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She was admitted to the ICU in a state of shock and anuria, receiving high doses of inotropic drugs and antibiotic treatment with ceftriaxone, gentamycin and metronidazole. She was also put under full anticoagulant therapy. A new transoesophageal echocardiogram in the 7 th post-operative day disclosed recurrence of thrombus remnants in the left atrial appendage. After 10 days she was weaned of the inotropic drugs and the veno-venous hemofiltration, and 15 days later she was also weaned of the ventilator. The patient during this period was afebrile. After 3 days of spontaneous breathing she had to be re-intubated because of progressive respiratory insufficiency due to intervening further neurological deterioration and fever reappearance. Lumbar puncture revealed findings consistent with a central nervous system infection, probably due to septic emboli. Antibiotic treatment was then switched to meropenem, but it was unsuccessful. The patient died 5 days later due to brain stem herniation.
Intensive Care Medicine
The case report presented is a representative example of a neglected and undertreated patient in refers to both surgical and medical aspects of her disease with extreme complications. In order to avoid such references in the future anticoagulant therapy must be used according to the currently recommended guidelines and standards and surgery must be considered in the setting of mitral valve disease complicated by systemic thromboemolism. In the presence of left atrial thrombus surgery should be offered in all patients with organized thrombus or suspicion of a tumor or an infected mass.
Cardiology
The proband’s grandfather, at 69 years of age, was examined and diagnosed with LCD. His symptoms initiated with sensitivity to sunlight, episodes of ocular pain, and redness at 42 years of age. Slit-lamp examination revealed irregularity of the epithelial surface with subepithelial and anterior stromal scarring resulting in diffuse clouding of the central cornea . Asymmetric commitment and vascularization of the cornea was observed. He was classified in maximal severity according to best-corrected vision (0.13 for both eyes) and corneal commitment. Typical, fine branching lattice lines could be seen in the peripheral anterior stroma, which was outside the area of central opacification. Central corneal sensation was significantly decreased, and the patient had a history of recurrent corneal erosions in the fourth and fifth decades of life.
Ophthalmology
The symptoms of the proband’s aunt began with episodes of acute ocular pain, redness, and photophobia at 30 years of age. The frequency and severity of these episodes increased coincident with a gradual deterioration of vision in both eyes. At 42 years of age, she was classified in maximal severity according to best-corrected vision (0.28 in the right eye [OD] and 0.13 in the left eye [OS]) and corneal commitment. Slit-lamp examination revealed an irregular epithelial surface with subepithelial and anterior stromal scarring, resulting in diffuse clouding of the central cornea. She showed a network of linear opacities associated with other smaller opaque spots and refractive lattice lines . No vascularization of the cornea was observed. Central corneal sensation was decreased.
Ophthalmology
The proband’s mother, at 40 years of age, initially presented a mild subepithelial scarring and opacification, which gradually progressed to include elevated subepithelial opacities, fine lattice lines, diffuse “ground glass” haze in the anterior stroma, and corneal grafts . Best-corrected vision was 0.5 OD and 0.4 OS. The left cornea contained opacifications from presumed recurrent disease. Fine branching lattice lines could be seen in the peripheral anterior stroma.
Ophthalmology
The proband had a history of recurrent corneal erosions in both eyes, which began when he was four years old. Slit lamp examination showed the presence of large, typical fine branching lattice lines in the anterior stroma in OD . A clear area was preserved around the corneal-scleral limbus. Best-corrected vision was 0.5 in the right eye (OD) and 0.67 in the left eye (OS).
Ophthalmology
Two patients died at dose level III, in both cases the deaths were not thought to be treatment-related. The first patient had been anticoagulated for a recently diagnosed proximal deep vein thrombosis. She collapsed suddenly 5 days after cycle one and a diagnosis of pulmonary embolism was made. The second patient died 15 days after cycle six and although a clinical diagnosis of pneumonia was made by the general practitioner, on further investigation the mode of death was more consistent with pulmonary embolism.
Hematology/Oncology
A Suffolk wether developed a head tremor at rest. This sheep had a stiff, mildly ataxic gait and responded to scratching of the dorsum with lip tremor and head tilt (inconclusive scratch test). Haematology and blood biochemistry were unremarkable. Degenerative osteochondritis of the femoro-tibial joints and acute oedema of the brain suggestive of enterotoxaemia following clostridial replication was diagnosed, although there was no clinical and pathological evidence of enteritis.
Veterinary pathology
A 30-year-old man with history of AD presented to the accident and emergency department of our hospital with fever and generalized skin rash. He complained of malaise and poor appetite for a week. He had a brief period of confusion on the day prior to admission. He also suffered from bronchial asthma that was well controlled with bronchodilator inhalers. There was no previous history of heart disease or rheumatic fever. He worked as an engineer in a plastic factory. He denied smoking or intravenous drug use. He had extremely dry skin with lichenifications affecting almost all areas of his body but worse on the elbows and knees for which he was applying moisturizers and 1% hydrocortisone cream. The exacerbations of eczema were treated with 2.5% hydrocortisone cream and varying dose of prednisolone tablets.
Dermatology
On examination, the patient was drowsy; temperature was 38°C, pulse rate 110/min and blood pressure 144/88 mm Hg. He had a generalized erythematous macular non-blanching skin rash and small purpuric haemorrhages over the palate. Systemic examination revealed a pansystolic murmur in the mitral area, splenomegaly and weakness of the right leg (power 3/5). Both plantar reflexes were extensor and sustained ankle clonus was present. There were no signs of meningeal irritation, cerebellar dysfunction or sensory deficit and fundoscopic examination was normal.
Cardiology
Laboratory investigations revealed haemoglobin 15.7 g/dl, WBC 14.5 × 10 9 /l, platelets 50 × 10 9 /l, ESR 19 mm/hour, C-reactive protein 292.9 mg/l, serum sodium 128 mmol/l, serum potassium 3.3 mmol/l, bicarbonate 27 mmol/l, blood urea 7.5 mmol/l and serum creatinine 207 μmol/l (patient's baseline – 118 μmol/l). Urine dipstick was positive for blood (++) and protein (+). Chest X-ray was normal. 12 lead ECG showed sinus tachycardia with features of left ventricular hypertrophy. Transthoracic echocardiogram showed mitral valve vegetation with severe mitral regurgitation and normal ejection fraction. CT scan of the brain was normal but MRI identified multiple areas of white matter abnormality suggestive of embolism around the periventricular area. Two sets of blood cultures grew Staphylococcus aureus sensitive to gentamicin and flucloxacillin.
Cardiology
The patient was commenced on intravenous gentamicin and flucloxacillin and later teicoplanin. After 48 hours he became afebrile; the rashes started to fade and his right leg weakness improved. CRP and ESR gradually reduced. Three weeks later, he had a recurrence of fever with elevation of CRP and ESR. Blood culture at that time showed no growth but his central venous line tip grew a coagulase-negative staphylococcus sensitive to rifampicin, vancomycin and netilmycin. His condition improved with removal of the central line and addition of rifampicin. The treatment was continued with teicoplanin for 6 weeks and rifampicin for 4 weeks. He was discharged 5 weeks later on treatment with ramipril and frusemide in view of significant mitral regurgitation.
Infectious diseases
Three months later, he underwent transoesophageal echocardiogram which showed perforation of the posterior mitral cusp with significant mitral regurgitation . He underwent successful mitral valve replacement 5 months following the presenting episode of infective endocarditis and was commenced on warfarin. Though he had an episode of eczema herpeticum two years following the infective endocarditis, he continues to do well.
Cardiology
This case report lends support to the association of infective endocarditis due to S. aureus and AD. In native valve endocarditis, S. aureus accounts for 30–35% of cases, whereas in a patient with atopic dermatitis, it is found to be exclusively due to S. aureus . This could be the result of frequent staphylococcal bacteremia in patients with AD.
