mechanical ventilator support for one night and was extubated later. Since the patient's hemodynamics were stable and she was conscious, after 24 hours of intensive care treatment, she was transferred to the service. The patient had poor and fragile peripheral veins. Thus, we left the catheter in its place while discharging her from ICU. During her follow-up in the Gynecology Service, the catheter was used as an intravenous route for crystalloid infusion and antibiotherapy. Her central venous catheter was decided to be removed on the postoperative sixth day since oral intake was adequate and the patient was mobile. While removing the catheter, resistance was noted and the catheter could not be withdrawn; therefore it was left in place without application of any force. Posteroanterior and lateral chest X-rays were obtained and the catheter was observed to be knotted in a location close to the subcutaneous tissue (Figures , , and ). The patient was transferred to the operating room and the catheter was withdrawn by the standby of cardiovascular surgery team without any difficulty and a need for surgery. |
A 74-year-old homeless male with a history of chronic alcoholic pancreatitis developed progressive epigastric abdominal pain and nausea that prompted his presentation to the emergency department at a community hospital. Laboratory studies showed mild transaminitis and hyperbilirubinemia (total bilirubin of 4.2 mg/dL) on initial evaluation. Computed tomography (CT) of the abdomen and pelvis revealed obstruction of the pancreatic and common bile ducts by what was felt to be a pancreatic head mass vs pseudoaneurysm of the GDA. Further evaluation with magnetic resonance cholangiopancreatography showed a 6-cm dilation of the proximal GDA consistent with the diagnosis of visceral artery pseudoaneurysm. Endoscopic retrograde cholangiopancreatography was performed with sphincterotomy and biliary stent placement. Pancreatic stent placement was also attempted but was unsuccessful because of significant external compression from the pseudoaneurysm that prevented cannulation of the major duct.\nFollowing the patient's transfer to our facility for a higher level of care, interventional radiology was consulted for visceral angiography and possible coil embolization of the pseudoaneurysm. The angiographic anatomy () was deemed unsuitable for a variety of endovascular interventions. CT angiography was obtained to facilitate thorough operative planning for definitive repair ().\nAfter review of all available imaging, the patient was taken to the operating room. The abdomen was entered via an upper midline laparotomy. A Bookwalter self-retaining retractor (Symmetry Surgical, Inc.) was placed to provide wide exposure. The hepatic flexure and transverse colon were mobilized and retracted inferiorly away from the head of the pancreas. The pulsatile GDA pseudoaneurysm was easily palpable at this point. The gastrohepatic ligament was opened, and the common hepatic artery proximal to the GDA was mobilized. The proximal right and left hepatic arteries were mobilized and controlled with vessel loops given the lack of a proper hepatic artery between the GDA origin and the bifurcation. The pylorus and first portion of the duodenum were then mobilized away from the head of the pancreas. The liver was noted to have good arterial inflow from the celiac trunk, both on palpation and intraoperative ultrasound with temporary occlusion of the GDA. Clamps were applied proximally and distally following heparinization, and the GDA was divided at its origin. At this point, the surgical team believed that primary closure of the arteriotomy created by division of the GDA would result in potentially hemodynamically significant stenosis and compromise hepatic artery inflow. Bovine pericardial patch angioplasty was performed, and the proximal and distal clamps were released without evidence of compromised inflow. Examination of the pseudoaneurysm revealed some continued faint pulsatility. The duodenum was further mobilized, and the anterior inferior pancreaticoduodenal artery distal to the pseudoaneurysm was identified. The artery was clamped with cessation of all pulsatility and subsequently ligated. The pseudoaneurysm was then opened, a large amount of thrombus was evacuated, and an aneurysmorrhaphy was performed. A Blake drain was placed, and the abdomen was closed in the typical fashion to complete the case.\nThe abdominal drain was removed on postoperative day 7. The patient's postoperative course was uncomplicated aside from extensive social work issues because of his transient social situation and difficulty with posthospitalization placement. |
A 79 years old male with multiple comorbidities including hypertension, valvular heart disease, diabetes mellitus and stage 4 chronic kidney disease with a baseline creatinine of more than 300 μmol/L presented with bilateral symptomatic large renal stones for which he underwent staged stone treatment. Prior to presentation to our hospital, he had bilateral double J stent (DJS) insertion and left extracorporeal shockwave lithotripsy followed by FURS and laser stone fragmentation of the left renal stones. Subsequently, he sought medical advice in our facility. Non-contrast CT scan showed multiple bilateral renal stones. In the left kidney, there were 3 stones distributed to middle and lower pole calyces with a stone burden of approximately 3.0 cm as measured using the CT scan. In the right kidney there were also three stones, two in middle calyces and one in the pelvis with a total stone burden of 3.2 cm. After stopping the aspirin for seven days, he underwent simultaneous bilateral FURS and holmium laser lithotripsy and insertion of bilateral DJS under general anesthesia with endotracheal intubation. The surgical procedure took 125 min (65 min for the left side followed by 60 min for the right one) and the procedure was similar in both sides. Following insertion of a hydrophilic tip guidewire (Sensor, 0.038 in), a ureteral access sheath (Inner diameter: 12 Fr, Length: 55 cm) was inserted and the tip was located approximated at the level of ureteropelvic junction. Karl Storz flexible ureterorenoscope (8.5 Fr) was used. During the procedure the normal saline was allowed to run from the bag (approximately 80 cm above the level of the patient pelvis without a pump) and the outflow of saline from around the scope was observed throughout the procedure. Laser energy between 1.0–1.2 joules with a frequency ranging between 8 and 12 Hz (short pulses) were used in both sides. 4200 and 4066 pulses were used in the left and right sides respectively. He was discharged home in a good condition the next day with almost clear urine. Four days later, he reported back to the emergency department with severe suprapubic pain and gross hematuria which required continuous bladder irrigation with a three-way catheter. His hemoglobin dropped dramatically from 121 to 84 g/dl requiring an initial transfusion of two units of packed red blood cells. CT scan showed left-sided subcapsular, perinephric and retroperitoneal hematoma (Fig. ), bladder clots and complete dislodgement of the left DJS. Cystoscopy revealed bleeding from the left ureteric orifice. Evacuation of clots and change of the DJS were performed. Postoperatively, he continued to have hematuria which was initially treated conservatively with bladder irrigation. His Hb dropped again from 113 to 85 g/dl and in view of the ongoing hematuria he required an additional five units of packed red blood cells. In view of the impaired renal functions, the patient was initially reluctant to have angiogram but ultimately it was performed and showed pseudoaneurysms in two small branches of the left main renal artery with extravasation of the contrast outside the renal parenchyma (Fig. ). Both branches were then selectively embolized using 3x2mm microcoils. As results, the hematuria ceased and he was discharged home two days later. On follow-up, his creatinine remained similar to the pre-operative values and in view of the patient comorbidities and of what happened during this procedure; he decided not to go for another procedures for the residual stones. |
A 27-year-old male presented with a 1-day history of intermittent gross painless hematuria. His past medical history included herniated lumbosacral disk with radiculopathy, otherwise unremarkable. Social history included current smoking, 6 pack/year, and occasional EtOH. The patient was single and had no children; family history was negative for genitourinary malignancies. Physical examination was unremarkable with BMI 23 and BP 120/86 and no prescribed medicines or drug use. Laboratory tests showed normal CBC, normal coagulation profile, and normal renal function.\nAxial, contrast-enhanced CT demonstrated a centrally located, 4 x 4 x 4.6 cm, lobulated mass invading the renal vein and extending into the lumen of the infrahepatic inferior vena cava (). CT angiography of the chest showed no evidence of detectable pulmonary emboli and bone scan was negative for metastases.\nRight radical nephrectomy, partial adrenalectomy, inferior vena cava tumor thrombectomy (infrahepatic), and extended retroperitoneal lymphadenectomy were performed; flexible cystoscopy performed during this surgery showed a bulbar urethral stricture (not clinically significant) and otherwise normal bladder. The intravascular tumor pedicle was easily removed intact from the vein lumen by pulling.\nGross examination of the nephrectomy specimen demonstrated a centrally located tumor with no gross invasion of adjacent tissue but with the pedicle extending into the inferior vena cava (). Grossly, the tumor pedicle had a smooth surface and no attachment to the renal vein. Grossly, the mass was partially cystic with variably sized cysts with a smooth lining. The intervening stroma formed grossly discernible nodules of variable thickness. No tumor necrosis was grossly apparent. The lesion appeared to be well demarcated with no invasion of adjacent kidney parenchyma.\nMicroscopically, the tumor was well demarcated with an elongated pedicle bulging into the renal pelvis and renal vein and a biphasic morphology with spindle cell stroma and a benign epithelial monolayer lining the cystic spaces (). The stromal component was composed of uniform spindle cells without cellular atypia, necrosis, or mitoses (). Focally, the stroma was densely cellular, resembling ovarian stroma, but no areas of scarring or fibrosis resembling corpora albicantia of the ovary were identified (). No blastemal, skeletal muscle or clusters of clear cells were seen. The cystic spaces were lined by a single layer of epithelium, which was cuboidal or flattened or, focally, had a hobnail appearance ().\nThe stromal cells were diffusely and uniformly positive for SMA () and desmin and, focally, for CD10, while stains for inhibin, CD34, WT-1, S-100, MART1, and HMB-45 were negative. The epithelial component was positive for CK7 (), for PAX-8, and, focally, for CD10. Immunostains for ER and PR were negative in stromal and epithelial components. The ki-67 index was low (<2%).\nThe tumor pedicle extending into the inferior vena cava showed similar morphology except for some edema and a focal procedure-related hemorrhage. Specifically, no epithelioid morphology and no tumor necrosis or mitoses were seen despite extensive sampling. The pedicle appeared to be floating in the vascular lumen without attachment to, or invasion of, the vascular wall (). The outer surface of the tumor pedicle was covered by endothelial (CD31/CD34 positive) cells (not shown).\nFISH studies for ETV6 rearrangement by an ETV6 break-apart probe on chromosome 12 at 12p13.2 and for SS18 by a synovial sarcoma break-apart probe on chromosome 18q11.2 were negative.\nA diagnosis of “mixed epithelial and stromal tumor (MEST) of the kidney with extension into IVC” was rendered. After surgery, the patient recovered uneventfully and no recurrences have been reported at 3 years' follow-up. |
A 50-year-old Asian woman who had undergone conjunctival and Tenon's capsule removal surgery on the right eye for cosmetic purposes at a local clinic 8 months prior to presentation, was referred to our University-associated tertiary-care eye center due to uncontrolled elevated IOP. She did not have any other significant past medical or social history. She did not take any systemic medications. Before conjunctival removal surgery, she had complained of redness in the right eye for 3 years, although she did not have ocular pain or irritation. The left eye did not show redness. Before surgery, IOP measurement was performed at two different visits. Goldmann applanation tonometry revealed that IOPs were 19 and 13 mmHg at the first visit, and 19 and 12 mmHg at the second visit, in the right and left eye, respectively. The optic disc and visual field (VF) were apparently normal. Average retinal nerve fiber layer (RNFL) thickness, determined by Stratus optical coherence tomography (OCT), revealed mild asymmetry, the average RNFL thickness was 91.77 microns in the right eye and 103.22 microns in the left ().\nHer prior surgery included extensive removal of nasal and temporal bulbar conjunctiva and Tenon's capsule on exposed areas of the sclera. Upper and lower triangular layers of conjunctiva and Tenon's capsule, from horizontal incisions at nasal and temporal canthal areas, were excised. The superior and inferior conjunctiva which were covered by the eyelid were not removed. The surgical area remained bare sclera and the sclera healed with thin overlying epithelium. After conjunctival removal surgery, the patient was treated with Fluorometholone (0.1%) for 2 months.\nThe IOP began to increase in the right eye 1 week after conjunctival removal surgery. The IOP ranged from 30 to 35 mmHg, despite prescription of maximum tolerated medications. An OCT scan performed 4 months postoperatively revealed reduction in average RNFL thickness in the right eye from 91.77 to 78.09 microns, while the left eye demonstrated minimal change, from 103.23 to 100.86 microns (). The patient was then referred to our clinic.\nAt first examination in our clinic, the IOP in the right eye was 38 mmHg with three anti-glaucoma medications (dorzolamide-timolol, latanoprost 0.005%, and brimonidine tartrate 0.15%). The superior and inferior conjunctival and episcleral vessels were severely engorged (), and the nasal and temporal bulbar conjunctival areas were covered with an avascular epithelium, within which a few nasal and temporal episcleral vessels were also engorged (). Gonioscopic examination revealed an open angle, with Schlemm's canal filled with blood to 360 degrees in the right eye (). The left eye was normal. Ocular examination revealed no evidence of bruit, chemosis, proptosis, or extraocular muscle limitation, suggesting no apparent causes for EVP elevation in either eye. Brain and orbital magnetic resonance imaging and angiography results were normal. The VF was also normal 8 months after surgery.\nWe prescribed timolol 0.5%/brinzolamide 1% combination twice a day, apraclonidine 0.5% three times a day, travoprost 0.004% once a day, and oral acetazolamide (250 mg four times a day). After 4 months, the IOP gradually decreased to approximately 25 mmHg, and the oral acetazolamide was tapered. Current treatment includes three topical anti-glaucoma medications, and the IOP remains approximately 25 mmHg. We will consider surgical treatment if a VF defect develops. |
A 14-year-old right-handed female fell from a 10th floor apartment, and presented to our emergency department with multiple trauma. She had no history of any previous illness or medication before the injury. She complained of pain in the abdomen, right elbow, pelvic area, and low back area. She underwent iliac artery embolization and exploratory laparotomy to repair the iliac artery and a liver laceration at another hospital, and was subsequently transferred to our emergency center for additional surgical intervention involving the pelvic bone, spine and anterior trans-olecranon fracture dislocation (). Fortunately, computed tomography (CT) confirmed that she had no brain injury and no systematic neurologic symptoms, although there was a chance fracture of the T10 vertebra. She was subjected twice to a closed reduction of the injured elbow joint, once at the first hospital, and then in our emergency room. According to the Mayo classification, the olecranon fracture was diagnosed as type IIIB. Sixteen days after the initial trauma, the anterior trans-olecranon fracture dislocation of the right elbow joint was treated by open reduction and internal fixation by applying a pre-contoured olecranon plate (Zimmer, Warsaw, IN, USA) using the posterior approach incised along the triceps, followed by application of a long arm splint ().\nAfter surgery, the elbow was immobilized in a long arm splint at 90 degrees of flexion and neutral rotation. Starting from the 3rd postoperative day, the patient was allowed gentle exercise of the right elbow joint, but developed aggravated pain during rehabilitation at 2 weeks post-surgery. The HO was first noted in radiographs, at 3 weeks. At 10 weeks post-surgery, she had increased difficulty in moving her right elbow joint, and had reduced range of motion. The size of HO had progressed and was clearly revealed in radiographs. Physical examination revealed total ankylosis of the right elbow at 45 degrees of flexion, at post-operative 6 months. However, since the radioulnar joint was intact, pronation and supination of the forearm showed the full range of joint motion. Standard anteroposterior and lateral radiographs of the affected elbow revealed an unusually large mass connecting the distal humerus to the ulna (, ). CT scan images revealed the progress of mineralization from the outer margins towards the center area (). After 6 months maturation of the heterotopic mass, surgical excision was carried out using the posterior approach through the previous incision scar. Skin, subcutaneous tissue, and deep fascia were incised in line with the skin incision. The ulnar nerve was identified and retracted. The extent of the mass was exposed adequately and was excised completely from the distal humerus and ulna (). Intraoperative elbow range of motion flexion 0 to 140 degree was checked.\nPostoperative radiographs showed no trace of HO (). One day after the operation, a 1-time dose of radiation therapy (800 cGy) was provided to prevent recurrence. Four days after surgery, the elbow range of motion exercise was started, as tolerated.\nAt 30 months after surgery, patient had no pain in the elbow; radiographs revealed no further development of the HO, and a residual flexion deformity of 10 degrees with flexion up to 145 degrees, and supination and pronation of the forearm were achieved with full range of motion (, ). The patient was permitted to return to play, without any restrictions.\nThe Patient was given an opportunity to review the manuscript and consented to its publication. |
A 42-year-old man presented to the surgical assessment unit with a five-day history of abdominal distention and vomiting and a two-month history of colicky and intermittent abdominal pain in the periumbilical region. He had no specific exacerbating factors for the pain, and he found some degree of relief by taking paracetamol and antispasmodics. He had been experiencing a profound decrease in appetite and had lost over 7 kilograms of weight over the previous few months. No other significant constitutional symptoms of fever or night sweats were positive in the history.\nThe patient had a significant past medical history of tuberculous monoarthritis of the left knee which was diagnosed 9 years prior to presentation. He was commenced on antituberculous therapy (ATT), which he prematurely stopped after four months of treatment without seeking medical advice due to abdominal discomfort. The patient also had a past history of acid peptic disease which was complicated by duodenal perforation for which he had a laparotomy over 18 years prior to the presentation. The patient required a blood transfusion but otherwise had a good postoperative recovery from the operation. When questioned further about this, he and his family were unable to provide further information. The social history confirmed that he was a smoker with a 20-pack year history of smoking. There was no history of alcohol use, and the patient was a taxi driver by profession.\nOn clinical examination, he had a cachectic appearance and was apyrexial and haemodynamically stable. On palpation of the abdomen, there was a mass in the umbilical region measuring 5 × 6 cm that was firm, nontender, and immobile and had indistinct margins. There was evidence of ascites. Bowel sounds were present and normal. No lymphadenopathy was found on physical examination and digital rectal examination was essentially unremarkable.\nBlood investigations initially found microcytic hypochromic anaemia (haemoglobin of 10.4 with MCV of 72). Leukocyte count was 8.4, C-reactive protein (CRP) count was 22, and he had an erythrocyte sedimentation rate (ESR) of 55. The liver function tests were mildly deranged with a raised alanine aminotransferase of 54, aspartate aminotransferase of 66, and a normal bilirubin of 1.0. The coagulation screen showed an international normalized ratio (INR) of 1.4, and the hepatitis serology was positive for antihepatitis C antibodies.\nAn ultrasound of the abdomen showed multiple well defined masses with matted gut loops and the presence of ascites. An ultrasound guided paracentesis was performed under local anaesthetic and revealed a turbid purulent fluid, which was sent for microbiology, cytology, Mycobacterium tuberculosis (MTB) DNA polymerase chain reaction (PCR), and acid fast bacillus (AFB) cultures. The fluid was negative for any organism on grams stain and had a high protein and white cell count. Cytology was negative for atypical cells. Whilst awaiting the results of the AFB cultures and MTB DNA by PCR, a provisional diagnosis of tuberculosis was made after discussion with the infectious diseases department. The patient was commenced on first line ATT, comprising isoniazid (INH), rifampicin (RMP), pyrazinamide (PZA), and ethambutol (EMB). He improved with conservative management and was discharged three days after admission.\nThe patient did not attend his follow-up appointment. However, it was later discovered that both cultures and MTB DNA by PCR was negative in the ascitic fluid.\nThe patient presented four months later to the emergency department with severe abdominal pain and vomiting for two days. On clinical examination, he was apyrexial and haemodynamically stable. Abdominal examination revealed generalized abdominal tenderness with no rigidity or guarding, mild splenomegaly, and sluggish bowel sounds. A repeat ultrasound demonstrated matted gut loops, coarse hepatic echo texture, moderate ascites, and splenomegaly. This raised the suspicion of chronic liver disease (CLD). A computerized tomography (CT) of the abdomen revealed a 9 × 5 cm mass in the right iliac fossa, matted gut loops, enlarged lymph nodes, and splenomegaly. The patient did not improve despite conservative management and an explorative laparotomy was performed five days after admission.\nPeroperatively, it was found that a dense membrane had enclosed the whole of the abdominal contents against the posterior abdominal wall to form three cocoons (). A large cocoon was found extending from the left hypochondrium to the epigastric region (cocoon 1), a second large cocoon was extending from the epigastric region to the pelvis (cocoon 2), and a third smaller cocoon was in the right iliac fossa (cocoon 3). There was also a moderate amount of purulent ascites found within the abdomen. The cocoons were dissected and the membrane was carefully excised to reveal densely adherent loops of gut (). Cocoon 1 was found to contain the spleen, a segment of the transverse bowel, small bowel, stomach, and greater omentum. Cocoon 2 also contained portions of the small bowel tightly matted against each other. Cocoon 3 contained the caecum and terminal ileum. The cocoons were separated by thick sheaths of fibrous tissue adherent to the posterior abdominal wall. The encasing membrane was sent for immediate histological analysis as a frozen section and samples of the membrane and ascites were also sent for routine cytology, histopathology, and microbiology. The frozen section came back negative for malignancy and so an extensive adhesiolysis of the gut was done from the duodenojejunal flexure to the ileocaecal junction and was found to be wholly viable (). The abdomen was closed after saline irrigation and placement of drain.\nThe samples taken during the surgical exploration were later found to be negative for MTB DNA by PCR and the histopathological report of the encasing membrane revealed fibrosis and chronic inflammation, negative for granulomatous inflammation and malignancy.\nPostoperatively, the patient was managed in the surgical intensive care unit for inotropic and ventilatory support. Despite optimal management, the patient developed hepatorenal shutdown and coagulopathy. The patient later developed an upper gastrointestinal (GI) bleeding and sepsis. Endoscopic gastroduodenoscopy (EGD) revealed grade III calcified varices but no evidence of fresh blood. He was transfused multiple units of PRBCs, platelets, and fresh frozen plasma (FFP). The patient then developed enterohepatic encephalopathy and died 14 days after his surgery. |
A 34-year-old Polish Caucasian male was admitted to the Dermatology Clinic of the Poznan University of Medical Sciences due to the presence of an erythematous, exfoliating, clearly distinct lesion located on the index finger of the right hand, spreading onto surrounding areas (Figs. , ).\nThe first signs of the disease had appeared in August 2012, after a contact with raw fish—the patient is a cook. Due to a suspicion of erysipeloid, the patient underwent an antibiotic therapy (intravenous clindamycin, 2 × 600 mg daily, for the period of 5 days) coupled with steroids administered topically.\nA series of diagnostic examinations were carried out during his hospitalization. A swab for bacteriological, skin biopsy for histopathological and a sample for mycological examinations were acquired. The bacterial culture was found to be positive for Enterococcus faecalis. The direct mycological examination was negative. Other examinations, such as the mycological culture and histopathology, were awaited for during the stay of the patient at the clinic. Due to the lack of significant improvement, the patient was discharged, while any further procedures were performed on an outpatient basis at the Dermatology Counseling Service of the clinic.\nThe results of the histopathological examination indicated a widened, swollen epidermis with a thickened stratum granulosum and a thick stratum corneum, in which there were numerous, round, fungal spores of 2.5 μm in diameter (Figs. , ). On the other hand, the result of the mycological culture was negative. Due to discrepancies in the results, a second sample for mycological examination was acquired during a follow-up appointment. This time the results of the direct mycological examination were positive.\nItraconazole (daily dose 100 mg) was administered orally. Additionally, an imidazole derivative was administered topically. The patient claimed an improvement in his dermatological condition after 2 weeks.\nThe mycological cultures were maintained for 20 days at 25 °C on two kinds of media: Sabo Sabouraud dextrose agar with chloramphenicol and Sabo Sabouraud dextrose agar with chloramphenicol and actidione. A fungal colony with a reddish brown color and a brownish reverse, strongly furrowed, suede-like surface and a raised center was observed (Fig. ). A microscopic examination revealed numerous cigar-shaped and thin-walled septate macroconidia. Pyriform microconidia were located on short branches along hyphae (Fig. ). A urease test, carried out on Christiansen’s medium, and dermatophyte test medium examination were both positive. Since the strain matched the original description, it was identified as T. rubrum var. raubitschekii [].\nThe following 3 weeks brought a further improvement in the dermatological status (Fig. ). It was decided to continue the itraconazole treatment while the topical drug was switched to a pyridinone derivative. The patient received itraconazole for a period of 2 months, yet due to low tolerance to the topical drug, the pyridinone derivative was no longer administered. Afterward, the patient showed no signs of the infection.\nIn order to reassess the diagnosis, we analyzed a ribosomal RNA genomic fragment [] and the urease gene of this isolate as described by Hiruma et al. []. Because the isolate ceased to grow on any new media, we isolated its DNA from a plate deposited for long-term storage at our clinic. One hundred and twenty milligrams of the colony was acquired from the deposited agar plate and ground in liquid nitrogen. DNA was isolated with the use of the column-based GeneMATRIX Plant & Fungi DNA Purification Kit (EURx, Poland) that includes both RNase A and proteinase K.\nFirstly, we analyzed a fragment containing partial 18S rRNA gene, ITS1, 5.8S rRNA gene, ITS2 and partial 28S rRNA gene with the use of common ITS1 and ITS4 primers for PCR and sequencing. PCR was performed with the following ingredients: 25 pmol of ITS1 and ITS4 primers each [], 0.5 µl of FastStart Taq DNA Polymerase (2.5 U, Roche), 10 µl of 5 × GC-rich solution, 5 µl of 10 × PCR buffer with 20 mM MgCl2, 1 µl of dNTPs (10 mM each), 5 ng of DNA sample, all in a volume of 50 µl. PCR was performed with the following program: pre-denaturation—4 min, 95 °C; amplification—35 × [denaturation: 1 min, 95 °C; annealing: 1 min, 55 °C; elongation: 1 min, 72 °C]; cooling: 1 min, 40 °C. Gel electrophoresis and paired-end sequencing revealed a 692-bp-long fragment, presenting a 100 % coverage with multiple GenBank T. rubrum entries and with our own T. rubrum reference strain. This result was expected as T. rubrum and T. rubrum var. raubitschekii were found to be identical in this genomic region [].\nSecondly, urease gene fragment was amplified by PCR with the following ingredients: 25 pmol of TrURE1S and TrURE1R primers each [], 0.5 µl of FastStart Taq DNA Polymerase (2.5 U, Roche), 10 µl of 5 × GC-rich solution, 5 µl of 10 × PCR buffer with 20 mM MgCl2, 1 µl of dNTPs (10 mM each), 0.5 µl of DMSO, 5 ng of DNA sample, all in a volume of 50 µl. Touchdown PCR was performed with the following program: pre-denaturation—4 min, 95 °C; amplification—35 × [denaturation: 1 min, 95 °C; annealing 1 min, 69–63 °C, step size 0.5 °C, step delay 0; elongation: 1 min, 72 °C]; cooling—1 min, 40 °C. Gel electrophoresis revealed that the product was approximately 330 bp long, suggesting a urease-positive T. rubrum rather than T. rubrum var. raubitschekii (Fig. ). According to Hiruma et al. [], the genomic sequence of a raubitschekii urease amplicon should also include a 68-bp intron and a 9-bp-long coding insertion (DDBJ AB719058, CBS 100084). The PCR product was sequenced from both ends. This analysis revealed a sequence of 329 bp (including primers) presenting a 100 % coverage with XM_003233873 (T. rubrum CBS 118892 urease (TERG_05790) mRNA) and with the corresponding genomic sequence (NW_003456423.1, Gene ID: 10376003). Both the 68-bp intron and the 9-bp insertion, specific to the CBS 100084 raubitschekii strain, were missing. Based on these results, it is more likely that the isolate was, in fact, a urease-positive T. rubrum. |
In August 2018, a 77-year-old woman presented to our Department of Breast Surgery, Hwa Mei Hospital, University of Chinese Academy of Sciences with a right breast mass for 1 year that rapidly enlarged in the past 2 months. The patient did not report a history of benign breast disease or a history of cancer. Physical examination revealed a large tumor (approximately 10×10 cm) in her right breast, and the tumor had an exogenous growth with an ulcer in the center measuring 5×5 cm and covered by a white and yellow exudate (). Enlarged lymph nodes were seen in the right axillary region. B ultrasonography uncovered a mass measuring 12.6 cm in diameter in the lateral quadrant of the right breast with enlarged axillary lymph nodes and was staged as BI-RADS 5 (). Right supraclavicular lymph nodes displayed unclear boundary and unclear structure. The tumor did not invade the left breast and left axillary region. Chest computed tomography (CT) showed two nodules in the upper lobe of the right lung; a large breast tumor with thickened skin; multiple enlarged lymph nodes in the right axillary region; and abnormal bone density on the left third posterior rib, thoracic 2,3 vertebral body (T2, T3), suggesting lung and bone metastasis (). Bone emission computed tomography (ECT) scan showed radioactive concentration on the left second and third posterior rib, thoracic 2–4 vertebral body, right inferior scapular angle, left ischium, and bilateral sacroiliac joints. Bone metastasis was suspected. Carcinoembryonic antigen (CEA) was elevated (13.67 µg/mL, normal range: 0–5 µg/mL) and other tumor markers were within the normal range. No obvious abnormalities were detected in abdominal ultrasound, cardiac ultrasound, brain magnetic resonance imaging, blood routine examination, and hepatic and renal function tests. Mammography was not performed because of size of the tumor and the exuding ulcer in the center. Core needle biopsy was carried out, which showed invasive ductal carcinoma. Immunohistochemistry showed triple negative breast cancer with ER-, PR-, HER2 0, KI-67 15%, and GATA-3+. The patient was diagnosed with right breast cancer with lymph node, lung, and bone metastasis (T4N1M1). The patient was treated with epirubicin and cyclophosphamide (EC) (E: 90 mg/m2, C: 600 mg/m2) every three weeks. After two cycles of chemotherapy, the patient complained of poor quality of life because of the stench emanating from the exuding ulcer and the need to re-dress several times a day. We advised palliative mastectomy to improve her quality of life. Pathological examination showed that the size of the right breast tumor was 13×9×9 cm and its histological type was metaplastic carcinoma with mesenchymal differentiation. The types of cancer were squamous cell carcinoma and invasive ductal carcinoma. The mesenchymal component comprised high-grade sarcoma with infarction (). It was diagnosed based on the morphology of the tumor. The histological grade was grade III. Immunohistochemistry showed: ER-, PR-, HER2(0), Ki-67: 30%, GATA-3+, CK5/6+, and vimentin (+) which helped confirm a mixture of squamous cell carcinoma, invasive ductal carcinoma, and sarcoma.\nThe patient was put on two cycles of EC followed by six cycles of paclitaxel (P) (80 mg/m2) every week. Unfortunately, the patient could not tolerate the intravenous chemotherapy and was changed to oral chemotherapy of capecitabine (1250 mg/m2 given twice a day from day 1 to day 14) every three weeks after evaluation of stable disease (SD). However, 7 months later, the patient developed lung metastasis. She was treated with nab-paclitaxel and carboplatin (nab-paclitaxel: 100 mg/m2, carboplatin: AUC 2) every week. The patient achieved SD for 4 months and discontinued therapy because of the on-going COVID-19 pandemic. A telephonic follow-up revealed that she was alive despite discontinuing all therapy. |
