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A 55-year-old man presented to our hospital complaining of fever, rash, and a sore mouth. Twenty-three days prior the patient had undergone orthotopic liver transplantation for chronic active hepatitis C with a T 1 N 0 M 0 hepatocellular carcinoma. His transplantation was without immediate complication, and he was discharged on the following new medications: bactrim ss daily, aspirin 325 mg daily, azothioprine 100 mg daily, tacrolimus 2 mg daily, and valgancyclovir HCl 900 mg daily. The patient had been treated with antithymocyte globulin during induction of immunosuppression.
Transplantation hepatology
The patient had no known drug allergies. His past history was notable only for type 2 diabetes mellitus and ethanol dependence (abstinent for 1 year). He was taking no alternative medicines. The patient's laboratory results were notable for a serum creatinine level of 1.4 mg/dL (increased over a normal baseline) and a white blood cell count of 0.1 with a differential showing no neutrophils. A skin biopsy revealed full-thickness epithelial necrosis, consistent with a diagnosis of Stevens-Johnson syndrome or TEN. There was a sparse lymphocytic infiltrate in the papillary dermis.
Dermatology
Although his airway was initially stable, within 24 hours of admission he was intubated for airway protection as his oropharyngeal mucosa began to disintegrate. Meanwhile, his bullous erythroderma progressed until most of his epidermis had sloughed off and he developed intermittent atrial fibrillation and a vasopressor requirement. In consultation with infectious disease medicine and transplant surgery services, the patient was begun on empiric vancomycin and aztreonam and all of his immunosuppressants were held except for tacrolimus (dose adjusted down).
Dermatology and Intensive Care Medicine
Over the next several days, the patient's condition continued to decline with anuric renal failure and Staphylococcal/Candidal septic shock. Filgrastim failed to evoke an increase in his circulating neutrophils. He became progressively hypothermic and his septic shock worsened. An ultrasound of the hepatic artery showed that it was patent. Continuous renal replacement therapy was started 4 days after admission. The patient gradually became increasingly acidotic and hypotensive and died 7 days after admission. The patient was given human intravenous immunoglobulin (IVIG) during his course because of a very low measured serum IgG level. At the request of the transplant surgery service, he also received a short course of high-dose solumedrol. Among candidate etiologic agents, sulfa and beta-lactam antibiotics stood out: both of these had been started within the 3 weeks prior to the patient's presentation with TEN and both are known to be frequently implicated in TEN. 1 , 2
Nephrology/Intensive Care Medicine
This patient's diagnosis may well have been TEN, and if so, this constitutes the first case report of severe TEN following liver transplant in the United States. There are 2 previous reports of TEN following liver transplant, one in the French literature 3 and the other involving a case of mild desquamation without mucous membrane involvement. 4 Because posttransplant GVHD bears such a strong clinical resemblance to TEN, GVHD is a diagnosis that must be maintained in the differential for all of these patients. Below, we review the clinical characteristics of both TEN and GVHD, illustrating the diagnostic ambiguity in our patient's case.
Dermatology/Transplantation Medicine
Lack of a good animal model for TEN 20 and the rarity of the syndrome have hampered investigation of its etiology at the cellular level, but, as noted above, the syndrome appears to be mediated by cytotoxic (CD8+) thymocytes. Consequently, the case reported here is surprising. The patient had just had a liver transplant and had received antithymocyte globulin as part of his immunosuppressive induction. Following the transplant, he was taking 3 immunosuppressive medications. Nonetheless, within 3 weeks of starting Bactrim pneumocystis prophylaxis and within 1 week of starting beta-lactam antibiotics he developed an illness indistinguishable clinically from TEN.
Immunology
A previously healthy 72 years old female presented with rotatory vertigo associated with nausea and vomiting. The symptoms had started five days earlier as short lasting vertigo attacks, her symptoms had been continuous for three days. Clinical examination showed BP 200/95. Otoscopy was normal No nystagmus in a neutral position, and no pathological findings by standard clinical neurological examination was found. The next day the patient was still nauseated and described continuous vertigo, with a slight improvement. Hallpikes manoeuvre did not evoke nystagmus or exacerbation of her vertigo.
Neurology
On follow up at four weeks later her symptoms had improved but not disappeared completely. ENG still showed right beating nystagmus in the left-rotated position, and still normal calorics. A new VEMP was recorded. This time the test was noted to be unpleasant for the patient, and cooperation was suboptimal. Absent responses on both sides were found.
Otolaryngology
77 years old female, previously healthy except for a short period of vertigo two years earlier. She presented with a two day long history of vertigo, nausea and vomiting, still nauseated and with inability to walk. Standard clinical exam showed no limb ataxia or other obvious pathological findings. Removal of fixation with the use of Barthels goggles revealed a counterclockwise nystagmus (with respect to observer), combined with nystagmoid jerks to the right. This nystagmus was completely suppressed by fixation. VEMP was normal on the right side, absent on the left. ENG was performed two weeks later. A weak nystagmus, fast beat to the right, was found, only when left ear was lower (slow phase velocity 10–14 degrees per second). Calorics demonstrated no significant asymmetry. MRI was normal except for minor white matter changes. Otoscopy and audiometry were normal. She made a good recovery but was still complaining of intermittent vertigo at follow up, two weeks after admission.
Neurology/Otology
A 64 yrs old female presented with vertigo, nausea and headache of two days duration. On admission she had no nystagmus, and normal clinical exam. including otoscopy. Audiometry and MRI was normal. VEMP showed attenuated/scarcely identifiable response on the left side . ENG showed slight nystagmus (7–8 degrees per second) in neutral (supine) position, which increased markedly, to a slow phase velocity of 40 degrees per second, on left rotation of the head, beating to the right . She reported no symptoms during these positional tests. Calorics showed no significant asymmetry.
Otolaryngology
Her condition improved but she had still symptoms at follow up two weeks after onset. On follow up five months later she reported some vertigo in episodes of 1–2 days duration, but was free of symptoms most of the time. ENG showed only slight spontaneous nystagmus is left position, and calorics was normal and symmetrical. VEMP was still absent on the left side, and normal on the right.
Otolaryngology
A 70-year-old man was referred for coronary revascularization with angina (Canadian Cardiovascular Society class 2), three vessel disease and good left ventricular function. He was otherwise well, with no significant past medical history and physical examination was unremarkable. Preoperative risk assessment scores were: EuroSCORE standard 3 (age), logistic 1.82. Preoperative investigations revealed a white cell count (WCC) of 15.6 × 10 9 /L (neutrophils 10.1 × 10 9 /L, monocytes 2.5 × 10 9 /L) and urinalysis positive for blood. He denied any urinary or respiratory symptoms but was treated empirically for a lower urinary tract infection and discharged home. Urine culture was negative. Two weeks later, the WCC remained elevated at 14.2 × 10 9 /L (neutrophils 10.5 × 10 9 /L, monocytes 1.6 × 10 9 /L), although he remained asymptomatic and was keen to proceed.
Cardiology
Coronary artery bypass grafting was performed using the left internal mammary artery and saphenous vein grafts. Cardiopulmonary bypass (CPB) time was 76 minutes with aortic cross-clamp for 39 minutes, using antegrade cold crystalloid cardioplegia. The patient came off CPB easily and remained haemodynamically stable thereafter. He was extubated expeditiously and returned to the ward on the first postoperative day. Routine blood tests demonstrated a leukocytosis (46.9 × 10 9 /L) with marked neutrophilia (38.5 × 10 9 /L) and monocytosis (8.4 × 10 9 /L).
Cardiac surgery
Late on the first postoperative day, his condition deteriorated with progressive hypoxia, oliguria and metabolic acidosis. He was readmitted to the intensive care unit for non-invasive ventilation and inotropic support. On the morning of the second postoperative day, he remained hypoxic and oliguric. A pulmonary artery catheter revealed low cardiac output state and low systemic vascular resistance. He was reintubated and mechanically ventilated, inotropic therapy was increased and continuous veno-venous haemofiltration and broad-spectrum antibiotics were commenced. A cardiac origin of low cardiac output was excluded and sepsis or splanchnic ischemia were considered as causes of his systemic inflammatory response syndrome (SIRS) and multi-organ dysfunction (MOD). Computed tomography of the thorax and abdomen showed bilateral ground glass shadowing of the lung parenchyma consistent with acute respiratory distress syndrome but no other significant findings. The leukocytosis increased to 91.7 × 10 9 /L (neutrophils 54.1 × 10 9 /L, monocytes 33.0 × 10 9 /L) and his condition deteriorated. A haematology opinion was sought and confirmed a diagnosis of probable leukaemoid reaction on a background of CMML. Despite maximal therapy, the patient died on the third postoperative day. Post-mortem findings were of an enlarged spleen and widespread SIRS, manifesting as pulmonary congestion with patchy hemorrhagic necrosis of the small and large bowel.