Infectious diseases
The other patient who had a CR was a 43-year-old woman who had no history of cirrhosis, alcohol abuse or hepatitis virus infection. She had previously undergone a partial hepatectomy for a highly differentiated HCC. The patient had three recurrent tumours at inclusion into the study, the largest of which was 35 × 50 mm. Complete response was first observed after 103 weeks of treatment. Alfa foeto-protein was high initially, decreasing gradually during treatment, and eventually ended within normal limits. When last seen, the patient had been on treatment for 36 months and still had a CR. The dose was between 15 and 20 μ g day –1 during the first 50 weeks, thereafter 10 μ g day –1 (disregarding short treatment pauses because of hypercalcaemia). Tissue was not available to determine VDR expression in this patient's tumour.
Oncology
During infancy, the child had undergone repair of an interrupted aortic arch and ventricular septal defect [VSD] closure as two staged procedures. Following the second operation, the child developed a complete heart block for which an adult epicardial pacemaker system (Sigma SSR 203, Medtronic, Inc. Minneapolis, USA) was implanted with the pulse generator in the epigastric region.
Cardiology
A seven-year-old girl presented with colicky abdominal pain of six days duration with no signs of localization or intestinal obstruction. She was afebrile with a normal white cell count. An electrocardiogram showed AV dissociation with a ventricular rate of 60 beats per minute. There were pacing spikes of 7 mm on the electrocardiogram ECG] with no relation to the QRS complexes. An antero-posterior abdominal X-ray showed a metallic shadow in the pelvic cavity and chest X-ray revealed fractured leads close to the heart leading to a diagnosis of a migrated pacemaker.
Cardiology
This child was delivered at 32 weeks of gestational age by an emergency cesarean section. Electrocardiogram showed a congenital complete AV block with a ventricular rate of 60 per minute. In the immediate neonatal period, patent ductus arteriosus [PDA], which was detected by transthoracic echocardiography, was ligated and an epicardial pacemaker system [Prodigy, SR 8160, VVI, Medtronic, Inc, Minneapolis, USA] with a pulse rate of 100 per minute was implanted. After 5 years the battery expired and the pacemaker was replaced with an adult sized rate responsive VVI pacemaker [Sigma SSR 203, Medtronic, Inc. Minneapolis, USA] that was set to discharge at a pulse rate of 100 per minute. This was implanted in the epigastric space behind the rectus abdominis muscle.
Cardiology
Under general anesthesia, a left anterolateral thoracotomy was done and a new VVI pacemaker [Microny II SR + Model 2525T St Jude Medical, Inc. Sylmar, CA, USA] with epicardial leads, programmed to deliver 100 beats per minute, was implanted in the left infraclavicular area superficial to the pectoralis major muscle. The migrated pacemaker and its fractured wires were retrieved by laparotomy. The postoperative course was smooth and child was discharged five days later.
Cardiology
A 2-year-old girl presented with complaints of diarrhea, vague abdominal pain, low-grade fever of one-week duration and history of passage of a foreign body per rectum. When this child was 8 months old, a permanent bipolar cardiac pacemaker [Prodigy, VVI, Medtronic, Inc, Minneapolis, USA] was inserted in an epigastric pocket below the rectus abdominis as she had developed complete heart block following repair of an atrioventricular septal defect.
Cardiology
In this child, the pacemaker generator was found extruded through the rectum but hanging by the pacing wires and was functional. There were no clinical or radiological signs of bowel perforation. Under antibiotic cover, a new epicardial pacemaker system was inserted through a left anterolateral thoracotomy and the pacemaker generator was hitched to the rib with non absorbable sutures to prevent migration. The old pacing wires were cut and were pulled out from the rectum. The postoperative course was uneventful and the child was discharged two days later.
Cardiology
The patient, a 50-year-old Asian man diagnosed with paranoid schizophrenia when in his late twenties, had been treated with a variety of first generation antipsychotics for several years. Whilst an inpatient at age 42 years, he had been prescribed SGAS. He was medically fit, apart from suffering from hypertension controlled with perindropril 4 mg/day. He was discharged once clinically stable on olanzapine 20 mg/day. His records revealed that at age 48 years, whilst on 20 mg olanzapine, his random glucose levels were 17.6 and his glycosylated haemoglobin was 10.9%. He was started on oral hypoglycaemic agents by his general practitioner, a combination of gliclazide MR 30 mg/day and metformin 500 mg twice daily, which was later increased to 1 g twice daily because of poor response.
Psychiatry
The patient was re-admitted to hospital 7 months later owing to poor compliance with medication and exacerbation of his psychiatric symptoms. Whilst in the community, his general practitioner restarted his diabetic medication (gliclazide 30 mg/day and metformin 500 mg twice daily) and combined it with a lipid-regulating drug (simvastatin 20 mg/day) and antihypertensive treatment (amlodipine 10 mg/day and perindropril 4 mg/day). On admission, his fasting glucose levels were between 3.7 and 6 mmol/l and he was overweight with a body mass index (BMI) of 27. Risperidone was added to the medication regime reaching a dose of 6 mg/day. His fasting glucose levels were between 4.2 mmol/l and 6 mmol/l. After 3 months, despite controlling his psychiatric symptoms, risperidone use resulted in intolerable side effects in the form of urinary incontinence, therefore it was discontinued and aripiprazole restarted reaching a dose of 15 mg daily. Six weeks later, the patient presented with symptomatic episodes of hypoglycaemia with fasting glucose levels between 2.1 and 3 mmol/l with no changes in body composition or other metabolic parameters. In consultation with his diabetologist, the oral hypoglycaemic medication was discontinued. The patient remained physically well with fasting glucose levels within normal range (3.5 to 6 mmol/l) during the 6 months that he remained in the unit.
Psychopharmacology
Our patient, who has diabetes treated with oral hypoglycaemic agents, experienced changes in body composition such as gradual weight loss and reversal of metabolic parameters towards normal levels associated with hypoglycaemia when the antipsychotic medication he was on was switched to aripiprazole for the first time. When his antipsychotic medication was changed to aripiprazole once again ten months later, following the discontinuation of risperidone owing to side effects, a second episode of hypoglycaemia occurred. On both occasions this led to review of his antidiabetic treatment. The hypoglycaemic episode could be explained by the discontinuation of olanzapine and/or risperidone as described in the literature or the combination of oral hypoglycaemic medication with aripiprazole. However, it is relevant to note that the patient did not require any antidiabetic treatment whilst on aripiprazole during the follow up period.
Endocrinology
Gastro-Intestinal stromal tumors (GISTs) are rare, and our understanding of their natural history and optimal treatment are continually evolving. This case report of a port site metastasis following laparoscopy for a malignant GIST is, to our knowledge, only the second documented case. As well as an outline of the case itself, the question as to why these arise and what the optimal treatment should be is also discussed.
Oncology
She is urgently referred for further investigation. Computed Tomography (CT) scanning demonstrates a 13 cm multi-loculated mass adjacent to the anterior wall of the stomach radiologically suspicious for a Gastro-intestinal stromal tumour (GIST). A subsequent PET-CT scan also demonstrated multiple peritoneal seedlings . Upper Gastro-intestinal Endoscopy showed extrinsic compression of anterior gastric wall, with possible communication to a pus filled cavity. Unfortunately, multiple biopsies failed to yield a histological diagnosis.
Gastroenterology
At diagnostic laparoscopy (performed to obtain tissue diagnosis and assess potential tumour stage), the large mass anterior to the stomach was biopsied as were the peritoneal seedlings. Histology demonstrated a malignant GIST, strongly positive for CD117. She was discussed at the regional Upper GI Multi-Disciplinary meeting, and the decision made to commence systemic oncological therapy with Glivec (Imatinib).