The patient is a 73-year-old male who was transferred from an outside hospital for repair of a right acetabulum fracture involving the femoral head after falling approximately 8-feet from a ladder while painting his house. Past medical history was significant for hyperlipidemia and osteoarthritis. Baseline metabolic equivalents were greater than four. Aside from a cataract extraction, the patient had no other operations. He denied any allergies. Prior to presentation, the patient was on Aspirin 81 mg daily for cardiovascular disease prevention and Atorvastatin 20 mg daily for hyperlipidemia. Computed tomography (CT) without contrast showed an acute, comminuted, and displaced fracture of the right acetabulum involving both posterior and anterior acetabular walls. The right femoral head was superiorly and laterally displaced with impaction fracture to its inferior and medial aspects. There were bone fragments within the right gluteus musculature, and the high attenuation in tissue density represented blood product within. Prior to surgery, the orthopedic team made an effort to reduce the patient's right acetabulum fracture with tibial traction pin under conscious sedation with midazolam, fentanyl, and ketamine in the emergency department. During the closed reduction, the patient experienced a brief period of respiratory depression. For approximately two minutes, his oxygen saturation was 85-86%, which improved to 95% with Narcan reversal. The emergency department record also noted that the patient had premature ventricular contractions on the electrocardiogram (EKG) at this time. After the closed reduction, the orthopedic team opted to monitor the patient on continuous telemetry for 24 hours and take the patient for open reduction and internal fixation of the right acetabulum the next day. He was not on prophylactic anticoagulation for this preoperative period.\nPrior to entering the operating room, the patient's vital signs were stable: blood pressure 120/64, heart rate 73 beats per minute, respiratory rate 18 per minute, oxygen saturation 95%, and temperature 98.6 Fahrenheit. Neurological exam was significant for limited right knee flexion (<30 degrees). Otherwise, overall sensation was intact and right deep tendon reflex was intact. Hemoglobin was 11.6, down from 13.4 on admission. Coagulation tests showed elevated prothrombin time at 12.8, partial thromboplastin time 26.7, and international normalized ratio 1.20. In the OR, the patient was induced with standard dosing of midazolam, fentanyl, lidocaine, propofol, and succinylcholine. Phenylephrine was given preemptively on induction to avoid hypotension. Intubation was uncomplicated. Patient had two 20g peripheral intravenous catheter in place, and a left radial arterial line was placed after intubation. Despite premedication with phenylephrine, the patient became hypotensive with systolic blood pressure (SBP) in the 80s, median arterial pressure < 65. Boluses of ephedrine and phenylephrine were given, and a phenylephrine infusion was started thirty minutes after induction. The patient initially responded appropriately to treatment (SBP > 100). Due to concern for sciatic nerve injury, intraoperative somatosensory evoked potential (SSEP) monitoring was performed, and general anesthesia was maintained with propofol at 100 mcg/kg/min and remifentanil at 0.2 mcg/kg/min, in addition to 0.5% end tidal sevoflurane.\nUpon patient positioning to the right lateral decubitus position from supine, the patient's SBP acutely declined into the 50s and the patient became hypoxic with an oxygen saturation of 88%. His hypotension was no longer responsive to phenylephrine or ephedrine. Norepinephrine and epinephrine drips were started. Also, the FiO2 was increased from 50% to 100%. There was a minimal and transient response to epinephrine administration. The patient was then returned to supine position, and he arrested shortly thereafter. Chest compressions commenced immediately upon loss of cardiac rhythm. The cardiac anesthesia team emergently responded and performed an intraoperative transesophageal echocardiography (TEE), which showed reduced left ventricular ejection fraction and reduced right ventricular systolic function. Due to unsuccessful cardiopulmonary resuscitation, the patient was placed on venoarterial ECMO via right femoral artery and vein; stable hemodynamics were achieved. A total of 8000 units of Heparin was given upon ECMO installation. At this point, the planned surgery was aborted. The patient was transported to the cardiac catheterization lab for pulmonary angiogram, cardiac catheterization, and antegrade perfusion of the right superficial femoral artery. A large thrombus in the left pulmonary artery was discovered on selective angiography (Figures –). Hypothermic protocol was initiated. Heparin therapy was also continued at this point.\nOn hospital day 5, a CT chest with contrast showed multiple bilateral pulmonary emboli and evidence of right ventricular failure. Right heart catheterization and placement of a Swan-Ganz catheter demonstrated that the patient was found to have severe pulmonary hypertension 55/30 and elevated central venous pressure (CVP) of 18. The patient underwent bilateral pulmonary artery embolectomy, exploration of right atrium with removal of clots, discontinuation of ECMO support, and repair of right femoral artery on hospital day 9. His pulmonary artery pressure and CVP improved to 31/12 and 8, respectively. One month later, the patient was discharged to a rehabilitation facility with a plan for conservative management of his fracture. |
Eleven years ago, when our patient was a 33-year-old unmarried nulligravida, she developed CCC of the ovary for the first time. At that time, her menstrual cycle was regular and 30 days long, with dysmenorrhea, manifesting as lower abdominal and lumbar pain, persisting for 5–7 days. There was no family or medical history of note. She had visited a local clinic with the chief complaint of lumbar pain, where a right ovarian tumor 8 cm in size that included solid internal components, was identified, and she was referred to the University of Tsukuba Hospital. Transvaginal ultrasound had revealed a right adnexal mass with solid components, 68 mm × 53 mm in size, and an intramural uterine fibroid 23 mm × 27 mm, with no hypertrophy of the endometrium, which measured 2.1 mm. Blood counts and blood biochemistry test results revealed no abnormalities. Her serum CA19-9 level was 45.9 U/ml, CA125 was 22 U/ml, and CEA level was 1.3 ng/ml. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) revealed an 80-mm unilocular cystic mass in the right ovary with a papillary protrusion of longest diameter 45 mm. The left ovary was not enlarged, and there was no obvious peritoneal dissemination or enlarged lymph nodes. Surgery, comprising right adnexectomy, left ovarian biopsy, partial omentectomy and uterine fibroid removal, was performed. The right ovary was enlarged to 8 cm in size, and it was removed without intraoperative rupture and with its capsule intact. The left ovary was not enlarged, but was seen to have a small endometrial cyst, which was resected. The absence of peritoneal dissemination and enlarged lymph nodes was confirmed during surgery. The pathological diagnosis was CCC localized to the right ovary and endometriosis was observed in the left ovary, with ascites cytology Class III. The cytology was composed of clusters that include atypical cells having somewhat nuclear enlargement, and it makes a diagnosis difficult to distinguish mesothelial cells from malignant cells. The patient expressed a strong desire to preserve her fertility. Hence, pelvic and para-aortic lymph node dissections were performed via staged laparotomy, which revealed no metastases in any of the 91 pelvic or para-aortic lymph nodes that were removed, leading to a diagnosis of Stage IA CCC. Four courses of postoperative combination paclitaxel (175 mg/m2) and carboplatin (AUC6) chemotherapy (TC therapy) were administered to complete the initial treatment.\nNine years after the initial therapy, MRI during regular 6-monthly monitoring revealed the appearance of a 95-mm polycystic mass with a mural nodule of longest diameter 53 mm in the left adnexal region. Those findings were never seen at the previous MRI. At this time, her serum CA19-9 level was 12.9 U/ml, CA125 was 18.3 U/ml, and CEA level was 0.5 ng/ml, all of which were within normal limits. Disease recurrence in the contralateral ovary was diagnosed, and total abdominal hysterectomy, left adnexectomy and omental biopsy were performed. At the time of surgery, the left ovary was enlarged to 95 mm in size, and it was removed intact without intraoperative rupture of its capsule. The internal lumen of the tumor contained several milky-white mural nodules extending around 15 mm into the cavity (Fig. ). The histological diagnosis was CCC, but in addition to the possibility of recurrence, it was also considered that the tumor might have developed de novo, and the fact that clear cell adenofibroma (CCAF) was also present (Fig. ) suggested that this might have provided the genesis for its development. In addition, there were no endometriotic lesions in the non-solid cyst wall, and it only consists of fibrous membrane. The tumor was localized to the left ovary, and since ascites cytology was negative, it was diagnosed as Stage IA disease. The treatment was completed with four courses of postoperative TC therapy.\nTwo years after the completion of treatment, the patient is continuing outpatient monitoring with no sign of recurrence to date. |
A healthy 11-year-old girl was presented to the National University of Malaysia (UKM) Paediatric Dental Clinic with a referral for further management of pulp necrosis of an immature lower right second premolar (tooth 45), secondary to the fractured tubercle of dens evaginatus. Two weeks earlier, she had treatment at a general dental clinic for pain related to tooth 45. Tooth 45 had spontaneous and lingering pain following cold and thermal stimuli. The tooth was diagnosed to have symptomatic irreversible pulpitis, and root canal therapy was initiated. The canal was accessed, and pulp extirpation performed before the placement of intracanal nonsetting calcium hydroxide by the general dental practitioner (GDP).\nAt the time of current assessment, her tooth-related symptoms had completely resolved. General oral examination showed the presence of generalised mild gingivitis with a basic periodontal examination (BPE) score of 1 in all sextants. The patient's oral hygiene was fair with a plaque score of 30%. The patient is still in her mixed dentition with the presence of the primary maxillary canines. Her upper dental arch was well aligned, and mild crowding of anterior teeth was noted in the lower arch. Tooth 45 has an occlusal glass ionomer dressing of the access cavity made for the pulp extirpation earlier by the GDP (). Cold and electric pulp sensibility testings showed positive responses to all fully erupted premolars indicative of tooth vitality expect for tooth 45. Tooth 45 also has slight tenderness to percussion. Periapical radiograph of tooth 45 showed an immature root with convergent open apex and small periapical radiolucency. The pulp space of tooth 45 is of an even width from the coronal to the apical portion (). Based on the assessments, tooth 45 was diagnosed with pulp necrosis secondary to fractured dens evaginatus and symptomatic apical periodontitis.\nOn the day of initial assessment, tooth 45 was isolated with rubber dam after infiltration of local anaesthetic solution (2% lidocaine with 1 : 80000 adrenaline). Pulp chamber was reentered through the previously prepared access cavity. The root canal was exposed and irrigated with saline. After that, the canal was dried with paper points and the tooth working length was estimated with a K-file No. 60. A working length, 2 mm short of the apical opening, was determined (17 mm). The canal was gently prepared with the K-file No. 60 and then irrigated with a copious volume of 1.5% sodium hypochlorite (NaOCI). After drying the wet canal with paper points, nonsetting calcium hydroxide was placed into the canal and the access cavity was double sealed with Cavit™ 3M, USA, and glass ionomer cement (GIC) (Riva Self Cure™ SDI, Australia).\nTwo weeks later, the tooth was reassessed for any signs and symptoms of infection. The tooth was no longer tender to percussion, and there was no indication of infection-related signs and symptoms. After isolation with a rubber dam, the root canal of tooth 45 was reaccessed and irrigated with a copious volume 1.5% NaOCl to remove the nonsetting calcium hydroxide. Then, the canal was irrigated with sterile water and dried with paper points. Subsequently, the canal was irrigated with 17% EDTA (Pulpdent™, Watertown, Massachusetts) for a minute and dried with paper points. Finally, under the guidance of a dental operating microscope (Carl Zeiss Surgical GmbH, S100), the apical region was filled using the EndoSequence (BC RRM-Fast Set Putty, Brasseler, USA) material up to 4 mm thickness to create an apical seal (). The orifice of the root canal was double sealed with a cotton pellet, temporary filling material (Cavit 3M, USA), and GIC (Riva Self Cure SDI, Australia).\nOnce again, the root canal was reaccessed two weeks later, irrigated with 1.5% NaOCl, and dried with paper points. Next, the dried canal was obturated with thermoplasticised gutta-perca using the Obtura III Max System (Obtura Spartan® Endodontics) (). After that, the access cavity was double sealed with GIC (Riva Self Cure SDI, Australia) and nanohybrid composite (AURA™ SDI, Australia), respectively.\nFollowing the obturation, tooth 45 was reviewed at three-month and six-month intervals. During both reviews, tooth 45 was asymptomatic. However, at the six-month review, a periapical radiograph of tooth 45 showed an unexpected finding. The apical root of tooth 45 continued to grow beyond the apexification level with a normal periodontal ligament space and lamina dura. No evidence of periapical radiolucency was noted (). However, regular annual monitoring of tooth 45 is essential to ensure that the coronal seal is intact and no apical complication further arises. |
The patient, an 18 years old Caucasian boy, is the second child of consanguineous parents (second degree cousins). His medical history was unremarkable until the age of 7 years, when he developed progressive gait, running disturbances and difficulties in playing soccer. At the age of 9.7 years, he experienced a severe ketotic, hypoglycemic attack. At the age of 10 years, due to the progressive worsening of the neurological symptoms, he was referred to the Unit of Neurology of the University of Messina, where a complex picture of upper and lower motor neuron involvement was recognized. Magnetic resonance imaging of the brain and cervical spinal cord was normal. On that occasion, the presence of marked skin pigmentation and brown spots of the tongue, together with a clinical history positive for ketotic hypoglycemia, suggested the diagnosis of adrenal insufficiency. This clinical suspicion was confirmed by a typical hormonal profile with very high ACTH levels and undetectable levels of cortisol, both basal and stimulated by i.v. ACTH. Further investigations showed an autonomic dysfunction with postural hypotension and abnormal heart reflexes. The oral treatment with hydrocortisone, at substitutive doses (10 mg/m2/day), caused a significant improvement of the patient’s general well being, although ACTH levels remained elevated. The coexistence of ACTH-resistant adrenal insufficiency and neurological manifestations suggested the hypothesis of triple A syndrome, that was confirmed by DNA analysis of the AAAS gene. In fact, sequencing the coding regions and exon/intron junctions of this gene identified a homozygous splice mutation in intron 14. This is a G > A replacement at the first nucleotide in intron 14, resulting in aberrant splicing (IVS14 + 1G > A). As expected, the parents were both heterozygous carriers of the mutation, as well as the two clinically healthy siblings [].\nDuring the clinical assessment, an ophthalmologic exam was performed, because the patient referred of symptoms characterized by foreign body sensation and itching, starting in the morning and worsening in the evening, mild dryness and light sensitivity. Before the clinical exam, an informed consent was obtained. The clinical procedures were in accordance with the Declaration of Helsinki.\nThe ophthalmologic exam included visual acuity assessment, ocular surface study, tonometry, and fundus examination. For the ocular surface study, the following tests were performed: tear osmolarity measurement (TearLab™ Osmolarity System Inc., TearLab™ Corp., San Diego, CA, USA), slit-lamp examination of the ocular surface, tear film break-up time (BUT), corneal fluorescein staining, Schirmer’s I test (SNO strips, Laboratoire Chauvin, Aubenas, France), lid margin assessment, corneal sensitivity, in vivo corneal confocal microscopy (Confoscan 4, Nidek, Vigonza PD, Italy) and conjunctival impression cytology (Supor 200 membrane filters, Gelman Sciences, Ann Arbor, MI, USA) [, ].\nThe best-corrected visual acuity was 20/20 in both eyes. Tear osmolarity was 300 mOsm/l in the right eye (RE) and 332 mOsm/l in the left eye (LE). The slit-lamp examination showed an unstable tear film with a BUT of 2 sec in both eyes. A mild conjunctival fluorescein staining was present in the LE conjunctiva in both interpalpebral areas; no signs of epithelial damage were present in the RE. A mild papillary hypertrophy was present in the upper tarsal conjunctiva in both eyes. Schirmer’s I test was 0 mm/5’ in both eyes. The lid margins showed a mild hyperemia, with meibomian gland expression giving rise to slightly turbid meibum. The keratoaesthesiometry was within normal values (RE = 1.08 g/mm2; LE = 0.96 g/mm2). In both eyes, the corneal confocal microscopy showed normal epithelial features. The subbasal corneal nerves presented a rectilinear course, with occasional beadings (Figure A). Stromal keratocytes, either in the anterior (Figure B) or in the posterior stroma (Figure C), showed morphological features typical of activated cells. Endothelial cells were normal (Figure D). The conjunctival impression cytology showed in RE the presence of islands of bilayered epithelial cells, together with areas of isolated cells. The cells within the bilayered tissue appeared of normal size, with N/C of 1:1 or 1:2. Also the isolated cells showed a normal N/C, although cells with reduced N/C could be seldom observed. Nuclear chromatin showed a condensed appearance. Goblet cells were normally represented. A mild keratinisation was present in some cells (Figure A). The LE showed the presence of islands of bilayered cells, together with areas of isolated and highly keratinized cells with a metaplasic appearance and pyknotic nuclei. Particularly in the isolated cells, the N/C was reduced (Figure B). In the multilayered areas some goblet cells were observed. The intraocular pressure was 14 mmHg in both eyes. The fundus exam was normal in both eyes. |
A 32-year-old man with a remarkable history of polysubstance abuse presented to the emergency room with a 2-week history of diplopia. Six weeks earlier, the patient had presented to the emergency department after being found unconscious secondary to a suspected drug overdose and a question of possible physical assault and head injury. Toxicology panel was positive for cocaine, cannabinoids, and opioids. The patient was observed and improved, returning to his neurological baseline level after overnight monitoring. He was unable to recall additional details of the event and was discharged the day after his presentation. Cranial imaging during this initial encounter was not obtained by the trauma team. Approximately 4 weeks after his initial presentation, the patient reported to his primary care physician because he was experiencing diplopia. Brain MRI was obtained as part of an outpatient workup 2 weeks following symptom onset, and the sagittal T1 sequence revealed an abnormal rim – hyperintense core – heterointense lesion on the sagittal T1 sequence []. Fluid-attenuated inversion recovery (FLAIR) T2 sequence more specifically revealed a 6 cm × 3.6 cm × 3 cm hyperintense [], diffusion restricting, and apparent diffusion coefficient hypointense extra-axial collection without surrounding edema [ and ]. Significant mass effect on the temporal lobe and left midbrain [] was observed, and the patient was referred to the emergency department for further evaluation.\nAt the time of neurosurgical evaluation, the patient complained of double vision with downward gaze in the bilateral lower visual quadrants. He denied any alleviating or exacerbating factors and described it as constant throughout the day. The patient was otherwise healthy, with no other medical conditions. Contrast-enhanced CT of the head was obtained to assess for the progression or change in the lesion observed on MRI and demonstrated the similar contrast rim-enhancing lesion with significant surrounding mass effect [ and ]. Given the history of possible remote assault as well as aforementioned imaging findings, the lesion was suspected to be a chronic epidural hematoma. There was also consideration given that this might be an abscess due to the patient’s history of polysubstance abuse, but no clinical signs or symptoms of infection were noted.\nDue to the significant size and mass effect on the left midbrain in addition to the diplopia, the patient was taken for a left temporal craniotomy for evacuation and removal of the lesion. A small temporal craniotomy was performed. On removal of the bone flap, normal dura was not clearly visualized. Instead, a rather thick, yellow fibrous-like tissue was observed []. This tissue was carefully dissected, and specimens were sent for pathological analysis. Underneath this tissue, a consolidated subacute hematoma clot was found. The clot was removed and another layer of the thick, yellow tissue was encountered, which was also carefully dissected. Once this capsule of tissue was removed, normal dura was encountered. Interestingly, no inflammatory or purulent material was encountered during the procedure. No clear sources of bleeding, including arteries or veins, were identified. The cavity was copiously irrigated with antibiotic instilled irrigation. The dura was tacked up; the bone flap was placed back; and the wound was secured. Pathology results revealed an organized hematoma with marked fibrosis.\nPostoperatively, the patient remained neurologically unchanged and was observed in the intensive care unit for 2 days. On postoperative day 1, a CT scan of the head showed gradual parenchymal expansion into the void that had previously been occupied by the hematoma. The patient was discharged on postoperative day 5. At the 6-week postoperative follow-up visit, he had near-complete resolution of his diplopia and complete resolution of his epidural hematoma on head CT [ and ]. |
A 32 year old male presented to us with complaints of pain and swelling of multiple joints of one month duration. The symptoms started in the right knee but soon involved the left knee, both ankles and hips. The pain was associated with morning stiffness which persisted for around one hour. Small joints of the hand were not involved. There were no ocular complaints. The patient also complained of backache which began at around the same time as the joint pain. No complaints of numbness, parasthesias, root pain, falls, and bladder or bowel symptoms were present. A fortnight later the patient developed skin lesions which began as fluid filled lesions over the back and later involved all limbs and the forehead. The patient also complained of some lesions over the penis. The patient gave history of multiple unprotected extramarital sexual contacts. He gave a history of dysuria prior to the onset of joint pains. He was a chronic smoker and occasionally took alcohol.\nOn examination he appeared pale but had no lymphadenopathy. He had multiple discrete to coalescent erythematous plaques with brown crust and dirty white scales on the back, forehead, all four limbs and buttocks (Figure ). Grattage test was positive but Auspitz sign was negative. Toe nails had nail plate discoloration and subungual hyperkeratosis. Multiple pin head sized erosions were present over glans penis and corona of penis in a circumferential manner suggestive of circinate balanitis. The chest expansion was reduced. The examination of cardiovascular and gastrointestinal system was normal. Testing for power was limited due to pain in joints. However, the patient had lower limb hypereflexia, sustained ankle clonus and extensor plantars. There was no evidence of any sensory loss, root pains, bladder or bowel involvement. Examination of spine revealed tenderness at second to sixth thoracic and the lumbar vertebrae. Straight leg raising test was negative. Modified Schober test revealed lumbar expansion of 3 cm. Locomoter system examination revealed swollen knees with a positive patellar tap. Direct pressure over the sacroiliac joints elicited tenderness. Joint movements were restricted at both ankles and knees.\nHe was anemic (Hemoglobin-8.7 g/dl) with a microcytic hypochromic picture. He had normal leukocyte count, raised Erythrocyte Sedimentation Rate (60 mm in 1st hour), normal serum uric acid, liver and renal functions. Rheumatoid factor was negative and ASLO titer was not raised. Semi quantitative assay for CRP titer was positive (>6 mg/L). Serology for HIV, HBV, HCV, syphilis and HSV-1 and 2 was negative. ANA was negative. HLA-B 27 was positive. Urethral smear revealed multiple neutrophils but urine culture revealed no growth. X rays of chest, hands, knees and feet were normal. However roentgenograms of spine and sacroiliac joints revealed sclerosis at both sacroiliac joints and marginal syndesmophytes on lumbar spine (Figure ). Echocardiography revealed mild aortic root dilatation and aortic regurgitation. USG abdomen was suggestive of cystitis. Skin biopsy was consistent with psoriasiform dermatitis. MRI spine revealed multiple hyperintensities in the spinal cord in the region of T9–T11 (Figure ) and in the medullary conus. The patient could not afford Chlamydial PCR. Lumbar puncture was done and the cerebrospinal fluid was clear with 35 cells/μL predominantly lymphocytes, with protein of 48 mg/dL and glucose 52 mg/dL. No oligoclonal band was seen on electrophoresis. Gram stain and cultures of CSF were negative. |
This case illustrates a 20-year-old female diagnosed with MS at the age of 17. She was initially treated with tocilizumab as neuromyelitis optica was suspected due to bilateral optical neuritis and the presence of spinal cord lesions. However, antibodies against aquaporin-4 and myelin oligodendrocyte glycoprotein were not detected and the magnetic resonance imaging (MRI) of the brain and spinal cord as well as cerebrospinal fluid (CSF) findings were supportive of MS. Apart from persistent bilateral severely reduced visual acuity she had no other signs of neurological dysfunction. She had previously been in good health and had no family history of PID, or other systemic inflammatory diseases.\nEighteen months prior to the episode of arthritis and skin symptoms reported here, she was started on off-label treatment with rituximab (RTX). RTX is the most frequently used immunomodulatory drug for MS in Sweden according to the Swedish MS registry (). Initially, she received 1,000 mg of RTX followed by 500–1,000 mg every 6th month, resulting in depletion of circulating B-cells (<0.001 ×109/L). During this period, there were no signs of neuroinflammatory activity of MS.\nA rheumatologist confirmed the diagnosis of monoarthritis. The right knee had typical signs of inflammation with rubor, tumor, and calor accompanied by a discretely reduced range of motion. The general status was good without fever. The lower right leg was diffusely swollen and two circular erythematous areas around the ankle were seen (). A dermatologist interpreted the skin symptoms as possible panniculitis with atypical erythema nodosum as a potential alternative diagnosis. There were no other clinical or laboratory findings of sarcoidosis.\nTreatment with RTX had been ongoing for approximately one and a half year prior to the onset of arthritis and the cutaneous symptoms had been present for at least 6 months prior to the diagnosis. The last dose of RTX was given 1.5 months before the onset of symptoms related to Lyme disease.\nAspiration of synovial fluid resulted in a limited volume, only sufficient for microscopic examination of crystals. Neither monosodium urate crystals nor calcium pyrophosphate crystals were detected in the joint fluid. Duplex ultrasonography of the lower leg showed no signs of deep vein thrombosis and there were no laboratory signs of systemic inflammation. Serological analysis performed 5 months after the last dose of RTX showed borderline levels of immunoglobulin (Ig)M and IgG antibodies against recombinant Borrelia antigens (Liason®, Borrelia IgM detecting OspC and VlsE; Borrelia IgG detecting VlsE). The results were interpreted to be of uncertain clinical significance. Laboratory results are detailed in .\nDespite the vague antibody results, there were an enduring clinical suspicion of Borrelia infection. Skin biopsies from one of the erythematous areas at the ankle were performed. Standard histopathology showed mild non-specific inflammation. Borrelia-DNA was detected in the biopsy analyzed by polymerase chain reaction (PCR). The method amplifies a 116 base-pair long fragment of the 16S rRNA gene. In addition, a lumbar puncture was done, and CSF was analyzed without presence of intrathecal Borrelia-specific antibodies or elevated levels of the B-cell chemokine CXCL13. Thus, the final diagnosis was Borrelia associated dermatitis and arthritis (Lyme disease).\nPrior to the diagnosis of Lyme disease, the patient was prescribed topical steroids for the skin manifestations and the joint symptoms were managed with paracetamol. Once the diagnosis of Lyme disease was confirmed, doxycycline 200 mg once daily for 3 weeks was prescribed. The knee and skin symptoms dissipated during the following month.\nAt the last follow-up 1 year after the antibiotic treatment had been ended, there was still minor swelling of the lower leg but no signs of arthritis or dermatitis. An MRI of the lower leg showed mild oedema in musculus soleus and gastrocnemius. Creatinine kinase in plasma was within normal reference. |