Intensive Care Medicine
The patient was referred for cardiac surgery with undiagnosed CMML. As the proportion of elderly patients undergoing surgery steadily increases, more occult chronic conditions are likely to be diagnosed on preoperative work-up. Despite reports that patients with chronic haematological malignancies undergoing cardiac surgery are at increased risk of complications, including bleeding and infection , a preoperative diagnosis would not necessarily have altered the decision to proceed with surgical revascularization. In retrospect, the blood count and film were suggestive of CMML in the chronic phase, with no evidence of acute transformation or the leukaemoid reaction to come. Indeed, urgent coronary artery surgery has been successfully performed in a patient with acute myeloid leukaemia . The key questions are whether patients with haematological malignancies with a poor prognosis should be candidates for cardiac surgery for prognostic benefit per se and why our patient responded to the inflammatory stress of surgery in such a catastrophic and unpredictable fashion. This may be related to the unfortunate timing of surgery in the natural course of his disease or the susceptibility of his particular subtype of CMML to inflammatory stress.
Cardiology and Haematology
As illustrated in figure 3 , between ages 10 and 11 years the patient's peripheral blood counts showed continuous decline requiring substitution with several erythrocyte concentrates. Bone marrow aspiration revealed strongly reduced cellularity with very few megakaryocytes confirming progression of bone marrow hypoplasia such that there was concern of beginning bone marrow failure (8% myelocytes, 9% band granulocytes, 9% segmented granulocytes, 2% monocytes, 50% erythroblasts, 20% lymphocytes). Due to poor cellularity a repeat bone marrow chromosome study was not attempted. Because of the patient's continuously decreasing peripheral blood counts, deteriorating general condition, and the known risk of LIG4-deficient patients for hematologic malignancies , the option of a timely BMT was considered. Her 15-year-old HLA-identical brother was healthy and carried no ligase IV mutation, making him available as donor and facilitating the decision in favor of transplantation. On admission to transplant, the patient's white blood cell (WBC) count was 2.7 × 10 9 /L, platelets were 22 × 10 9 /L, and hemoglobin was 6.6 mmol/L. Immunoglobulin (Ig) levels before transplantation were IgM 0.35 (g/L), IgG 8.83 (g/L), and IgA 0.74 (g/L). Lymphocyte subsets were CD3 960, CD4 412, CD8 531, CD19 11, and NK 222 (cells/microlitre). Total lymphocytes (CD45+) were 98.1%, monocytes (CD14/45+) were 1.1%, NK cells (CD16+56+) were 17.8%, and T cells (CD3+) were 76.8%.
Hematology
The patient received 5.43 × 10 8 /kg nucleated cells of unmanipulated bone marrow from her HLA-identical brother. GvHD prophylaxis consisted of cyclosporine A at 3 mg/kg/d from day -1 until day 64 but without additional methotrexate. On day 5, granulocyte colony- stimulating factor (G-CSF) (5 μ g/kg/day) was started and given until day 22. The patient did not suffer from any severe side effects and did not even loose her hair. The leukocyte nadir was reached on day -1 with 0.1 × 10 9 /L. Engraftment was observed as early as day 10 with an absolute neutrophile count (ANC) of > 0.5 × 10 9 /L (74% segmented granulocytes; 10% band granulocytes) and a WBC of 1.1 × 10 9 /L. Prophylaxis for fungal, viral and bacterial infections was with acyclovir (30 mg/kg/day from day -2 until day 11), liposomal amphotericin B (1 mg/kg/day from day 6 until day 10), fluconazol (5 mg/kg/day from day 12 until day 16), metronidazol (20 mg/kg/day until day 11), ceftacidim (100 mg/kg/day until day 15), and penicillin V (50,000 units/kg/day from day 18 until today). Pneumocystis jiroveci prophylaxis was performed with cotrimoxazol (5 mg/kg/day; 3 days per week from 1 until 6 months after BMT). Immunoglobulins were given at a dose of 250 mg/kg/day on days 2, 8, and 19. The patient received a total of 7 platelet concentrates, the last on day 13. The girl was discharged on day 22 in good clinical condition with a WBC of 1.2 × 10 9 /L, platelets of 32 × 10 9 /L, and hemoglobin of 6 mmol/L.
Hematology
Blood chimerism analysis showed 100% donor cells on day 46. The percentage of donor cells declined to 91% on day 60 but resurged to 100% following withdrawal of cyclosporin A. Complete chimerism was established within 4 months after BMT, remaining stable since that time. As shown in figure 4 , sequential flow cytometric studies of mitogen response vs . radiosensitivity confirmed the replacement of radiosensitive patient cells by radioresistant donor cells. Currently at age 16, her immune system functions well, with a good immune response towards all common vaccinations. During the course of two years after BMT the patient gained considerable weight. She had always been below the 20 th percentile, whereas now at age 16 she is overweight with 44 kg at 146 cm. Head circumference is 44 cm. Her height is still below the 3 rd percentile, but her general physical condition is excellent. She is fully active and can lead a normal life with no restrictions (100% Lansky-Play-performance). Due to her mild to moderate mental retardation, she currently attends grade 9 of a special school for mentally handicapped children, with good success. There are no skin or genital abnormalities, but puberty is markedly delayed. There is little pubic hair and breast development, corresponding to Tanner stages P3 and B3, and the patient has not yet started menstruating. The hormonal profile is suggestive of hypergonadotrophic hypogonadism (FSH is 55.5 IU/L, LH 24.2 IU/L, estradiol 73 pmol/L).
Hematology and Immunology, with aspects of Pediatrics and Endocrinology
A 52-year-old female presented to an otolaryngologist with a hoarse voice. An endocrine opinion was sought to exclude an underlying endocrinopathy. She had been investigated three years previously by a neurologist for right-sided facial pain. A magnetic resonance imaging (MRI) scan had shown a small “cyst” in the pituitary gland. She had been reassured that this was a coincidental finding. The patient did not volunteer any specific symptoms and had no complaints other than a hoarse voice. Her appearance is shown in Figure 1 .
Endocrinology
On closer inspection, there was a suggestion of some broadening of the nose and prominence of the lips, rather large hands, and increased skin thickness . Visual fields were normal and Tinel's sign was positive. Blood pressure (BP) was 160/96 mm Hg. Blood tests showed normal urea, creatinine and electrolytes, fasting glucose of 6.6 mmol/l (normal range, <5.6 mmol/l), prolactin 330 imu/l (normal <550 imu/l), and thyroid stimulating hormone (TSH) of 1.4 mIU/l (normal 0.3–4.7 mIU/l) with free T4 of 16 pmol/l (normal 11–23 pmol/l).
Endocrinology
The patient's random GH level was 55 mu/l (0–20) along with an elevated IGF 1 level of 78 nmol/l (age-matched normal range being 12–44). She then proceeded on to an OGTT with glucose and GH level measurements; the results are outlined in Table 1 .
Endocrinology
This patient's case highlights the dilemma clinicians are facing today as a result of the increasing use, availability, and precision of imaging techniques. She had a MRI scan previously that detected a pituitary tumour. Pituitary tumours that are detected coincidentally (so called “incidentalomas”) are not uncommon with reports of prevalence of up to 10% in adults. Given the limitations of diagnostic testing, biochemical screening requires sufficiently high pre-test probability to make it effective. Apart from the cost factor, an imaging-first principle in any field in medicine that relies on biochemical proof of disease leads to a cascade of tests and pursuit of false-positive results. Therefore, appropriate clinical evaluation should precede biochemical investigations, which then may need to be confirmed by imaging techniques .
Endocrinology
She was then started on somatostatin analogue therapy to achieve better GH level control. This reduced mean GH levels to 2.9 mu/l and normalised IGF 1 levels to 32 nmol/l. After discussion with the patient, it was decided not to proceed with radiotherapy since the acromegaly was well controlled with medical therapy and due to the high probability of hypopituitarism associated with radiotherapy. She remains asymptomatic and is under regular endocrine follow-up.
Endocrinology
Macroscopically the tumour measured 4 × 3 × 2.5 cm, was white and well circumscribed. Microscopically it was moderately cellular and it contained spindle cells with irregular and focally pleomorphic nuclei. No mitoses or necrosis were seen . No Antoni A areas were present. A definitive histological diagnosis of a benign cellular neurofibroma was made. After 5-years no recurrence has been observed.