Oncology
In this particular case, laparoscopy (and hence gas insufflation) was required because of failure of alternative techniques to establish the diagnosis. The tumour was biopsied and the biopsy instrument removed through the port (in theory protecting the specimen from contacting the layers of the abdominal wall), although the port itself then required removal. The advanced nature of the tumour also increased the risk of a PSM but as far as we know, the port did not enter the peritoneal cavity at the site of an existing peritoneal metastasis.
Oncology
Three patients died during the trial: one patient developed mucositis during the first course of chemotherapy and underwent radiation therapy involving the already inflamed oral mucosa. The event was classified as probably related to trial treatment. The patient was admitted to a regional hospital with sepsis, suspected to be related to the injured oropharyngeal mucosa. No agranulocytosis was found. The patient's clinical condition rapidly deteriorated and he died due to a presumed septic shock. The second patient died during the second course after somnolence progressed into coma with a clinical diagnosis of brain metastases or cerebral infarction. Due to the poor clinical status at hospital admission, no further work-up had been done. The third patient died during the first course due to treatment-related toxicity. This 60-year-old obese patient with extensive osseous and hepatic metastases was treated with 5 g of capecitabine daily. He developed mucositis and diarrhoea and was then admitted to the hospital 10 days after starting chemotherapy. Administration of capecitabine was stopped. He was neutropenic and was treated with antibiotics. He succumbed to infection on the tenth day of hospitalisation.
Oncology
The expected post-treatment pain and swelling subsided in 2–4 weeks. There was no treatment-related airway obstruction. The only major treatment-related complication was a carotid blow out 2 weeks after PDT in a 33-year-old woman with recurrent neck disease. On an MR scan taken 1 month prior to IPDT, the tumour was judged to be close to, but not involving the carotid artery. Post-mortem examination showed malignant cells along the intima of the carotid artery implying tumour invasion. Skin photosensitivity was noted in one patient who failed to comply with the recommended regime for ambient light exposure.
Oncology
A young man of Asian origin first presented at the age of 8 years with left knee pain. Initial treatment with simple analgesics failed and he presented again with ongoing knee pain that also involved the right knee. On investigation, no evidence of inflammatory arthropathy was found. A skeletal survey and bone scan were reviewed by a paediatric radiologist with expertise in children's skeletal dysplasias, who diagnosed an unusual variant of multiple epiphyseal dysplasia with relative sparing of the hips and severe changes in the knees, hands and feet. Radiographic changes in the knee joint were subtle and included genu valgum, osteoarthritic change and tibial plateau depression. There were no known affected family members and genetic testing was not carried out.
Orthopedics
By the age of 16 years, the symptoms had worsened and included pain, stiffness, fixed flexion deformity of 10°, reduced range of movement with flexion restricted to 90° and subjective instability in both knees but mainly the left, as well as intermittent pain in both hips. At 18 years of age, plain imaging found no abnormality in either hip joint with both capital femoral epiphyses fused. Radiographs of the left knee at this time showed a slight depression on the joint surface of the lateral distal femoral condyle with some surrounding sclerosis, but no evidence of loose bodies . Magnetic resonance imaging (MRI) showed features consistent with MED with an interesting finding of a dysplastic distal femur with an overgrown medial femoral condyle impinging into the notch over which the anterior cruciate ligament (ACL) was being abraded. There was also an abnormal interposition between the lateral aspect of the medial femoral condyle and the ACL.
Orthopedics
Examination under anaesthesia confirmed restriction of motion as detailed; however, it revealed no objective abnormal anterior movement of the tibia with respect to the femur in either the left or right knee and was unable to explain the reported symptoms of instability. Arthroscopic examination of the left knee was consistent with the MRI findings, revealing an abnormal prominence of the lateral aspect of the medial femoral condyle, as well as narrowing of the notch due to an abnormal lateral femoral condyle . The ACL was clearly impinging within the notch and appeared to restrict the range of motion. A notchplasty was performed. The patient made an uneventful recovery, with reported reduction in pain and subjective instability. The range of joint motion had improved at 6 months to allow full extension and flexion to 110°. The Oxford knee score improved from 45 pre-operation to 21 post-operation, while the SF36 physical component scale improved significantly from 24.1 pre-operation to 43.8 post-operation. This improvement was maintained for 2 years; however, the patient developed symptoms in the contralateral knee. A further MRI revealed a similar appearance and a subsequent arthroscopic notchplasty of the right knee provided a comparable level of improvement with reduced pain and increase in range of movement. This improvement was maintained at the most recent review at 6 months.
Orthopedic surgery
Arthroscopy has been used to successfully resect a symptomatic peri-articular osteochondroma of the distal femur and is used commonly in patients suffering from MED to clear loose bodies or repair meniscal damage. In this case, we report the success of arthroscopy in treating structural ACL impingement alone resulting in marked symptomatic improvement. We speculate that the reported symptoms of instability were due, at least in part, to pseudo-instability secondary to early osteoarthritic change.
Orthopedic surgery
MED is well known but ACL impingement leading to fixed flexion deformity and reduced range of motion have not been described in the literature to the best of our knowledge. This case highlights the scope for subjective symptomatic improvement following a minimum of surgical intervention in the form of arthroscopic decompression and/or notchplasty to resolve symptoms and to improve quality of life.
Orthopedic surgery
A 50-year-old man was admitted with a suspicion of an acute coronary syndrome because of progressive dyspnea and positive Troponin I (9.5 ng/ml). A two-dimensional echocardiogram revealed severe left ventricular hypokinesis with an ejection fraction of 27%. Upon coronary angiography, coronary artery disease was excluded. Because of a white blood cell count of 17000/mm 3 with 41% eosinophils, endomyocardial biopsies were taken from the left ventricle. Histological evaluation showed marked endomyocardial eosinophilic infiltration and areas of myocyte necrosis . Further evaluation revealed no evidence of secondary hypereosinophilia (malignant diseases, allergy, vasculitis, parasitic infection). The patient was diagnosed with hypereosinophilic myocarditis due to idiopathic hypereosinophilic syndrome. Medication with steroids and heart failure was initiated promptly and the patient improved rapidly.
Cardiology
This case highlights the clinical importance of CMR which is the only noninvasive method to visualize the extent of endomyocardial involvement in the diagnosis and treatment of eosinophilic myocarditis.
Cardiology
Metastases involving the TMJ are rare, and only 40 cases have been reported in the international literature . The primary site of the carcinoma was the breast in 9 cases, the lung in 9, the prostate in 5 and the rectum in 3 , followed by the liver, cardia , uterus and pancreas (1 case each). In 2 cases the primary tumour was a melanoma , the nose being the primary site in one case and the hallux in the other. In 6 cases, the primary site was not identified. As a rule, TMJ metastases manifest clinically even years after the onset of the primary tumour. In this study, we present a very unusual case, unique in the literature, in which the non-reducible dislocation of the mandible was the first clinical manifestation of pulmonary adenocarcinoma.
Oncology
The patient, a 60-year-old male, underwent extraction of the lower first right molar. From that moment he suffered from right TMJ pain. At first his symptoms were associated to the intervention. Following this episode, the patient reported limitation of mandibular movements, with reduced mouth opening, inability to perform lateral movements, right crossbite and edge-to-edge occlusion. The oral examination revealed Class III malocclusion and left mandibular deviation. On palpation the glenoid cavity was found to be empty.
Orthodontics
The joint capsule was accessed through a pretragal incision, observing that the condyle was displaced past the eminence. An unsuccessful attempt was made to perform bimanual reduction. It was finally observed that there was a mass that completely occupied the intra-articular space. The tumour was thus circumscribed and resected, after which the dislocation was successfully reduced manually; Deutrey's procedure was then performed to constrict the condyle.
Orthopedic surgery
A post-operatory chest X-ray appeared within the normal range, and only a post-operatory spiral CT scan succeeded in identifying the primary tumour The patient was referred to the pulmonary and oncology departments. Based on bronchoscopy and biopsy results, pulmonary adenocarcinoma was diagnosed. Given the presence of distant metastases, the patient was classified as Stage IV (TNM) . However, a total-body CT scan revealed that the condylar lesion was the only distant metastasis. The patient underwent a cycle of radiotherapy, but died 6 months later.