The patient was a 55-year-old African American male with a significant past medical history of known breast cancer, who presented to an academic teaching hospital in February of 2018 after a surveillance computed tomography (CT) scan of his chest, abdomen, and pelvis showed incidental acute appendicitis. The patient was asymptomatic on his original presentation but subsequently developed nausea and vomiting along with right lower quadrant abdominal pain in the following days resulting in admission to the general surgery service for treatment of appendicitis. The patient did not have any contributory family, drug, or psychosocial history.\nOn chart review, the patient had an extensive past oncological history dating back to 2014 after resection of an enlarging, exophytic, ulcerating chest mass on the right side just lateral to the midline. Pathology from the wide local excision of this mass demonstrated estrogen receptor (ER) positive, progesterone receptor (PR) positive, and HER2/neu negative metastatic adenocarcinoma with an unknown primary source at the time. He underwent esophagogastroduodenoscopy (EGD) and colonoscopy to rule out a primary gastrointestinal malignancy, however, both were normal. He was followed closely by medical oncology and treated with tamoxifen for hormone therapy. Due to an enlarged anterior mediastinal wall lymph node discovered on routine CT surveillance in May of 2017, the patient underwent CT-guided biopsy and positron emission tomography (PET). The biopsy was negative for malignancy, however, the PET demonstrated abnormal, hypermetabolic activity within a retrosternal nodule, intense activity in a mixed focus in the manubrium, and several hypermetabolic nodes within the mediastinum. It also showed an abnormal hypermetabolic focus in the cecum suspicious for a colon primary (, ). An attempt was made to complete a colonoscopy at the time, but the gastroenterologist could not advance the colonoscope past the transverse colon due to technical reasons. Medical oncology elected to continue close surveillance.\nIn January 2018, the patient was noted to have right axillary lymphadenopathy and an ultrasound-guided biopsy of a 1.5 cm right axillary lymph node was performed. Pathology results showed an infiltrating, moderately-differentiated ductal carcinoma. This specimen was also ER and PR positive as well as HER2/neu negative.\nA CT scan of the patient’s chest, abdomen, and pelvis was completed on February 15, 2018 for further monitoring. At this time, the patient was incidentally found to have a dilated appendix with no wall thickening or periappendiceal stranding. He did not have a leukocytosis and clinically he did not have any fever or any symptoms including pain, nausea, or vomiting at this original presentation. However, he returned two days later with nausea, vomiting, and right lower quadrant pain consistent with appendicitis. On a repeat CT of the abdomen and pelvis with IV and oral contrast, the appendix was dilated to 1.8 cm with appendiceal wall thickening and periappendiceal stranding. In addition, a 2.3 × 1.9 × 2.3 cm bowel mass was identified in the right lower quadrant either adjacent to or within the wall of the cecum that correlated with the previous PET-CT scan performed in May 2017 (). He was therefore admitted and treated with IV antibiotics for acute appendicitis with plans for appendectomy during his hospitalization along with further workup of the cecal mass.\nBecause of his oncological history, a colonoscopy was performed on hospital day one to evaluate the cecum to rule out metachronous colon tumors given his CT imaging findings and previous PET-CT. The colonoscope was advanced to the cecum without issue. Cecal inflammation was noted and several biopsies were taken of this area, however, no discernable tumors were noted. Pathology returned with no significant pathologic changes to the biopsied tissue.\nThe following day after receiving intravenous antibiotics and fluid resuscitation, the patient underwent laparoscopic appendectomy. The procedure was converted to open due to the extensive inflammatory process in the right lower quadrant and retrocecal position of the appendix. The appendix was divided from the cecum using a GIA stapler and the appendiceal vessels were suture ligated and divided with electrocautery. The specimen was removed from the field and sent to pathology for evaluation. Prior to abdominal closure, the colon was run from the terminal ileum to the rectum and a large walnut-sized mass was discovered in the cecum proximal to the area of the appendiceal orifice. It was hard, mobile, and suspicious for a primary colon lesion. A right hemicolectomy was then performed including adequate mesenteric lymph node resection. The operation was performed by a general surgeon and a chief resident. The patient tolerated the procedure without complications and was returned to the surgical floor postoperatively. His post-operative course was unremarkable, and he was discharged five days after the procedure.\nPathology from the surgical specimen returned shortly after the surgery. The appendix showed findings suggestive of acute appendicitis with organizing periappendicitis. The right colon specimen returned as metastatic breast carcinoma involving cecal submucosa clear of margins with overlying benign colonic mucosa (). Twenty lymph nodes were harvested and were all negative for disease. The specimen stained strongly and diffusely for GATA3 which was consistent with metastatic breast carcinoma and negative for CDX2 (A–D).\nAfter discharge, the patient had close surveillance with medical oncology. He was transitioned to anastrazole secondary to the ER/PR + receptor status of his tumor and its refractory response to tamoxifen. Prior to his first outpatient appointment in April 2018, he complained of worsening skeletal pain at multiple sites. Bone scan showed left posterior rib and sternal involvement for which zoledronic acid was initiated. He also underwent CT scan of his chest, abdomen, and pelvis to monitor progression of his metastatic disease which showed no significant changes. During his appointment in June 2018, he was noted to have an enlarging left lateral neck mass. Ultrasound-guided biopsy of this mass returned positive for metastatic carcinoma consistent with breast origin. No significant changes to his treatment regimen have occurred since this time. |
A 64-year-old Caucasian male who was treated over the course of 7 months for multiple intrahepatic abscesses, left portal vein and segmental hepatic arterial thrombosis ultimately underwent an extended left hepatectomy and was found to have XGC. The patient had a medical history significant for non-insulin dependent diabetes, chronic renal failure, chronic hepatitis C and coronary artery disease. He initially presented to the hospital seven months prior to his eventual surgery with diabetic ketoacidosis and at the time was found to have hypodense areas in the lateral and medial segments of the left lobe with extension to the hilum concerning for evolving phlegmon of the liver. Subsequent ultrasound of right upper quadrant revealed acute left portal vein thrombus (Fig. ). The patient was started on heparin infusion and transitioned to coumadin. He was subsequently readmitted with sepsis and underwent an attempt at image-guided drainage of the liver abscess (Fig. ) where no purulent fluid could be aspirated. A solid lesion was noted which was biopsied with final pathology noting organizing hematoma with acute and chronic inflammation.\nRepeat CT imaging revealed continued thrombosis of the left portal vein and anterior branches of the right portal vein and right hepatic artery (Fig. ). Due to persistent left intrahepatic abscess and concern for an underlying obstructive process in the left biliary system, the patient underwent an endoscopic retrograde cholangiopancreatography (ERCP) which revealed choledocholithiasis which was removed followed by sphincterotomy and stent placement. He subsequently underwent a spyglass™ procedure which noted a left biliary tract occlusion secondary to a mass which was biopsied. Pathology, however, was noted to be benign with debris and bile crystalline material and rare fragments of fibrous tissue with biliary-type epithelium.\nAfter evaluation and review of his complicated hospital course with no clear diagnosis but high suspicion for cholangiocarcinoma, a decision was made to undergo a diagnostic laparoscopy with peritoneal washings to rule out carcinomatosis given ascites. The laparoscopy noted inflammation in segments 4 and 5 of the liver, with the gallbladder not visualized since it was covered by omentum. Several biopsies which were obtained from both the right and left liver segments including washings were negative for malignant cells. Thus, the decision was made to proceed with hepatectomy.\nPreoperatively patient reported mild right upper quadrant pain, nausea and diarrhea. He denied any fevers, jaundice or weight loss. On physical exam, his vitals were stable, the abdomen was soft and non-tender to palpation. Labs showed white blood cell (WBC) of 3.84 k/uL, hemoglobin 9.4 g/dL, aspartate aminotransferase (AST) 56 U/L, alanine aminotransferase (ALT) 63 U/L, alkaline phosphatase 261 U/L and total bilirubin 1.0 mg/dL. Tumor marker noted cancer antigen CA 19-19 at 53 U/mL (normal limit: <36 U/mL) and carcinoembryonic antigen (CEA) at 1.7 ng/mL (normal limit <2.9 ng/mL).\nIntraoperatively, the surface of the liver was noted to be quite inflamed. There were dense pericholecystic adhesions, and the gallbladder was diffusely thickened. A hard mass with surrounding inflammatory and fibrotic changes was encountered in segment IV. After mobilizing the liver and controlling all hilar structures, in addition to Doppler confirmation of right hepatic artery flow, an extended left hepatectomy was performed. The right hepatic artery was carefully dissected off the mass, and the left liver including segments 5 and 8 as well as the caudate lobe was resected (Fig. ). We were able to preserve the main bile duct. Pathology subsequently revealed XGC extending into the hepatic parenchyma (Fig. ). After an uneventful post-operative course patient was discharged to extended care facility in stable condition 8 days after his surgery. |
A 60-year-old male patient reported to the outpatient department of our institute, with the chief complaint of burning sensation in the mouth for the past 3 months. He had no abusive habits, and his past dental history and family history were noncontributory. The patient was not under any medications. He had initially visited a general practitioner, who had prescribed him multivitamin tablets. When the lesions did not subside, he consulted a general dentist, who had first treated him symptomatically, with topical anesthetic gel and mouthwash. Later, he was advised antifungal drugs by the same dentist. However, since there was no real improvement in his condition, he was referred to us, 3 months after his initial oral lesions. A thorough intraoral examination revealed numerous erosive lesions on the right and left buccal mucosae, gingiva, floor of the mouth, soft palate, and oropharynx []. Similar erosive lesions were also observed in the nasal mucosa. Few of these eroded areas showed tissue tags over them. However, no intact vesicles or bullae were noticed. A general examination to identify similar lesions elsewhere in the body turned out to be negative. Based on the history and clinical findings, a vesiculobullous lesion was suspected. Pemphigus, mucous membrane pemphigoid, bullous lichen planus, erythema multiforme, and chronic ulcerative stomatitis were considered under differential diagnosis.\nUnder local anesthesia, an incisional biopsy was performed from the edge of the eroded area in the buccal mucosa, taking care to include apparently unaffected mucosa also. Two samples were taken, and one was fixed in 10% neutral buffered formalin for routine histopathology. The other sample was immersed and transported in Michel's medium for direct immunofluorescence study.\nHistopathology revealed the presence of a parakeratotic stratified squamous epithelium that showed features of intraepithelial blister formation. Most areas showed suprabasilar clefting with only the basal layer of epithelium attached to the underlying connective tissue. Within these clefts, numerous round-to-ovoid epithelial cells with large hyperchromatic nuclei and minimal eosinophilic cytoplasm, suggestive of acantholytic (Tzanck) cells, were also evident [Figures and ]. The histopathological features were suggestive of pemphigus. Direct immunofluorescence revealed intercellular staining of the epithelium with IgG, in a typical fishnet pattern, which was diagnostic of pemphigus []. Therefore, on the basis of clinical, histopathological, and immunofluorescence findings, a final diagnosis of pemphigus was made.\nThe patient was initially prescribed tablet prednisolone 10 mg twice daily for 15 days. However, no marked response was noticed. In the meantime, the patient also started complaining of pruritus in the skin of the trunk region and he was referred to a dermatologist for review. The patient was admitted as an inpatient and was advised pulse steroid therapy. As part of the first phase of this treatment, he received 100 mg of dexamethasone in 500 ml of 5% dextrose on 3 consecutive days. Cyclophosphamide (500 mg) was also administered intravenously on day 2. In addition, the patient also received 50 mg of oral cyclophosphamide daily. The patient has been scheduled to receive this treatment once every month, until all lesions disappear. Between the monthly visits, the patient was advised to take tablet prednisolone (30 mg in the morning and 10 mg in the night), along with topical betamethasone for the skin lesions and triamcinolone gel for oral lesions. The patient has already undergone two courses of the first phase of the pulse steroid therapy and is scheduled to receive the third course shortly. His signs and symptoms have shown marked improvement, and the number of lesions has reduced considerably []. However, as a side effect of the pulse steroid therapy, the patient has developed hyperglycemia, for which he is under antidiabetic medications currently. |
Our patient was a 64-year-old, right-handed man. The time course of his illness is summarized in table . His symptoms began 2 years before his presentation to our service. He developed depression, anxiety and agitation, which led to his retirement from construction work. Shortly thereafter, he developed episodes described as hyperventilation, lasting from seconds to minutes, mostly in the morning, which were followed by confusion and a severe ‘pounding’ headache affecting the whole head, both of which lasted for hours.\nOne year prior to presentation, his family described personality changes (it was hard for them to characterize this further) and worsening confusion, particularly over the preceding 3 months. When these changes began, he was diagnosed with hypothyroidism and diabetes insipidus.\nThree months before presentation, he developed weakness of the right leg, which had been slowly progressing since. Two weeks prior, it became so bad that he needed help walking. At this time, he also noticed weakness of the right arm and slurring of speech, which led him to seek medical help.\nHe had been diagnosed with peripheral arterial disease (claudication in his left leg) 4 months prior to presentation, and paroxysmal atrial fibrillation 1 month prior, for which he was taking warfarin. He continued to smoke 2–3 cigarettes a week. His initial review of systems revealed that he was anorexic and had lost 30 lb over the last 2 years. He also complained of a loss of libido and an occasional shortness of breath when walking.\nOn initial exam, he was unable to sustain upward gaze; vertical saccades were followed by a slow downward drift, then a compensatory saccade. He had a weakness of the right side of his face, sparing the forehead. He was weak throughout, with strength of 4–/5 on his right side in an upper motor neuron pattern with signs of spasticity; Babinski's reflex was present on this side. The left side was 4+/5, with a similar pattern. Cerebellar function and sensation were unimpaired.\nHis initial complete blood count, comprehensive metabolic panel, serum protein electrophoresis and folate were normal apart from a white cell count of 13 × 109/l (95% neutrophils) and platelets of 570 × 109/l. His sodium was initially normal, but, during his admission, it was generally in the range of 145–160 mmol/l.\nHis international normalized ratio was 2.9; the C-reactive protein was 30 mg/l (<10), and the erythrocyte sedimentation rate 60 mm/h (<20). His B12 level was low at 375 pg/ml (>400). He was hypothyroid with a thyroid-stimulating hormone (TSH) level of 5.6 μIU/ml (<4.9) and a free T4 of 0.6 ng/dl (>0.7). He had hypogonadism (presumably secondary) with total testosterone of <20 ng/dl (>200). His morning cortisol and ACTH levels were normal. Additional pituitary tests were deferred.\nA lumbar puncture was done shortly after admission, showing 4 red cells, 9 white cells (56% lymphocytes, 43% macrophages), and a protein level of 45 mg/dl (<35). His glucose was normal as was his IgG synthesis rate. Oligoclonal bands were absent and cytology showed no signs of malignancy.\nShortly after admission, a complete serum paraneoplastic panel was sent; this was negative. The antibodies reported were: anti-neuronal nuclear types 1–3, anti-glial nuclear type 1, Purkinje cell types 1–2 and type Tr, amphiphysin, CRMP-5-IgG, striatal, T/Q and N-type calcium channels, ACh receptor-binding, ACh receptor ganglionic and neuronal voltage-gated K+ channels.\nBrain images on presentation are shown on the left in fig. . A CT showed lytic bone skull lesions. MRI is notable for the presence of T2-hyperintense lesions in the left frontal lobe, the right mesial temporal lobe and the brainstem, which were minimally enhanced. An MR perfusion scan of the brain showed no significant increase in relative blood volume or relative blood flow in these lesions. A CT of the thorax, abdomen and pelvis showed 5 small lung nodules (maximum diameter 6 mm) and a pneumatocele in the left lower lobe. As there was nothing further on history, exam or initial investigations to suggest cancer anywhere else, a PET/CT was not performed.\nA biopsy of the left frontal bone lesion was performed. This is shown in figure . Immunohistochemistry shows the lesion to be CD1a- and S100-positive, which is characteristic for histiocytosis.\nInitially, we were unsure of the cause of his right temporal lesion. Given the rarity of LCH with onset in the central nervous system (CNS) in adults, the possibility of another tumor was raised, and so a second biopsy was performed. In retrospect, it would have been better to avoid performing this, as the diagnosis was already clear and his other lesions were attributable to LCH. Furthermore, the procedure precipitated status epilepticus and led to preventable complications which ultimately contributed to his death. The biopsy is shown in figure . This illustrates mild edema, hypercellularity and reactive changes, all of which are consistent with the edge of an inflammatory process. No Langerhans or neoplastic cells are seen.\nFollowing the biopsy, it was necessary to induce coma to control his seizures. He was also re-intubated. Septic shock (likely due to ventilator-associated pneumonia) and a pulmonary embolism complicated his course thereafter. He remained intubated for 3 weeks. At the end of this period, he was given methylprednisone 1,000 mg i.v. for 3 days, with no sign of improvement. Thereafter, he started cladribine 1.4 mg/m2/day for 5 days. Cycles were planned every 3 weeks, but delayed due to pancytopenia and infection. He remained clinically stable after 4 cycles of this regimen. Histiocytosis affecting his skull was thought to be in remission as a result of the treatment, as there was no sign of new or enlarging lytic lesions.\nAfter discharge from the ICU, he showed signs of critical illness myopathy/neuropathy. There was widespread muscle atrophy and generalized weakness. He was unable to stand without support and his tracheostomy remained in place.\nGiven the suspicion of paraneoplastic brainstem inflammation, he was started on intravenous immune globulin (IVIG) 400 mg/kg × 5 days. Two weeks after the initial treatment, the brainstem showed some improvement in the degree of FLAIR hyperintensity (fig. ). An MRI 1 month after a second course of IVIG showed these changes to be stable overall, with some increase in pontine enhancement (fig. ).\nHe then developed recurrent pneumonia and septic shock, again requiring an ICU admission. Following this, his family elected a palliative approach to care and he died 6 months after his initial presentation. |
A 72 year-old man with no significant family medical history and a personal history of hypertension that had been treated for years with amlodipine, fosinopril and hydrochlorothiazide was diagnosed with PV in July 2012. Hydroxyurea and acetylsalicylic acid were prescribed one month later and he was immediately referred to our department to rule out skin cancer in the context of a prospective study of cutaneous manifestations associated with PV and associated treatments. The patient had a Fitzpatrick skin type II and a long sun-exposure history. We detected a facial lentigo maligna, two basal cell carcinomas and one cSCC that had been present for several months. All these lesions were surgically removed with no recurrences. The cSCC was located on the left leg and histologically showed poor differentiation (case 1 in ). In January 2013, five months after hydroxyurea had been prescribed, it had to be withdrawn due to hematological resistance. Ruxolitinib 10 mg twice a day was then initiated. The dose of ruxolitinib was progressively escalated to 15 mg twice a day, dose that was achieved on August 2013. In May 2014, the patient started developing rapidly growing invasive cSCCs. He developed seven primary lesions and one recurrence in sun-exposed areas that corresponded to cSCCs over an 18-month period of time (). All the tumors were rapidly growing crateriform lesions that clinically suggested keratoacanthomas (KA) (). Histologically, all these tumors consisted of cSCCs and six out of seven lesions also showed KA-like features (, ). Areas of fibrosis at the tumor invasion fronts were observed in all the lesions (). All the tumors were treated with surgical excision () with no recurrence except for lesion number 6, located on the left parietal area, which showed recurrence 2 months after removal. This lesion (case 9) was excised and irradiated with no further relapses. No decrease in the count number of leukocytes or lymphocytes per mm3 was observed during treatment with ruxolitinib. This treatment was discontinued in December 2015. Four months after ruxolitinib being withdrawn, the patient still developed a new KA-like cSCC (case 10). In a further 12 month follow-up, the patient has only developed a poorly differentiated cSCC with no KA-like features on the leg (case 11).\nMutational analysis was performed on the 6 lesions in which good quality DNA could be obtained by next-generation sequencing with a small targeted panel covering the exonic regions of TP53, KRAS, NRAS, HRAS, BRAF, PIK3CA, EGFR and KIT genes (GeneRead; Qiagen, Hilden, Germany) and sequenced on a Miseq (illumina, San Diego, CA, USA). The mutational analysis could not be performed in 6 additional lesions due to inadequate DNA quality. Frequent mutations in TP53, previously reported in cSCC, were found in 5 cases and a HRAS mutation in one case (). The recurrent cSCC (case 9) showed the same mutational profile (mutations in TP53) than that found in the primary lesion (case 6). None of these mutations were observed in granulocytes from this patient, which were mainly characterized by the presence of the p. JAK2V617F mutation. The study was approved by the local ehtics committee (Hospital del Mar. CEIC 2010/3913/I). We received the patient's consent form about publishing all photographic materials. |
A 50-year-old female with a past medical history of hypertension, type 2 diabetes, gastroesophageal reflux disease, paraesophageal gastric hernia, diaphragmatic hernia, and sickle cell trait presented to the emergency department (ED) with acute onset left-sided pleuritic chest pain and severe dyspnea. Relevant past surgical history includes Nissen fundoplication and appendectomy. Upon her arrival she was hemodynamically unstable with a heart rate of 162 beats per minute, blood pressure 75/62, and respiratory rate of 36 per min. Her oxygen saturation was 98% on room air.\nThe patient was seen immediately. Upon initial examination she was noted to be in severe respiratory distress and could not speak more than one or two words between breaths. While attempting to obtain an initial history from the patient her BP began to drop further and she became more tachycardic. Monitor revealed continued sinus tachycardia with no ectopy. Upon auscultation it was noted that breath sounds were completely absent in the left hemithorax. Bedside ultrasound was immediately available and showed an abnormal pleural line, lack of comet-tail artifacts, and absence of lung sliding motion. This was felt to be consistent with pneumothorax. Due to her worsening cardiovascular instability it was decided that there was not sufficient time to obtain a portable chest radiograph and emergent needle thoracostomy was performed. After applying betadine solution to the overlying skin, a 14-gauge intravenous catheter was inserted in the left second rib interspace in the mid-clavicular line. Immediately a large release of air was appreciated. The patient’s BP increased from 63 systolic to 100 systolic and her heart rate began to decrease. Additionally, she was no longer exhibiting signs of respiratory distress as she was much less tachypneic and no longer using accessory muscles. The patient now demonstrated appreciable, however diminished, breath sounds on the left. Of note, this was the only intervention given to this patient at this time. At this point, the patient had not yet received any fluid resuscitation, vasoactive medications, nor antiarrhythmics.\nAt that point it was felt that the patient was stable enough to obtain a chest radiograph. The radiograph showed some residual pneumothorax but also showed what appeared to be bowel in the left hemithorax (). Lung markings could not be visualized 3 centimeters from the apex, although the exact size could not be determined due to overlying bowel. The radiologist’s report noted that the splenic flexure of the colon was herniated through the left hemidiaphragm. There was some aeration in the left upper lobe of the lung, the remainder of the left hemithorax was opacified. Cardiothoracic surgery was consulted, reviewed the chest radiograph and made the recommendation to replace the needle thoracostomy with a Heimlich valve to temporize the patient as we obtained an axial computed tomography (CT). After placing the Heimlich valve, a CT of the chest was obtained and showed a left diaphragmatic hernia with an intrathoracic loop of colon along with a loculated hydropneumothorax suspicious for empyema (). This was compared to a CT from 12 days prior when she presented to the ED with abdominal pain and vomiting. The previous scan showed no evidence of incarceration of the bowel loop or the presence of an empyema. She had been following the diaphragmatic hernia with a surgeon on an outpatient basis. On that visit, her symptoms resolved and was discharged. She returned 2 days prior to this presentation with posterior thoracic pain. At that time, chest radiograph was obtained and showed the known hernia but no acute process, specifically no pneumothorax. She was felt to have musculoskeletal pain and was once again discharged. However, the CT scan obtained on the current visit showed an increased amount of colon within the thorax and the colon appeared to be significantly more distended when compared to the prior CT. This was suspicious for incarceration of the colon.\nThe patient continued to remain hemodynamically stable while in the ED. Her surgeon that she was seeing regarding the diaphragmatic hernia was contacted and came to see her in the ED. Upon reviewing the radiograph and CT results, plans were made to take the patient to the operating room for an exploratory laparotomy. This revealed a 3.5 cm diaphragmatic defect approximately 3 cm lateral to the esophageal hiatus through which a 10 cm segment of transverse colon was incarcerated. The segment of incarcerated transverse colon appeared ischemic and there was a 2 mm perforation. General surgery could not reduce the hernia. Therefore, cardiothoracic was called in to perform a thoracotomy and radial incision of the diaphragm “because of the narrowness of the diaphragmatic hernia.” Bowel, as well as omentum, was found to be “edematous and matted,” and was reduced and passed into the abdomen. No frank pus or stool was observed in the thoracic or peritoneal cavities. The ischemic segment of bowel was resected to healthy colon both proximally and distally and a side-to-side anastomosis was created. A chest tube was inserted into the left posterior thoracic cavity and the diaphragmatic defect was repaired.\nCultures of serosanguineous secretion from the chest tube showed growth of Escherichia coli and Enterococcus Avium. She was placed on antibiotic therapy. She gradually regained bowel function and continued to improve quite well. She was discharged after a 9-day hospital stay in good condition. |
A 31-year-old male was injured by baamboo and was admitted to Fuding People’s Hospital (Fuding, China) for wound debridement and suturing. However, the patient was transferred to The Affiliated Hospital of Wenzhou Medical College (Wenzhou, China) due to progressive swelling and numbness of the right chest wall and absence of pulses in the right upper limb. Physical examination showed that the patient was anemic, with a reduced level of consciousness, clear breath sounds bilaterally and blood pressure <70/40 mmHg in the left upper limb following volume expansion. Oxygen saturation of the right upper limb was 0% and the pulse rate was 110 bpm. There were two right thoracic wounds; a 2-cm wound located 1.5 cm below the medial end of the clavicle, and a 3-cm wound located below the lateral end of the clavicle in the anterior axillary fossa. The wounds were sutured, with evident capillary hemorrhage in the wound at the lateral end of the clavicle. The right chest wall and the right upper limb were swollen. The right axillary, brachial and ulnar pulses were absent, and there was no capillary return in the fingers. Traction on the limb produced pain and increased numbness. Muscle strength was grade II and muscle tone was normal. Thoracic CT scan images are shown in . The patient was diagnosed with hemorrhagic shock, right axillary artery injury, right brachial plexus injury, right scapular fracture, damage to the right shoulder muscles and a large right shoulder hematoma. This study was approved by the ethics committee of First Affiliated Hospital of Wenzhou Medical College. The informed consent was obtained from the patient.\nColor ultrasonography showed an injury at the junction of the right subclavian artery and the axillary artery. Emergency repair of the injury was performed under general anesthesia with tracheal intubation. A longitudinal incision was made on the medial aspect of the right upper arm and an endovascular stent delivery catheter system was inserted into the brachial artery. C-arm fluoroscopy-guided arteriography showed leakage of contrast medium from the proximal segment of the axillary artery and the distal vessels were not visualized (). A 5-cm Wallgraft artificial coated endovascular stent with Unistep Plus propulsion system (Boston Scientific Ireland Ltd., Galway, Ireland) was placed in the injured vessel. Arteriography following stent placement showed contrast medium passing normally through the proximal axillary artery, with distal and collateral vessels clearly visualized (). Radial and ulnar pulses were palpable following the procedure, but limb swelling increased following the intervention, possibly due to ischemia/reperfusion injury and venous injury. Considering the signs of brachial plexus injury, an exploratory surgery was performed immediately. During the surgery, exploration revealed a false aneurysm in the proximal segment of the axillary artery. Following removal of the hematoma, a 1.5-cm U-shaped wound was covered with a coated endovascular stent. Gauze was used to stop bleeding and the ruptured accompanying vein was ligated. There was no disruption of the brachial plexus and the surrounding hematoma was removed. Clopidogrel was administrated orally for 2 weeks to inhibit platelet aggregation postoperatively.\nOne week after injury, a CT scan of the right clavicular region showed that the stent was correctly positioned and patent, with no surrounding false aneurysm. A second-look surgery was performed to remove the gauze and the organized blood clot surrounding the stent. Doppler ultrasonography () and CT arteriography (CTA; ) were performed at 1, 3 and 6 months after the second-look surgery, and showed a patent coated endovascular stent, normal blood flow wave pattern, and the right upper limb with 97–100% oxygen saturation on finger pulse oximetry.\nAfter injury, traction on the right upper limb produced pain and increased numbness, and muscle strength was grade II. During the first surgery, a false aneurysm was found in the proximal segment of the axillary artery, with surrounding hematoma causing brachial plexus compression. The hematoma was removed to decompress the brachial plexus. One week after the initial surgery, a second-look surgery was performed to examine the brachial plexus and remove the organized blood clot. At 1 month after the initial operation, the patient had developed atrophy of the pectoralis major, pectoralis minor, deltoid and infraspinatus muscles (). Right upper limb function was examined () and evaluated using various clinical scales; the Gilbert score () for shoulder joint function was stage I, the Gilbert and Raimondi score () for elbow function was 3 points (Grade II), the Raimondi score () for hand and wrist function was stage II and the disabilities of the arm, shoulder and hand (DASH) score () was 53.33 (). After 3 months of oral neurotrophic medication, the Gilbert score for shoulder function had increased to stage II and the DASH score had decreased to 48.33, indicating partial recovery of shoulder function. However, the elbow, hand and wrist function scores had not improved, and the region innervated by the ulnar nerve had not recovered. Electromyography (EMG) showed injury to the right cord of the brachial plexus, including severe injury to the median, ulnar and axillary nerves and mild injury to the radial and musculocutaneous nerves. The second brachial plexus neurolysis was performed at 3.5 months after injury. The right upper limb function was re-evaluated following the second brachial plexus neurolysis, and the shoulder, elbow, wrist and hand function and DASH scores are shown in and .\nThe patient was followed up until 13 months postoperatively. Color B-mode Doppler ultrasonography showed that the stent at the junction of the right axillary artery and the subclavian artery had a 5.3–8.7 mm internal diameter, with intimal thickening of ≤2.7 mm, and slower blood flow than that on the contralateral side. Part of the right axillary vein was reversed and used to form a collateral branch. CT angiography (CTA) showed the stent and the normal size and appearance of the right subclavian and axillary arteries, with no evidence of stenosis. These results indicate that the endovascular stent was stable and remained patent in vivo, and may be used to repair injured great vessels in the clavicular region. Notably, stent distortion or deformation due to the large range of motion of the shoulder joint did not cause stenosis during the follow-up period. However, the possibility of stenosis resulting from the large range of motion should be considered when repairing vessels close to the shoulder joint. All parameters of EMG at 13 months were significantly improved compared with the results at 3 months. The right upper limb function was almost restored to normal, with the exception of hypothenar muscle atrophy, limited interphalangeal joint extension, limited intrinsic muscle function and numbness of the fourth and fifth fingers and ulnar palm. The DASH score at 13 months was 7.5, indicating minimal influence on the life and work of the patient. |