Pathology
The microdebrider is a recent addition to the armeratarium available to the interventional bronchoscopist for central airway occlusion, following the successful application of laryngeal microdebriders for over a decade . However, limitations of the microdebrider include maneuverability and length (37 cms) which limits use to the trachea and proximal main-stem bronchi. We report a case of malignant distal left main-stem obstruction treated with a new elongated rotating tip microdebrider (45 cms) via rigid bronchoscopy with sufficient length to reach distal bronchial lesions.
Pulmonology
Physical exam revealed reduced breath sounds in the left lower zone. Chest x-ray demonstrated a left hilar mass with loss of volume and post obstructive pneumonia in the left lower lobe . Axial Computed tomography (CT) angiogram of the chest also revealed multiple segmental and subsegmental pulmonary emboli and the patient was admitted for anticoagulation with low molecular weight heparin (enoxaparin). The CT study also confirmed total occlusion of the left mainstem bronchus by a tumor invading from the left hilum .
Pulmonology
After two days of anticoagulation, rigid bronchoscopy was performed using an Efer-Dumon rigid bronchoscope. Normal vocal cords, trachea, carina and right bronchial tree were confirmed. A large endobronchial lesion with complete occlusion of the left mainstem bronchus was identified . After an unsuccessful attempt to pass the lesion using a pediatric video bronchoscope, debulking was attempted using a combination of polypectomy snare and electrocautery. There was still 100% obstruction of the airway from the residual tumor and therefore the ERBE argon plasma electrocoagulator ® (APC) was utilized to devitalize the tissue. The devitalized tissue was then debulked using the Straightshot ® microdebrider with rotating tip and the bevel of the rigid bronchoscope. The length of the microdebrider is 45 cm and outer diameter is 4 mm. The rotating tip is controlled by a fly-wheel on the hand-piece, which allows for better maneuverability and is angled at 12 degrees providing better visualization. The blade was serrated and an oscillator mode was used at 1000–1200 RPMs. In this manner the left main stem was recanalized and the channel was opened into the left lower lobe bronchus. Hemostasis was achieved using a combination of suction and APC with less than 20cc blood loss. Visual examination revealed that there was some degree of extrinsic compression in the left lower lobe bronchus as well; however, the superior segment bronchus and the basal segmental bronchi were noted to be patent. Post procedure residual obstruction in the left main stem bronchus was estimated at less than 20%. Since the left upper lobe had been completely replaced by tumor, a guidewire was then placed into the left lower lobe bronchus and a 4 cm × 14 mm covered nitinol self-expandable metal stent was placed over the guidewire under direct vision into the left lower lobe bronchus. The stent was deployed under direct vision and adjusted in terms of its position using rat-tooth forceps. At the end of optimal positioning, the distal aspect of the stent was noted to be just inside the left lower lobe basal segmental bronchus with the superior segment being ventilated through the uncovered portion of the stent. The proximal aspect of the stent was noted in the left main stem bronchus well above the proximal extent of the tumor . Follow-up chest x-ray revealed aeration of the left lower lobe with resolution of dyspnea and hemoptysis.
Pulmonology
In conclusion, this case report details the successful management of a distal left mainstem obstructing malignant lesion with a combination of therapies including a novel elongated microdebrider with rotating tip. Prospective trials are required to identify specific indications for this therapy along with long-term outcomes in the management of central airway obstruction.
Pulmonology
A 50-year-old female presented with complains of painless progressive swelling in right cheek region of two months duration. Examination revealed a 6 × 4 cm bony hard swelling in right zygomatic region near and below lateral canthus of right eye Roentgenogram of the paranasal sinuses showed a lesion arising from right maxilla and right zygoma. Computerized tomographic (CT) scan revealed two separate masses one in right orbit and other infiltrating zygoma and maxilla , radiographic picture was suggestive of osteogenic sarcoma or Ewing's sarcoma. A fine needle aspiration was carried out that showed typical polygonal and spindle cells with moderate amount of cytoplasm and vesicular nuclei with nucleoli, many with intracellular pigment; seen singly and clusters, the picture was suggestive of malignant melanoma . The ophthalmologic consultation revealed that there was no useful vision in the right eye. A B-scan of the right eye showed an organized mass within the vitreous cavity indicating vitreous haemorrhage . A detailed clinical examination failed to show any other melanocytic lesion on skin and other mucus membranes.
Oncology
With a provisional diagnosis of melanoma of the maxilla and zygoma with vitreous haemorrhage and no useful vision in right eye a wide excision of the lesion including anterolateral maxillectomy, zygomectomy, and removal of superolateral wall and floor of orbit with en bloc exenteration of the right eyeball was carried out . Histopathological examination of the resected eyeball specimen showed a neoplasm in choroid, filling posterior chamber with no extraoccular extensions, composed of sheets, nests and cords of polygonal cells and vesicular nuclei. Prominent nucleoli and eosinophilic cytoplasm containing abundant melanin pigment were noted . Similar tumours were seen in the extra ocular soft tissue and resected maxilla and zygomatic bones. A final diagnosis of choroidal melanoma metastasizing to maxillofacial bones was made. Differential diagnosis of primary bone melanoma with metastasis to choroids was kept.
Ophthalmology and Oncology
An 11-year-old boy was referred for evaluation of an exertional dyspnoea. He reported recent history of few episodes of shortness of breath associated with moderate entity physical activity. At physical examination a mild continuous murmur could be heard mainly at the level of the second intercostal space of the left parasternal area. A transthoracic echocardiogram showed a continuous flow at color Doppler analysis in the high parasternal short axis view, originating from a small entry site on the wall of the main pulmonary artery (arrow). A selective left coronary angiography revealed a fistula connecting the proximal portion of the left anterior descending coronary artery (solid white arrow) with the main pulmonary artery (blank white arrow); black arrows indicated drainage into the pulmonary circulation.
Cardiology
This was a 44-year-old male patient who presented to the Plastic Surgery outpatient clinic with a chronic non-healing wound on the left leg (10 × 15 cm) due to venous stasis. The wound had been present for three months and exhibited positive methicillin resistant staphylococcus aureus (MRSA) cultures on two occasions. The MRSA was treated with parenteral as well as oral antibiotics. Past medical history was significant for moderate rheumatoid arthritis for which the patient was taking 5 mg prednisone daily. The patient's wound was initially treated using wet to dry dressings for five days. Next, a single debridement with the Versajet™ hydrosurgery system (Power setting: 5) was performed followed by immediate split-thickness skin grafting. Early postoperative course was uneventful with full graft take .
Plastic Surgery
This was a 42-year-old female patient who had a third degree burn injury to the left hand following a motor cycle accident. The injury affected the volar and proximal aspect of index, middle, ringer, little finger and distal palmar regions. An initial escharectomy was followed by two debridements using the hydrosurgery system (Power setting: 2–3). Subsequently, a thick partial thickness skin grafting was performed with full take . Physical therapy was started within ten days after skin grafting with no disability or contracture formation at 3 month-follow-up.
Plastic surgery
This was a 50-year-old female patient with a traumatic left elbow wound due to a motor vehicle accident. The soft tissue defect was 5 × 7 cm in size with exposed olecranon. Because of her additional abdominal injury, a wound vacuum assisted closure (Vacuum-assisted closure (V.A.C), KCI, San Antonio, TX) was placed. Within three days after VAC treatment, debridement with the hydrosurgery system (Power setting: 5) was performed followed by immediate reconstruction with lateral arm island flap . Physical therapy was initiated at one week after the surgery. The patient regained elbow functions in 2 months.
Orthopedic surgery
This was a 30-year-old female patient with a traumatic degloving injury (15 × 25 cm) involving distal thigh and popliteal fossa extending to the lateral aspect of the knee on the right side. The mechanism of injury was a motor vehicle collision. The Versajet™ hydrosurgery system was used twice for wound debridement. The wound edges had 6–7 cm of dissection medially, laterally, and superiorly. The Versajet™ was also used for debridement under the dissected skin edges (Power setting: 6). The underneath of the avulsion skin flaps were tacked down to the wound bed to obliterate the dead space. The distal thigh wound was skin grafted, and the popliteal wound and lateral aspect of the knee were reconstructed with medial gastrocnemius muscle flap covered with split-thickness skin graft . Minor wounds healed with no complications at three weeks.
Plastic surgery
The emergency department doctor's examination found a small cut to the nose. The patient was disorientated, exhausted with generalised weakness and subsequent difficulty in moving either arm. Both shoulders were documented as symmetrical with no injury to the soft tissues and grossly neurovascularly intact but were uncomfortable and had limited range of movement. A 'first fit' was diagnosed, bloods were requested and a referral was made to the medical team. The doctor starting the next shift performed a full musculoskeletal examination because of the persisting pain in the shoulders. Radiographs of the shoulders were taken and confirmed bilateral anterior shoulder dislocations . The dislocations were reduced under sedation and the patients' upper limbs were placed in poly-slings. After four weeks of physiotherapy shoulder movements returned to normal.