Oncology
A 75-year-old woman with a 1-week history of diplopia was referred to the ophthalmology clinic from the medical ward, where she was being treated for atypical pneumonia. She had been unwell for a few months with loss of appetite and weight. Sputum acid-fast bacillus smear and culture tests for tuberculosis were negative. There were no specific symptoms or signs suggestive of giant cell arteritis (GCA) but the erythrocyte sedimentation rate (ESR) was 98 mm/hour and the C-reactive protein was more than 139 mg/dl, which prompted the physicians to arrange a temporal artery biopsy. This was later reported as normal.
Ophthalmology
Ophthalmic examination showed corrected visual acuity of 6/6 bilaterally. There was subtle localized episcleral injection near the right lateral rectus muscle insertion. The posterior segment revealed a normal optic disc and macula bilaterally. Ocular motility revealed an abduction deficit in the right eye .
Ophthalmology
Computed tomography (CT) scanning of the head and orbits revealed a mass in the belly of the right lateral rectus suggestive of a metastatic lesion . Subsequent CT and magnetic resonance imaging (MRI) of the thorax and abdomen showed multiple secondary metastatic lesions in the lung and enlargement of retroperitoneal lymph nodes. The patient underwent an endoscopic retrograde cholangiopancreatography and the cytology of the brushings was highly suggestive of anaplasia. Pancreatic carcinoma was suspected but abdominal CT scanning and ultrasonic studies revealed no evidence. The patient later developed a supraclavicular lymph node mass, bilateral axillary lymphadenopathy and enlarged spleen. A supraclavicular lymph node biopsy was inconclusive. The suspected diagnosis of pancreatic cancer remained unconfirmed. The general condition of the patient had deteriorated, precluding further invasive investigations such as orbital biopsy. The patient died 4 months after her initial diagnosis of orbital metastasis. Autopsy was not performed on the body.
Oncology
In April, 2006 a 53-year-old Hungarian tourist was involved in a severe terror-attack in Egypt. He was initially hospitalized because of his burn, mechanical injuries and sepsis syndrome in Egypt. Five days later he was transferred in comatose, hypoxic and hypothermic condition to the Burn Unit of State Health Center, Budapest, Hungary. On the day of admission, bacterial cultures taken from burn wound. Based on the different colony morphology and antibiotic susceptibility patterns three different P. aeruginosa strains – an ESBL-producing P. aeruginosa (PA1), an imipenem-resistant P. aeruginosa (PA2), an MBL-producing P. aeruignosa (PA3), were observed and furthermore ESBL-producing Klebsiella pneumoniae (ESBL-KP), methicillin-resistant Staphylococcus aureus (MRSA) and Enterococcus faecalis (EF) were isolated. Next day ESBL-producing A. baumanii (ESBL-AB) and PA2 were cultured from the patient's canul. During his hospitalization from his nose PA1, ESBL-AB, ESBL-KP, MRSA, from his throat PA1, ESBL-AB, ESBL-KP, from his trachea PA3, ESBL-AB, ESBL.KP,-MRSA and from his wound PA1, PA3, ESBL-AB, ESBL-KP, MRSA and EF were isolated. Blood cultures were taken nine occasions and PA1, ESBL-KP, MRSA and EF were subcultured. The patient received adequate supportive treatment and empirically cefepime, subsequently meropenem and vancomycin were administered intravenously at high dosage. The aminoglycosides were synergy resistant. On the 8 th day of the hospitalization in Hungary the patient died.
Infectious diseases
Laboratory investigations revealed that serum prostate specific antigen was 5.1 ng/ml and serum creatinine was 86 μmol/l. Urine microscopy revealed no atypical cells and no haematuria. Prostate core biopsies showed benign prostatic hyperplasia.
Urology
An incidental exophytic lesion measuring 3 × 3.2 × 4.2 cm was discovered in the right kidney on abdominal ultrasound. This was a homogeneous hypoechoic structure with a well-defined margin seen lying towards the lateral border of the ventral aspect of the mid-zone of the kidney. The lesion appeared as a soft tissue abnormality on contrast renal computed tomography . There was a small area of calcification within it. The maximum diameter was approximately 2.17 cm.
Radiology
The lesion was removed by laparoscopic partial nephrectomy with a small amount of renal parenchyma and surrounding fat with clear surgical margins as a curative approach. The specimen comprised a nodule surrounded by fat and attached with a small pedicle to a piece of renal tissue. The nodule was well-circumscribed, seemingly encapsulated, firm in consistency and measured 2.5 × 1.4 × 2.5 cm. The cut surface was grey-white with microcystic areas.
Urology
We have reported the case of a renal schwannoma. To the best of the authors' knowledge, this is the first case of a schwannoma reported towards the lateral border of the kidney rather than in the hilar region. On microscopic examination, this was a cellular schwannoma and hence did not show the typical features of a classical schwannoma, including the Antoni A and Antoni B areas. On immunostaining, the cells showed diffuse and strong positivity for cytokeratins. Focal cytokeratin expression has been reported in schwannomas , but in this case, the cytokeratin expression using a broad range cytokeratin cocktail was strong and seen in all cells. However, the cells were all negative for cytokeratin 7 and EMA. The strong cytokeratin expression with the atypical histological features could have led to a diagnosis of an epithelial renal neoplasm as a sarcomatoid carcinoma.
Pathology
To the best of our knowledge, this is the first case of a renal schwannoma presenting towards the lateral border of the kidney. This was a cellular schwannoma, which showed, in addition to the expression of S-100 protein and GFAP, strong and diffuse expression of cytokeratins.
Pathology
Renal schwannomas, although rare, must be considered in the differential diagnosis of spindle cell lesions of the kidney. We report a case of an incidentally identified perirenal schwannoma, which was treated by partial nephrectomy. In 1988, Somers et al. reported a case of renal schwannoma recommending radical nephrectomy as the treatment of choice as then very few cases had been reported and the natural history and potential for malignancy was uncertain. Now given the benign clinical course of schwannomas, nephron-sparing surgery seems to be appropriate if technically feasible and the diagnosis is considered pre-operatively.
Urology
Thickening of the infrapatella fat pad following minor injury has been described by Hoffa. Calcification and ossification of the fat pad may be the end stage of this process . The association between minor injury and the formation of a para-articular osteochondroma has also been described . The rapid development of bone within soft tissues is termed heterotrophic ossification (HO). It is well appreciated that HO can occur following central nervous system injury including iatrogenic insult, although its aetiology is unknown. A search of the literature has not revealed an upper duration of this association although we appreciate that it is likely to be short term. The knee injury with development of the osteochondroma has occurred only several months after the diagnosis of the second acoustic neuroma. We appreciate that it is possible that the patient may have had the lesion for many years and however he was aware of it several months after his injury. Our patient clearly recalled suffering a minor knee injury and subsequently noted anterior knee discomfort and then gradually developed fullness to the front of his knee over five months. We are unaware of any association between osteochondroma and acoustic neuroma but since both entities are rare this is unlikely to be a coincidence.
Orthopedic pathology
We report the rare occurrence of a para-articular osteochondroma of the knee developing over short duration, 5 months, following minor injury. Predisposition to heterotrophic ossification after previous neurosurgery and a second acoustic neuroma may have accelerated the growth of this benign tumour. The development of these two rare entities suggests they may be associated.