A 31 years old right hand dominant gentleman presented to our orthopedic specialty clinic with fracture distal end radius on left side. On further evaluation, there was deformity in ipsilateral shoulder joint and asymmetry was present as compared to opposite side. There was an anterior globular bony swelling palpable with well defined margins. Transmitted movements from humerus were present confirming it to be bony humeral mass. There was wasting of deltoid muscle as compared to opposite side. Range of motion were flexion upto 170 degrees (), extension up to 10 degree (), internal rotation up to L3 (), and external rotation up to 30 degree () and abduction up to 120 () in both active and passive movements, further movements were restricted and mildly painful. Patient did not have any history of significant trauma to shoulder in past. However he gave a history of seizure attacks three years back following which he had shoulder pathology. He had taken antiepileptic treatment; however no treatment was taken for the shoulder. There were no further epileptic episodes and he was taking antiepileptic treatment. Radiographs of shoulder joint were taken which confirmed anteriorly dislocated humeral head (). Further views of shoulder joint were taken to check for any bony changes. They didn’t show any significant abnormality. CT scan showed anteroinferior dislocation with neocavity with pseudojoint formation (,,). Chronic hillsachs lesion was also seen. MRI Scan showed anteroinferior shoulder dislocation with intact rotator cuff muscles (,) Since the patient had good functional range of movement we explained him the treatment options. He opted for non surgical line of management. Hence was given shoulder mobilization exercises. Presently patient is being followed up with no fresh complaints and maintained join movements.\nAnterior dislocation of shoulder unlike posterior dislocation is most commonly traumatic in nature. The mechanism of anterior dislocation following trauma is that greater tuberosity abuts against acromion when arm is abducted and extended, causing leverage forces leading head to come out of glenoidcavity []. Posterior dislocation secondary to seizure attack is caused due to imbalance between strong internal rotators and weak external rotators and deltoid. The cause of anterior dislocation following seizure is postulated to be direct trauma due to collapse of patient hitting the floor []. Anterior dislocation of shoulder is commonly missed after seizure because of its unusual occurrence, post seizure drowsiness and subsequent medical management[].\nThe term chronic dislocation of shoulder is applied to condition where there is loss of recognition of injury for at least 3 weeks [] or 4weeks []. Many authors have described chronic dislocation with varying amount of duration at presentation. Rowe and Zarin presented eight patients with anterior dislocation with seven patients presenting at 3 week to 2 year interval and one patient at 10 year duration []. Goga presented 31 patients with chronic anterior dislocation with longest duration of failed recognition at 2 years []. Postacchini and Facchini presented five patients with 6 weeks as longest duration of failure of diagnosis[].\nMansat et al showed five patients with 6 weeks to 3 years as the duration of missed diagnosis[]. Mancini et al presented a case with 24 years of missed anterior shoulder dislocation []. Our case presented 10 years after the dislocation.\nTreatment options for neglected shoulder dislocation include observation, manipulation, open reduction with or without allograft reconstruction, bankarts repair, capsulolabial repair and arthroplasty [, ]. Surgical treatment for chronic dislocations is usually advocated for better functional outcome, however the results can be poor and unsatisfactory[].\nVery few cases describe chronic dislocation of shoulder with good functional range of motion which were treated nonsurgically. Table shows epidemiological details of patients reported with asymptomatic neglected anterior shoulder dislocation. Essi et al showed a case with 15 years old neglected anterior shoulder dislocation in a 35 year old lady with good functional range of movement and neocavity formation leading to preserved movements []. Similar neocavity formation seen in a case described by Mancinni et al with 24 years of neglected dislocation ion a 74 year old lady with low functional demand []. Jerosch et al [] presented a case of a young male with preserved movements 4 years after missed diagnosis. This case didn’t show neocavity formation, but had a large Hill Sachs lesion which locked the head in dislocated position. In our case we found neocavity formation with large hill Sachs lesion. |
A 39-year-old Thai male patient presented with progressive pain and swelling of seven-month duration over the antecubital fossa of the right elbow. Initially, there was only slight swelling. Three months later, he complained of dull pain. The patient went to a private clinic where the diagnosis was distal biceps tendinitis. The first doctor gave a local steroid injection, but the symptoms recurred about one month later. Four months later, the patient complained of pain at night and weakness on supination of the forearm and flexion of the elbow. He had no underlying disease, chest symptoms, fever, weight loss, or history of contact with patients suffering from pulmonary tuberculosis.\nPhysical examination of the right elbow when patient visited the hospital in Thailand demonstrated swelling at the antecubital fossa, mild tenderness at the distal biceps, and muscle weakness or pain when attempting to supinate the forearm and flex the elbow. All other systemic examinations were normal. There was a high white blood cell count (12,710 cells/mcL); neutrophil count was 72% and lymphocyte count 17%. Erythrocyte sedimentation rate was 17 mm/hr, and C-reactive protein was 6.69 mg/L. Radiography of the right elbow showed swelling at the antecubital fossa, and chest radiographs showed infiltration of the left upper lung. Magnetic resonance images showed disruption of the distal biceps tendon with an associated ill-defined soft tissue mass (about 2 × 2 cm). A less enhanced area was observed at the inferior part, which was likely to be necrotic or cystic. An abnormal marrow signal was detected at the proximal radius with focal cortical erosion at the radial tuberosity ().\nIn this case, we suspected that the patient had disseminated tuberculosis because preoperative chest radiographs demonstrated left upper lung infiltration, which was likely pulmonary tuberculosis, and there was a soft tissue mass at the distal biceps tendon. We performed an open excisional biopsy and debridement using the single-incision anterior approach. The finding was a soft tissue mass involving the distal biceps tendon with complete tendon rupture. There was also a small focal cortical defect at the radial tuberosity. The ruptured distal biceps tendon was debrided. The tendon was repaired to the long-head tendon insertion, which was proximal to the focal defect by about 5 mm, using a TWINFIX Ti 2.8 mm Suture Anchor with one #2 ULTRABRAID Suture (Smith & Nephew Inc.). Antituberculosis chemotherapy started one day after the surgery, following a positive test of the fluid for acid-fast bacilli and a positive polymerase chain reaction for Mycobacterium tuberculosis. The patient received a total of 6 months of a rifampin-based regimen, which is recommended for musculoskeletal tuberculosis []. The patient initially received isoniazid 300 mg, rifampicin 600 mg, ethambutol 800 mg, and pyrazinamide 1500 mg daily for two months and then reduced to isoniazid and rifampicin for the remaining four months. The elbow was immobilized in a posterior elbow slab with the forearm supinated for four weeks. Mycobacterium culture revealed Mycobacterium tuberculosis. Microscopic examination of the soft tissue revealed granulomatous inflammation with multinucleated Langhans giant cells and caseous necrosis.\nAt the 1-year follow-up, erythrocyte sedimentation rate was 10 mm/hr, and C-reactive protein was 2 mg/L. Motor power of supination and flexion showed grade V and the hook test was negative. This study was approved by the Khon Kaen University Ethics Committee for Human Research (KKUEC) in which the study ID was HE611179. |
A 38-year-old right-handed woman, originally from Sri Lanka, who was G2, P2, and A0 presented to our emergency department 2 weeks post-spontaneous vaginal delivery with a history of sudden onset of right arm jerking movement for 2 days lasting 2–3 min each. CT scans with and without contrast were carried out and showed no abnormalities, including no thrombus in the sagittal sinus. The patient had no events during a 12-h observation period in the emergency department and was sent home the next morning.\nFive days later, the patient returned to the emergency department with sudden onset of right hemiplegia of the face, arm and leg with onset at 9:30 a.m. The hemiparesis began while she was standing up in the kitchen. She did not wake up with any symptoms, and she had no headache or seizure activity. There was no history of clotting disorder and no history of abortions, and there was no history of photosensitivity or rashes. She had had two spontaneous vaginal deliveries with no complications and both delivered at term. There was no history of alcohol or drug abuse. The patient is non-smoker and is not on any medications.\nThe clinical suspicion was high for cerebral sinus venous thrombosis. MRA and MRV did not reveal venous thrombosis. No parenchymal abnormality was noted on the MRI. The patient was admitted to the NICU for observation and was started on IV heparin, as the suspicion was that this patient did have a small thrombus that was overlooked by imaging.\nThe patient was alert and oriented with a stable hemodynamic and respiratory status. She had a dysarthric speech with intact comprehension and expression. Pupils were equal and reactive to light and accommodation. There was no INO and no relative afferent pupillary defect. There was no nystagmus. EOMs were normal. Visual fields were normal to confrontation. There was decreased sensation to light touch and pinprick in the right V2 and V3 distribution. There was a right upper motor neuron facial weakness. Cranial nerves 9, 10, and 11 were normal. The tongue was deviated to the right.\nMotor examination revealed decreased tone on the right upper and lower extremities. Bulk was normal. Fine-finger movement was not present in the right hand. There was upper and lower extremity weakness in the corticospinal distribution at the level of 1–2/5. Reflexes were diffusely brisk, but more so on the right with positive Hoffman’s sign bilaterally, with a clear right up-going toe.\nSensory examination revealed decreased sensation in all modalities to the right side. Coordination examination was limited by the weakness on the right side but was normal on the left. It was not possible to examine gait.\nThis clinical examination localized the lesion to a left subcortical event, either in the corona radiata or the internal capsule. Review of the MRI revealed some ischemic changes in this region and a totally occluded left internal carotid artery. For that, and her overall clinical condition, IV t-PA was deferred.\nFurther MRA analysis revealed the site of the occlusion of the left internal carotid artery to be just above the bifurcation, and this most likely represented carotid dissection. Doppler ultrasound was carried out confirming this finding and also revealed significant vascular disease in both vertebral arteries. A digital subtraction cerebral angiogram was carried out that confirmed severe 3-vessel disease with fibromuscular dysplasia affecting both vertebral arteries and the right internal carotid artery, with a completely occluded left internal carotid artery with no run-off flow seen in the left middle cerebral artery.\nA SPECT study was carried out showing symmetrical perfusion in both hemispheres, a finding which, coupled with the relatively stable course, deferred the decision for urgent surgical or interventional revascularization (Fig. ).\nThe patient continued on the IV heparin. She interestingly developed a pattern of recurrent worsening right hemiplegia with no hemodynamic explanation (BP 150/70) and despite being appropriately anticoagulated (PTT > 80). In an attempt to preserve and improve global cerebral blood flow, we instituted IV milrinone therapy as per our NICU protocol (5 mg bolus followed by 0.75 μg/kg/min infusion). This resulted in significant improvement in her right-sided power such that the next morning the patient had a near-normal power in the right side with a slight pronator drift and was able to ambulate. The patient was maintained on the milrinone and heparin drips. After 7 days of infusion, the milrinone was weaned with significant recurrence of her hemiplegic symptoms with significant weakness in the right side, necessitating a new bolus of milrinone of 3 mg with a return of the basal rate of milrinone of 0.75 μg/kg/min with resolution of her deficits. After a total of 14 days of milrinone infusion, a SPECT scan was repeated revealing preserved global hemispheric blood flow. On the 15th day, milrinone was weaned off with no recurrence of her weakness. She was maintained on the heparin drip and observed for 36 h off milrinone with no recurrence of her symptoms. A SPECT scan was repeated at this time showing normal perfusion of both hemispheres.\nThe patient was transferred to the neurology ward and was switched to coumadin. She continued to improve in her fine motor skills for which she was discharged to an outpatient rehabilitation center. She continues to follow in our outpatient clinic showing no deficits and is currently maintained on 81 mg of aspirin daily. |
A 75-year-old woman (46.2 kg, 153.7 cm tall, and American Society for Anesthesiologists physical status II) was scheduled for a partial duodenectomy. She provided written informed consent for participation and publication. Her previous surgical history included ovariectomy under general anesthesia at the age of 38 years, for which the surgical and anesthetic procedures were uneventful. She also had a history of hypertension, which was treated with angiotensin II receptor blockers. She had no other past medical history. At presentation, she was not taking any herbal preparations or anticoagulants. She had no family history of bleeding tendency.\nFour months prior to her surgery, she developed epigastric pain and an endoscopic examination revealed a duodenum tumor; she was scheduled for a partial duodenectomy. Laboratory findings on admission were as follows: normal electrocardiogram and chest X-ray, hemoglobin 13.4 g/dL, platelet count 21.5 × 104 μL, and the coagulation tests showed prolonged activated partial thromboplastin time (APTT 89.7 s); however, the prothrombin time and the international normalized ratio of prothrombin time were normal (PT 10.3 s, PT-INR 1.01). Hemophilia A and B are usually considered first, especially in males, whenever a prolonged APTT is noted; however, our female patient had no family history of bleeding. Further investigation revealed severely deficient levels of factor XI, and factor XI deficiency was diagnosed. The levels of activity of her factors VIII, IX, XI, and XII are shown in Table .\nIt is obvious that patients with severe clotting factor deficiencies are at risk of bleeding from the surgical site, and anesthetic management is one of the most important concerns for the attending anesthesiologist. To avoid massive bleeding during the surgical procedure, 4 units of fresh frozen plasma (FFP) was transfused (in Japan, the estimated volume per unit of FFP is 120 mL/unit) 1 day prior to the surgery. We performed another coagulation test immediately before entering the operating room, and her APTT level improved to 36.2 s.\nIn our facility, epidural anesthesia is the first choice of analgesia for open upper abdominal surgery. However, because the patient had a potential risk of epidural hemorrhage, we avoided epidural anesthesia and selected ultrasound-guided subcostal transversus abdominis plane (TAP) block with rectus sheath block. General anesthesia was induced by target-controlled infusion of propofol at a target plasma concentration of 3 μg/mL. Fifty milligrams of rocuronium was administered after loss of response to verbal commands. Remifentanil was initiated at a rate of 0.3 μg/kg/min, and propofol was adjusted to maintain a bispectral index of 40–60. Subcostal TAP block with rectus sheath block was performed in the supine position. After skin preparation with 0.5 % chlorhexidine, the ultrasound probe was placed parallel to the subcostal margin near the xiphoid process, the needle was advanced to the TAP, and 20 mL of 0.25 % levobupivacaine was administered to the TAP. After subcostal TAP block, the rectus muscle was imaged with the ultrasound probe on the level of the umbilicus and the needle was advanced to the posterior rectus sheath, and 10 mL of levobupivacaine was administered.\nThe surgical procedure itself was uneventful, and recovery was satisfactory. The operation duration was 289 min, and the anesthesia duration was 406 min. The blood loss was 222 mL, and the urinary output was 1250 mL. The patient received 3600 mL of fluid administration. The patient reported no pain at the surgical site after extubation. Postoperative supplemental analgesics were administered. The patients received intravenous flurbiprofen axetil and acetaminophen on the first postoperative day. After started oral intake, loxoprofen sodium was administered and reported no pain.\nAfter the surgery, thin, watery-pink exudate was seen from surgical drain, but on the seventh postoperative day, the color of the fluid changed to have a more slightly reddish consistency. Although amount of exudate from the surgical drain did not increase, the drain was removed on the same day. The levels of exudate from the surgical drain after surgery are shown in Table . Coagulation tests were performed every day after the surgery. The PT and PT-INR levels were normal throughout the perioperative period. The perioperative APTT levels are shown in Fig. .\nFactor XI deficiency, which is relatively common among Ashkenazi Jews, is associated with injury-related bleeding []. This is sometimes called “hemophilia C,” is distinguished from hemophilia A and B by the absence of hemorrhage inside joints and muscles, and has equal incidence in individuals of either gender. The level of the deficiency does not determine the bleeding risk [], and bleeding tendency varies among individuals. Patients who have severe factor XI deficiency are at risk of massive hemorrhage; however, some do not have this tendency [].\nPatients with factor XI deficiency need specific management during surgery. Previously, adverse surgical outcomes of patients with factor XI deficiency have been reported, including cerebral hemorrhage [] and spinal epidural hematoma []. Different surgical management strategies, each with varying degrees of risk, have been reported, including FFP, factor XI concentrates, and desmopressin [].\nTo manage hemorrhage tendency during the perioperative period, anesthesiologists must determine an appropriate method and tailor it to each patient. We used 4 units of FFP prior to the surgical procedure. The estimated half-life of factor XI is 45 h, which is similar to standard FFP []; therefore, we judged FFP transfusion 1 day prior to surgery which would prevent intraoperative and acute phase massive hemorrhage. As shown in Fig. , APTT levels improved from 89.7 to 36.2 s after FFP transfusion. We investigated the transition level of APTT after surgery. The level of APTT was gradually prolonged to 52.6 s by postoperative day 7. After the surgery, the color of the exudate fluid from surgical drain had changed to slightly reddish, which may have been related to the change of APTT. Considering the estimated half-life of factor XI, we anticipated that APTT would rise to the same level as the preoperative value. Fortunately, APTT remained shorter than expected, and we did not need to use additional FFP.\nThe disadvantages of plasma which are the large volumes are required and the potential for allergic reactions and infections. The patient described in our case report had no cardiac disease, and only 4 units of FFP were used, so fluid overload was not an issue; however, FFPs should be transfused carefully, especially in patients who have cardiac failure. Although factor XI concentrates are an effective treatment, they are not currently available in Japan.\nIn addition to medication, safe and reliable anesthesia is required to manage perioperative hemorrhage. Epidural anesthesia is one of the most useful methods to alleviate surgical pain during the perioperative period. Although the incidence of neurologic dysfunction resulting from hemorrhagic complication associated with neuraxial blockade, including epidural anesthesia, is unknown [], the risk of bleeding is increased, especially in the presence of coagulopathy. In recent years, peripheral nerve blocks using ultrasound have gained popularity. It serves as an effective alternative analgesia when an epidural is contraindicated or refused. Although guidelines for regional anesthesia in the patient receiving anticoagulants are available, regional anesthesia for patients with a clotting factor deficiency is dependent on the careful decision-making by the attending anesthesiologist. In this case, we opted to avoid the potential risk of a spinal hematoma associated with epidural anesthesia and decided to control perioperative pain using a peripheral nerve block. Subcostal TAP block provides analgesia in the upper abdominal area [], and combined with a rectus sheath block that provides effective pain relief for the umbilical are, we were able to alleviate pain in our patient. Neurologic dysfunctions after epidural anesthesia are very rare but devastating. Appropriate management for patients with clotting factor deficiency should result in an uneventful recovery. |
A 16-year-old man was struck by a car while riding a bicycle and was taken to the emergency room with chief complaints of chest pain and dyspnea. He was mentally alert and his vital signs included a blood pressure of 130/83 mmHg, pulse of 93 beats per minute, respiratory rate of 22 breaths per minute, body temperature of 36.6°C, and 90% oxygen saturation. The patient presented with an ill appearance, decreased respiratory sounds in both lungs, and acute pain in his right chest. From the portable cervical lateral view, deep neck subcutaneous emphysema was observed in the soft tissues of the anterior cervical spine; chest images revealed subcutaneous emphysema in the right chest. Chest CT was performed immediately after confirming that the patient’s paroxysmal events appeared to be stable. From the axial view, mediastinal emphysema was observed in the right lung contusion and main bronchus areas along with a small amount of bilateral pneumothorax in the apex of the lung (). The main bronchial laceration was clearly seen from the coronal view, but there was a mistake of not checking the view (). Despite the oxygen supply, the patient complained about worsening dyspnea and developed cyanosis, diaphoresis, and palpitations along with symptoms of anxiety. His oxygen saturation level decreased to 80%. The hypophonesis of both lungs appeared to have decreased, suggesting the progression of tension pneumothorax. An 18-gauge needle was used to perform emergency needle aspiration between the second and third ribs on both thoraces and chest tubes were inserted on both sides of the chest. However, the oxygen saturation level still did not improve and the deep neck subcutaneous emphysema in the cervical spine and chest wall were aggravated; therefore, an endotracheal tube was inserted, after which gas analysis of the arterial blood revealed a pH of 7.29, pO2 of 69 mmHg, pCO2 of 51 mmHg, and 91% O2 saturation. Despite the endotracheal tube, the oxygen saturation did not improve and the subcutaneous emphysema progressed. Conscious sedation was performed and a ventilator was attached; however, evacuation of air through the chest tubes remained poor and the oxygen levels did not fully recover. Subsequently, a self-inflating bag was used to supply oxygen. Despite this, the air deflation was not smooth and there was large resistance to the ventilations. Suspecting an error in the placement and the function of chest tubes, a third tube was inserted in the intercostal space between the fifth and the sixth ribs on the right side. The patient’s oxygen level briefly rose to 90%, but the resistance during bagging persisted. A suction tube was inserted but did not go deeper than 25 cm from the incisor. In order to identify the bronchial injury, a portable bronchoscopy was performed. The endotracheal ends were almost blocked by blood clots. A guide wire was used to remove the blood clots and a right bronchus proximal rupture was confirmed. A double lumen intubation tube (35-Fr left Broncho-Cath, Mallinckrodt, St Louis, MO, USA) was then inserted to the left bronchus (). The patient’s oxygen level increased to 95% and his other vital signs stabilized. The patient was referred to the thoracic surgery department. He was hospitalized in the intensive care unit and an emergency operation was performed. During the operation, a right bronchus rupture was discovered 2 cm directly under the carina. An end-to-end anastomosis with interrupted suture was performed using 3.0 Vicryl. The patient recovered after the operation and was discharged from the hospital after 21 days without complications. |
A 13-year-old Italian girl was referred by her pediatrician because of a retained deciduous canine in her right mandible. During an earlier visit to the pediatrician, the doctor, considering the age of the patient, asked her about the exchange of deciduous teeth, and she reported that the tooth had not yet changed. She was not alarmed, neither was her mother, but the pediatrician insisted that the tooth would probably have already dropped. The pediatrician therefore encouraged her to contact her dentist.\nThe girl was in good health, and her dental and medical history was unremarkable with only the usual childhood maladies.\nAn extraoral clinical examination disclosed a symmetrical face with balanced vertical thirds.She shows a dental-skeletal class I with normal mandibular divergence, with no bad habits, and her cephalometric values are all normal; even her lower and upper incisors are normal-inclined. Her profile is standard for Italian people. An intraoral examination revealed that her dental midlines were concordant with each other and with her face, and no mandibular shift was detected on closure. Except for some lower incisor crowding the overall occlusion was fair with acceptable overjet and overbite. Her clinical periodontal parameters were normal. A radiographic examination revealed that the mandibular right canine was in an oblique position with its crown tip near the apex of the lower right first incisor root (Figure ). An occlusal radiograph confirmed that the crown of the impacted canine was vestibular (Figure ). The canine angulation to the midline was 55° (Figure ). This value suggested a very difficult problem, which might not be orthodontically treatable. After careful evaluation of this case, in view of the age of the patient, the clinical decision was to treat this impacted tooth orthodontically. Full mouth orthodontic treatment was suggested.\nIt was our goal to treat this case with a non-extraction orthodontic approach using upper and lower jaw appliances, while doing our best to correct the impacted tooth, to maintain the profile and reaching as good a final occlusion as possible. The objectives of orthodontic treatment for this patient were to bring the impacted mandibular right canine into her dental arch, level and align the arches, maintain the normal overjet and overbite, and achieve a bilateral Class I canine and molar occlusion. First, the oral surgeon had to eliminate the retained mandibular deciduous canine. At the same time, a vestibular repositioned, full thickness mucoperiosteal flap was elevated, and the crown of the canine was exposed (Figure ). In the same session a fishing-rod (it is a lingual arch of bands positioned on the first permanent molar with the addition of an arm in titanium-molybdenum alloy wire with a trend from the lingual to the buccal side; it is used for traction on canines) was cemented (Figure ); this appliance is fixed, and previously prepared by the technician; the appliance was used to tie up and drive into the canine’s eruption (Figure ). After 5 to 8 months, the cusp of the canine was visible in her mouth (Figures and ), so the fishing rod was replaced with a vestibular rigid arch, welded on the band, to continue the orthodontic traction. Pre-informed brackets and straight archwires were used; for the first 15 days a 0.356mm (0.014 inch) nickel-titanium alloy (NiTi) archwire was used; then it was replaced with a 0.016×0.022 inch NiTi archwire (Figure ). When the canine was present in the oral cavity, a bracket was added to it and linked directly to the arch by an elastic ligation. To keep the space in the arch for the canine, since it was not aligned, we used an open coil spring (Figure ). Finally after approximately 18 months, the canine was well positioned in the arch (Figure ). At this time, she was advised that she needed an attached gingiva graft on her restored tooth to improve esthetics and the periodontal health compromised by the treatment (Figure ).\nThe last step to improve the intercuspidation was the use of criss-cross elastics between her upper right first molar and her lower right first molar (Figure ). When an acceptable occlusion with adequate root angulation had been achieved, the fixed appliance was removed.Retention was established with removable appliances (Figure ). Then, here is the smile of the girl (Figure ).The post-treatment radiographic view (Figure ) showed that the roots of her teeth in her upper arch were well angulated and aligned. No apical root resorption was evident on the radiograph. The midline as well as the overjet and overbite had been maintained during the treatment. Periodontal health was not compromised. One year after treatment follow-up there was no obvious relapse. Her midline, overjet, and overbite are still in good position. No tooth morbidity is evident. One year after debonding, only a partial recurrence was observed in the position of the upper first right molar, as she had not observed the restraint protocol (Figure ). Also the periodontal problem (the lack of attached gengiva) at the level of the lower right canine was confirmed. Her gums are healthy, and although the lack of attached gengiva in the canine region is intact, she is satisfied with the treatment results. |