Orthopedics
Cooper in 1839 first reported an association between seizures and posterior shoulder dislocation . In 1902 Mynter first described bilateral posterior shoulder dislocations in a patient following a seizure with numerous cases reported since. Aufranc reported the first bilateral anterior shoulder dislocations following a seizure in 1966 . Only seven further cases have subsequently been reported in the literature . This is the first published case to be missed on initial examination. Because of the absence of any obvious shoulder asymmetry, the patients' generalised weakness and exhaustion, the discomfort and difficulty in moving his arms was initially attributed to a post-ictal state. Full musculoskeletal examinations are not routinely performed following a seizure .
Orthopedics
Minocycline is widely prescribed for the treatment of inflammatory forms of acne vulgaris . Although it is considered to be a safe drug , it has been reported to cause serious adverse events such as hepatitis, auto-immune syndrome and HS . To our knowledge, lymphocytic meningitis and brain edema associated with minocycline-induced HS have not been reported in the literature. This presentation, which is probably under-recognized, may lead to a diagnostic delay. We report herein one case.
Dermatology
A 31-year-old female native of Africa was hospitalized with fever, weakness, nausea, headache, facial edema, and rash, for 4 days. She had been diagnosed as HIV positive 2 years previously. CD4 cell count was between 300 and 400 cells/mm 3 , viral load was near 150,000 copies/mm 3 , both steady for several weeks. She had no other relevant medical history, and was on no treatment for HIV. Three weeks before the onset of the symptoms, she had been started on oral minocycline to control an inflammatory form of acne vulgaris .
Infectious diseases
At admittance she was unwell and vital signs were: blood pressure 100/60, temperature 40°C, heart rate 120 beats/min., respiratory rate 22/min., and oxygen saturation 97% (room air). On physical examination, she had multiple erythematous, oedematous and coalescing plaques on the upper trunk, and on proximal segments of the limbs. Her palms and soles were erythematous. She also had injection of the sclera, and edema of the eyelids. Buccal and genital mucous membranes were unaffected. Palpation revealed enlarged tender lymph nodes at all sites, but no accompanying hepatomegaly or splenomegaly. Cardiovascular and pulmonary systems were normal, with no sign of septic shock. Neurological examination was normal.
Dermatology
Minocycline was discontinued on the fourth day after the onset of the symptoms. Two days after minocycline withdrawal, treatment with steroids was introduced (methylprednisolone 60 mg daily) because of the severity of the symptoms. The patient improved quickly after steroid administration. Temperature dropped to 37°C within three days. Within one week the eruption cleared and lymphadenopathies disappeared. Biological abnormalities (eosinophilia, liver enzyme elevation) resolved within three days. A second brain CT-scan, undertaken 14 days after the onset of the symptoms, was normal. Lumbar puncture was not repeated. The steroids were steadily tailed off (5 mg per week) but a relapse occurred on week 6, when on a dose of 30 mg per day. This relapse was under the form of a transient and isolated generalized pruritus with no cutaneous nor neurological signs. Skin tests were not carried out due to the potential risk of severe reaction. The patient was weaned off steroids over a period of 8 months and was free of symptoms at discharged form care.
Dermatology
The case described had several features suggesting a diagnosis of HS secondary to minocycline treatment. The history was characteristic (interval of 3 weeks between the introduction of the drug and the symptoms) and the condition resolved promptly following cessation of minocycline. Moreover, differential diagnoses due to the most likely infectious diseases were excluded and no evidence of pseudotumor cerebri syndrome, as it may be observed with minocycline, was found. While lymphocytic meningitis and cerebral edema have not been described in association with minocycline-induced HS, they have been reported following use of allopurinol .
Pharmacology
All patients presented with abdominal pain and epigastric tenderness. Both handle bar injuries were associated with bruises in the epigastric region; one of the MVA patients had lap seat belt marks of bruises on the anterior abdominal wall and was found to have first Lumbar vertebral (L1) compression fracture. All patients who had MVA had associated injuries which included head injury , L1 compression fracture, rupture spleen , fracture femur , Right Kidney , one patient had multiple fractures. All were haemo-dynamically stable except one who had multiple fractures.
Traumatology
The two patients with duodenal perforation had emergency laparotomy and closure of the perforation with an omental patch followed by total parenteral nutrition (TPN) which was initiated due to delayed recovery from associated injuries and prolonged ileus; TPN was used for 10 days in one patient and 14 days in the second one. No major complications were seen and the average hospital stay was12.2 days. There were no deaths.
Gastrointestinal surgery
Six patients had colonic injuries, 5 males and one female; with median age 14 (range 5–16). Three had hematoma of the right colon and caecum, 2 caused by MVA of which one was treated conservatively and one sustained by knee to the abdomen during cricket match which needed right hemicolectomy. All were diagnosed on CT scan. 2 had stab wound injuries to the descending colon and sigmoid colon both were treated by resection and end to end anastomosis. The sixth (9 year old female) patient had severe multiple injuries including aortic dissection, fracture L3 spinal cord injury, head injury and perforation of the right colon treated with right hemicolectomy and ileostomy she died after 19 days from multiple organ failure related to sepsis.; the rest recovered well 2 of them were readmitted 2 years later with partial small bowel obstruction which resolved without operative treatment.
Traumatology/Surgery
We identified one patient with perforation of the distal jejunum. He was 21 month old who was previously admitted with subdural hematoma presented on this occasion with abdominal distention, vomiting and bruises on the anterior abdominal wall and scrotal bruises and altered liver function test. Abdominal ultrasound exam (done before surgical involvement) showed large amount of free fluid in the abdominal cavity, CT scan was then obtained and showed free air in the peritoneal cavity. At laparotomy there was jejunal perforation and a defect in the mesentery. A delayed perforation had probably occurred due to mesenteric vascular injury and ischaemia of the bowel wall, the increased liver enzymes were caused by paracetamol poisoning (part of child abuse). The child has recovered well from surgery.
Pediatrics
A 43-year-old man with a history of moderate hypertension and hypercholesterolemia consulted his physician for non-specific retrosternal pain. The first diagnostic tests ruled out myocardial ischemia. Due to the persistence of the symptoms, the patient underwent endoscopy of the upper gastrointestinal tract which revealed the presence of gastric adenocarcinoma in the antrum and corpus. The patient underwent a laparotomy. However, the direct infiltration of the tumor into the pancreatic head and the presence of small metastatic nodules in the omentum did not allow for a surgical resection.
Oncology
At the first examination by the medical oncologist the patient complained of moderate pain in the epigastric region with dorsal irradiation and dyspepsia. Codeine and acetaminophen were prescribed for symptom control. To complete tumor staging, a contrast-enhanced spiral computed tomography (CT) of the chest, abdomen, and pelvis was carried out which showed the presence of three pathological lymph-nodes. The lymph nodes had diameters larger than 10 mm and were located in the lesser curvature of the stomach, in the peripancreatic region, and in the mediastinum. Three pulmonary nodules that were smaller than 10 mm were also observed. A very small amount of ascites was present in the abdomen, but neither hepatic nor lytic bone lesions were detected. A representative bone window scan of the pelvis is shown in Figure 1A . The results of hematological and biochemical tests were normal with the exception of serum alkaline phosphatase, which was 2.5 times the upper limit of normal.
Oncology
We reasoned that the observed bone lesions were due to the strong osteoblastic response of preexisting bone metastases not visible on the baseline CT scan. In agreement with the patient, treatment was continued for two additional cycles. At the second tumor re-evaluation 6 weeks later, the target lesions were in confirmed partial response, and the bone metastases remained stable and no new blastic lesions appeared.
Oncology
We report a rare case of a GA, in which the patient's high level of serum ornithine was responsive to therapy with vitamin B6 dietary supplement and was reduced to near normal level.
Biochemistry
A 28-year-old man presented with the complaint of gradual visual loss during the past five years. He had noticed night vision difficulties since the age of 15 years but did not seek medical advice. His visual acuity in each eye was at the level of counting fingers; his refractive error measured -6.00-1.50 × 180 for the right eye and -5.00-2.00 × 170 for the left eye. His best corrected visual acuity was 3/10 in the right eye and 1/10 in the left eye. Bilateral 2+ posterior subcapsular cataracts were present. Fundus examination of both eyes revealed sharply demarcated areas of choroid and retinal atrophy in gyrate shape and involving the midperiphery with the macula not affected. A tilted disc in right eye was also presented . Clinical diagnosis identified gyrate atrophy of the choroid and retina. He had mild muscle weakness, but his intelligence and electroencephalography (EEG) were normal. Aminoacid analysis revealed a high serum ornithine level (629 nmol/mL), with the normal range being 28-110 nmol/mL. Arginine, creatinine, lysine and glutamine levels in the serum were within the normal range. He was treated with vitamin B6 at a dose of 300 mg/day for six months. This dietary supplementation successfully reduced his serum ornithine level by more than 50% to 293 nmol/mL.