Orthopedic Oncology
A 6 year old girl who was sleeping on the floor woke up at 3 AM with pain and swelling over the left hand. This was initially ignored by the parents. The pain and swelling gradually increased, and she complained of difficulty in breathing about 2 hours later. At this point she was brought to the hospital. There was no history suggestive of a snake bite. Examination revealed a drowsy child with a massively swollen left hand and forearm with shallow breathing. There were two clean puncture marks on the left thumb but no bleeding from them. There was no blistering or necrosis of the swollen limb. The local lymph nodes were enlarged. Examination of the central nervous system showed the child to be drowsy but arousable, not obeying verbal commands and was localizing pain. She had ptosis and grade 2–3/5 power in all four limbs and sluggish deep tendon reflexes with extensor plantars. Her pulse rate was 80/min, BP-100/60 mmHg SpO 2 – 60% on 5 liters of oxygen by face mask and temperature 98.4°F. Endotracheal intubation was done and she was ventilated on synchronized intermittent mandatory ventilation (SIMV) mode as she had some spontaneous respiratory efforts. Polyvalent anti snake venom therapy was started and a total of 100 ml was given (Haffkine Institute, Bombay), after a provisional diagnosis of snake bite with severe envenomation was made. Over a period of about 4 hours, the weakness progressed and involved proximal muscles first and then distal muscles. She was comatose, had no motor response to painful stimuli, became areflexic and her plantar reflex was not elicitable. Her pupils were dilated with no response to light; she had absent oculocephalic and corneal reflexes. She had no spontaneous respiratory effort, no response on tracheal suctioning and went onto full ventilatory support. Ventilation was continued despite findings suggestive of brain stem dysfunction and after about 36 hours of ventilation she showed a flicker of movement of her fingers and toes. The paralysis improved distally first then proximally. She was weaned off the ventilator and extubated after 5 days of ventilation. From about 36 hours of ventilation she had been noticed to have high blood pressure recordings up to 160/100 mm Hg which over a period of three days returned to normal without any specific drug therapy. When she was shifted out of the PICU she had grade 3 power in the lower limbs grade 4 power in the upper limbs and was breathing well but had truncal muscle weakness. She had mid-dilated and very sluggishly reacting pupils. She had no dysphagia or dysphonia. At discharge from hospital she had truncal muscle weakness, grade 4 power in the lower limbs, grade 5 power in the upper limbs, her pupils were still mid dilated and reacting sluggishly to light. The local reaction in the left upper limb had settled.
Toxicology
This case highlights the occurrence of both internal and external ophthalmoplegia which would mimic brain death in many ways, thus prompting many a pediatrician to consider withdrawing ventilatory support, which would be disastrous. In such a case other confirmatory tests of brain death like electroencephalography, four vessel cerebral angiography, Transcranial Doppler ultrasonography or radionuclide imaging (Technetium Tc 99 m hexametazime) should be resorted to. Supportive care needs to be continued till the effects of the venom wear off with excellent outcomes.
Neurology
The cat was treated with injectable piroxicam, (in order to better dose the drug), given at the dose of 0.3 mg/kg SC every other day for 4 months and carboplatin at the dose of 180 mg/m 2 (every 3 weeks) for a total of four doses. Carboplatin was chosen since systemic administration of CDDP causes fatal pulmonary edema in cats . The patient had a complete blood cell count, biochemical profile and urinalysis performed 7 days after each chemotherapy. A ultrasonographic exam was performed on a monthly basis to check for effusion secondary to malignancy.
Oncology
A 56-year-old, moderately obese (body mass index of 35) man with a past medical history of sleep apnea, hypertension and hyperlipidemia presented to his primary care physician for routine health maintenance. He mentioned that he had recently been very busy with overtime work, which had left him exhausted. He informed the physician of an incident that occurred one evening as he entertained his colleagues in a fine restaurant. While waiting for the meals to be served, a guest had told a very amusing joke and the patient began to laugh heartily, "Ha, ha, ha, ha... " in decrescendo until he was out of breath. To everyone's surprise, he then fell forward resting his head on the table and remained unresponsive for a few seconds before regaining consciousness. Prior to losing consciousness, he described feeling short of breath and noted that his surroundings were becoming dark. No seizure-like activity or incontinence was witnessed. After the episode he denied nausea, diaphoresis or otherwise feeling sick and proceeded to eat when his entrée was served. The remainder of the evening was without incident.
Cardiology
Intimal sarcoma of the pulmonary artery (PA) is a very rare tumor with poor prognosis. It is frequently misdiagnosed as pulmonary thromboembolism, and in most cases the definitive diagnosis is made during surgery or upon autopsy. The defining feature of this sarcoma is local growth with slight ability to metastasize . Here we present and discuss a case of PA intimal sarcoma with extensive metastases 10 months after the initial diagnosis was made during surgery.
Oncology
The patient, a 54-year-old woman, presented with shortness of breath on exertion over the previous three months. Physical examination revealed slight jugular vein distention (JVD); a grade III to VI systolic murmur, which was heard at the tricuspid area; and mild edema of both legs. Laboratory reports were within normal ranges. Echocardiography showed that the right PA (RPA) was almost completely obstructed by a low echogenic mass, and the left PA (LPA) was also partially obstructed, and the superior vena cava (SVC) was dilated with a diameter of about 23 mm. Right ventricle enlargement and tricuspid insufficiency were also revealed. A helical computed tomography (CT) scan showed that the RPA trunk was blocked by a soft tissue mass which grew circumferentially, with only line-like contrast passing through it . The LPA was thickened interiorly, with favorable contrast perfusion to its branches. On the basis of these findings, the patient was diagnosed with PA thromboembolism.
Cardiology
During surgery, a local soft mass measuring approximately 1.5 × 1.8 × 4.0 cm 3 was completely resected from the RPA. The diagnosis of intimal sarcoma was made by pathological examination . Atypical spindle cells were observed. Immunohistochemical analysis was positive for vimentin and weakly positive for CD34, while CD117, S-100, smooth muscle actin, desmin, and CD68 were negative. Following surgery the symptoms were relieved, and echocardiography showed normal blood flow in the PA trunk, RPA and LPA as well as in their proximal branches. The patient was then discharged.
Cardiology
Ten months later, the patient was referred to our department with complaints of shortness of breath on exertion, facial swelling and pain in the right upper quadrant. On physical examination no abnormalities were found except for facial swelling and JVD. Helical CT scan revealed a solid mass in the right pulmonary hilum, with no clear boundary to the mediastinum. The mass could be enhanced asymmetrically, with a central low-density necrosis invading the SVC and RPA trunk . In addition, a low-density cycloid mass with a clear boundary was detected on the quadrate lobe of the liver. Based on these findings, a diagnosis of a local recurrence of intimal sarcoma of the PA was made, with suspected metastasis to the liver. Three-dimensional conformal radiation therapy (3D-CRT) to the mediastinum was performed with a total dose of 60 Gy using 15 MV X-ray in 30 fractions. Dyspnea and facial swelling were relieved.
Oncology
In this case, the patient had a 10-month symptom-free period after surgery, even though the surgery was a simple local mass resection, confirming the positive role of surgery in the management of PA intimal sarcoma. When tumor relapse occurred in the pulmonary hilum, local 3D-CRT to the mediastinum successfully controlled symptoms of dyspnea and facial swelling. This suggests that intimal sarcoma of the PA may be sensitive to radiation in some cases. In addition, TACE of a metastasis in the liver also relieved pain for 2 months. Unfortunately, further systemic metastases finally developed, and the total survival time for this patient was approximately 15 months from the onset of symptoms.
Oncology
The volunteer was challenged by another infected mosquito on day 0. The mosquito was tested by PCR, confirming the presence of P. vivax only. The volunteer was continuously monitored in Mahidol University Hospital for Tropical Diseases in Bangkok, a non-malarious region. The volunteer experienced microscopically positive parasitaemias of P. vivax from day 14. From day 20, a regimen of artemisinin (200 mg/day for 3 days) and doxycycline (100 mg/day for 6 days) was administered to radically terminate the blood-stage parasites. The blood collection was resumed on day 29, when the anti-malarial drugs should have been completely eliminated from the volunteer's blood . Subsequently YN relapsed, and parasites were detected in the peripheral blood microscopically on day 36. After experiencing an acute phase, he was treated with chloroquine (1,500 mg on day 52; 500 mg on day 53 and 54), and with primaquine (15 mg per day for 14 days) to radically kill both the blood-stage parasites and the hypnozoites. The illness, especially headache, was subjectively much milder than in the previous infection one year before; nevertheless, the peak parasitaemia was three-times higher than the previous one.