Our case report describes a 32-year-old woman diagnosed with lymphangioleiomyomatosis who was placed on an organ transplant waiting list in February 2014, due to rapidly progressing respiratory insufficiency. Her history was significant for multiple lymphangioleiomyomas in the retroperitonium along with repeated spontaneous pneumothoraces that required drainage. Transesophageal echocardiography (TEE) was performed during preoperative assessment and the patient was found to have a low ejection fraction. In March 2014, the patient was intubated and ventilated due to respiratory failure. She went on to develop bronchopneumonia caused by multiresistant Pseudomonas aeruginosa, followed by sepsis and disseminated intravascular coagulopathy with severe bleeding from the nose, hypopharynx, and lower respiratory tract. At this point, mechanical ventilation was no longer sufficient to sustain oxygenation and venovenous ECMO (VV-ECMO) was commenced, as a bridge to urgent lung transplantation (). Six days later a donor was found and sequential bilateral lung transplant with a clamshell incision was performed with the support of the arteriovenous ECMO (AV-ECMO). The cannulae from the VV-ECMO were left in place and an arterial cannula was inserted centrally into the ascending aorta. Cannulation of the vena jugularis interna dextra was terminated. Due to anatomic disproportion, the donor lungs were partially resected at the right middle lobe and lingula.\nPerioperative TEE showed severely hypokinetic right and left ventricles along with a large thrombus in the left atrium near the pulmonary veins (). The procedure was complicated by diffuse bleeding due to coagulopathy present in the septic state, which resulted in a 10 L blood loss. Postoperatively, the AV-ECMO was left in place due to the poor myocardial function; therefore, the sternotomy was not closed to allow the arterial cannula to remain in the aorta. During admission to the intensive care unit, severe pulmonary edema was noted with the continuous leakage of edematous fluid from the endotracheal tube. Tidal volumes were 30–40 mL with a peak pressure of 30 cm H2O and a positive end-expiratory pressure of 10 cm H2O. Cardiovascular support with high-dose norepinephrine (>1 µg/kg/min) was in place. Arterial blood gas results showed good oxygenation due to the support of AV-ECMO. Immunosuppression therapy was commenced with tacrolimus, mycophenolate mofetil, and corticosteroids.\nAnticoagulation with heparin was initiated with a target of activated coagulation time (ACT) 150–200 seconds. Six hours later, a TEE was performed and thrombi were noted in both the left and right atria. Due to the high risk of embolism, we were faced with making a crucial decision: to start thrombolysis, which is generally contraindicated in immediate postoperative period, or not. We evaluated that the risk of massive embolism was superior to the risk of bleeding when the sternotomy was not closed. The primary thrombolysis was undertaken with 25 mg alteplase given over 2 hours into the pulmonary artery as the rescue procedure declared in medical documentation. The left atrial thrombus was quickly reduced, after which a second identical dose of alteplase was given. Pulmonary edema began to resolve after the completion of thrombolysis. A further TEE showed no thrombi in the left ventricle or pulmonary veins, although small thrombi remained in the right and left atria. The following day a full dose of thrombolytic agent was given – 50 mg alteplase over 2 hours intravenously – after which no further thrombi could be detected on TEE (). Lung function continued to improve along with radiological findings within 1 week following the thrombolysis ().\nIn the following days, the patient developed acute kidney injury for which she was placed on continuous renal replacement therapy. Gradually, her heart and lung function improved and on postoperative day 8, ECMO support was stopped. The sternotomy was closed after resolution of the P. aeruginosa infection. Weaning and rehabilitation were complicated by poor muscle strength. Due to worsening lung function, a transbronchial biopsy was performed which confirmed tissue rejection A 1–2 (histologic grading of lung allograft rejection- the Classification of Pulmonary Allograft Rejection: Lung Rejection Study Group) and was successfully treated with pulses of steroids. The following days were significant for the loss of consciousness that was thought to be caused by posterior reversible encephalopathy syndrome. An electroencephalograph showed an epileptic status, and antiepileptic medications were prescribed. Three months postoperatively, stenosis of the right main bronchus was diagnosed bronchoscopically, and a biodegradable stent ELLA® (ELLA-CS s.r.o. Milady Horakove, Hradec Kralove, Czech Republic) was implanted. Another setback of pulmogenic sepsis and multiorgan failure, with a need for continuous renal replacement therapy occurred. Six months postoperatively, the patient was extubated and began to actively rehabilitate. Seven months postoperatively, the patient was discharged to home. Currently, she enjoys a good quality of life without any signs of stenosis of the upper respiratory tract and with stationary spirometric values (last forced expiratory volume in 1 second [FEV1] 38%) (). |
A 13-year-old female of Asian Indian origin reported to our hospital for evaluation of swelling and pain on the right side submandibular region of the face. History obtained from the parents revealed that the swelling was present for past 2 months. The patient was prescribed multiple courses of antibiotics, but there was no significant improvement. The patient was moderately built, nourished, and afebrile at the time of examination. The patient gave a history of generalized weakness, weight loss for 2 months, and pain associated with the swelling.\nGeneral physical and systemic examinations did not give any significant diagnostic information. Extra oral examination revealed a unilateral diffuse solitary swelling over the right submandibular region of mandible measuring 2 × 3 cm in size. The overlying skin was normal in color, but swelling was tender on palpation with a discharging sinus []. On pressing the swelling, thick yellow pus discharge with blood was noticed. Margins of sinus opening were hyperemic. Multiple cervical lymph nodes on the right submandibular group of lymph nodes were enlarged and tender. A panoramic radiograph revealed an ill-defined, radiolucent, osteolytic lesion surrounding the developing right permanent second molar []. A provisional diagnosis of residual chronic dento alveolar abscess was made, while the differential diagnosis included tuberculous osteomyelitis of mandible or malignant bone tumor.\nHer chest radiograph did not reveal any evidence of TB []. Hematological finding included WBC counts of 8800 cells/mm3 and Hb of 11.50 gm/dl. Erythrocyte sedimentary rate raised to 38 mm and Mantoux test was 10 × 10 mm. Her serum HIV test was negative. Magnetic resonance imaging of neck showed evidence of osteomyelitis of right mandible involving the inferior margin of distal body of the mandible (from 2nd molar root abscess) [Figures –]. The length of the tract measured 2.34 cm and thickness was 0.6 cm. Computed tomography (CT) scan showed enlarged submandibular lymph nodes measuring 1 × 1 cm (nonspecific lymphadenitis).\nFine needle aspiration cytology of the swelling was also performed. A typical ceseous material with white cheesy appearance was aspirated, which on microscopic examination demonstrated necrotic calls, a large number of neutrophils, few lymphocytes, and few clusters of epitheloid cells, suggesting tuberculoid or caeseating granuloma. Right submandibular lymphadenopathy was also noted. Based on the above clinical findings, corroborated by history and various investigations, a diagnosis of primary tuberculous osteomyelitis of mandible was made.\nSinus tract excision with scooping of the cavity was done under local anesthesia. An extraoral submandibular incision was given to approach the mandible in the affected site. Necrotized bone was found distal to 46, involving the body of mandible with sequestrum formation in the angle area. The lesion was surgically drained and curetted and sent for histopathological examination.\nThe patient was started on standard antitubercular chemotherapy (2HRZE/4HR). The dosage was INH 75 mg, rifampicin 150 mg, ethambutol 275 mg, and pyrazinamide 400 mg. After 2 months of therapy, she improved markedly with decrease in swelling. Panaromic radiograph shows radio-opacity in relation to 47 (bone formation). She is under regular follow-up with continuation of AKT []. |
A 65-year-old man was admitted to our hospital for “discovering a nodule adjacent to the stoma in April that had enlarged for 1 wk, and then ruptured 1 d prior”.\nFour months ago, the patient accidentally found a small nodule on the right side of the colostomy that was approximately 0.5 cm in size without redness, swelling, or tenderness, and did not go to the doctor. Two months ago, the nodule enlarged, accompanied by redness, swelling, heat, and pain. After oral administration of cefdinir, he improved and did not take antibiotics regularly. One week ago, the patient had an enlarged nodule on the right side of the stoma, accompanied by a mass on the left side of the stoma that was approximately 4 cm × 3 cm in size, with redness, swelling, heat, pain, and a black mass of pus in the middle; intravenous infusion of cefoxitin did not alleviate symptoms. Three days ago, the nodule on the right side of the stoma ruptured, and a fistula of approximately 1.5 cm in size appeared, which caused a large amount of overflowing stool. A day ago, the mass on the left side of the fistula ruptured and released pus, so he went to our hospital.\nThirteen years ago, the patient underwent Hartmann surgery due to a sigmoid colon tumor with acute obstruction. Postoperative pathological diagnosis was stage II sigmoid colon adenocarcinoma (T4N0M0). Due to the patient's own reasons, he did not undergo regular treatment and not reverse the stoma in time. The patient was admitted to our hospital for paraostomy hernia and right inguinal hernia 3 years ago, and underwent tension-free inguinal repair and paraostomy hernia repair via an open operation. The abscess was cut for drainage 1 year ago due to a paraostomy abscess.\nThere was a 10-cm surgical scar (from the Hartmann surgical incision) on the right abdomen, a 15-cm surgical incision scar (paraostomy hernia repair incision) in the middle abdomen, and a 5-cm surgical scar on the right inguinal area. The colostomy port was located in the left lower abdomen. On the right side of the colostomy port, there was an abscess of approximately 5 cm × 5 cm in size. The skin was red, swollen, and hot. A middle ulcer was also found. The place where the mass overflowed with pus was located at the anterior upper pole of the iliac and was approximately 1.5 cm × 1.5 cm, at a depth of approximately about 2 cm, and there was pus outflow. On the left side of the stoma, there was a fistula of approximately 1.5 cm × 1.5 cm in size, located next to the belly button that had much stool overflow. A mass of approximately 5 cm × 3 cm was palpable in the left groin area, which was obvious when standing, soft in quality, and did not protrude into the scrotum.\nRoutine blood markers and tumor markers were normal, but Calcitonin original was 0.53.\nEnhanced computed tomography: The right sinus of the stoma was formed, which was the same as the intestinal cavity (orange arrow), and the parastomal hernia can be seen (Figure -). The left side of the stoma had a suspicious sinus formation (yellow arrow), with surrounding soft tissue swelling (Figure and ) and left inguinal hernia (white arrow). No tumor recurrence was seen (Figure ).\nColonoscopy: The proximal bowel (Figure and ) was not significantly abnormal; the distal bowel (Figure and ) was approximately 25 cm, and showed changes from disuse, with a large number of white mucus masses (The white mucus masses were formed by the absorption of water by intestinal fluid secreted by the colon).\nRetrograde transanal digestive tract radiography: Front view (Figure and ) and lateral view (Figure and ) showed that the distal bowel was approximately 25 cm, with no bowel stenosis and a smooth bowel mucosa. |
A 43-year-old Caucasian man, with a 4-week history of right upper quadrant and right flank pain radiating to his right testicle, presented to his general practitioner. There was no preceding trauma, haematuria or any other systemic symptoms. The past medical history included osteoid osteoma in the left upper femur, which was removed in 2007. He also had L4/5 microdiscectomy. There was no positive family history of medical problems or cancer. He was a non-smoker, had no allergies and was not taking any regular medications. The general practitioner organized an ultrasound, which revealed a large subcapsular haematoma measuring 7 × 5 × 7 cm in the right kidney compressing the renal cortex. Further investigation with computed tomography (CT) scan of the abdomen with intravenous contrast revealed enlarged right kidney with subcapsular haematoma and a mass lesion within the right lower pole measuring up to 7.4 × 7.5 × 9.2 cm with associated paracaval lymphadenopathy producing compression of the right renal vein and inferior vena cava (). The patient was then referred to an urologist for further management.\nPhysical examination revealed that the right kidney area was filled, with minimal tenderness. The haemoglobin concentration was 140 g/L. There was a mild elevation of creatinine (116 µmol/L), and the estimated glomerular filtration rate was 66 ml/min. CT staging showed a large right renal tumour and significant paracaval lymphadenopathy producing compression of the right renal vein and inferior vena cava. The size of the tumour was substantially increased in size to 11.5 × 11.6 × 11.5 cm when compared with that in the previous CT scan.\nA preliminary diagnosis of renal cell carcinoma was made based on the clinical findings and investigations. The patient underwent open radical right nephrectomy, retroperitoneal lymph node dissection and partial excision, and reconstruction of vena cava. The renal samples were sent for histopathology. The right kidney specimen (with fibro adipose tissue) weighed 1291 g and 175 × 140 × 110 mm in size. The tumour was located in the mid-pole and lower pole of approximately 100 × 95 × 100 mm. On gross inspection, the tumour was cream and pink in colour with mixture of solid and cystic components (). There was an extensive haematoma within the lumen of the cyst and possible necrosis. The retrocaval lymph node measured 4 cm. Histologically, the tumour comprised of blastemal, stromal and anaplastic elements. The blastemal elements were present in nodular fashion and had syncytial pattern with no diffuse infiltrative component (). The anaplastic elements were focally present in stromal elements (nuclear sized 3× tumour cells and tripolar mitosis; ). The stromal elements were present with focal anaplastic elements. There were features of necrosis, haemorrhage and cystic degeneration. The morphologic and histopathology features favoured adult Wilms’ tumour with synovial sarcoma as a differential diagnosis. A fluorescence in situ hybridization analysis showed loss of heterogeneity (LOH) for 1p 16q, which is associated with poor outcomes in paediatric Wilms’ tumour. The pathological staging was pT3a, pN1 and cM0 (pT3a – tumour invading renal sinus fat but not beyond Gerota’s fascia, pN1 – metastasis in single regional lymph node, cM0 – not stated). The patient was subsequently treated with adjuvant combination chemotherapy and radiotherapy.\nBased on the age of presentation, unfavourable histology and chromosomal LOH for 1p16q, the tumour was considered high-risk, and chemotherapy was initiated as per the Children’s Oncology Group UH-1 protocol, including early radiation to the tumour bed, and multiagent chemotherapy including actinomycin D, vincristine and doxorubicin. Radiation and chemotherapy were commenced concurrently within 3 weeks of surgery. The intensity of chemotherapy was maintained as per protocol with granulocyte support. The patient has been followed up at regular intervals with CT scans and routine blood tests. There has been no radiological or clinical recurrence at the end of 12 months of initial diagnosis. |
A 25-year-old male reported to our department for opinion regarding a massive, asymptomatic enlargement of the right half of the face since childhood (). The asymmetry had increased with age and ceased to grow after 18 years of age. Family history was unavailable since he was an orphan. Medical examination did not reveal any other health issues. The patient had deferred treatment until now due to the lack of financial resources. Extraoral examination revealed a massive and diffuse enlargement of the right side of the face. The enlargement extended from the midline to the preauricular region, superiorly to the frontal bone and inferiorly to the inferior border of mandible. The nose and chin were deviated towards the left side of the face, with the facial midline describing a gentle arc from nasion to gnathion instead of the usual vertical straight line. The right corner of the mouth was drooped and lips were larger on the right side. The skin of the involved right side of the face was coarser than the unaffected left side. The right pinna was larger than that of the left side. There was monstrous enlargement of the soft tissue over the zygoma, the infraorbital region and the symphyseal region. The enlarged soft tissue mass on the right side of the face caused closure of the right eye, compromising the patient's vision. There appeared to be an excessive increase in size of the right side of the mandible, maxilla, zygoma, and frontal bone, as compared to the contralateral side. On palpation a nontender, soft tissue swelling over the right parietal bone was noticed. No temporomandibular joint disorder or dysfunction was detected. No discrepancy in range of mandibular motion was noted.\nIntraoral examination disclosed an obvious alveolar enlargement of the right maxillary and mandibular quadrants as compared to the left quadrants (). The surface of the gingival tissue and palate on the right side was granular in appearance. Right half of the tongue showed an obvious enlargement to the midline with polypoid excrescences representing enlargement of the fungiform papillae (). The right buccal mucosa was thickened and hung in pendulous folds (). A distinct tooth size discrepancy was observed between right and left side. Detailed coronal dimensions of the teeth were measured on the casts with vernier calipers, which revealed major variations in size between the teeth of the affected side compared to the uninvolved side. Right permanent maxillary canine, first premolar, second premolar, first molar, and second molar were larger cervicoincisally, mesiodistally, and labiolingually than those of the left side (). Similarly, right permanent mandibular lateral incisor, canine, first premolar, and second premolar were larger cervicoincisally, mesiodistally, and labiolingually than those of the left side (). The above mentioned right maxillary and mandibular teeth were considerably enlarged than their contralateral counterparts. The right permanent maxillary canine, first premolar, second premolar, first molar, and second molar demonstrated 224%, 100%, 63%, 110%, and 75% increase in volume, respectively, than their contralateral counterparts, while the right permanent mandibular lateral incisor, canine, first premolar, and second premolar demonstrated 337%, 150%, 116%, and 57.14% increase in volume, respectively, than their contralateral counterparts. The maxillary and mandibular midline was shifted to the left. A generalised crossbite was present due to a prognathic mandible (). The occlusal plane on the right side was canted downwards.\nThe panoramic radiograph showed enlarged right body of the mandible with the widening of the right inferior alveolar canal (). PA Skull showed enlargement of right half of mandible, maxilla, and zygoma (). Soft tissue enlargement was seen on the right side of the face and also encircling the symphysis.\nComputed tomographic (CT) scan of face revealed enlargement of the bony structures, including right half of maxilla, mandible, condyle, zygoma, bony walls of external auditory canal, and pterygoid bone (Figures and ). There was bony overgrowth of the glenoid fossa with irregularity of the articular surface. The right condyle was found to be irregular and flattened; however, the temporomandibular joint space was maintained. The right external auditory canal appeared stenosed due to bony overgrowth. The right frontal and parietal bones were thinned out with irregularity of inner table. The right foramen ovale, spinosum, rotundum, mental and infraorbital foramen, vidian canal, and greater and lesser palatine canals were widened as compared to those of the left side (). An intracranial lipoma in the quadrigeminal cistern of the right side was also seen (). Bony orbit on right side was deformed; however, globe, intra-, and extra-orbital structures were normal. Deformation and deviation of the nasal bone and chin were seen towards the left side due to enlargement of overlying soft tissues. Prominent vessels and few nodular serpiginous areas were seen within right parotid gland which was enlarged with heterogenous appearance (). The right submental region, submandibular region, parapharyngeal space, pterygopalatine fossa, soft palate, tongue, and floor of mouth were involved (). All the muscles of mastication and the anterior belly of diagastric on the right side were enlarged with fatty infiltration (). Soft tissues of the right half of the face were hypertrophied, which demonstrated predominantly fat HU (Hounsfield unit) value (Figures and ). A 5.4 × 4.7 cm sized soft tissue swelling was seen in right high parietal region with fat HU value suggestive of lipoma ().\nBased on clinicoradiological findings, the diagnosis of THFH was established. Multiple reconstructive procedures were advised in view of correction of the massive facial deformity. The patient, however, refused to undertake the extensive surgeries, since the enlargement was asymptomatic. |
A 14-year-old female was referred to emergency service with the symptoms of nausea and headache. In her oropharynx examination, only poor oral hygiene was seen by the pediatrician. There were no infection findings in her ear, nose, and throat. Her chest graphy was normal. Her urine tests and CBC were in normal limits. Analgesics were prescribed and control examination was suggested. After three days toothache started. Her parents took her to a dentist. It was said that there was a tooth abscess around right inferior first molar tooth decay. Amoxicillin and paracetamol were started and control examination was suggested. However, after two days with medication, the patient was getting worse. Submental swelling, dysphagia, and chest pain started. WBC was 20.700 per mm3, CRP was 35,7 mg/L, and her fever was 39°C. PA chest graphy was performed and a minimal increase in cardiothoracic index was detected. Neurological examination was normal. There were no pathology about eye movements, muscle strength, and tendon reflexes. Throat and blood cultures and antibiogram were performed. The patient was hospitalized in pediatrics clinic because of a lack of oral intake. Intravenous ceftriaxone and metronidazole medications were started at the first day of hospitalization. As the patient consulted otolaryngologist, in spite of impaired mouth opening abscess formation and purulent secretion from right inferior first molar tooth decay were seen. Impaired mouth opening is more likely because of the ache during the activity of the mandibula, not like trismus. Multiple lymph nodes were palpated in the submental region and bilateral submandibular and anterior cervical regions. There was tenderness in both cervical areas. Consultation by a dentist was suggested. In dental examination, abscess formation, an opening in periodontal tissue, and purulent secretion from right inferior first molar tooth decay were seen. Oral hygiene was poor but no other significant pathology was seen in oral cavity. Tooth extraction was performed and a Penrose drain was applied by the consultant dentist at the third day of the hospitalization in order to avoid chronical process of the infection (). The results of the throat, abscess, and blood cultures were negative. There was no bacteria colonisation. However, there was no clinical improvement. In fact the clinic of the patient got worse. Vancomycin was added to the antibiotherapy at the fourth day of the hospitalization, not to ignore the possibility of MRSA infection until evaluating the culture results. Parents of patient denied any systemic disease. Total Ig A, Ig M, Ig G, and Ig E levels were within normal limits. Serologic studies for hepatitis and HIV were negative. Cervical MRI revealed multiple abscess formation in left carotid space from skull base to the level of carina as obliterating the aortopulmonary window in mediastinum and multiple lymphadenopathy in submental, bilateral, submandibular, and anterior cervical areas (). Percutaneous drainage under ultrasonography was performed. It was intended to be therapeutic. However, it was not successful because of the dense contents of the abscess. Because of that, in general anesthesia left deep neck spaces were explored. Carotid sheath was opened from the level of carotid bifurcation to the level of clavicula and abscess was drained by an aspirator and a catheter placed in mediastinum through carotid sheath (Figures -). For proximal and distal end, two drains were placed into spaces. Postoperatively place of the drain was checked by PA chest X-ray. Cardiothoracic index was increased and the right costophrenic angle blunting was seen. By echocardiography, pericardial effusion was seen 6.1 mm. There was no cardiac tamponade or any other intracardiac mass. Because of the pleural effusion, chest tube thoracostomy was performed to the right side by the consultant pediatric surgeon. Bacterial cultures of pleural effusion were positive. Proliferation of alpha hemolytic streptococcus was seen and there were only resistances of cefazolin and trimethoprim/sulfamethoxazole in antibiogram. Vancomycine was stopped at the fifteenth day of the hospitalization. For pericardial effusion, clinical followup without treatment was suggested by the consultant pediatric cardiologist. After eight days from the surgery, the general situation of the patient got much better and all drains were removed. WBC was 5.500 per mm3, CRP was 2.2 mg/L, and her fever was 36.7°C. After a few days, the periodontal tissue was seen normal. There was no problem about oral intake. |
A 16-month-old boy presented with growth retardation and hypotonia. He was the second child who was born to non-consanguineous Chinese couple. His elder sister developed normally. He had an uncle and a cousin on his mother's side. His parents claimed that their relatives had no similar medical history.\nThis child was born 5 weeks prematurely by cesarean section. His birth weight was 2.0 kg with no history of asphyxia. He often experienced the symptoms of coughing, snoring and stuffy nose after birth. The first 3 months after birth, he developed relatively normally. Nevertheless, it was found that his independent activities were less than his peers when he was 3 months old. After that, he began to present with crying weakness, limb weakness and hypotonia, accompanied by diurnal symptom marked fluctuation. Then he was diagnosed as “growth retardation” and suspected as “cerebral palsy”. Two months later, he still had a poor strength to grip. He could not suck his finger or look up more than 45 degrees in the prone position. He was also unable to keep his neck stable vertically when he was pulled up. He could recognize parents and understand the meaning of their talk. There were no remarkable symptoms such as abnormal eye movements, convulsion, hidrosis, and ptyalism.\nThere were no obvious peripheral nerve abnormalities in electromyography (EMG) examination. Brain magnetic resonance imaging showed bilateral widened frontotemporal extracerebral space which in line with imaging manifestations of premature children. After physical examination and neostigmine test, the possibility of myasthenia gravis (MG) was ruled out. It was suspected that he may suffer from spinal muscular atrophy (SMA) in children because of myasthenia and dyskinesia, but no relevant genetic pathogenic mutations were detected by molecular genetic study.\nHe was hospitalized in Beijing at the age of 6 months. It was considered that he suffered from congenital hereditary metabolic disease based on the aforementioned characteristics. His blood cell counts, hemoglobin, electrolytes, C-reactive protein, lipids, blood glucose, liver function tests, serum ceruloplasmin, urine gas chromatography-mass spectrometry, tandem mass spectrometry, and biotinidase were unremarkable. 3-hydroxybutyric acid level elevated which suggested ketonuria. Urine neopterin level was within normal limits and biopterin level was slightly lower. As an end product from adrenaline and norepinephrine, the urine vanillylmandelic acid (VMA) level was significantly decreased [2.46 μmol/24 hours, (reference level:11.7–84.6 μmol/24 hours)].\nAfter obtaining informed consent, the genetic analysis revealed 2 heterozygous mutations c.457C>T (paternal) and c.698G>A (maternal) (Figs. and ), which resulted in amino acid changes p.R153X and p.R233H. His parents were subjected to genetic tests, and they proved to be healthy carriers afterwards (Figs. and , Figs. and ) while his elder sister was not subjected to the test. This child was diagnosed with DRD and treated with levodopa.\nAccording to our follow-up, his condition improved dramatically after the treatment with 1/12 tablet of a levodopa 200 mg/benserazide 50 mg combination twice daily. The patient adhered to treatment with help from his parents. He weighed 7.5 kg and could raise his head at the age of 11 months. One month later, he was able to turn over and call “mom” and “dad”. Sitting alone can be maintained for about 10 seconds with the armrest when he was 14 months old. The dose was increased to 1/8 tablet, 3 times per day at the age of 15 months. His parents found no obvious problem during the treatment course. |
A 48-year-old morbidly obese woman with a history of metastatic endometrial carcinoma who was status post-total abdominal hysterectomy and bilateral salpingo-oophorectomy presented for evaluation of a large right-sided goiter found incidentally on computed tomography (CT) performed as part of her metastatic cancer evaluation. CT revealed a 10 cm right thyroid mass with extension deep to the right clavicle and associated tracheal compression (). Positron emission tomography revealed no evidence of metastatic disease but displayed diffuse thyroid hypermetabolism.\nAt initial presentation, she was undergoing chemotherapy but recently discontinued one of the agents due to thrombocytopenia. Initial work-up included thyroid-stimulating hormone and free T4 which were within normal limits. A surgeon performed thyroid ultrasound revealed a large 8.9 × 5.0 × 9.7 cm solid, isoechoic, homogeneous nodule without calcifications encompassing the right thyroid lobe and extending into the substernal/subclavicular location (). Fine needle aspiration of the large nodule revealed colloid and follicular groups consistent with colloid nodule or goiter. Given the large size and associated decreased diagnostic accuracy as well as tracheal deviation, excision was recommended after completion of her course of chemotherapy.\nApproximately 7 months after the initial evaluation, the patient returned to clinic to discuss surgery since recent completion of chemotherapy. Repeat surgeon performed thyroid ultrasound revealed slight growth of the large right nodule, measuring 9.4 × 5.2 × 9.9 cm. Her platelets were 50 000.\nAn experienced, high-volume endocrine surgeon proceeded with right thyroid lobectomy using intraoperative nerve monitoring. On the morning of the case, her platelets were 62 000, an acceptable preoperative level per well-established preoperative guidelines [, ]. As the strap muscles were swept off the right thyroid lobe, extremely large vessels (approximately the size of normal internal jugular veins) were encountered coursing through the thyroid capsule. The middle thyroid vein was at least 1.5 cm in diameter and the superior thyroid artery measured >0.5 cm in diameter. Tedious dissection and mobilization of the lobe proceeded for 2 hours without blood loss. However, the nodule was not able to be elevated or delivered to expose the recurrent laryngeal nerve (RLN). The non-nodular isthmus was divided to allow for additional medial to lateral mobilization. On the first spread with the dissecting instrument in the (typically) avascular pretracheal plane, considerable bleeding was encountered. The isthmus was divided with the ultrasonic dissector, and direct pressure was applied. The bleeding originated from a high-pressure arterial tracheal perforator that was flush with the tracheal surface. There was no vessel or soft tissue to grasp/ligate, and the bleeding was too significant for electrocautery. A second faculty endocrine surgeon scrubbed to assist. Bleeding was slowed and hemostasis eventually achieved using multiple figure-of-eight prolene sutures on the tracheal surface. Bleeding from additional vessels in the thyroid bed and from the right lobe remained problematic but not high volume. The additional mobilization allowed for successful completion of the right thyroid lobectomy. While visual identification of the RLN is standard for the operating surgeon, identification was not possible due to the degree and pace of blood loss. A meticulous capsular dissection technique was used to prevent inadvertent injury to the unidentified RLN. Once the lobe was removed, the vagus nerve was identified and it stimulated appropriately, indicating appropriate RLN function.\nEstimated blood loss was unprecedented at 2 L. Notably, all the bleeding encountered during the case was surgical in nature and all bleeding stopped following lobectomy (no concern for platelet-based oozing). The patient preemptively received a six-pack of platelets in the operating room when significant bleeding was encountered. The patient remained hemodynamically stable, requiring no other blood products. She recovered from surgery without incident.\nPathologic examination of the right thyroid lobe revealed a dominant adenomatous nodule with Hürthle cell and degenerative changes. A proliferation of large arterial and venous structures was seen predominantly at the periphery of the nodule and in the adjacent soft tissue (). The findings were consistent with an AVM. Consultation with an institutional expert on AVM syndromes resulted in recommendation for vascular imaging of the head and neck to evaluate for other possible associated AVMs. Follow-up imaging revealed no additional head or neck AVMs. |