Ophthalmology
This report describes the cases of two patients who experienced unusual trivial penile trauma resulting in infection and ultimately extrusion of a successfully inserted and well functioning penile prosthesis.
Urology
At presentation, both patients displayed fever (38.6°C and 39°C, respectively), and reported receiving broad spectrum antibiotics from general practitioners in their home towns. They denied any previous similar episodes since prostheses implantation. Physical examination in both cases revealed an erythematous, edematous and indurated penis with mildly macerated skin. The first patient also had ischemic spots over the penile shaft and localized soft swelling (3 × 2.5 cm) on the left side of the peno-scrotal junction . Penile and perineal palpation indicated intact devices in place, and this was further confirmed by radiography of the pelvis. However, the appearance of the patients' organs looked abnormal with poor concealment of the devices. The patients' white blood counts were elevated (13.200/mL and 14,100/mL, respectively). Urine analyses and cultures yielded negative results. Diabetes was well controlled in both patients as evidenced by normal levels of fasting and postprandial blood sugars and glycosylated hemoglobin levels. Ultrasound examination of the genitalia was performed to identify any possible hematoma but yielded no relevant results other than edema at the peno-scrotal junction of the first patient. Blood examination for bleeding, coagulation, prothrombin and partial thromboplastin times yielded normal results.
Urology
One patient of the phase II developed cough and dyspnea three weeks after radiochemotherapy. Radiographic pulmonary infiltrates according to an atypical pneumonia were diagnosed. These infiltrates were distributed symmetrically in both lobes of the lung. There was no accordance to the irradiation fields, making radiation induced pneumonia improbable. The patient died of progressive respiratory failure. Refusing post-mortem examination it was impossible to clarify the exact causes of pneumonia.
Oncology
21 years old female was referred as complete vaginal agenesis. She was examined and Rokitansky Kustner was diagnosed. She was operated under general anesthesia in october 2004. Full-thickness skin graft taken from inguinal region was used. The prosthesis was secured in place for 5 days after the operation and then it was gently removed for first look to the grafts. For the first postoperative three weeks, wound care was done with serum physiologic, furacine (nitrofurantione) cream and rifocine (rifampisine). Then the prosthesis kept in place for 7 months with cleansing it for one time in every week. The only problem encountered during postoperative course, a granulation tissue formation along with the graft suture lines and electrocautery was applicated for 2 times. Additionaly aloe-vera cream was used for wound care during the period of 2nd–7th postoperative months in which a complete epithelization was achieved. She has been sexually active without any problem . Hair growth problem in the newly created vaginal space was solved with an epilatory cream application.
Gynecology
30 year-old female was also referred for amenore. Gynecologic examination showed that vaginal agenesis was present and a rudimentary uterus was palpated with rectal examination. Ovaries were seen normal dimentions by ultrasounographic evaluation. A further Caryotype analysis showed 46XX and Mayer-Rokitansy-Küster-Hauser syndrome (mullerian agenesis) was diagnosed for the patient. She was operated at january of 2006 with the same technique. A split-thickness skin graft was used and a monoblock skin-graft was wrapped around the prosthesis and placed into the pouche which was dissected according to Mc Indoe thechnique. 4/0 chrome catgut (Dogsan, Turkey)was used for suturing the edges of grafts. The same wound care protocol was applied to the patient and no skin graft loss was seen. She was followed up for 5 months no complication was seen except a minimal granulation tissue formation. This was also managed with electrocautery. An vaginal depth of 11 × 3 cm was obtained and maintained in the patient. The patient is still under follow-up at early postoperative period and an ideal wound healing course was also noted .
Gynecology
All the mucinous cystadenomas were histopathological diagnoses. Three were suspected on gross examination by the pathologist and confirmed on sectioning. The remaining 3 showed the characteristic mucin-filled lumen on sectioning. Four mucinous cystadenomas were benign lesions and were fully resected. However, 2 showed severe dysplastic change and were later discussed at the multi-disciplinary team (MDT) meeting. One patient went on to have colonoscopy and caecal biopsies, which were negative for tumour. The other underwent CT abdomen and pelvis, which revealed no abnormalities. Both patients are currently being monitored as out-patients with regular colonoscopies in line with the colon cancer protocol.
Pathology
A 49-year-old African American female with a past medical history of hypertension, migraine headaches and mild intermittent asthma presented to emergency room with a severe right-sided headache for the last 3 days. The patient gave a 14-year history of migraine headaches described as a slow onset unilateral throbbing headaches without aura accompanied by nausea and lasting 2–3 days. The patient stated that her migraine attacks were decreasing in frequency and severity for the past 2–3 years until the past year when she started experiencing different kind of headache. At the time of examination, she described the headache as sharp, intermittent, non-radiating pain with sudden onset behind her right eye lasting 1–2 days. During the last 3 days the pain became more intense, reported as 10/10 on the pain scale 1 to 10, with associated photophobia, dizziness and nausea, with diplopia that was worsened with gaze to the right lateral field. Her past medical history was significant for hypertension and asthma which were stable. Her past surgical history is significant for adenoidectomy at age of 12. Her family history is significant for a sister with breast cancer, and a father who died of renal cancer. A complete review of systems was otherwise negative.
Neurology
On physical examination the patient displayed right lateral rectus muscle palsy (6 th cranial nerve palsy) with inward deviation of her right eye. There was numbness in all three divisions of the right trigeminal nerve, suggesting involvement of the fifth cranial nerve. There was no evidence of right 3 rd , 4 th , 7 th or 8 th nerve involvement. Motor and sensory examination of her extremities was normal. The rest of the examination was negative. The patient was admitted to the inpatient service. Her pain was controlled with medications. All laboratory studies, including complete blood count, biochemical studies and syphilis screening were negative. A Magnetic Resonance Imaging (MRI) of the brain and brain stem revealed a large mass in the clivus extending anteriorly, involving the nasopharynx and sphenoid sinus, and posteriorly destroying the clivus laying anterior to the pons. There was destruction of the pterygoid plate and right cavernous sinus . Neurology consult recommended starting the patient on phenytoin and dexamethasone for seizure prophylaxis. A pituitary profile was ordered which revealed a slight elevation of prolactin level 39.1 ng/ml (normal 20 ng/ml). On day number 2 of admission, neurosurgery and otolaryngology were consulted and the patient underwent sinus endoscopy with biopsy of the right sphenoid sinus. From days 3 to 6, the patient was stable, and pain was controlled. On day 6th pathology revealed a tumor with a cribriform pattern. The neoplastic cells were monotonous and intraluminal basophilic material was noted. No perineural invasion was identified. The neoplastic cells were strongly positive for cytokertain AE1/AE3 and positive for S-100 and CD117. This histology indicated ACC of the minor salivary glands. Oncology consultant recommended resection followed by radiation. Additional work up included a thin cut computerized tomography (CT), which revealed bone invasion. Chest, abdomen and pelvis CT scans were negative for metastatic disease. The patient underwent total gross surgical resection. A postoperative CT scan showed partial resection of the tumor and the patient was scheduled for a second surgery followed by postoperative radiation.
Neurology/Oncology
Our patient's course prior to diagnosis is unusual. The patient had a history of 14-year headaches which changed in character for the past year, and symptoms of acute 6 th and 5 th cranial nerve involvement. It is difficult to determine whether the 14-year history of headache was due to slowly growing ACC with gradual invasion of clivus, nasopharnx sphenoid, and cavernous sinus. It is possible that when the patient started experiencing "different" kind of headache was a warning sign of intracranial invasion of vital structures. This is likely due to the fact that ACC is a very slowly growing tumor , and the size of the patient's tumor was 3.8 × 3 × 2 cm. when discovered. In our case, direct invasion of cavernous sinus could explain 6 th and 5 th cranial involvement, as histopathology revealed no perineural invasion. Based on the literature review, there are only two other cases of intracranial ACC with 6 th nerve involvement. One case was reported 6 th nerve involvement due to perineural spread and second case was duo to invasion of the cavernous sinus .
Neurology
We report a case of ACC arising from the minor salivary glands and invading the clivus nasopharynx, sphenoid, and cavernous sinuses. Controversy exists regarding the most effective treatment of ACC and there is lack of reliable information about the clinical behavior of ACC in response to treatment. Further clinical trials needed to evaluate the effectiveness of treatment on improving quality of life and survival rates. This case is a reminder that careful monitoring of headache symptoms in patients is essential and a change in characteristics should prompt further investigations.