Parasitology
A 75-year-old man with a history of persisting abdominal pain and mild diarrhoea for three months was referred by a general practitioner to the hospital. During this period the patient observed a weight loss of 2 kg, but did not show any B symptoms. A colonoscopy was performed without pathological findings. A subsequent CT examination of the abdomen showed two separate fat-containing retroperitoneal masses one in the adrenal gland and the second lateral of the psoas muscle inferior to the right kidney, outside the peri-renal adipose tissue. The lesions were separate and had no connecting tissue in between them . To further confirm the obtained CT scan results an MR imaging was performed and showed again a fat-containing lesion in the adrenal gland and a 7 × 5 × 7 cm soft tissue-tumour inferior to the right kidney without invasion of the right kidney, urethra or renal vasculature. Both lesions appeared similar in the CT and MR imaging and were consistent with a well-differentiated liposarcoma . Aware of the fact that a liposarcoma of the adrenal gland is extremely rare, a primary benign and fat containing tumour derived from the adrenal gland itself, such as a lipoma or myelolipoma was considered. Regarding tumour markers, only increased levels of CA 19-9 were detected (81.86 U/ml reference < 37 U/ml, CEA 2.2 μg/l, reference < 5 μg/l). To exclude a neuroendocrinological pathology additional tests were performed but did not reveal any noticeable conspicuities.
Oncology
According to our described strategy, the patient received a laparotomy. As expected, the adrenal tumour appeared well-encapsulated in accordance with a benign lipomatous tumour. Following the above mentioned second option first an adrenalectomy was performed. The rapid section of the adrenal lesion revealed the result of a myelolipoma. Having in mind that both lesions presented morphologically identical in CT and MR imaging and the assessed intraoperatively analogousness of the masses, we resigned a biopsy of the infrarenal tumour and removed it in toto without the kidney. Once more the rapid section revealed a myelolipoma. The final histology described a 4 cm sized and in weight 46 g well-encapsulated myelolipoma of the adrenal gland. Further a 14 cm sized and in weight 250 g well-encapsulated retroperitoneal myelolipoma was determined, without capsule involvement. Both tumours were composed of predominantly mature adipose tissue with mature myeloid elements, allowing the diagnosis of an extra-adrenal and adrenal myelolipoma. Consequently no other treatment than surgery was indicated and has not been performed in this case.
Endocrinology and Surgery
In our case the intraoperative detection of the macroscopically benign and encapsulated lesions, the pre-operative identical CT and MR imaging of both tumours followed by intra-operative rapid section of the adrenal tissue, prevented us from a radical en bloc resection or even from a second operation to remove the retroperitoneal mass after pre-treatment.
Surgery
Nevertheless, the appearance of a simultaneous adrenal and extra-adrenal myelolipoma is a rare incident. Therefore, we conclude that such lesions should be considered in the differential diagnosis of a fat-containing tumour in the retroperitoneal tissue/compartment. Summarized, this case underlines the importance of pre-operative CT and MR imaging, intra-operative rapid section and the importance of the surgeons' intraoperative judgement of the tumour, concerning an unknown primary tumour located in the adrenal gland and the retroperitoneum.
Oncology
Here we report the case of an adult patient with mild IAEE, who has recovered without any neurological deficit. The complete follow-up study of MRI and the serum/CSF cytokine assay are presented. MRI revealed a reversible lesion of the central portion of the corpus callosum splenium. Levels of IL-6 and IL-10 in his blood serum and the IL-6 levels in his CSF were initially elevated and later decreased to normal.
Neurology
A previously healthy 35-year-old man contracted an influenza type A virus infection. He had a high fever with a mild painful throat, myalgia and arthralgia throughout his whole body. He was diagnosed with an influenza type A infection by a positive result from an influenza antigen detection kit with a sample taken from a throat swab. He started taking oseltamivir phosphate (three 75 mg capsules) within 24 hours of the onset of high fever. The next day, he had an acute progressive tetraplegia and transcortical motor aphasia with mildly altered mental status. He was then transferred to our emergency room for further evaluation.
Neurology
Initial MRI at day 1 showed lesions diffusely throughout the white matter and especially on the central portion of the corpus callosum splenium, with a slight hyperintensity on T2-weighted fluid-attenuated inversion recovery and markedly high signal intensity on diffusion-weighted images. These findings resolved completely on follow-up study at day 8 and day 146 .
Radiology
His cerebrospinal pressure was high (235 mmH 2 O), but cytological and biochemical analyses of CSF were within normal limits: number of cells was 3/3 mm 3 (all monocytes), protein level was 30.6 g/dl and the glucose level was 67 mg/dl. The influenza genome was not detected by polymerase chain reaction in CSF samples from day 1 and day 8. Blood count showed a mild thrombocytopenia (12.0 × 10 4 cells/ml) and leukopenia . Electroencephalography showed normal basic activity with no paroxysmal discharge. He was treated with methylprednisolone pulse therapy for 3 days; his condition improved quickly following this treatment. After a 2-week rehabilitation, he made a complete recovery and was discharged from the hospital on day 24.
Neurology
Here, we have presented a case of adult IAEE with transient reversible CNS manifestations and MRI findings revealing a reversible lesion of T2 prolongation and reduced diffusion in the central portion of the corpus callosum splenium. During the initial examination, our first impression of this distinctive MRI finding was an acute disseminated encephalomyelitis (ADEM), which was foremost in our differential diagnosis. Corticosteroid pulse therapy was undertaken and, upon initiation of treatment, the MRI findings rapidly disappeared in conjunction with clinical improvement, although the lesion in the white matter recovered more slowly.
Neurology
We have reported a mild case of IAEE in an adult patient with a transient neurological deficit and interesting reversible lesions in the central splenium of the corpus callosum. It is important for clinicians to recognize that, even in adulthood, the subcortical encephalopathy observed during therapy for influenza type A infection can occur in conjunction with a reversible lesion of the corpus callosum, which may recover quickly. In addition, the cytokine storm in the blood system and the corticospinal cavity may play an important role in the etiology of this disease process.
Neurology
A 62-year-old male patient presented in May 2001 with initially unresectable metachronous liver metastases of a surgically treated rectal carcinoma. Cholecystectomy and implantation of an infusion system were carried out by laparotomy. From June 2001 to October 2001 he received four cycles of neoadjuvant HAC with FUDR monotherapy in a total dose of 1100 mg. In November 2001 hemihepatectomy of the right lobe could be performed after downsizing of the metastases. Five months later, he developed a solitary liver metastasis. In April 2002 a wedge-resection was performed, followed by one cycle of adjuvant HAC with FUDR in May 2002. 15 days after restart of regional therapy the patient presented with abdominal pain. Computed tomography revealed a large retroperitoneal hematoma and a ruptured pseudoaneurysm of the hepatic artery at the site of the catheter tip.
Oncology
In a subsequent angiography via a transfemoral access a stent-graft was placed into the hepatic artery in order to preserve arterial hepatic perfusion. This led only to a partial exclusion of the aneurysm and was complicated by a dissection of the hepatic artery, reflecting its friability. Definitive treatment was finally achieved by coil embolization . After clinical stabilization, the pump system was removed. Regional therapy was discontinued and the patient recovered uneventfully.