A 68-year-old man presented to our institution. He previously underwent laparoscopic intersphincteric resection of the rectum with diverting loop ileostomy for lower rectal cancer at the age of 56. The immediate postoperative course was uneventful. A pathological examination revealed that the patient had T1, N1a, M0, Stage IIIA rectal adenocarcinoma. He did not receive adjuvant chemotherapy, and there was no recurrence of cancer during the follow-up period. However, soon after surgery, he developed an anastomotic stenosis requiring repeated endoscopic balloon dilatation.\nHis loop stoma was not reversed because these treatments were unsuccessful. Therefore, the surgeon proposed surgical resection of the anastomosis, but the patient did not agree to undergo permanent colostomy. Consequently, he had lived with loop ileostomy since undergoing surgery.\nTwelve years postoperatively, he felt swelling and pain in the scrotum. He was seen by a local physician and diagnosed with a perineal abscess. He was referred to a urologist at our hospital for treatment. Computed tomography (CT) scans revealed a low-density area and air-fluid level in the perianal region (Fig. ), which was consistent with the signs of a perianal abscess. Drainage was performed, and the abscess was resolved. The urologist was concerned about the cause of the perineal abscess, and anastomotic complications were considered to be associated with the abscess. The patient was then referred to the department of surgery for further evaluation and treatment.\nDuring examinations, we found that the patient did not have a significant medical history, including inflammatory bowel disease. He denied having any allergies. He had undergone surgery for an inguinal hernia and duodenal ulcer. He quit smoking after undergoing rectal cancer surgery. He took oral antibiotics after the perineal abscess was drained; otherwise, he denied taking any routine medications.\nHe was 161 cm in height and 50 kg in weight, and his body mass index was 19 kg/m2. A physical examination revealed a well-healed scar on his abdomen and a loop stoma in the right lower quadrant. A digital examination revealed severe stenosis at 2 cm from the anal verge. The patient was afebrile and did not have pain in the scrotum or anus. Given his stable medical condition, we judged that urgent surgical intervention was unnecessary. Close observation with a radiological evaluation was planned.\nOne month later, the patient developed skin ulcers in the right ankle (Fig. ) and around the stoma (Fig. ), with a fever over 39 °C. He also presented with bloody discharge per anus. Laboratory data showed there was an elevated C-reactive protein level (11.1 mg/dL) and white blood cell count (7710/mL), while the hemoglobin and albumin levels were decreased (9.4 and 2.6 g/dL, respectively). Regarding the skin ulcers, the culture studies were negative for bacteria, whereas a pathological examination of the biopsy specimen revealed pyoderma gangrenosum. These findings suggested that the patient’s acute illness may have reflected an inflammatory response rather than infectious disease.\nColonoscopy revealed that the bowel lumen had narrowed at 2 cm from the anal verge and extended to 10 cm from the anal verge, where a fistula was incidentally found (Fig. ). Moreover, edematous and easily hemorrhagic mucosa was observed throughout the proximal colon (presumably the transverse colon). The endoscopic examination was terminated soon after we detected a mucosal laceration in the transverse colon that was possibly caused by air insufflation (Fig. ). A pathological examination of a biopsy specimen of the colon revealed inflammatory mucosa with infiltration of neutrophils, eosinophils, and lymphocytes. These findings were interpreted as nonspecific colitis and did not indicate a definitive diagnosis of a condition such as UC, DC, or infectious colitis. Contrast-enhanced abdominal CT did not show signs of cancer recurrence or bowel ischemia.\nA contrast enema confirmed that the distal bowel was narrowed with a fistula, whereas the remnant colon appeared to be consistent with the “lead pipe” phenomenon (Fig. ). We observed active and diffuse mucosal inflammation that indicated UC; pyoderma gangrenosum of the skin is well-known to be an extra-intestinal feature of UC. Given the findings of the physical, laboratory, and imaging studies, we hypothesized that the pathogenesis of the disease was either UC in the diverted colon or DC mimicking UC.\nAfter reviewing the patient’s clinical episodes and extensively discussing the case with gastroenterologists, surgeons, stoma nurses, and the patient, we proposed total colectomy with end ileostomy. Then, the patient finally agreed to undergo this procedure.\nWith the patient under general anesthesia in the lithotomy position, we performed total colectomy with open laparotomy and transperineal resection of the rectum, in accordance with the abdominoperineal resection. The loop ileostomy was taken down and the end ileostomy was reconstructed at the same stoma site. The operative time was 267 min and the estimated blood loss was 210 mL.\nThe postoperative course was uneventful, and he was discharged home on the 11th postoperative day.\nA macroscopic view of the resected specimen is shown in Fig. . The coloanal anastomosis had significant stenosis. There was a fistula proximal to the anastomosis. There was atrophic mucosa, and haustra had disappeared from the distal colon. In contrast, the mucosa appeared to be reddened, edematous, and rather dilated in the proximal colon. The pathological findings included erosion and ulceration in the edematous wall, a crypt abscess, and inflammatory infiltration into the mucosa throughout the colon (Fig. –), without any evidence of dysplasia or carcinoma. The pathological diagnosis was consistent with UC. Given that he developed these inflammatory mucosal alterations after the surgery requiring fecal diversion, we concluded that DC mimicking UC was appropriate for the final diagnosis.\nThe skin ulcers in the right ankle and around the stoma healed over time. Six months after the last surgery, the ulcer areas were significantly reduced (Fig. –). Three years after the last surgery, the skin ulcers were completely healed with some scar tissue (Fig. –).\nWe reported the rare case of a man with acute and severe DC mimicking UC with extra-intestinal manifestations, and he was successfully managed with surgical treatment. His clinical symptoms and histological findings were consistent with the findings of UC, suggesting there is a possible pathogenetic link between DC and UC.\nDC is a frequently seen consequence of the interrupted fecal stream and is characterized by nonspecific mucosal inflammation. In patients who undergo fecal diversion surgery, 70–91% have the endoscopic diagnosis of DC, and 70–100% of those have some histological changes [, , , ]. Typically, the altered bacterial flora decreases luminal SCFA in the diverted intestine, and this may play a role in the development of DC [, ]. Despite the high incidence of DC, most patients are asymptomatic [, ]. Surgical re-anastomosis is the most favorable treatment, whereas some medications are also available [–]. However, acute and severe colitis may occur [, ], and a careful assessment with a prompt decision about the treatment is required in clinical settings.\nIn our case, the decision-making process was as follows. Initially, we considered nonsurgical, conservative treatment targeting UC. However, an enema or oral agents could not reach the diseased colon directly, and steroids or immunomodulators may have provoked a secondary infection and/or recurrent perineal abscess. The presence of a fistula also prohibited the use of these agents. There does not appear to be sufficient evidence supporting cytapheresis for the treatment of DC.\nSecond, the friability of the colonic mucosa represented by insufflation-induced lacerations required urgent and effective treatment. Although the patient underwent diversion, he was nearly at risk of experiencing colonic perforation. Moreover, imaging studies had already shown the presence of a mucosal fistula.\nThird, pyoderma gangrenosum of the skin had worsened rapidly. These features were the patient’s chief complaints, and delayed or prolonged treatment would have diminished his quality of life. Finally, we considered reversing the fecal stream with loop ileostomy closure. However, we deemed it impossible because of the anastomotic stenosis and colonic fistula. Thereafter, we decided to perform surgical resection of the residual colon, including the coloanal anastomosis.\nTo diagnose DC, it is necessary to exclude other bowel disorders such as IBD, radiation colitis, infectious colitis, and nonsteroidal anti-inflammatory drug-associated colitis. A histological examination is paramount for the diagnosis; however, a comprehensive assessment of the patient’s disease, social, and medical histories is also required to determine the treatment strategy.\nDC is regarded as a multifactorial disease []; the causative factors include alterations in intestinal bacterial flora [, , , ], overproduction of oxygen free radicals, impairment of butyrate oxidation [, ], defects in the transport of SCFA, and immunological factors []. Regarding immunological factors, some intestinal mucosal changes or disruption may produce inflammatory or immunoregulatory cytokines, and they induce predominant T helper cell type 1 (Th1). A previous report demonstrated that the Th1 phenotype might be the pathogenesis of DC []. Others indicated that tumor necrosis factor alpha, interleukin 1 beta, interleukin 6, and transforming growth factor beta are important in the treatment of DC [–].\nThe endoscopic features of DC include erythema, friability, and edema, and in severe cases, ulcers, filiform and inflammatory polyps, and strictures are found [–]. The most common pathological features include expanded lymphoid aggregates and inflammation in the lamina propria with lymphocytes and plasma cells [, ].\nIn some cases, follicular lymphoid hyperplasia with crypt distortion and basal lymphoplasmacytosis were observed in DC [, , ]. In such cases, the crypts may appear atrophic and short and are displaced by lymphoid infiltrates. Lymphoid hyperplasia, in which the germinal centers of B and T cell lymphocytes are enlarged, is found in pediatric patients but not in all adult patients with DC [, , , , ]. The microscopic abnormalities found in those with DC may also occur in those with UC, and these diseases cannot be distinguished from one another []. One study hypothesized that the histological resemblance of DC to active UC suggests there is a pathogenetic link between the two diseases [].\nImmunological factors could play important roles in the pathogenesis of both DC and UC. In our case, the differential diagnoses of DC and UC remain unclear. The acute illness of this patient was represented by a perineal abscess, high-grade fever, bloody discharge per anus, and skin ulcers, which could be interpreted as UC with extra-intestinal manifestations. The colonic fistula may indicate chronic inflammation in the diverted colon. These findings suggest that DC may mimic UC, and acute progression of the disease can eventuate in a long-term period in patients without a pre-existing IBD.\nThis report has some limitations. There is still controversy about how to distinguish DC and UC, and there are concerns about total colectomy in patients with acute and severe diseases. Larger case series are required to compare symptomatic and asymptomatic patients with DC and should include periodic histological examinations of the diverted colon. This will help to elucidate the pathogenesis of DC. |
A 70-year-old woman was referred to our hospital because of right knee dislocation after TKA was performed on her right knee due to severe varus deformity and flexion contracture (figure ). Before TKA, the patient had a 45° flexion contracture and severe varus deformity, according to the patient's records. The patient's knee had dislocated when she first tried to stand and walk on the second day after surgery. The day after the dislocation, the patient was referred to our institute (figure ). We attempted closed reduction of the dislocated knee in the operating room, but this was unsuccessful. In the first operation, size 5 femoral and size 4 tibial components (Genesis II, Smith & Nephew) of a non-posterior-stabilized prosthesis with a size 11 liner were inserted. Paramedian arthrotomy was performed via the previous longitudinal incision, and the knee was exposed. Eversion and lateral dislocation of the patella were attempted; however, the surrounding soft tissues were very tight and further proximal dissection and patellofemoral ligament resection was required to enable patellar eversion and knee flexion to 90°. The liner was then removed. After placing the retractors in the appropriate sites, we observed that the lateral collateral ligament, arcuate ligament, and popliteus tendon were severely torn, with inadequate release of the medial structures, including the medial collateral ligament (MCL) and intact medial sleeve with pes anserine tendons. The tibia dislocated laterally, even in the absence of a laterally applied force. First, a periosteal elevator was used to strip the superficial MCL from the proximal tibia, up to the diaphyseal region. Subsequently, the insertion of the pes anserine tendon was released with a cautery. The posterior capsule was released from the top of the tibia and the back of the posteromedial femur with an electrocautery and the curved tip of the osteotome, respectively. A second incision was made, extending from the lateral femoral condyle to the head of the fibula. Since grade III acute injury of the posterolateral corner of the knee was present, we preferred direct repair of the injured structures []. Upon incision of the fascia between the iliotibial tract and the biceps femoris, midsubstance tears of the lateral collateral ligament, arcuate ligament, and popliteus tendon were revealed. These were treated by performing an end-to-end repair with Bunnell-type crossing sutures []. The popliteus tendon was also found to be avulsed from the femur, and transosseous sutures were used to attach the tendon to its proximal insertion site. Posterolateral capsular repair and plication were performed, and the tendon of the lateral gastrocnemius muscle was sutured to the adjacent capsule. Subsequently, a size 15 deep-dish liner was inserted in order to optimize soft tissue tension. Rotational alignments of the tibial and femoral components were normal so they were not revised; further, there was no malalignment of the limb after the reduction of the knee prosthesis.\nDuring knee flexion, the patella subluxated laterally; hence, lateral retinacular release was performed. Furthermore, 2 suction drains were applied for 4 days until hematoma drainage ceased, and the patient's knee was kept in a brace for 2 months. Culture of the synovial fluid that was collected during the second operation revealed methicillin-resistant Staphylococcus aureus (MRSA); therefore, parenteral teicoplanin (Targocid®) treatment was started. Antibiotic therapy was continued for 1 month, and the eradication of the infection was confirmed clinically and with the help of laboratory tests. During this period, the patient was enrolled in a physical therapy program that included. Only isometric quadriceps exercises with no flexion and walking without weight bearing for 1 month. After 1 month, range of movement (ROM) exercises was begun, and the patient started to walk with full weight bearing and was able to achieve full knee extension. After the end of 14 postoperative months, the patient had a knee flexion of 100° and no varus or valgus instability (Figure ). |
A 4.5-year-old boy reported to the dental clinic with bleeding from oral cavity following fall from bicycle. Clinical examination revealed bruise on the chin, open mouth appearance with profuse bleeding from the oral cavity and derangement of occlusion []. Step deformity with tenderness and mobility was elicited along the lower border of the mandible on the left side canine region. Preoperative orthopantamogram (OPG) could not be taken due to the profuse bleeding needing immediate intervention.\nUnder sedation, upper and lower arch alginate impressions were taken and stone casts were poured. An open occlusal acrylic splint was fabricated [], and under general anesthesia, the mandibular body fracture was immobilized, fixed with the acrylic splint which was retained by circum mandibular wiring [Figures and ]. Patient was reviewed every week, and on the third postoperative week, the circum-mandibular wiring and splint was removed under local anesthesia. No mobility was present at the fracture site. Postoperative recovery was uneventful and occlusion achieved was satisfactory, although spacing was seen in incisor–canine region []. Patient was reviewed monthly for 6 months. On 2 months follow-up, the spacing had closed and the child had good alignment of teeth []. Patient had perfect occlusion and good masticatory efficiency.\nFacial fractures in children account for the approximately 5% of all facial fractures.[] A male predilection is seen in all age groups. The most common fracture in children requiring hospitalization and/or surgery generally involves the mandible and, in particular, the condyle. Fractures in the condylar region are the most common, followed by angle and body fractures. The etiologies of mandibular fractures in children are usually falls and sports injuries.\nThe protective anatomic feature of a child's face decreases the incidence of facial fractures. In young children (less than 5 years of age), the face is in a more retruded position relative to the “protective” skull, therefore, there is a lower incidence of midface and mandibular fractures and a higher incidence of cranial injuries. With increasing age and facial growth directed in a downward and forward direction, midface and mandible becomes more prominent; thus, the incidence of facial fractures increases, while that of cranial injuries decreases.[] The high elasticity of young bones, a thick layer of the adipose tissue covering them, a high cancellous-to-cortical bone ratio and flexible suture lines are some of the reasons contributing to the low incidence of facial fractures and minimal displacement of the fracture fragments.[–]\nThe clinical features of a fractured mandible in a child are the same as in an adult, which includes pain, swelling, trismus, derangement of occlusion, sublingual ecchymosis, step deformity, midline deviation, loss of sensation due to nerve damage, bleeding, TMJ problems, tenderness, movement restriction, open bite and crepitus. Thorough clinical examination, however, may be impossible in uncooperative young trauma patients. Lacerations should be evaluated to reveal injuries to underlying structures. General palpation should be applied over all bony surfaces of the mandible. The mandibular range of motion must be examined as patients actively open and close their mouth.\nFor children, the imaging technique which is of value, especially following trauma, is a computed tomography (CT) scan, since plain radiographs in young children are less helpful than in adults due to unerupted tooth buds obscuring fractures, the increased incidence of greenstick fractures and the fact that the cortex is underdeveloped, leading to difficulty in visualizing fractures.[]\nTreatment of mandibular fracture in children depends on the fracture type and the stage of skeletal and dental development.[] Mandibular growth and development of dentition are the main concerns while managing pediatric mandibular fractures. In adults, absolute reduction and fixation of fracture is indicated, whereas in children minimal manipulation of the facial skeleton is mandated. The small size of the jaw, existing active bony growth centers and the crowded deciduous teeth with permanent tooth buds located in great proximity to the mandibular and mental nerves, all significantly increase the therapy related risks of pediatric mandibular fractures and their growth related abnormalities.\nIntact active mandibular growth centers are important for preserving mandibular function, which have a significant influence on future facial development. Thus, restoration of the mandibular continuity after fracture is important not only for immediate function but also for future craniofacial development. Accordingly, the goal of treatment is to restore the underlying bony architecture to its pre-injury position in a stable fashion as non-invasively as possible with minimal residual esthetic and functional impairment.\nLoose anchorage system due to attrition of deciduous teeth and physiologic resorption of roots.[] Precarious dental stability in the mixed dental development period. Difficulties in securing IMF using arch bars and eyelets as primary teeth are not sufficiently stable and may be avulsed due to the pressure exerted. In addition, the partially erupted secondary teeth are not sufficiently stable in the pediatric soft bone.[] Shape of the primary teeth: Conical shape with wide cervical margins and tapered occlusal surface makes placement of wires technically challenging.[] Restricted normal dietary intake in children on IMF was reported to result in significant weight and protein loss and reduced tidal volume.[] Children on IMF are at an increased risk of aspirating gastric contents should they vomit.[] The wires cause discomfort and damage periodontal tissues.[]\nDespite the fact that the goal of treatment is to establish the bony architecture to pre-injury state as non-invasively as possible, the challenges with closed reduction and IMF are many. However, some authors have indicated that IMF using arch bars is safe in children, especially those older than 9 or 11 years.\nSeveral studies have recommended the use of pre-fabricated acrylic splints as a treatment for pediatric mandibular fractures. These splints are more reliable than open reduction or IMF techniques with regard to cost effectiveness, ease of application and removal, reduced operating time, maximum stability during healing period, minimal trauma for adjacent anatomical structures and comfort for young patients.[]\nLaster et al,[] described nickel titanium staples which are inserted in a relatively non-invasive and pain free manner and their eventual removal, if required, is done as quickly as their insertion, facilitated by the fact that the staples are not osseointegrated. Due to their superficial location, there is little risk for inhibiting and deforming facial bone development or having any proximal strategic structures such as nerves and developing dentition. Furthermore, the reduced compression rendered by the staples on the bony fragments result in primary healing with no callus produced.\nModified orthodontic brackets have been used for maxillomandibular fixation (MMF).[] Orthodontic resin has been used for fixation of mandibular fractures in children.[] Orthodontic rubber elastics in combination with fixed orthodontic brackets were used to create compressive horizontal force marginally over the mandibular fracture site from one side to the other.[] A modified orthodontic splint appliance has been applied to fractures where two orthodontic bands are fit on the primary second molars with rounded stainless steel arch wires soldered to them on the buccal and lingual side.[]\nA greenstick fracture is a fracture in which one cortex of the bone is broken and the other cortex is bent. Pediatric patients are more likely than adults to sustain greenstick or incomplete fractures. The relatively high elasticity of the mandibular body's thin cortical bone and a thick surrounding layer of adipose tissue and the relatively larger amount of medullary bone held by a strong periosteal support results in a high incidence of greenstick fractures in children.[]\nA greenstick fracture will ensure stability of the undisplaced segments in children less than 5 years.[] Furthermore, the osteogenic potential of the periosteum in the developing craniofacial skeleton is very high and will lead to somewhat rapid and easier healing which occurs under the influence of masticatory stress, even when there is imperfect apposition of bone surfaces.[] Thus, there is a greater degree of tolerance permissible in the alignment of fragments and restoration of occlusion, which will subsequently be corrected by alveolar bone growth at the time of eruption of permanent teeth.[] Therefore, management of greenstick fractures without displacement and malocclusion would merely be close observation, a liquid-to-soft diet, and avoidance of physical activities (e.g. sports and analgesics).[] There may be cases in which the fractures can be snapped back into a good reduced position and held by the periosteal sleeve, the fracture surfaces and even by the occlusion.[] For greenstick/minimally displaced fractures, conservative closed reduction is the most recommended treatment.[]\nThe closed reduction and immobilization approach can be achieved by means of acrylic splints, circumferential wiring, arch bar or gunning splints.[] These techniques provide a good reduced position, continuity of periosteal sleeve and maintenance of the soft tissue, thus creating a positive environment for rapid osteogenesis and remodeling processes as well as prevention of any type of non-fibrous union.[] Furthermore, in the splinted mandible, the fracture segments are tightly fixed and serve in reducing tenderness and pain reactions during a child's daily activity.[]\nTill the mid-seventies, closed reduction by means of IMF was used for all types of pediatric fractures.[] Today, open reduction and rigid internal fixation (ORIF) has become the standard of care for management of displaced fractures.[] ORIF includes micro or miniplates or biodegradable devices which significantly increase the therapy related risks previously mentioned. Nonetheless, this technique provides stable three-dimensional reconstruction, promotes primary bone healing and shortens treatment time. Posnick et al,[] claimed that a decreased dependence in IMF improved postoperative respiratory care, nutritional intake and oral hygiene measures.\nThe treatment modality for displaced mandibular fractures is debatable between closed reduction and open reduction. While different open reduction techniques have been the standard of care for adults for a long time, its suitability for children remains controversial.[] The effect of implanted hardware in the mandible of a growing child is not completely understood. Damage to the periosteum and surrounding soft tissues and potential damage to primary teeth and permanent tooth gums is to be considered when choosing internal fixation.[] Rigid internal fixation might create artifacts on CT scans or magnetic resonance imaging (MRI) and may be visible or palpated through the child's thin skin and cause pain and early or late infection.[]\nTherefore, the decision to use ORIF in children should be taken with great caution and only if other means of reduction and fixation are not attainable. Miniplate and screw devices have revolutionized the modern management of facial fractures by enabling precise anatomical reduction and fixation under direct vision.\nPresence of developing tooth germs, though the inferior border of the mandible can be plated either through an intraoral/extraoral approach. Additionally, the extraoral approach carries the risk of scarring and damage to the marginal mandibular nerve.[] Interference with growth due to placement of miniplates.[] General anesthesia and hospitalization is needed for removal of the hardware after complete healing.[] Allergic reactions to the metal resulting in inflammation that needs removal of plate have been reported.[] Stress shielding, especially after rigid plate fixation, has been reported and may cause weakening of the bone after removal of the implant.[] Corrosion and release of metal ions can lead to removal of the fixation device.[]\nCurrently, ORIF with resorbable osteosynthesis plates and screws is increasingly being used in children. These biodegradable materials do not interfere with radiodiagnostic techniques due to their radiolucency and they are sufficiently rigid and stable. They eventually degrade, resorb and are eliminated from the body. Although the secondary implant removal operations are avoided and there are no side effects on the growing skeleton, the risk of damaging tooth buds in the pediatric jaw is still present due to drilling for direct application of the resorbable plates and screws.[] Eppley[] claims that this risk is minimal since the drill hole and the tapping of the screw penetrate only the outer cortex of the bone. Even if the resorbable screw tip encroaches upon a tooth, its tip is blunt and non-penetrating. Subsequent resorption of the screw removes any potential obstruction to tooth eruption.\nDespite the above reports, the literature advocates conservative management of mandibular body fractures at young ages with the added advantage of decreased immobilization time, decreased muscular atrophy and better oral hygiene.[]\nComplications per se are very rare in pediatric trauma due to the child's greater osteogenic potential, faster healing rate and less frequent requirement of ORIF. Furthermore, a greater number of fractures are minimal to non-displaced. Late complications such as damage to permanent teeth may occur in 50% of mandibular fractures. TMJ dysfunction (recurrent subluxation, noise and pain, limited condylar translation, deviation on opening, ankylosis) and growth disturbances (e.g. secondary mid face deformity, mandibular hypoplasia and asymmetry) usually occur in pediatric patients with severely comminuted fractures.[]\nMalocclusion as a complication of pediatric facial fractures is rare. It has been attributed to short fixation time in alveolar fractures and may be caused by growth abnormalities following condylar fractures.[] Spontaneous correction of malocclusion is seen as deciduous tooth shed and permanent teeth erupt.[] Ellis et al,[] did not find occlusal complication associated with the use of closed treatment and IMF.\nLois et al,[] found no difference in the complication rates of fractures treated by MMF versus ORIF (4.3% and 5.45%, respectively). They concluded that in fractures with displacement in the range of 2–4 mm, there is no difference between MMF and ORIF.\nA recent study of mandibular fracture complications in children[] noted a lower complication rate (9.1%) with closed treatment of mandibular body, angle and parasymphyseal fractures, while open reduction using miniplate, mandibular plate and mandibular/miniplate fixation revealed a higher rate of complication (30%, 28.6% and 29.2%, respectively). Infection and wound dehiscence occur less frequently with closed versus open.\nNonunion occurs significantly less often in closed reduction than in open treatment. Nonunions of the mandible may develop due to a number of factors, including poor patient compliance with postoperative care, metabolic disturbances and generalized disease states, which can all lead to inadequate bone healing.[] Other local causes for non union may be related to inadequate immobilization of fracture segments, infections at the fracture site, tissue or foreign body between the segments and the inadequate reduction the fracture segments.[]\nHowever, Ellis et al,[] found lower complication rates in patients with comminuted mandibular fracture, who underwent open reduction and fixation (10.3%) than in those who were treated with closed reduction with IMF (17.1%). Yerit et al,[] found uneventful healing and no complications when using resorbable osteosynthesis plates and screws for ORIF, while others described the same type of complications as mentioned for non-resorbable miniplates infections due to mucosal exposure of the plates, premature occlusal contact and TMJ disorders.[]\nA long-term follow-up period is recommended postoperatively in cases of mandibular body fractures in children.[] Facial growth pattern and mandibular movements should be recorded. Ranta and Ylipaavalniemi[] pointed out that teeth in which root development has already begun at the time of fracture, appear to erupt normally; however, marked deformation of the crown and roots occur in teeth located on the fracture line when the calcification process is still in progress at the time of fracture.\nDevelopmental disturbances occur in the lower tooth buds at the pre-calcification stages involved in the fracture and internal fixation site, which include damage to the pulp causing pulp obliteration and extensive root resorption as well as impaction. Koenig et al,[] pointed out that the developing follicle is more elastic than the surrounding bone and better able to survive mechanical injury. Nevertheless, it is difficult to predict the facts of tooth buds and fracture and the implanted hardware fixation.\nSuei et al,[] mentioned that the presence of infection in the fracture site is a crucial factor affecting odontogenic cells in the dental follicle. Surgical procedures as well as fixation and reduction are also potential causes of impaction. Eleonora Schiller et al,[] report that trauma occurring between 0 and 3 years of age is likely to disturb the formation and mineralization of the permanent teeth.\nNixon and Lowey[] concluded that mandibular fractures which occur during mixed dentition can be associated with subsequent failed eruption of permanent teeth when the fracture line is reduced using an open surgical approach.\nYocheved Ben Bassat et al,[] reported discoloration of the crown of permanent tooth in 16% of the children with the incisal one third being the most common site. Hypoplasia was evident in 9% of the permanent teeth.\nIn our case, the deciduous teeth seem to have aligned well over a period of 2 months, thereby closing the spacing initially seen between the teeth. We opted for the open occlusal acrylic splint because of its ease in lab fabrication, preservation of periodontal tissue integrity, patient compliance and ease in assessing the occlusion after reduction. |