Oncology
During the initial course of management she was given 50 mg of ranitidine as a slow intravenous bolus for epigastric discomfort. Few minutes after the injection, the patient complained of itching at the injection site that spread to involve the entire upper limb. She also complained of swelling of her tongue and difficulty in breathing. Within minutes her level of consciousness deteriorated and she became comatose. The initial examination revealed the following features: GCS 6/15, a grossly oedematous face, neck and extremities, a grossly swollen tongue, congested conjunctivae, cyanosis, diffuse rhonchi over both lung fields.
Allergology/Anesthesiology
The anaphylactic reaction in our patient was due to ranitidine as she developed signs of anaphylaxis a few minutes after receiving the intravenous dose the drug. She was not given any other medication prior to ranitidine. The management was directed towards combating the severe anaphylactic reaction. All the cases reported in literature were treated along the same lines. No mechanisms have been identified for this adverse reaction. As the patient was known to be allergic to other medications, this may suggest that allergy to ranitidine may develop in patients with known multiple allergies.
Allergology
Ischemic Mitral regurgitation (IMR) continues to be a complex surgical problem. Choosing the most optimal therapy remains a challenging issue in cardiac surgery. There is wide variation in the clinical spectrum of IMR due to varying location and chronicity of ischemia and anomalies in annular and ventricular remodeling. As a result, there is lack of consensus in treating these patients. Treatment has to be individualized for each patient. Three-dimensional (3-D) echocardiography is a potentially valuable tool in gaining the mechanistic insights into the pathogenesis of IMR by precise evaluation of the complex three-dimensional interactions between the mitral valve apparatus and the left ventricle. Noting the exact geometric perturbations in each individual clinical setting will enable us in the rational design of the specific repair methods and in making the choice amongst the different techniques and devices that are currently available. We present a case of severe IMR in which ring annuloplasty combined with chordal cutting was planned based on the findings on 3-D echocardiography with a good result.
Cardiac Surgery
A 66-year-old African-American male with cardiomyopathy and IMR, presented to the emergency room with a 3 day history of breathlessness and lower extremity edema. Chest radiography displayed marked cardiomegaly. A 2-dimensional echocardiography demonstrated severe (3–4+) mitral regurgitation, moderately severe aortic valve insufficiency, tricuspid regurgitation (3+) with moderate pulmonary hypertension (right ventricular systolic pressure was 58 mm of Hg). The LV was markedly dilated with the end diastolic diameter of 6.7 cm and end systolic diameter of 6.0 cm. The ejection fraction was only 15% with severe global systolic and diastolic dysfunction of the LV. Cardiac catheterization showed triple vessel disease with severe diffuse stenosis of the right coronary artery, left coronary artery and first diagonal artery. Intra-operatively, transesophageal 3-D echocardiography (TEE) was performed by multiplane TEE probe using Sonos ® 7500 (Philips Medical Systems, N.A, Bothel, WA, USA). The 3-D processing was performed with 4-D Cardio-view ® 1.3 (TomTec Imaging Systems, Munich, Germany). The three-dimensional left ventricular model was generated. It produced a shell reconstruction of the LV allowing to calculate total and segmental left ventricular volumes during the cardiac cycle. The end systole frame was determined as frame with a closed aortic valve just prior to the opening of the mitral valve. This time point was used to make all valve measurements. Frame by frame analysis yielded the valvular area, circumference, commissure-to-commissure (c-c) diameter, antero-posterior (a-p) diameter and mitral annular volume (measured from annulus to cusp) . The 3-D reconstruction of the mitral annular volume showed tethering of the lateral scallop (P1) and medial scallop (P3) of the posterior leaflet of the mitral valve due to the secondary chordal attachments. There was an eccentric jet due to inadequate apposition of the mitral leaflets. .
Cardiology
Clinical examination and mammography of the right breast revealed a palpable, retroareolar lesion of diameter equal to 5 cm. In ultrasound, the consistency of the lesion was solid and multilobular. Additionally, mammography of the left breast demonstrated a newly developed, non-palpable lesion, consisting of clustered microcalcifications of diameter equal to 0.7 cm in the upper outer quadrant. Axillary lymph nodes of small size were detected on the mammogram in both sides.
Radiology
At the 20th month after surgery, the patient developed symptoms of cholecystitis (a first, sudden attack of biliary pain in the right upper abdomen, which resolved within 48 hours, but was followed by mild episodes of abdominal pain within the next month). Biochemical tests were within normal range (SGOT, SGPT, gamma-GT, ALP, conjugated and unconjugated bilirubin, LDH, CPK, amylase). Ultrasound examination and CT revealed the presence of gallstone disease and thus the patient underwent routine cholecystectomy. At that point, there was no indication of metastasis to the gallbladder.
Gastroenterology
To our knowledge, this is the first case of two synchronous breast carcinomas (lobular invasive and ductal invasive carcinoma), one of which metastasized to the gallbladder.
Oncology
The present case is extremely rare. Between the two synchronous breast carcinomas (lobular invasive and ductal invasive carcinoma), the lobular one metastasized to the gallbladder. This clinical observation is in line with larger series having demonstrated the preferential metastasis of lobular cancer to the gallbladder. Symptoms of cholecystitis in patient with a diagnosis of lobular carcinoma may indicate metastasis to the gallbladder.
Oncology
We here present a case of ipsilateral reexpansion pulmonary edema occurring after the insertion of a chest tube in a patient with spontaneous pneumothorax. The case is illustrated with unique radiological figures as well as a figure showing the course of the oxygen saturation in correlation to the oxygen administered over the initial 12 hours.
Pulmonology
A 25 year old male patient presented to our emergency department 24 hours after the onset of a sudden left-sided inspiratory chest pain without any further associated symptoms. His medical history was unremarkable except for a smoking history of nine pack years. The patient was in good general health with normal vital signs, including respiratory rate (16/min) and oxygen saturation (98% when breathing ambient air). Physical examination was normal except for diminished breath sounds on the left side, as were laboratory values. Chest X-ray confirmed the clinical suspicion of a left sided spontaneous pneumothorax with slight mediastinal shift as a possible sign of a beginning tension pneumothorax . A chest tube was inserted in local anesthesia in the left midaxillary line without complication. A negative pressure of 20 cm H2O was applied. Ninety minutes after the insertion of the chest tube with drainage and relief of symptoms, the patient complained of dyspnea and increasing pain in the left chest. Chest auscultation revealed left-sided inspiratory crackles. Oxygen saturation decreased to 85% despite the addition of oxygen (initially 2 l/min via nasal cannula). Later oxygen saturation stabilized at 90% with 12 l/min oxygen via a non-rebreather face mask. The arterial blood gas analysis under 12 l/min oxygen showed hypoxemia: pO2 7.9 kPA (>10.7 kPA), pCO2 5.5 kPA (5–5.5 kPA), pH 7.36 (7.38–7.42), bicarbonate 23 mmol/l (21–26 mmol/l) and oxygen saturation of 90%. A second chest X-ray demonstrated an expanded left lung, but also an ipsilateral pulmonary edema .
Pulmonology
The patient stabilized under continuous oxygen (12 l/min via a non-rebreather face mask) with his oxygen saturation steadily increasing. No other treatment was necessary. The patient was transferred to the medical floor where the negative pressure on the thoracic drainage was continued. The patient's further hourly clinical observation was unremarkable over the course of 12 hours . Finally, the patient was in a good condition without requiring any additional oxygen. The chest drainage was removed when no more air leakage was detected on day 2 of hospitalization. A third chest X-ray 12 hours after the removal of the chest drainage showed a completely reexpanded left lung and a decreased pulmonary edema .
Pulmonology
We report a case of a REPE after the insertion of a chest tube for a spontaneous pneumothorax. Our patient suffered from this rare complication but showed a benign clinical course. He was successfully treated with the sole administration of oxygen via a non-rebreather face mask. His oxygen saturation improved steadily and normalized after 12 hours. Usually REPE is self limited and can even be asymptomatic . However, a mortality rate as high as 20% has been described . This rare complication and the treatment of REPE should be known by clinicians and particularly considered when a patient's condition declines after initial amelioration . Treatment is supportive, mainly consisting of the administration of supplemental oxygen and morphine if needed . Occasionally, non-invasive continuous positive pressure ventilation or even mechanical ventilation are required to provide adequate oxygenation . In severe cases – i.e. failure of mechanical ventilation – differential lung ventilation might be a treatment alternative .