Interventional Radiology
A 38-year-old female patient was diagnosed with a multifocal hepatocellular carcinoma in July 2000. Hemihepatectomy of the right lobe, resection of segment I and cryosurgery of a satellite lesion in segment II were performed one month later. Because of recurrence of the tumor, a wedge resection of segment III/IV followed in February 2001. Concurrently, an Arrow pump system was implanted in a subcutaneous pocket on the lower right side of the abdomen. The catheter tip was placed at the origin of the gastroduodenal artery. From May 2001 to September 2001 she received HAC with 5 cycles of FUDR in a total dose of 600 mg to slow down tumor growth until orthotopic liver transplantation could be performed. In October 2001 the treatment had to be discontinued because of biliary toxicity with elevated liver enzymes and bilirubin. Five months later she had progressive disease and HAC was started again. Because of the previous toxicity, FUDR was administered in reduced dose. After completion of the fourth cycle, she presented to our emergency room with jaundice, fever and epigastric pain in June 2002. She was hospitalized and antibiotic treatment with ciprofloxacin was started. After isolation of Klebsiella pneumoniae from the blood culture, antibiotic treatment was changed to piperacillin and tazobactam. Percutaneous transhepatic cholangiography showed multiple liver abscesses. A few days later the patient developed severe hematemesis and melena necessitating blood transfusions. Gastroscopy showed blood remnants without any lesions of the gastrointestinal mucosa and hemobilia was seen in a repeated cholangiogram. Finally, computed tomography revealed a pseudoaneurysm of a segmental left hepatic artery, about 5 cm from the site of the catheter tip close to the hepato-enteric anastomosis . The development of a hepatic artery-biliary fistula was assumed to be the cause of the gastrointestinal bleeding.
Oncology
Cerebral arterial air embolism (CAAE) has been reported as a rare complication of medical intervention. We present a case of a 7-month-old girl who was admitted to our emergency department; post-mortem computed tomography (CT) showed a CAAE.
Neurology
At 5:00 a.m., she was pronounced deceased. Although the clinical history and findings during resuscitation suggested food aspiration, questions regarding the cause of death remained. Therefore, according to our battered child protocol, the standard radiographs, following the guidelines of the American College of Radiography were performed . In addition, a head CT, which was a standard in our hospital in children under the age of 2 years with unexplained trauma/death, was performed within 1h after death. The skeletal radiographs showed no abnormalities, except for the IO infusion in the proximal right tibia . Neither fractures nor signs of malnutrition were noted. The head CT, however, showed a considerable amount of air within the arterial circulation . Fig. 1 Intraosseous infusion needle correctly positioned within the proximal right tibial metaphysis Fig. 2 CT at the level of the circle of Willis showing air within the circle of Willis ( open arrow ) anterior cerebral artery ( small arrow ) and peripheral cerebral arteries ( solid arrow )
Radiology
A full judicial autopsy was performed in The Netherlands Forensic Institute, as is mandatory in children with a possible non-natural cause of death. The body showed normal measurements (length, 64 cm, and 6,900 grams, both p50) and a known omphalocele. Autopsy showed that the omphalocele contained a large segment of the right liver lobe and the ascending colon including the appendix, with adhesion to the abdominal wall . The intestines showed no signs of ischemia. Inspection of the heart revealed only minor congenital abnormalities, which consisted of a defect in the interatrial septum, fossa ovalis type with deficient flap valve, and slight hypertrabeculation of the ventricles, particularly the right ventricle . The latter findings, however, were clearly insufficient for a diagnosis of ventricular non-compaction. There was some pulmonary oedema, possibly due to the vigorous fluid resuscitation. The trachea was without abnormalities and, in contrast to the clinical history, showed no signs of food aspiration . Additional toxicological, microbiological and biochemical analyses of body fluids and tissues were all inconclusive. Fig. 3 Omphalocele containing a large section of the right liver lobe ( asterisk ) and the ascending colon including the appendix ( arrow ) Fig. 4 View of the atrial septum showing a persistent foramen ovale ( arrow ) and a fenestration ( arrowhead ) Fig. 5 View of the trachea devoid of food remnants, a small amount of saliva is seen ( arrow )
Forensic pathology
Recently, investigators from the Berne Institute of Forensic Medicine (Switzerland) reported on a case of air embolism after resuscitation and IOI in a 4-month-old boy . In their case, resuscitation was considered as the cause for CAAE; however, whole-body CT showed air in the lower extremity, which was used for IOI. As in our case, IO infusion was the only vascular access. Given the patent foramen ovale and atrial septal defect, leading to a right to left shunt, we feel that the only logical explanation for the CAAE is that air is introduced into the bloodstream via this route. To the best of our knowledge, our case is the second reported case of an air embolism after the use of an IO infusion.
Forensic medicine
On admission, he had mild anemia. Blood chemistry and coagulation were normal. A full colonoscopy was performed, which detected a 5 cm long, non stenosing rectal tumour, starting after the dentate line, in addition to a sigmoid polyp. Biopsies revealed a rectal adenocarcinoma and a non dysplastic adenomatous polyp in sigmoid colon. Staging studies were completed with tumour markers (CEA and CA 19.9) measurement, echoendoscopy and a thoracic-abdominal-pelvic CT. Tumour markers values were within normal range, echoendoscopy showed a 6 cm long uT3N0 rectal cancer, and CT detected an eccentric thickening of the rectal wall, compatible with a rectal cancer with no lymph node or visceral involvement. Final diagnosis was a rectal adenocarcinoma located in the middle-inferior thirds, clinical stage T3 N0 M0 .
Oncology
His clinical case was discussed shortly after in our Digestive Tumours Commitee, and neoadjuvant combined chemoradiotherapy followed by surgery were planned. The patient received capecitabine 900 mg/m2/12h d1-5/7d concurrent with radiotherapy, 45 Gy (180 cGy/d) . Treatment was generally well tolerated, with moderate cystitis and mild epithelitis as major adverse effects. Reassessment after neoadjuvant treatment showed neither blood analysis abnormalities, nor CT suspicion of residual disease .
Oncology
Fifty-three days after completing chemoradiotherapy patient underwent surgery. An abdominoperineal (AP) resection, including with mesorectal excision (TME), was performed with no surgical complications. Subsequent pathological analysis revealed a complete pathological response (TRG 1) ; with none out of four isolated lymph nodes involved; mesorectal excision was complete and the circumferential margin was greater than 1 cm.
Oncology
Five weeks after surgery the patient was again referred to Clinical Oncology, were, once we had confirmed the absence of disease by blood analysis and imaging, adjuvant treatment was planned. Adjuvant fluorouracil-based chemotherapy was then administered in order to complete a total of six months neoadjuvant and adjuvant treatment . Thoracic-abdominal-pelvic CT performed shortly after completing adjuvant chemotherapy, and this showed only surgical changes; colonoscopy through the end colostomy was normal, and blood analysis, including CEA and CA 19.9 levels, were also normal. The patient entered into our three-monthly periodic follow-up program .
Oncology
Eighteen months after initial diagnosis, the patient was asymptomatic. Programmed review detected increased CEA value (25 u/ml, normal value < 10) associated with a left lobe liver metastatic lesion of size 6 cms. in thoracic-abdominal-pelvic CT . PET scan performed showed no other metastatic sites . The patient received neoadjuvant chemotherapy with combination of intravenous fluorouracil c.i., oxaliplatin . After completing four cycles, revaluation tests showed normalisation of CEA levels and complete disappearance of the liver lesion . Patient was then referred to surgery. He subsequently had a liver trisegmentectomy, involving segments 1, 2 and 3 . Pathological analysis revealed a 1 cm residual focus of metastatic non-expressing EGFR colorectal adenocarcinoma. Recently, six weeks after liver surgery, and in the absence of residual disease, patient has started adjuvant chemotherapy with FOLFOX-4, planned to last for four months.