A 45 years old male patient, attended the neurology emergency department on 3rd January 2016 due to right limb numbness for 6 days and convulsive seizure for 3 days. Six days before the hospital visit, the patient experienced numbness in the right upper limb and instability while holding without obvious incentive but did not seek immediate medical intervention. Three days before admission, the patient experienced convulsive seizure in the right upper limb while remaining conscious, which was relieved after 1 min. Similar attacks occurred intermittently on six further occasions. The patient had a 3-year history of hypertension with the highest blood pressure being 180/110 mmHg. He also had a history of smoking and drinking lasting more than 30 years. He was born and has always lived in Beijing, with no history of contact with infested water, infectious zone, other radioactive substances or toxins. Upon admission, the patient was examined to be obese with no subcutaneous nodules. Neurological examinations showed full level muscle strength in the right upper limb, accompanied with diminished needling response. Emergency head CT scan (2016-1-1) showed lower density in the left parietal lobe. As the patient manifested as an acute onset of right limb weakness and hemiparesis,with low density lesions in the left occipital lobe on CT and a history of hypertension, the patient was hospitalised with a preliminary diagnosis of acute stroke and secondary epilepsy.\nAfter hospitalisation, head MRI scan (2016-1-4) displayed a lesion in the left parietal lobe of unknown nature. After enhancement in the magnetic field, a larger area of oedema was found around the lesion in the left parietal lobe which could indicate glioma or other inflammatory diseases. Since the nature of the brain lesion did not match the characteristics of common cerebrovascular diseases, intracranial angiography DSA was used but found no obvious vascular abnormalities or stenosis. Further examinations including lumbar puncture, immune rheumatoid factors and parasite antibody detections were carried out. A raised cerebrospinal fluid pressure was found to (215 mm H2O) with no red or white blood cells present. After consultation within the neurology department, intracranial tumor was considered and therefore prepared for stereotactic biopsy of the brain. At this point, pathology results came back positive for Spirometra mansoni IgG. On further questioning the patient admitted that he had drank tap water and eaten frogs when travelling in another province during June–September 2015. Given his medical history, and results from head MRI and blood tests, the patient was considered to be infected with Spirometra mansoni and surgical intervention or antihelmintic chemotherapy was recommended .\nThe patient accepted pharmaceutical treatment and was given praziquantel (1600 mg, 20 mg/kg) 3 times a day for 10 days. During these 10 days, the patient reported occasional headache and was treated for dehydration before discharge from the hospital. The patient was also administration oral sodium valproate 500 mg 3 times a day to control seizures.\nThe patient was hospitalised again in March and July 2016 and treated with praziquantel (1600 mg,20 mg/kg) 3 times a day for 10 days. His headaches were eased with intravenous infusion of 20% 250 ml mannitol twice a day.\nLumbar puncture (Table Examination of cerebrospinal fluid), head MRI, blood biochemistry, conventional blood analysis and parasite antibody examinations (Table Spirometra mansoni IgG antibody) were also carried out on both occasions. On 13th Jul 2016, the patient was free from numbness and seizures in the upper limb.\nTable displays the laboratory results of lumbar puncture performed on the three occasions when he was admitted to hospital and during the 1-year follow-up appointment. CSF analysis showed normal results except for a few white and red blood cells during his second hospital stay most likely resulting from the procedure itself. Table shows the Spirometra mansoni IgG result on three occasions. Note that the IgG result became negative during his third hospital stay following three courses of praziquantel treatment.,.\nEnhanced head MRI scans were performed during the three hospital stays in January, March, and July 2016. Figure A-A3display head MRI scans performed on 8th January. The scans showedan abnormal horseshoe signal in the left parietal lobe with a low T1WI signal and a high T2WI + FLAIR signal. Enhanced scan showed irregular wreath in the lesion without enhancement in the surrounding. This type of abnormality in the left parietal lobe may indicate glioma. During the second hospital stay, the MRI scan carried out on 17th March showed abnormal small stripes of signal shadow in the left parietal lobe with a decreased range. It also showed clearer abnormal veil-like signal shadow in the left parietal lobe as compared to the previous MRI scan. These results may indicatethe presence of Spirometra within this area of the brain. During the third hospital stay, MRI scan on 7th July detected only minor abnormalities in the bilateral frontal lobes and parietal lobes indicating that the lesions has reduced significantly or resolved. The follow-up on 12th December showed no abnormality in the head MRI scan. MRI scans from each hospital visit are shown in Fig. . |
A 53-year-old woman with a known history of metastatic breast cancer managed with docetaxel/bevacizumab and Zometa presented with three weeks of upper cervical pain. She also had complaints of right-sided numbness to her face and neck and decreased range of motion. Her upper neck pain was so severe that she could not sit up for more than one minute before she needed to lay down for pain relief. Due to the severe pain, she was admitted to the hospital for workup and management. Her neurological examination was normal. CT and an MRI of the cervical spine revealed diffuse osseous metastatic disease of the cervical spine as well as a pathological fracture of axis with collapse and retropulsion causing significant atlantoaxial subluxation and deformity at the craniovertebral junction. There was also evidence for bony disease involving the skull base at the level of the foramen magnum as well as the subaxial spine (Figures , ).\nCase management\nA multidisciplinary meeting that involved medical and radiation oncology and neurological surgery was conducted. The patient’s estimated survival was at least one year, and her Karnofsky score was 100. Given that the pathological fracture was causing atlantoaxial instability, the treatment algorithm included surgical stabilization.\nUse of Crown Halo Traction\nDue to evidence of instability of the craniovertebral junction requiring surgical stabilization, the patient was initially transferred to the neurosurgical ICU for crown halo traction to reduce the chronic subluxation. After being placed in a 24-hour crown halo traction of 15 pounds, both her upper cervical pain and C1/C2 alignment on lateral x-ray improved. With a successful reduction of C1-C2 subluxation, the crown halo traction was converted into a crown halo vest. The patient was locked into the crown halo vest for a day before operative stabilization was pursued. Subsequent CT of the cervical spine showed an appropriate reduction. The patient’s neck pain improved, and her swallowing was formally assessed as normal (Figure ).\nOperative Details\nThe patient was subsequently taken to the operating room for posterior occipitocervical fusion to stabilize her upper neck. The patient was placed in the prone position on the open OSI Jackson table (Mizuho OSI, Union City, CA) while in her crown halo vest, and the crown halo was connected to the OSI table with an appropriate OSI adapter. Both arms and shoulders were tucked (Figure ). An incision line was then made from the inion to the spinous processes of C6, and the posterior arch of C1 and the C1-C2 joint were exposed carefully by staying in the midline. The occipital plate was then placed with three screws along the midline keel with a transverse bar on either side to augment and maximize the points of fixation. Bilateral C2 pars intra-articular screws were then placed. Following that, bilateral C3 and C5 lateral mass screws were placed. No hardware was placed at C4 due to extensive bone involvement with metastatic disease. Two hinged rods were then used to connect the occipital plate with the lateral mass screw heads on both sides. Posterolateral fusion was then completed by applying a mixture of corticocancellous bone allograft and demineralized bone matrix that was laid above decorticated bone at the level of the occiput, posterior arch of the atlas, and facets of C3-C5. The wound was then closed in layers. The patient was then placed in the supine position and taken off the crown halo vest and placed in a rigid Miami J collar (Ossur hf, Reykjavík, Iceland). She was extubated successfully. The estimated blood loss was 100 mL.\nPostoperative Details\nThe patient recovered well and was transitioned from intravenous pain medications to oral Tramadol. The patient was discharged with home physical therapy after clearance from physical and occupational therapy.\nShe returned to the radiation oncology center six weeks postop to receive palliative radiation therapy of 30 Gy in 3 Gy daily fractions over 10 days. The treatment was tolerated well and completed without any complications. At her one-year follow-up appointment, her neck pain was minimal. An upright cervical spine X-ray showed posterior fusion from occiput to C5 with intact hardware and normal alignment (Figure ). CT showed improved alignment of the pathological fracture of the C2 vertebral body and decreased widening between the posterior arch of C1 and C2 spinous processes. There was also reconstitution of bony integrity at the upper and subaxial cervical spine (Figure ). Unfortunately, the patient passed away 20 months following surgery due to hypoxic respiratory failure secondary to metastatic breast cancer. |
The patient is a 28-year-old male with prior history of a grade 3 astrocytoma, who presented to our emergency department (ED) after having a general tonic-clonic seizure (GTC). Initially, his disease was appreciated four years prior when he presented with a GTC and was found to have a grade 3 astrocytoma of the left occipital lobe (Figure ). He underwent maximal safe resection followed by adjuvant EBRT to 45 Gy in 25 fractions at an outside institution. Due to reasons outside the patient’s control, he received only two cycles of adjuvant temozolomide following EBRT. He developed disease recurrence, presenting as a GTC six months prior to this ED presentation, where imaging demonstrated tumor progression with increased extension into the parietal lobe (Figure ). He subsequently underwent a second maximal safe resection later that month. Final pathology returned as GB. Follow-up imaging four months afterward demonstrated disease progression and he was started on bevacizumab as well as a tumor treatment field (TTF) device. He unfortunately only tolerated TTFs for one month, ending one month prior to the ED presentation.\nUpon admission to our hospital from the ED, MRI demonstrated progression of disease in the left occipital-parietal lobes with extension into the splenium and anterior-inferior extension into the left thalamus and basal ganglia (Figure ). His physical exam was notable for mild right-hand weakness, but he was otherwise neurologically intact. Despite changes to his antiepileptic medication, he had seizure recurrence a few weeks following admission. Presuming that the area of tumor recurrence received a definitive dose in the past, the consensus decision was to proceed with repeat maximal safe resection with GammaTile placement. A dose of 60 Gy was prescribed to a 5 mm depth using a total of eight tiles, each containing four Cesium-131 3.5U seeds, to line the post-operative cavity volume of 17.6 cc. A significant portion of the occipital-parietal disease was debulked, with final pathology again demonstrating a grade 4 astrocytoma with molecular studies indicating an IDH-mutated, ATRX mutated, and MGMT promoter methylated phenotype with hypermutation. Postoperatively he was noted to have right upper and lower extremity weakness/spasticity with right foot drop and mild right face weakness. He required a cane to assist with ambulation and reported word-finding difficulties and decreased short-term memory. He was planning to start adjuvant temozolomide; however, he developed a severe GTC with increased muscle weakness and altered mental status two months later. MRI following this episode demonstrated mildly increased enhancement to the tissue surrounding the surgical bed with the progression of disease in the splenium and left thalamus/basal ganglia (Figure ).\nGiven this symptomatic disease progression, a treatment plan was made to take the progressive regions of disease outside the irradiated GammaTile volume to 35 Gy in 10 fractions. As shown in Figure , an initial planning target volume (PTV) was delineated consisting of the T1 post-contrast-enhancing disease with a 5 mm margin. The volume that received greater than 35 Gy from the GammaTile treatment was excluded from the PTV. The PTV was then separated into two portions by subdividing the remaining volume between tissue that received less than 17.5 Gy (PTV1), and that which received 17.5-35 Gy (PTV2). Using a volumetric modulated arc therapy plan with five arcs, one non-co-planar, a homogeneous dose of 35 Gy in 10 fractions was delivered to PTV1. This dose was calculated to have a biologically equivalent dose in 2 Gy fractions (EQD2) of 45 Gy. Dose painting gradually decreased the dose from 35 Gy to as low a dose as achievable approaching the resection bed border of PTV2 (Figure ). A composite of the GammaTile dose volume (Figure ) with a dose delivered from PTV1 and PTV2 resulted in a homogeneous dose of approximately 50 Gy EQD2 to the residual disease extending into the splenium and the left thalamus/basal ganglia (Figure ). Digital imaging and communications in medicine (DICOM)-RT data from the initial 45 Gy delivered from the outside institution were obtained, and cumulative dose to critical organs at risk (OARs), including the brainstem, ocular structures, and cochlea, were within established constraints.\nDue to the hypermutation phenotype of his tumor, the patient was started on CCNU (100 mg/m2) and completed his EBRT with no issue or progression of his current neurological symptoms. He was last seen in follow-up by our team three months after his most recent EBRT, at which point he reported significant improvement in the right leg tremors/spasms and improved headache. He demonstrated decreased right foot drop but continued to have word-finding difficulties and short-term memory deficits. He has not developed any new areas of muscle weakness or paresthesia, visual loss, or other forms of altered sensoria. The current plan is to complete six cycles of CCNU with alternative systemic therapy thereafter upon disease progression. While temozolomide could be used to this effect, the high mutational burden found in his most recently resected disease would permit entry into currently available trials of immunotherapy. |
A 17-year-old adolescent male patient applied to Marmara University, Faculty of Dentistry, with complaints in mastication, speech, and aesthetics; dryness on the oral mucosa, and thermal hypersensitivity. Moreover, he was depressed about his appearance of his teeth, thus affecting his social life.\nAccording to his medical history, at the age of 12 the patient had noticed a solid swelling on the left side of his neck and lesion was diagnosed as NPC by incisional biopsy. Following 6-month period chemotherapy regime, 3-month of radiotherapy was accomplished. He has been under the routine control of his oncologist. During the oncologic treatment, tooth brushing was prohibited and he has not performed any oral hygiene procedures since then. Moreover, the patient was neither referred to a dentist nor informed about any oral hygiene instructions until applying to our clinic. The patient disregarded the cautions of the physicians and started smoking nearly 15 cigarettes a day and the body mass index was 16.3 kg/m2 regarded as underweight [].\nThe patient exhibited heavy plaque accumulation, xerostomia, and excessive dental caries (), as well as moderate gingival inflammation with no periodontal attachment loss. There was limited mouth opening with 25 mm interincisal distance. The radiographic examination revealed that the teeth 14, 24, 25, 26, and 36 had periapical lesions, teeth 27 and 37 were impacted, and altered teeth development was observed in teeth 37 and 47 due to oncologic treatment [] ().\nFollowing the oral examination, the patient was referred to his oncologist for consultation. The physician reported that he did not have any current signs or symptoms of NPC, so there was no contraindication for any dental or surgical treatments and there was no need for antibiotic prophylaxis.\nA sequential multidisciplinary treatment that consisted of the extraction of the hopeless teeth, initial periodontal therapy (IPT), endodontic treatments, preprosthodontic periodontal surgery, and full mouth fixed prosthetic restorations was planned for oral health, function, and aesthetics. Written informed consent was obtained from the patient's parents after the detailed explanation of all treatments.\nDue to excessive dental tissue loss and periapical lesions, the teeth 26 and 36 were extracted. Although the tooth 47 had insufficient root development and lack of bone support, the patient refused the extraction of 47 since he had no complaint about the tooth. Regarding the patient's medical history, extraction of the impacted teeth was avoided aiming not to traumatize the patient.\nThe patient was informed about dental plaque and its role in the gingival disease. Instructions for adequate tooth brushing and interdental cleaning were given in detail to the patient. Besides oral hygiene instructions, chewing gums with xylitol and drinking water frequently during the day were recommended to dissolve the effects of xerostomia. Moreover, the patient was encouraged to quit smoking. Microbial deposits were removed by using ultrasonic scalers (Cavitron, Dentsply Professional, USA) and hand instruments (Gracey curettes, Hu-Friedy, USA) and at the end of 6 weeks, the mean plaque score [] decreased from 2.56 to 0.35 and gingival score [] from 1.95 to 0.5.\nEndodontic treatment was performed between the teeth 15–25 and 35–45 except 17 and 25 (). The root canals of the teeth were instrumented manually under asepsis with 2.5% sodium hypochlorite irrigation and aspiration. The root canals were obturated with resin canal sealer (AH-Plus, Dentsply, Konstanz, Germany) and gutta-percha points. Lateral condensation was performed with the aid of the finger spreader. The teeth 17 and 25 were extracted since 17 had highly curved root canals and insufficient working space to perform endodontics and 25 failed to respond to endodontic treatment.\nFull mouth metal ceramic veneers over postcore substructures were planned for the patient. In order to provide ferrule resistance for postcores [] and to obtain adequate biological width for restoration margins [], crown lengthening procedures from tooth 15 to tooth 24 and from tooth 33 to tooth 43 were performed. Labial frenectomy operation was performed prior to osseous recontouring procedure on maxilla (). Since the width of the keratinized gingival tissue was 2 mm from tooth 33 to tooth 43, apically repositioned flap technique was applied to the area to keep the keratinized tissue () []. Postoperative recalls were scheduled at the 1st, 2nd, and the 6th weeks ().\nPrefabricated metal posts (Svenska Dentorama AB, Sweden) were placed and then composite resin cores were built (Ælite, Bisco, USA). In the same visit, exposure of the tooth 27 to the oral cavity was observed and full eruption of the tooth was decided to wait for using as an abutment. Core restorations were prepared and impression was made for provisional restorations. New occlusal vertical dimension (OVD) was established and provisional restorations were prepared in regard to new OVD (). The provisional restorations were fitted and adjusted in the mouth. Interdental hygiene products suitable for fixed restorations (Proxybrush TePe, Sweden, Superfloss Oral-B, Procter & Gamble, USA) were instructed to the patient. Provisional restorations were maintained for 6 months, while the tooth 27 was erupting. After the final preparations were performed, impressions were taken with condensation silicone impression material (Zetaplus, Oranwash, Zhermack, Italy) using Putty-Wash technique. Trial of the ceramic veneers () was performed a week after the test of the metal framework. OVD and centric relations were adjusted on the permanent restorations. Restorations were maintained intraorally with provisional cementing for the orientation of the patient. At the end of the 4-week orientation period, final adjustments were made on the proximal surfaces to facilitate proper interdental brushing and flossing, and then restorations were cemented permanently using glass ionomer cement (Kavitan Cem, Spofa Dental, Czech Republic) ().\nThe patient was satisfied about his new appearance and his mastication ability was significantly improved. He was controlled at the end of the first month and then maintenance visits with 3-month intervals were scheduled. |
A 41-year-old female presented with complaints of right arm claudication, weakness, and pain associated with serous drainage from a previous incision site to the right anterior chest. At age 16, this patient was involved in a motor vehicle accident, which resulted in a right innominate artery and brachiocephalic vein avulsion. The two vessels were immediately ligated and oversewn. The perfusion to her right arm was supplied by cerebral collateral circulation down the right vertebral to the right subclavian artery.\nThe patient was stable until the age of 35 years when symptoms of ischemia set in and she developed right arm pain. This led to her readjusting her right arm frequently and an inability to sleep secondary to ischemic pain. It required surgical intervention done at the University of Chicago with the placement of a right aortic to axillary bypass graft.\nSix years passed before the patient began developing her recent symptoms of right arm claudication and weakness. Upon the patient’s recent presentation, a computed tomography (CT) angiogram of this graft showed occlusion of the aortic-axillary bypass graft due to thrombosis. Interventional radiology decided to thrombolyse the clot and re-canalize the graft. However, the patient developed an infection of the graft with serous discharge, bacteremia, and persistent symptoms of right upper extremity arterial occlusion (Figure ).\nThe persistence of these symptoms and the infection of the graft led to the decision to perform a two-staged procedure: 1) removal of the infected/occluded aorto-subclavian bypass graft with the exception of the aortic stump; this was performed without sternotomy. The patient was treated with antibiotics and once the infection was cleared, the next stage involved redo sternotomy with 2) removal of the old aorto-axillary graft stump and creation of a new aorta to right subclavian artery stump with a direct path using an open harvest of a piece of the left greater saphenous vein.\nProcedure\nThe previous incision over the right anterior chest was reopened. The sternum was not opened at this time. The graft was controlled and the subclavian artery was exposed. A large number of adhesions was noted, requiring extreme care to liberate and identify the right subclavian artery and vein. The patient was heparinized using 8000 units. The left greater saphenous vein was harvested open. Once the subclavian artery was isolated, all graft material was removed up to the incorporated graft material just medial to the aorta. A remnant of the graft was left attached to the lateral position of the aorta, as it appeared to be incorporated into the surrounding tissue. The laterally surrounding tissue was noted to have induration but no gross pus present. However, once the lateral graft was incised, pus spilled out. All material was sent for culture. Proximal and distal control of the subclavian artery was obtained and the defect of the arterial wall was closed using a saphenous venous patch and 6-0 Prolene sutures. The distal flow was noted following repair and clamp removal. Hemostasis was obtained. Due to active infection, it was deemed unsafe to remove the stump and re-implant another graft. The incision was left open and a wound vacuum was placed medially. The incision over the axillary artery was loosely closed and a drain was placed.\nPost-operative care and follow-up procedure\nThe drain was removed at three days post-procedure. Home wound vacuum and intravenous antibiotics were initially started with vancomycin for two weeks followed by Bactrim DS twice daily for two weeks. The infected graft and pus were tested for aerobic, anaerobic, and fungal cultures. The patient did not show signs of infection after the antibiotics (Table ).\nOn follow-up after the first staged procedure, the patient denied fever, chills, weight loss, or any other constitutional symptoms. There was excellent granulation tissue, and the incision was approximately 50% closed with no surrounding erythema, odor, drainage, or fluctuance. The incision subsequently healed and the wound vacuum was removed.\nTwo months after the initial procedure to remove the graft, the patient underwent redo sternotomy to remove the remainder of the old graft. During this same procedure, a right subclavian to aorta graft was placed using an 8 mm Gore-Tex graft (W. L. Gore & Associates, Inc., Arizona, US). This procedure resulted in total relief of the symptoms. The patient continues to do well. |
A 68-year-old male with past medical history of coronary artery disease status post coronary artery bypass graft (CABG), aortic valve replacement along with replacement of the root of the ascending aorta 10 months prior to presentation and recent hemorrhagic cerebrovascular accident (CVA), came to the hospital with complains of acute onset of severe abdominal pain and melena for 1 day. He also attested to chronic abdominal pain and a 30-pound weight loss over the last 8 months prior to these acute symptoms. His physical exam on presentation was positive for severe bilateral lower abdominal tenderness. Apart from a hemoglobin of 10 mg/dl and a positive stool occult blood test, the rest of his basic lab work up was unremarkable (white blood cell/platelet count, comprehensive metabolic panel, and PT/INR included). Hepatitis B, hepatitis C, and human immunodeficiency virus (HIV) testing were negative. The electrocardiogram (EKG) showed sinus rhythm and left ventricular hypertrophy (). An emergent computerized tomography (CT) scan of the abdomen revealed features suggestive of an embolic infarct in the left kidney (Figures and ) and within the mid-one-third of the superior mesenteric artery causing luminal narrowing and also suspected to be extending to the takeoff of small bowel branches. Segmental mural thickening of at least one small bowel loop was noted which strongly favored acute bowel ischemia as a cause of his abdominal pain (). Incidental findings on CT of the abdomen were also strongly suspicious for large eccentric thrombus in the ascending aortic graft and the aortic root which were further investigated and confirmed with a CT scan of the chest (). Cardiology and cardiothoracic surgery were consulted. A CT scan of the head was performed to assess the recent CVA and showed a subacute hemorrhage along the left-sided temporal parenchyma (). CT head imaging was obtained from the facility where the patient presented 3 months prior for cerebral hemorrhage and in comparison, to the most recent CT scan of the head, the hemorrhage appeared stable. The hemorrhage was suspected to be secondary to thromboembolism. After a review of the risks and benefits of anticoagulation to prevent extension of this suspected thrombus, heparin was initiated. An echocardiogram revealed dilatation of the ascending aorta and mild paravalvular leak around the bioprosthetic aortic valve. Gastroenterology was consulted and an emergent esophagogastroduodenoscopy was performed which was negative for any causes of upper gastrointestinal bleed. A hypercoagulable workup was performed which did not reveal any apparent cause of a prothrombotic state. Anticoagulation was held and subsequently, the patient underwent a redo sternotomy under cardiopulmonary bypass with extensive lysis of adhesions, removal of the thrombosed aortic valve and graft, ascending and proximal aortic arch replacement utilizing a 30 mm Dacron graft, and aortic valve replacement with a 25 mm Edwards Magna Ease bovine pericardial valve. The patient also underwent an explorative laparotomy as a part of a staged procedure to address the ischemic bowel caused by the presumed septic emboli. Intraoperatively, the patient was found to have a portion of small bowel that had become necrotic. The necrotic bowel was excised and an end to end anastomosis was performed.\nThe aortic graft and thrombus were sent for culture and pathology. Histopathological examination of the aortic graft and cusps of the aortic valve revealed chronic inflammation and was also notable for abundant acute angle branching septate fungal hyphae (). The patient was immediately started on amphotericin B and voriconazole pending finalization of cultures and sensitivities. On postoperative day four, three culture reports from the graft came back positive for dematiaceous mold, suggestive of Bipolaris species. The minimum inhibitory concentration (MIC) was 0.25 ug/dl for voriconazole and 0.03 ug/ml for amphotericin B. A decision was made to continue the same antifungal regimen on the basis of sensitivities and further speciation was not performed. Patient had a good postoperative course and was later discharged on amphotericin B and voriconazole for at least 1 month with continued follow-up with an infectious disease specialist. |
A 40-year-old female attended the hospital with a brief episode of chest tightness radiating toward her back, immediately followed by severe headache, neck stiffness, nausea and vomiting, and photophobia. She was previously fit and well with no past medical history and no history of trauma. She was not on any anticoagulation therapy and had no significant family history of cancer or neurological disorder.\nOn examination, she demonstrated neck stiffness and occipital tenderness. There were no other positive findings in her initial neurological examinations. Her Glasgow Coma Scale (GCS) was 15, her mental state and speech were normal, her cranial nerves were unaffected and she had good power on all four limbs and a normal gait.\nA head CT was performed on arrival (level I evidence), which showed acute subarachnoid blood predominantly perimesencephalic in distribution with extension into the ventricular system (). A CT angiography (CTA) intracranial and a cerebral digital subtraction angiography (DSA) was arranged within 24 h of admission (level I evidence) (); both showed no convincing evidence of intracranial aneurysm or other vascular abnormalities.\nThe patient was subsequently closely managed under the joint care of neurology and neurosurgery. She was being monitored for signs of hydrocephalus and vasospasm during the acute stage (level I evidence). Her case was discussed in the first neuro-radiology MDT and was recommended for baseline MRI/MR angiography (MRA) 1 week into her SAH (level I evidence). The MRI/MRA result showed normal brain parenchyma and no evidence of an intracranial vascular malformation or aneurysm. However, there was a find of a small rounded lesion with enhancement at the level of C2–3, anterior to the cervical cord (, left panels). This is consistent with an arteriovenous malformation (AVM)/AVF. A spinal DSA was organised, which showed a dural AVF situated at the ventral aspect of the cervical spine at the level of C2–3. Two radicular branches were coming off the left vertebral artery (VA) at the same level, travelling deep into the spinal canal and feed into the dural AVF. No anterior spinal artery (ASA) involvement was noted. It was a small 3–4-mm dilated varix in the midline and ventral to the cervical spinal cord, likely on the venous side, which is likely to represent the site of a recent haemorrhage (, middle and right panels).\nHer case was further discussed during the second neuroradiology MDT meeting, which included a neurologist, an interventional neuro-radiologist and neurosurgeons. Given that the two feeding branches are too small to navigate the micro-catheter, it was deemed unsuitable for endovascular treatment (level V evidence). Disconnection of dural AVF (level IV evidence) with intraoperative indocyanine green (ICG) angiogram guidance (level I evidence) was recommended by senior consultant neurosurgeons (). The MDT decided to offer this patient surgery.\nThe patient fully consented and agreed to have surgery. Following surgery, she was neurologically well but complained of severe head and neck pain that had not improved since onset and very poor neck stiffness. Her pain was not controlled by regular paracetamol, codeine and morphine. Our pain team recommenced a ketamine trial (level I evidence), to which she responded well. Her headache dropped from a rating of 10/10–2/10 ~15 min after 25 mg of ketamine was administered. She subsequently commenced a 6-day course of ketamine 25 mg every 4 h for pain control, which was replaced with pregabalin 75 mg twice a day afterwards. Our physical therapist/occupational therapist (PT/OT) reviewed her neck stiffness and concluded that it was mainly due to subsequent fear of harming and avoiding activity/guarding of movement, which is fairly common in patients after spinal surgeries. They provided information for self-managing exercise and provided her with information on how to adapt movement to limit the pain and encourage a gradual return to normal activity (level II evidence). Other than physical symptoms, the patient had been very anxious during her postoperative recovery, mainly with the restrictions on inpatient management and no visitor rules due to COVID-19 restrictions. All patients found it difficult due to the lack of family support during this period. Our specialty nurse used iPads to arrange for family calls to ensure she has proper access to her loved ones, which helped calm her down and involve the family in her care.\nWith her treatment completed and symptoms fully controlled, she was discharged home from the hospital on the 10th postoperative day. The PT/OT had arranged for ongoing support from the community. Outpatient follow-up used virtual stream 6 weeks after discharge. The patient was well, was no longer using painkillers and had returned to her normal lifestyle.\nIt was planned for her to receive a follow-up angio-spinal 6 months post operation per our neurovascular MDT recommendation. However, the procedure was delayed due to the COVID-19 restrictions. She eventually had follow-up spinal DSA 10 months post-surgery. The result showed that the C2–3 no longer demonstrated dural AVF, and no further arteriovenous shunting presented ().\nsummarises the timeline for this case. |