Pulmonology
We describe a 21-year-old patient with BPI who received a vascularized free gracilis muscle transfer innervated by a part of the vascularized ulnar nerve connecting CC7 to the affected median nerve. This successfully restored wrist motion and sensation to the palm.
Neurosurgery
A 21-year-old man sustained contusion injuries to both lungs and the brain in a motorcycle accident. He was transferred to a hospital, where he was treated for the lung and brain injuries. As his consciousness recovered, his left arm was found to be completely paralyzed. He was diagnosed as having a total left BPI, and underwent surgery for this three months after the injury. During surgery, the left third, fourth and fifth intercostal nerves were transferred to the left musculocutaneous nerve , the sixth and seventh intercostal nerves to the left thoracodorsal nerve, and the left accessory nerve to the left suprascapular nerve .
Neurosurgery
Twelve months after the BPI surgery performed in the previous hospital, he visited our clinic complaining of complete loss of sensation and motor function distal to the left elbow joint. Reasonable recovery of the shoulder and elbow joints was seen from a physical examination. The left elbow flexion range was 100° against gravity and the abduction and flexion ranges of the left shoulder against gravity were 80° and 85°, respectively. However, the elbow extension was M0 according to the Medical Research Council Scale (MRCS) and the range of left shoulder extension was 15°, which was mostly achieved by motion of the left scapula. No active motion was observed in the left wrist and fingers. The patient demonstrated complete numbness in the area innervated by the left C5-T1 nerve roots. He had slight stiffness in the MP joints of the left index finger to the little finger, and started rehabilitation exercises to soften the joints. No other joints of his left upper extremity showed restriction of the range of motion in the passive movement. Elbow flexion was recovered to M4 level according to MRCS at 15 months after surgery thanks to muscle-strengthening exercises.
Neurosurgery/Orthopedic surgery
Eighteen months after the injury, he underwent reconstructive surgery to his left wrist and hand. As the first step of the reconstruction, the CC7 was transferred to the median nerve through a vascularized ulnar nerve taken from the affected arm. The left ulnar nerve was elevated based on the superior ulnar collateral vessels, including the ulnar vessels distally. A monitor skin flap (2.5 × 1 cm) supplied by perforators arising from the ulnar vessels was also harvested from the distal third of the forearm together with the ulnar nerve. The ulnar nerve and vessels were sectioned at the wrist level distally. The ulnar nerve was also sectioned just proximal to the insertion of the superior ulnar collateral vessels into the ulnar nerve in the upper arm level proximally . The median nerve was sectioned at the same level of the proximal section of the ulnar nerve. The proximal stump of the transected ulnar nerve segment was approximated to the distal stump of the median nerve. The distal stump of the ulnar nerve segment including the ulnar vessels was transferred to the right neck through a subcutaneous tunnel and approximated the posterosuperior portion of the right seventh cervical nerve root as described by Songcharoen et al. . The distal stump of the ulnar artery accompanying the transplanted ulnar nerve segment was approximated to the transverse cervical artery in the right neck followed by ligation of the distal stumps of the ulnar veins. After surgery, the monitor flap survived completely. The patient showed transient paresthesia in the tips of right index and middle fingers, which lasted for two months. Grip strength was not affected. The sensory recovery of the affected limb was rapid, and touch sensation was restored to the midpalm level of the affected hand by four months after the CC7 transfer.
Neurosurgery
Postoperative recovery was uneventful. After the muscle graft operation, the patient's sensation in the left palm was not compromised and showed continuous further recovery, even though almost 10% of the nerve fibers had been taken to innervate the transplanted muscle. Contraction of the transplanted muscle was observed eight months after the transplantation. Fifteen months after the muscle graft, the dorsiflexion of the left wrist was 35° against gravity with the elbow joint held fully extended (approximately at a 30° -flexion position) when the patient applied forces to the flexor muscles of his right wrist and ulnar fingers (mainly, right and little fingers). His right fist formation and slight palmar wrist flexion was synchronously sccompanied by the dorsiflexion of his left wrist . The left elbow joint was restricted in extension after the muscle transplantation; the active range of the joint was -30° in extension and 130° in flexion. The flexion force of the joint was augmented by the muscle transplantation and recovered to an M5 level. Recovery of touch sensation advanced to the base of the index and middle fingers and thumb. The sensation in the left palm was S2 on the MRCS. When the tips of the fingers and thumb were touched or scrubbed, he did not recognized it as a sensation of his left digits but as a weak tingling pain at his right middle fingertip. The sign of motor recovery of the left median nerve has not been observed yet.
Neurosurgery
This patient with BPI involving the total brachial plexus had undergone intercostal nerve transfer to the musculocutaneous nerve and a spinal accessory nerve transfer to the suprascapular nerve in a previous hospital. We restored sensation of the palm and motion of the wrist to the patient by transplantation of a vascularized ulnar nerve between the CC7 and the median nerve and a free vascularized gracilis muscle innervated by a part of the transplanted vascularized ulnar nerve. Nineteen months after our first operation, the affected wrist was flexed dorsally by the transplanted muscle innervated by a part of the vascularized nerve connecting to the CC7 and touch sensation recovered up to the base of each fingers.
Neurosurgery
Leiomyosarcoma is a soft-tissue tumor that differentiates from smooth muscle. Primary leiomyosarcomas of vascular origin are relatively rare and frequently arise within the IVC . Rarely, leiomyosarcomas may arise from the renal or adrenal vein. We report the case of a leiomyosarcoma of the adrenal vein and a novel approach to surgical resection involving the use of deep hypothermic circulatory arrest (DHCA).
Oncology
A 64-year old woman who presented with a 9-month history of persistent, non-productive cough, bilateral lower extremity edema, and multiple spider angiomata extending from umbilicus to ankles. The patient was otherwise healthy with no significant past medical, surgical, or family history. She denied recent weight loss, fevers, chills, nausea, vomiting, abdominal or back pain, and she had no difficulty with ambulation. Physical exam also was significant for hepatomegaly extending 4 fingerbreadths below the costal margin. There was no jaundice or virilization. Routine laboratory studies were unremarkable. Computed tomography (CT) scan revealed a 13 × 6.5 × 6.6 cm heterogeneous mass arising in the region of the right adrenal gland and extending into the IVC and the right atrium .
Endocrinology/Oncology
The differential diagnosis included adrenocortical carcinoma, renal cell carcinoma, retroperitoneal sarcoma, or hepatoma. The patient was brought to the operating room for a planned adrenalectomy, possible right nephrectomy, cholecystectomy (for cholelithiasis), and tumor resection via IVC cavotomy under DHCA. Diagnostic laparoscopy revealed no evidence of peritoneal or hepatic metastases. Exploratory laparotomy was performed through a midline incision, followed by a Köcher maneuver. The adrenal lesion was noted to be separate from the right kidney. Intraoperative ultrasound confirmed that the tumor did not involve the caudate lobe of the liver.
Oncology and Surgery
A median sternotomy was performed, and the diaphragm was divided in the midline anteriorly. The adrenal gland was mobilized from the liver, and the IVC was fully exposed. The patient was then placed on cardiopulmonary bypass with DHCA, and her core body temperature was lowered to 19°C. The IVC was incised, and intramural tumor was dissected free from the walls of the IVC and excised en bloc with the adrenal gland . There was no evidence of residual disease within the IVC or right atrium on transesophageal echocardiogram (TEE). After completion of the IVC cavotomy, the patient was taken off DHCA and re-warmed. Total circulatory arrest time was 14 minutes, with a bypass time of 115 minutes. The aorta was cross-clamped for 25 minutes. She required 6 units of packed red blood cells, 4 units of fresh frozen plasma, 4 units of platelets, and 5 liters of crystalloid. The total procedure time was 7 1/2 hours.
Cardiothoracic surgery
The patient required pharmacologic cardiovascular support for the first 12 hours postoperatively, but was awake within 24 hours. She was extubated on the morning of first postoperated day and displayed no neurocognitive deficit. The balance of her postoperative course was complicated by the development of a symptomatic pulmonary embolus, diagnosed by spiral CT of the chest. An ultrasound of the IVC also was performed to exclude tumor embolus; no evidence of thrombus was seen. She was discharged on 12th postoperative day on oral anticoagulation. Pursuant PET-CT showed no evidence of uptake in the lungs, providing further confirmation that this was a pulmonary embolus, and not tumor thrombus.