Oncology
A 51-year-old man was admitted to the hospital for treatment of benign prostatic hyperplasia (BPH). The patient's anamnesis was negative for allergic events. Before hospitalization he was being treated with alfuzosin, which belongs to a group of medications known as alpha-1A-receptor antagonists used to treat the symptoms of enlarged prostate and BPH. On admission to the hospital alfuzosin treatment was suspended and the patient underwent transurethral resection of the prostate under epidural anesthesia, followed by post-surgical administration of antibiotics (modivid) and lactated Ringer's solution. Twenty-four hours after surgery, routine prophylaxis for stress ulcer (one phial of Zantac ® 50 mg, intravenous, in normal saline solution) was prescribed. Within minutes of the injection of ranitidine, the patient developed a combination of wheezing, dyspnea and hypotension followed by loss of consciousness. Despite intensive resuscitation attempts, no cardiac activity reappeared and death was certified 30 minutes later. As the circumstances of death appeared suspicious to the treating emergency physician, a forensic investigation was initiated and the public prosecutor ordered a forensic necropsy.
Urology
The autopsy revealed pulmonary congestion with widespread upper airway edema, the presence of petechial hemorrhages and brain swelling with diffuse petechial hemorrhages. There was no evidence of recent myocardial infarction or other structural heart diseases. The rest of the organs were unremarkable. Histological sections confirmed the presence of widespread hypolaryngeal and pharyngeal mucosal and submucosal edema with inflammatory cells and an abundance of mast cells . Testing for specific IgE antibodies and mast cell tryptase was not performed because of post-mortem degradation of the serum.
Pathology
We have presented the case of a 51-year-old man with negative anamnesis of allergic events, who was admitted to the hospital for treatment of BPH. During post-surgical recovery the patient received antibiotics (modivid), lactated Ringer's solution and, 24 hours after surgery, routine prophylaxis for stress ulcer (one phial of Zantac ® 50 mg, intravenous, in normal saline solution) was added to the therapy. Within minutes of the injection of ranitidine clinical symptoms manifested in a combination of wheezing, dyspnea and hypotension followed by loss of consciousness and, despite intensive resuscitation attempts, no cardiac activity reappeared and death was certified 30 minutes later.
Pharmacology
Despite PMs and ICDs have protective systems , the effect of EMI are unpredictable and include: software reprogramming; inappropriate inhibition; triggering or asynchronous pacing (V00 or VVI setting); acute loss of capture or chronic increase in stimulation thresholds of ventricular and atrial leads; and also damage of internal circuits . When a DC shock must be performed in these patients, the defibrillator pads or paddles should not be placed close to or on top of the device. Most of PMs and ICDs are placed in the left subclavicular region; thus, the conventional defibrillator paddles position (left apical and right subclavicular position) is appropriate. However other sites can be used for device implantation (e.g. right subclavicular area) and they can be difficult to be detected in emergency situations. We report a case of sudden and complete PM battery discharge following two consecutive transthoracic DC shocks applied near the pulse generator in a patient with the device placed in the right pectoral region.
Cardiology
A 76-year-old lady was admitted in the cardiac surgical department due to severe mitral valve regurgitation and moderate-severe tricuspid regurgitation. Because of brady-tachy syndrome, a dual chamber PM was previously implanted in 2002 and then replaced in 2006. The pulse generator was located in the right pectoral region and, via right subclavian vein, two unipolar leads were placed in right atrial appendage and in right ventricle apex respectively. Just before surgery, in the presence of normal sinus rhythm, the PM was programmed in V00 mode. The surgical procedure consisted of a mitral valve replacement with mechanical prosthesis and a tricuspid valvuloplasty with a standard sternotomic approach. As a routine measure, atrial and ventricular epicardial pacing electrodes for external pacing were positioned.
Cardiac Surgery
During the night the patient, sedated and connected to mechanical ventilator, developed a broad QRS complex tachycardia episode (complex cycle 310 ms) associated with severe hypotension (arterial blood pressure: 70/20 mmHg). An emergency synchronized cardioversion using a 100 Joules DC shock was performed using a biphasic wave defibrillator, with an immediate return to sinus rhythm. After few seconds, a second DC shock (150 J) was necessary due to a relapse of the tachycardia. Lidocaine, 1 mg per Kg, was then administered to avoid tachycardia recurrences.
Cardiology
Following the procedure, the patient showed a spontaneous sinus rhythm at 100 beats/min with correct inhibition of pacing. A new test of the implanted PM was performed showing a complete battery depletion of the pulse generator with end-of-life characteristics, while all the other programming parameters were unchanged (impedance, pacing and sensing thresholds of both leads were unchanged compared to pre DC shock test values). The device was replaced before patient discharge from the hospital.
Cardiology
An increasing number of patients with an implanted PM or ICD could need a DC shock cardioversion or defibrillation for atrial or ventricular tachycardias. We present a case of life-threatening arrhythmia in which, after two consecutive DC shocks with a paddle positioned near the pulse generator, we noted a complete battery discharge of the device. Some suggestions to prevent such device failure are presented. In emergency situation first responder must be aware of the exact device site. Generic biaxillary paddle position can not be enough to reduce the risk of device damage. We suggest that patients with implanted ICD/PM, especially those at risk of developing malignant arrhythmias, must be checked at admission in hospital to highlight the actual device position. This should be marked clearly to allow safe DC shock delivery, in case of necessity.
Cardiology
A 77 yr old gentleman was admitted with an acute coronary syndrome with modest troponin rise. He was known to have aortic stenosis and coronary artery disease and the aortic gradient had accelerated by 50 mmHg over a 6 month period to 80 mmHg with good systolic but poor diastolic function. Valve area was calculated at 0.7 cm 2 and coronary angiogram showed significant two vessel disease involving the LAD and circumflex territory.
Cardiology
He had an uneventful anaesthetic induction and institution of cardiopulmonary bypass. During bypass it was noted that he required large doses of vasoconstrictors (pitressin infusion) to maintain perfusion pressure. He underwent a successful aortic valve replacement with a size 21 Tophat mechanical valve, reversed saphenous vein graft to obtuse marginal of circumflex and LIMA to LAD with a crossclamp time of 70 minutes.
Cardiac surgery
Similar flow and pressure measurements were obtained on three separate occasions of closure and release of compression. Eventually the fistula was explored and tied off together with 3 large collateral vessels. Flow characteristics at baseline and with arterio-venous fistula occluded are shown in Figure 1 & 2 . The patient was successfully weaned off bypass on IABP, NO and minimal inotropic support and was transferred to the ICU in a stable condition for further monitoring and care.
Cardiac surgery
This case serves to highlight a potential problem of cardiopulmonary bypass in patients with chronic large proximal A-V fistulae which have developed collaterals. In such cases it may be wise to measure fistula flow prior to surgery and place an uninflated sphygmomanometer in anticipation of temporary occlusion.
Cardiology
Pulmonary talc granulomatosis is a rare disorder characterized by the development of foreign body granuloma secondary to talc exposure. Several case reports have documented the disease in known intravenous drug abusers who present with respiratory symptoms. We present the diagnosis in a patient with a remote history of intravenous heroin use, and initial symptoms and imaging suggestive of malignancy.
Pulmonology
Laboratory results including complete blood count, renal function and liver function tests were all within normal limits. Human immunodeficiency virus (HIV) serology was negative. A chest X-ray showed an ill-defined density close to the right heart border, and a computed tomography (CT) scan confirmed a 4.5 × 2.2 cm opacity in the medial aspect of the right middle lobe, with emphysematous changes and spiculated opacities in both lung fields suspicious for malignant spread. A CT scan of the abdomen and pelvis was unremarkable.
Radiology
The patient underwent autofluorescence bronchoscopy and the visualized portions of the upper and lower airways were widely patent with no abnormalities. Ultrasound of the mediastinum using ultrasonic bronchofibervideoscope located the echodensity inferior to the right hilum, and fine needle biopsy of this structure was obtained via endobronchial ultrasound-guided transbronchial needle aspiration. Biopsies were also obtained from four further echodense areas suggestive of malignant lesions involving both lungs.
Pulmonology