A 74-year-old female patient who had been followed at our private clinic for AD for the past two years, recently presented to her follow-up visit with the complaints of displaying hypersexual behavior to the females around her, including her female caregivers and adult granddaughters. According to the history obtained from her caregivers, this hypersexuality involved not only flirtatious remarks to females but also included attempts to touch their breasts and genitalia. The family was at first terrified and baffled by these new symptoms and preferred to ignore when they first became apparent in the past few months, but later on wanted to seek professional help as it was getting harder for them to find professional female caregivers to take her on; and also leading to social isolation of the family members. Eventually she was taken to a nursing home, where she continued to display ISB toward the female staff. During the clinical visits, she also made compliments to the female staff members and expressed a desire to be physically intimate with them.\nPast history obtained from her son and daughter-in law revealed that she married with her first boyfriend when she was 17 years old and stayed married for 43 years until her husband passed away 14 years ago. Both of them were born and raised in Romania, but then immigrated to Turkey when she was at the age of 40. Reportedly, she had a decent and happy marriage and raised three children with her husband. She continued to work as a nurse in Turkey for 17 years until her retirement. After her spouse’s death, she moved back and forth between Romania and Turkey spending half of the year at two homes in each country for a while but she was finally settled down permanently in Turkey in the past 8 years. She did not have another boyfriend after her spouse’s passing and refused getting married, stating that her husband was the one and only love in her life.\nShe was reported to act oddly for a brief period of time soon after her spouse’s death, blaming him for leaving her alone or trying to look attractive during the funeral; but they did not seek medical attention for these and she was reported to be “normal” until she had a hip joint replacement surgery 7 years ago. After the surgery, she became increasingly forgetful, started repeating the same sentences over and over, and lost her purse several times. There was a change in her character as well; always being a cheerful and outgoing person, she started to be less social. With these complaints, they saw a neurologist 5 years ago and a diagnosis of AD was made and she was started on donepezil 5 mg initially which was increased to 10 mg after a month. Two years ago, she developed anxiety symptoms and an increasing restlessness and visual hallucinations and a 20 mg of citalopram was added to her drug regimen which helped with her anxiety symptoms but paranoid delusions associated visual and auditory hallucinations spiked up and that was when she was referred to our clinic for the first time.\nAt her first presentation two years ago, she was on 10 mg of donepezil, 40 mg of citalopram daily. She had severe memory deficits and visual and auditory hallucinations during the night time. She also had paranoid delusions. She closed her windows and curtains tightly because she supposed someone was chasing after her. She reportedly also started to show interest in clothes and underwear with flamboyant colors. The patient had three siblings, two of which were also diagnosed with AD; however genetic testing was denied by the family. Neuropsychological assessment demonstrated severe memory impairment, moderate deficit in executive functions, visuospatial ability, attention, and concentration. She scored 13 on the Mini-Mental Status Examination (MMSE) and 2 on the Clinical Dementia Rating Scale (CDR). She was recommended to take memantine (with a dose to be gradually increased to 20 mg within a month) and 25 mg quetiapine at night time. After 15 days from her new medication, hallucinations and delusions were gone.\nHer present visit was mainly due to these recent ISB. Her neuropsychological evaluation remained unchanged with a MMSE of 13 and a CDR of 2.\nRoutine blood tests (complete blood count, electrolytes, renal and liver function tests and vitamin B12 and folate levels) were within normal limits. Her EEG demonstrated diffuse background slowing without epileptiform activity.\nAn MRI scan was ordered to rule out any superimposing organic pathologies such as a recently developed infarct or a space occupying lesion; however it was unremarkable except signs of small vessel ischemic disease involving the periventricular white matter with scattered foci of T2 hyperintensities throughout the deep and subcortical white matter and moderate volume loss more prominently involving the mesial temporal structures ().\nHer medication was re-evaluated and quetiapine 25 mg that she had been on for 2 years, was stopped and she was prescribed aripiprazole with a dose of 2.5 mg to be gradually increased to 10 mg within a month. Phone interview about 2 weeks after the initiation of the aripiprazole treatment revealed that sexual remarks to the female staff members and other disinhibitory features subsided. She was less likely to get into physical contact with the female staff and other female residents of the nursing home, and her restlessness also improved. During the follow up visit after two months of the treatment, she was reported to be free of the symptoms without any ISB in the nursing home. She had increased personal interactions with other residents and the staff of both genders, without evolving into a disturbing point. No side effects were reported. |
A 43 year-old woman was admitted to hospital with headache of two months' duration and left-sided pulsatile tinnitus that occurred suddenly one month ago and had become severe. She complained of a constant rhythmic sound that beat in synchrony with her heartbeat. The sound became louder in her ear, making it difficult to sleep. On admission, the patient did not have any otologic symptoms such as otorrhea, dizziness and hearing loss except ear-fullness in the affected ear. She had no previous history of surgery, head and neck trauma, or systemic diseases. Based on the results of a questionnaire in which the answers were measured on a 10-point visual analog scale (VAS), her intensity of tinnitus was 7, tinnitus-induced annoyance was 8 and the effects of the tinnitus on her life was 6, showing a high level of discomfort. The tinnitus handicap inventory (THI) was also high, as much as 76. The physical examination revealed no mass in the head and neck, and the otoscopy exhibited no abnormal characteristics of the tympanic membranes. No sound waves were detected by auscultation. The tinnitus sound became weaker when the patient turned her head to the left and when her neck was pressed with the hand. The result of the pure tone audiometric evaluation was 11 dB in the right ear and 18 dB in the left ear. The impedance audiometry produced a Type A curve for both ears. In the tinnitogram, the patient showed pulsatile tinnitus in the left ear. And the sound pressure was 24 dB over the frequency of 1000 Hz. The results of the auditory brainstem response test and distortion product otoacoustic emission test were normal.\nBased on the patient's condition and test results, pulsatile tinnitus was suspected, and CTA was performed as a screening test. In the CTA, a prominent vascular structure was observed along the left tentorial margin, and focal stenosis at the transverse-sigmoid junction was present. Distension of the left occipital artery was also observed (). The CTA findings suggested the possibility of a DAVF, and a 4-vessel CA was performed for the diagnosis.\nAngiography of the left internal carotid artery revealed no abnormal findings in the arterial phase. However, the left external carotid angiogram revealed an enlarged occipital artery. Also, the blood drained to the left transverse sinus and the meningeal vein passing through the transcranial route, extending from the left occipital artery, middle meningeal artery, posterior auricular artery, and superficial temporal artery. The diagnosis of DAVF, fed by the left external carotid artery (ECA), and draining into the left transverse sinus, was therefore made (). Although excessive drainage into the cerebral cortex or brainstem was not observed, transarterial embolization was performed due to the severity of the tinnitus symptoms and the high level of discomfort. First, a 6-Fr guiding catheter was placed through the left ECA to identify the enlarged occipital artery for catheterization. Embolizations with ethylene-vinyl alcohol copolymers (ONYX) were performed to embolize the four feeding arteries. Embolization of the middle meningeal artery was performed to embolize the fifth feeder, followed by transcranial arterial embolization in the proximal portion of the superficial temporal artery. A total of six feeding arteries were embolized. Post-embolization angiography revealed blood flow in fine arteries branching off from the posterior auricular artery and occipital artery. However, the feeders were too small and numerous to perform embolization on. During embolization, the pulsatile tinnitus disappeared, and no post-embolization complications were found. During the outpatient follow-up, the patient did not show tinnitus symptoms but claimed minor fullness in the ear. A THI questionnaire was conducted 3 months postoperatively, and the score for tinnitus-induced discomfort was 0. However, the VAS score for annoyance associated with ear-fullness on the affected side was 2. |
A 59 year old male presented with a 2 week history of progressively worsening exertional dyspnea (NYHA class II), bilateral lower extremity edema and abdominal distension. He denied any fever, chills, chest pain, cough, orthopnea, paroxysmal nocturnal dyspnea, palpitations, nausea, vomiting, abdominal pain, syncope or weight loss. He presented to the Emergency Room after his cardiologist found a right atrial mass on echocardiogram during an office visit. His past medical history was significant for treated colorectal cancer, hyperlipidemia and gout.\nThe patient was diagnosed with colonic cancer 5.5 years earlier for which he underwent surgical resection with right hemicolectomy. The patient did not receive any adjuvant chemotherapy or radiation. He continued to follow up with his primary oncologist with surveillance CT scans and had completed his 5 year surveillance.\nFamily history was significant for colon cancer in his uncle and breast cancer in two of his sisters. He denied any tobacco use or alcohol intake.\nThe physical examination was remarkable for decreased lung sounds at the lung bases, moderate ascites and bilateral 1+ pitting edema up to the knees. EKG and cardiac enzymes were unremarkable. The patient underwent a CT angiogram of the chest which revealed a 4.0 cm low attenuating right atrial mass. The lung parenchyma had some ground-glass opacities bilaterally which were thought to be related to motion artefact or less likely suggestive of pulmonary edema. Deep venous thrombosis was ruled out with a negative lower extremity Doppler. The following day, he underwent paracentesis which showed a Serum Ascitis Albumin Gap of 1.4 and 726 nucleated cells (41% lymphocytes, 54% macrophages and 5% neutrophils). Cytologic examination was negative for malignancy, with findings notable for mesothelial cells and macrophages. Cardiac MRI showed a 8.3 cm × 6.5 cm right atrial mass extending from the suprarenal IVC into the hepatic veins and occupying the majority of the right atrium. The mass was noted to be isointense to myocardium on T1 weighted imaging, hyperintense to myocardium on T2 weighted imaging; with positive uptake of Gadolinium contrast in the peripheral segments of the tumor on first pass perfusion imaging, and had heterogeneous uptake on Late Gadolinium Enhancement imaging. A small circumferential pericardial effusion was also seen. Other laboratory studies were majorly unremarkable; with a normal CBC, CMP, PSA and serum CEA levels. The major differentials at this point were atrial myxoma, sarcoma or metastatic disease. It was believed that the shortness of breath was liked related to the increased right atrial pressures.\nCardiothoracic surgery performed an echocardiographic and fluoroscopic guided biopsy of the right atrial mass. The biopsy showed metastatic moderately differentiated colonic adenocarcinoma (Fig. ). Mismatch repair protein expression analysis by immunohistochemistry showed no loss of MLH1, MSH2, MSH6 or PMS2 expression. No RAS, HRAS, NRAS or BRAF mutation was detected on next generation sequencing. MRI of the brain was negative for metastasis. However, MRI bone scan showed that the patient had a low T1 signal intensity in the left distal clavicle that was suspicious for metastatic disease. PET/ CT, was performed to evaluate if the disease was oligometastatic, confirmed metastatic disease in the left distal clavicle with a pathological fracture. The right atrial mass was also noted on PET/CT with abnormal uptake (SUV of 11). Treatment was initiated with plans for 4 cycles of FOLFIRINOX/ bevacizumab. Radiation therapy to the distal clavicle was deferred at this point pending response to chemotherapy.\nColonoscopy was performed during the hospitalization to rule out a second primary colorectal carcinoma. It showed a patent end to side ileo-colonic anastomosis with no endoscopic evidence of colon cancer was noted in the entire colon.\nOn his clinic visit to receive his second cycle of chemotherapy, he was noted to be doing much better with his symptoms of dyspnea and lower extremity edema being significantly better. However, he noted that the left clavicle was a little more painful, so oral opioids were added. At this point, radiation to the clavicle was planned. Repeat echocardiogram performed a month later showed a decrease in the size of the right atrial mass from 4 cm to 3 cm. On his most recent clinic visit, he continues to be asymptomatic and tolerated cycle 7 of chemotherapy with bevacizumab. A cardiac MRI is pending for him with plans to attempt surgical removal by the cardiothoracic surgery team if the tumor has regressed to the supra-diaphragmatic area. |
A 34-year-old female with recurrent ischaemic stroke, type 1 diabetes mellitus, hypertension, dyslipidaemia and stage 2 chronic kidney disease, presented to the outpatient stroke clinic in June 2020 with a history of recurrent transitory right leg weakness and right hand shaking. These episodes occurred several times per day lasting approximately 30 s whilst mobilising and resolved on sitting. Neurological examination demonstrated recovering dysphasia and mild pyramidal right-sided weakness requiring a walking stick for gait assistance.\nPreviously, in March 2019, she presented with slurred speech, left-sided weakness and visual neglect with confirmed bilateral embolic middle cerebral artery (MCA) territory infarcts on magnetic resonance imaging (MRI). Computer tomography (CT) carotid angiography and Digital subtraction angiogram (DSA) identified severe ICAD with bilateral occlusion of the supraclinoid segment of the internal carotid arteries but no features of moyamoya disease (Fig. ). She had no family history of stroke, a negative thrombophilia screen and no cardioembolic source identified. Secondary prevention was commenced including aggressive lipid lowering with rosuvastatin aiming for low density lipoprotein < 1.8 mmol/L, dual antiplatelet therapy with aspirin and clopidogrel for three months, antihypertensive treatment aiming for blood pressure < 130/80 mmHg and augmentation of her diabetic regime. She recovered to a point of supported independent living but was unable to return to work.\nIn March 2020 she represented to the emergency department with reduced consciousness in a state of diabetic ketoacidosis secondary to a lower respiratory tract infection and episodes of vomiting. On presentation her blood pressure was 70/40 mmHg and she was found to have new right-sided weakness. Diffusion weighted MRI identified multiple acute embolic infarcts in the left MCA territory. Her deficits improved with a period of neurorehabilitation but she had ongoing high-level language and cognitive difficulties, marked post stroke fatigue as well as reduced high-level balance. She was independent in personal care.\nIn neurorehabilitation initial concerns were for post stroke focal motor seizures causing her transient symptoms and she was commenced on levetiracetam. However, these brief episodes spared the face, did not exhibit Jacksonian march and were precipitated by periods of reduced cerebral perfusion such as changing position and dehydration. When reviewed in stroke clinic the leading differential diagnosis was cerebral hypoperfusion syndrome. A 99mTc-ethyl cysteine dimer cerebral perfusion single photon emission CT (SPECT) with CT acetazolamide challenge was performed (Fig. ). Baseline perfusion was reduced in the left frontal, temporal and parietal lobes in keeping with recent infarction as well as a small fixed perfusion defect in the right frontal lobe corresponding to previous established infarct. Although asymptomatic during scanning, worsening hypoperfusion to the left frontal lobe and to a lesser extent the left temporal and anterior parietal lobes, was evident with acetazolamide challenge, indicative of reduced vascular reserve in these territories. Given her ongoing symptoms and declining quality of life a repeat DSA was performed to assess for possible sites of surgical revascularisation. The second DSA demonstrated progressive intracranial stenosis with opacification of the left posterior communicating artery and collateral supply to the left hemisphere predominantly from the posterior choroidal artery and posterior cerebral artery (Fig. ). No feasible site for surgical revascularisation was identified. She was managed on maximal medical therapy including long term dual antiplatelet therapy, consolidated antihypertensive regime, tight glycaemic control and avoidance of dehydration. Over the following six months a systolic blood pressure between 120 and 130/70 mmHg was achieved, with a low-density lipoprotein of 1.0 mmol/L and high-density lipoprotein of 1.2 mmol/L. Glycaemic control remained an ongoing challenge, with a glycosylated haemoglobin level of 9.5% (80 mmol/mol). The patient has recently trialled a continuous infusion pump device. Levetiracetam was ceased. During this period the limb-shaking TIAs settled. |
A 16-year-old left handed girl suffered a spontaneous brain hemorrhage into her left occipital lobe. She lost consciousness and was resuscitated from respiratory arrest. Imaging showed left occipital and left frontoparietal subdural hematomata. Drug induced coma was implemented and maintained for eight days prior to left parietooccipital craniotomy and excision of the causative left occipital arteriovenous malformation (Figures and ). At surgery small arterial feeders were evident 5cm from the midline. The hematoma cavity was evacuated. The arteriovenous malformation extended into the lateral horn of the left lateral ventricle and across the surface of the tentorium, draining into the transverse sinus. The medial aspect of the left occipital lobe was disconnected from the midline and the affected 4 x 4cm area was hinged up onto the draining vein. The vascular pedicles were coagulated and divided, and the vascular malformation was removed. Shortly following the surgery, the patient displayed intermittent jerking of the right upper limb, as well as marked weakness of the left side. EEG testing revealed irregular brain waves in the posterior temporal and parietal regions with a right-sided bias. Both the jerking and the weakness resolved over 4 days. A right-sided homonymous hemianopia became evident. On discharge, the patient and her parents were informed of a possibility of spontaneous recovery of the hemianopia, but this was deemed unlikely. Her neuropsychological tests showed focal visual dysfunction, suggestive of difficulties in visual search (), but their origin was not identified at the time. For the following 17 years, the patient experienced challenges with many aspects of her life, including her mobility, her ability to access learning material at school, her social interactions, specific daily living activities including finding items on a supermarket shelf or clothes in her wardrobe, and the control of emotion. In 2013, at a conference presentation on identifying and supporting children with CVI, the patient recognized the difficulties described as being identical to her own. The patient introduced herself to the presenter and explained this.\nVisual assessment confirmed persisting right hemianopia. No evidence of visual impairment in the sighted visual field [] was elicited. A body turn to the right (without a head turn) slightly extended the field of vision to the right, suggestive of an additional element of right inattention. Nevertheless, repeated small discreet finger movements made by the examiner in multiple locations within the hemianopic field were correctly intuited on more than 70% of occasions, consistent with blindsight. Uncorrected visual acuities and stereopsis were in the normal range. Structured history taking for evidence of perceptual visual impairment [] revealed great difficulty finding an item from within clutter or within a pattern and great difficulty copying, consistent with simultanagnostic vision. Standard eye movement examination was normal, with normal convergence, pursuits, and saccades to command (e.g., ‘look right'), but instruction to look at specific items nominated by the examiner resulted in anarchic visual search movements until the specified item was eventually found. This feature is consistent with apraxia of gaze, a phenomenon known to accompany simultanagnostic vision []. When asked to look at a large group of people, the patient could only identify the presence of one person. Careful observation of eye movements during such search consistently showed them to be sparse and apparently random. In an outdoor café she was asked to count the number of support poles for the sun-shading roof. She randomly found and identified 3 out of 11, with the same lack of visual search strategy.\nThe accuracy of the patient's visual guidance of reach was evaluated. Careful observation of the in-flight gap between the fingers, and their orientation while reaching for discreet targets in the intact visual field showed consistent features of a gap much wider than necessary, with inaccuracy of orientation []. These features are consistent with a degree of accompanying optic ataxia.\nThe patient was given a detailed tutorial concerning the nature of her vision and strategies that she could employ to make best use of her vision. |
A 30-year-old female with a medical history of hypertension and smoking sustained a left mandibular angle fracture after a fall from a ladder. The patient underwent open reduction and internal fixation of her left mandibular angle through an intraoral approach without complications. The patient developed an infection at the site of the hardware and nonunion of the fracture site as a result of noncompliance—lack of smoking cessation and poor oral hygiene—with postoperative instructions. For treatment of the nonunion and infection, the patient returned to the operating room (OR) approximately 1 month after the initial surgery for debridement and removal and replacement of the hardware. Two months from the time of injury, the patient sustained a fist-to-face assault and developed an abscess at the site of the hardware. Incision and drainage of the abscess were performed at an outside hospital. However, the patient developed an extraoral fistula at the site of the infected hardware. She was scheduled for an elective fistulectomy, debridement of the previous fracture site, and removal and replacement of hardware 4 months from the time of her initial injury.\nIn the OR, all standard American Society of Anesthesiologists monitors were placed. The patient underwent preoxygenation for 8 minutes prior to intubation, and induction of general anesthesia was facilitated with 80 mg lidocaine, 160 mg propofol, 150 mcg fentanyl, 120 mg succinylcholine, and 10 mg rocuronium. Nasoendotracheal intubation was performed with a 7.5 mm nasal right angle endotracheal (RAE) tube, and a MacIntosh 3.5 laryngoscope was placed with direct laryngoscopy; however, 2 minutes after intubation during confirmation of tube placement, the patient was found to have a leak related to cuff rupture. An Eschmann-guided exchange was performed with a 7.0 mm nasal RAE tube and a McGrath video laryngoscope because a Cook airway exchange catheter was not available. Placement was confirmed with bilateral breath sounds, symmetric chest rise, and capnography; however, the patient had diminished breath sounds throughout auscultation with expiratory wheezes bilaterally. Approximately 10 minutes following confirmation of endotracheal tube placement, the patient's peak inspiratory pressures increased from 23 cm H20 to 64 cm H20, resulting in difficult ventilation. Numerous measures were taken, including suctioning the nasal RAE tube and administering 0.5 mg terbutaline subcutaneously and 0.5 mL of 2.25% nebulized racemic epinephrine; however, no improvement in ventilation and peak pressures was noted. Six minutes after the elevation in peak pressures, the 7.0 mm nasal RAE tube was removed, and bag-mask ventilation was initiated. Peak pressures elevation and ongoing difficulty with ventilation continued. The patient's oxygen saturation declined from 98% to 54%, and her heart rate decreased from 104 to 64 bpm, but no significant decline in blood pressure was noted. The patient was reintubated using a McGrath video laryngoscope and a 7.0 mm oral endotracheal tube, and ventilation was initiated via Ambu bag and supplemental oxygen. The patient's oxygen saturation increased to 96% and her heart rate to 92 bpm.\nShe began to develop significant left-sided facial and bilateral neck and chest edema with crepitus, indicating subcutaneous air. Decreased right breath sounds were noted with auscultation. Intraoperative chest x-ray revealed significant bilateral subcutaneous air, pneumomediastinum, and a large right-sided tension pneumothorax (). An attempt at needle decompression failed because of large chest wall thickness. General surgery emergently placed a 32-French chest tube on the right side without complication, resulting in an almost immediate improvement in ventilation and tidal volumes and resolution of the tension pneumothorax. The fistulectomy, debridement, and removal of hardware procedures were aborted.\nThe patient was transferred to the trauma intensive care unit (TICU) where she remained intubated and sedated. Bronchoscopy performed intraoperatively in the TICU following chest tube placement revealed no obvious injuries. Postoperative computed tomography (CT) maxillofacial scan without contrast revealed extensive air within the right parotid, masticator, retropharyngeal, and bilateral carotid spaces, along with right preseptal periorbital, bilateral neck, and anterior chest wall soft tissues. CT of the neck without contrast demonstrated a large amount of soft tissue emphysema within the anterior and posterior neck, superior mediastinum, and chest wall compartments, and CT of the chest without contrast demonstrated persistent right-sided pneumothorax ( and ).\nThe following day, flexible fiberoptic laryngoscopy performed by the TICU service revealed a tissue flap in the posterior hypopharynx with overlying blood clot. After removal of the clot, a tissue defect consistent with a right pyriform sinus tract laceration injury was identified (). Otolaryngology confirmed the pyriform sinus injury. Enteral feeding through an orogastric tube was initiated, and intravenous (IV) ampicillin-sulbactam was administered for aerodigestive organism coverage.\nThe patient remained intubated and sedated until day 5 when she was extubated over an Eschmann catheter by the anesthesia service. The following day, the patient's chest tube was removed, and she was transferred to the floor for monitoring of her hypopharyngeal injury. She remained an inpatient for 2 days and then requested discharge from the hospital. The patient was advised to remain hospitalized until the otolaryngology service could perform a barium esophagram with laryngoscopy to reevaluate the pyriform sinus laceration, but the patient left the hospital against medical advice on postoperative day 8 after the risks had been discussed with her. The patient completed 8 of the recommended 10 days of IV ampicillin-sulbactam and 8 days of enteral feeding.\nThe patient was successfully contacted 1 week after leaving the hospital and agreed to return for follow-up evaluation and another attempt to perform the procedure. One month later, the patient underwent fistulectomy, debridement of the previous fracture site, and removal and replacement of hardware after nasoendotracheal intubation without complication using a C-MAC video laryngoscope. Preoperative evaluation with awake nasolaryngoscopy performed by the otolaryngology service revealed a well-healed pyriform sinus. Surgery was completed without incident, and the patient was extubated postoperatively without incident.\nThe patient was seen postoperatively 3 times in 1 month for outpatient follow-up. She continued to smoke cigarettes daily and did not comply with the recommended no-chew diet. At her final visit 1 month postoperatively, she had developed a superficial intraoral surgical site dehiscence. She did not return for additional follow-up visits. |
A 15-year-old male adolescent, an amateur swimming athlete, visited our clinic. His chief complaint was pain in the right shoulder when swimming. He first felt pain in his right shoulder 5 years before, but the pain disappeared just after a one-day rest, so he did not see a doctor. Four years after the incidence, the pain recurred during swimming. It was still mild but occasional from then on. Several days prior to his first visit to our clinic, the pain suddenly got worse and he consulted an orthopaedic doctor, who took x-rays and MRI of his right shoulder and then referred the patient to our clinic.\nHis pain was located at the lateral portion of the right shoulder. The intensity of pain assessed by visual analog scale was 00/100 at rest, 25/100 during motion, and 00/100 at night. There was no muscle atrophy and no positive impingement sign. There was a slightly limited range of motion of the right shoulder: flexion, 150 degrees; abduction, 120 degrees; external rotation, 75 degrees; and internal rotation, T4.\nAn anteroposterior radiograph showed osteolysis of the subchondral area of the medial part of the humeral head. MRI (T1- and T2-weighted images) revealed an inhomogeneous low signal area in the medial part of the humeral head with high signal in the joint space on T2-weighted image. Although administration of any kind of steroid, history of sickle-cell disease and other risk factors for osteonecrosis were denied, based on the clinical findings, we primarily established a diagnosis of osteonecrosis of the right humeral head. According to the classification system of Cruess \n[], the lesion was classified as stage 3, which was characterized by the crescent sign (subchondral fracture). We talked to the patient to stop swimming or any kind of sport activities. The shoulder was immobilized in a sling until the pain disappeared. We decided to observe the patient, expecting the lesion would be remodeled, but also with an option of core decompression in case it would not be remodeled. However, on May 28, 2007, when the patient revisited us, the plain x-rays and MRI both showed that the lesion became clearer in shape and a little larger in size. A sclerotic band appeared surrounding the lesion on the x-ray (Figure \n). The low signal area on MRI scan became homogeneous and the margin became clear (Figure \n). The patient complained only of slight motion pain with the range of motion well preserved. Due to a rapid aggravation of the lesion, a malignant disease was also suspected and bone scintigraphy was performed, but no abnormal findings were observed. We talked to the patient that a biopsy might be necessary if the lesion would still expand in 2 months. On the following visit, the pain subsided completely with a full range of motion of the right shoulder. Both plain x-ray and MRI showed that bone remodeling had started and the lesion had become smaller. These findings confirmed the diagnosis of osteonecrosis. Still the patient was not permitted to perform any sport activities. On December 25, 2007, new MRI scans revealed that the lesion had become even smaller and there was only a low signal band left on several slices in the coronal oblique view. On June 30, 2008, the healing progressed even further, and abnormal signal had disappeared on all but 2 slices, on which only a thin and vague low signal line could be observed on the posteromedial subchondral area (Figure \n). Because the healing process was almost completed, the patient was permitted to start swimming in a gradual manner. On the latest visit of March 31, 2009, the lesion was totally healed with normal appearance on plain radiograph (Figure \n). There was no pain and the range of motion was full. |
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