Cardiology
To our knowledge, this is the first reported case of the use of DHCA for the resection of an adrenal vein leiomyosarcoma. The use of DHCA for resection of other retroperitoneal tumors, including renal cell carcinomas and adrenocortical carcinomas, has previously been reported . Total circulatory arrest is only required for the critical period of tumor removal from the IVC and right atrium. Neurological sequelae of DHCA can be transient (i.e. post-operative confusion, delirium and agitation) or permanent, secondary to postoperative stroke and resulting in gross dysfunction; the upper limit of DHCA for brain protection is 40 minutes . Our patient did not immediately exhibit any of these symptoms, but she developed minor disturbances in gait and difficulty with slurring of her speech six weeks postoperative. It is unclear if DHCA was related to her symptoms; CT and MRI of the head were unremarkable.
Cardiothoracic surgery
Literature on amyloid angiopathy globally is scanty and specific literature on the involvement of the submandibular salivary gland and the floor of the mouth is even rarer. It is the purpose of this communication to report a case of amyloid angiopathy involving the submandibular salivary gland and the floor of the mouth, the first report of its kind from the West African Sub-region. The problems with diagnosis and management are highlighted.
Pathology
A 56 year old retired male civil servant who had been referred from the surgical outpatient department with a Fine Needle Aspiration Cytology (FNAC) report of benign salivary gland tumour, presented to our ENT department in the same institution, with a one year history of gradual but progressive bilateral swellings of the submandibular region. There was no associated variation in size with feeding. It was initially painless but the patient had developed some burning sensation involving the head and neck region. There were no associated purulent discharge, halitosis, dryness in or of the mouth or eyes and no upper lid swelling. About the same time, he noticed progressive tongue enlargement that almost filled the oral cavity and resulted in affectation of his speech. There were no other laryngologic, rhinologic or otologic symptoms. His appetite was good with no weight loss or abdominal discomfort. He had no symptoms referable to the musculoskeletal system.
Otolaryngology
Physical examination revealed an anxious ill-looking middle-aged man with an open mouth posture and" hot potato" speech. In addition, he had thickened skin of the head and neck region and massive macroglossia obscuring the visualization of the oropharynx. The tongue appeared depapillated but moist and pink. There was bilateral submandibular salivary gland enlargement, each measuring 4 by 3 cm, bosselated, firm to hard in consistency and attached to skin. There was no lacrimal gland swelling or slanting of the palpebral fissures.
Endocrinology
Investigations done included: Full blood count with PCV-38%, WBC-6,400/cmm; neutrophil-42% (low), lymphocytes-46% (slightly above normal), monocytes-7% (normal), platelets-123,000/cmm (low). Electrolytes, Urea and Creatinine were within normal limits while urinalysis, was normal. HIV 1 and 2 screening by the rapid kit method was non-reactive. An Ultrasound scan (USS) of the neck revealed a normal thyroid gland and diffuse enlargement of the submandibular glands devoid of stones, cysts, or lymph node enlargement.
Hematology
He had an open biopsy of the submandibular gland in which a 2.0 by 0.5 centimeter wedge biopsy was taken from the left submandibular gland. This provoked a torrential bleed giving the impression of a major arterial injury but careful search revealed no identifiable blood vessel and the idea of external carotid artery ligation was jettisoned after having finally secured haemostasis by mass suturing (tissue was friable). Pressure dressing was also applied. About 250–500 mls of blood was lost and patient was subsequently admitted for observation.
Surgery
He was then planned for a CT-angiography while an Interventional Radiologist was consulted for a possible selective embolisation; the facility however was not available. Independent reviews by the Surgical Oncologist and the Cardiovascular Surgeon were not suggestive of the likelihood of a named vascular structure at the region in question. However the swelling had become massive almost compromising the airway, making neck exploration hazardous. Preliminary histology of the initial specimen revealed chronic inflammation with presence of thick walled vessels suspicious of amyloid angiopathy for which special stain with Congo red was done.
Radiology and Vascular Surgery
A 57-years-old male patient was admitted to the general surgery department with abdominal distention, weight loss and hematuria. During physical examination, significant distention and multiple palpable tumor masses were identified on the abdomen . Contrast-enhanced abdominal multi detector CT imaging was performed with a four-detector row CT scanner (Somatom Sensation 4 ® , Siemens Medical Solutions, Erlangen, Germany). Multiple, well-defined, soft tissue masses with homogenous pattern, measuring maximum 6 × 5 cm in size were observed in the mesenteric and retroperitoneal areas . In some masses, central necrosis and coarse calcifications were seen . There was grade 3 hydronephrosis in the left kidney due to the left ureteric invasion . However, CT did not reveal liver metastasis and/or the lymph nodes with pathological size.
Radiology
Ultrasonography (US)-guided true-cut ® biopsy was made, histopathologic and immunohistochemical analyses demonstrated stromal tumor which, C-KIT (+), CD34 (-), S-100 (-), desmin (-), actine (-) Ki-67 (+) and with a proliferation index of <%1. During the operation, not only the tumor filling the whole abdomen but masses invading small bowel mesentery, left hemicolon and the left ureter have been detected. The patient underwent left ureterectomy, left nephrectomy and total colectomy. Proximally from the treitz, small bowel resection from a distance of 200 cm was applied. The mass have been totally removed and taken out of the abdomen . The operation has been completed with an end-ileostomy .
Surgery
A 58-year-old woman presented with a one-day history of malena and light-headedness. She did not have any known co morbidities. Apart from being anaemic she was haemodynamically stable and her general physical and abdominal examination unremarkable. Hematological examination revealed low hemoglobin levels of 7.3 g/dl with normal clotting profile. After initial resuscitation, an esophagogastroduodenoscopy (OGD) was performed which excluded any cause of upper gastrointestinal bleed. This was followed by colonoscopy, which although did confirm the presence of blood within the gastrointestinal tract but did not localize the site of bleeding. However, the patient's hemoglobin level continued to fall over the next two days, which was treated with a transfusion of six units of packed red cell.
Gastroenterology
Subsequently, she underwent selective superior mesenteric angiography, which demonstrated a large hyper vascular mass with an extensive contrast blush, near the terminal ileum in the right iliac fossa . It was supplied by a large feeding artery and drained via a solitary large vein into the portal venous system . No extravasation of contrast was seen suggesting that the lesion was not actively bleeding at the time of the study. Inferior mesenteric and coeliac axis angiography revealed no synchronous lesion or evidence of any vascular deposit in the liver.
Radiology
She thereafter underwent laparotomy through midline incision, which identified vascular solid tumor along the anti-mesenteric border of the ileum about 30 cm from the ileo-caecal junction, measuring 7 × 7 cm . A segmental small bowel resection was performed, and intestinal continuity established with an end-to-end small bowel anastomosis. The patient had an unremarkable postoperative recovery; her hematocrit remained stable and she was discharged from the hospital on the 7 th postoperative day.
Surgery
Arteriovenous malformations (AVMs) occur mainly in the central nervous system, although they have been described in other organs as well. AVMs rarely involve the testis or the scrotal components, presenting mainly in the form of para-testicular or intra-testicular masses. This case reveals an uncommon clinical presentation of a non-palpable AVM of the spermatic cord. To our knowledge we present the first reported case of an arteriovenous malformation of the spermatic cord being the cause of recurrent acute scrotal pain.
Urology
Four months later the patient presented with the same intractable right scrotal pain. Clinical examination and ultrasound investigation were insignificant. Orchiectomy was suggested this time and was performed through an inguinal incision after obtaining patient's consent. The surgical specimen consisted of a 16 ml testicle with its spermatic cord structures. The testis and spermatic cord were macroscopically unremarkable. Detailed pathology examination of the testicle and vas deferens revealed insignificant pathological changes, except for a small, smooth vascular mass (0.5 cm maximum diameter) in the lower part of the spermatic cord with the characteristics of an arteriovenous malformation . The patient was uneventfully discharged the following day. The patient has not experienced another episode of scrotal pain during the last 2 years.
Urology
The case presented highlights a rare and unique cause of recurrent acute scrotal pain, attributed to the presence of an arteriovenous malformation of the spermatic cord. Therefore there is reason to believe that AVMs of the scrotum should be considered in the aetiology of otherwise inexplicable, recurrent scrotal pain. In that case consideration should be given to a trial of superselective angio-embolization of the lesion before one resorts to orchiectomy.
Urology
A 27 year old female presented with a one year history of progressively worsening epigastric pain, reflux, and fatigue. She was initially treated with acid suppression therapy by her primary care physician, which temporarily relieved her symptoms. However, her symptoms became refractory to medication and she noted the onset of dysphagia. She reported a remote history of bulimia nervosa (BN) of approximately one year duration at the age of 17. She reported episodes of binge eating and self-induced vomiting, at least once a day. She denied any further history of bulimia since that time, which was corroborated by her mother. The patient reported smoking approximately 10 cigarettes per day since the age of 20, and had recently quit. She denied alcohol and drug abuse, and had no family history of malignancy.
Gastroenterology