Inflammatory myofibroblastic tumour of the spinal cord: case report and review of the literature

Inflammatory myofibroblastic tumours (IMT), also called inflammatory pseudotumours, nodular lymphoid hyperplasia, plasma-cell granuloma and fibrous xanthoma, are rare soft-tissue lesions characterised by inflammatory cells and a fibrous stroma. Clinically and radiologically, they may look like malignant tumours. They rarely affect the central nervous system and are very rare in the spinal cord. We report an IMT of the spinal cord in a 22-year-old woman presenting with spinal cord compression and a cauda equina syndrome. MRI showed a lesion at T9 with extramedullary and intramedullary components giving low signal on T2-weighted images and enhancing homogeneously. Pial lesions on the lumbar enlargement and thoracic spinal were present 11 months after surgery, when the lesion recurred. We present the radiological, operative and pathological findings and review the literature.     

Papillary cystic neoplasm of the pancreas: a report of three pediatric cases and literature review

BACKGROUND/PURPOSE: Papillary cystic neoplasms are rare pancreatic tumors that typically present in women in their third decade of life. Few cases have been reported in children. METHODS/RESULTS: The authors report on three pediatric patients: a 10-year-old boy, an 11-year-old girl, and a 14-year-old girl. The authors have reviewed the existing literature on papillary cystic neoplasms of the pancreas and suggest that these tumors probably arise early in life, grow slowly, and metastasize infrequently. CONCLUSION: Even when these tumors metastasize, patients seldom die as a result of the malignancy.     

Cutaneous reactions following herpes zoster infections: report of three cases and a review of the literature

Three patients, one healthy and two immunocompromised, developed cutaneous reactions that histologically mimicked granuloma annulare at sites of resolved varicella-zoster virus (VZV) reactivation infections. Variable latency periods between the infection and the granulomatous reaction were noted. As in other case reports, the presence of VZV DNA in these lesions was inconsistently demonstrated by the polymerase chain reaction (PCR) and appears more common in early, as opposed to late, post-zoster granulomas. In addition to various granulomatous reactions, vasculitic and neoplastic eruptions following resolved VZV infections have been described and are reviewed here. Therapeutically, topical, intralesional and systemic corticosteroids, as well as acyclovir, have been tried with inconsistent results. Although the pathogenesis remains unclear, the presence of VZV DNA in early lesions that histologically do not display viral cytopathic changes, suggests the virus induces an atypical delayed hypersensitivity reaction not affected by antiviral therapy.     

Leri's melorheostosis: three pediatric cases and a review of the literature

Three pediatric cases of melorheostosis are described. Growth disorders of the limbs are often the first signs in children. The radiological appearance consists of long tracks of increased cortical radiodensity in the long bones and of small dense islets in the epiphyses.     

Megalourethra: a report of four cases and review of the literature

Megalourethra is a rare congenital anomaly characterized by severe dilatation of the penile urethra. Four cases of congenital megalourethra were seen at Sir Padampat Mother and Child Health Institute, Jaipur, during the last 10 years. Three cases of scaphoid megalourethra had no other associated congenital anomalies and were treat-ed successfully without any complications, while one patient with a fusiform megalourethra had severe associated congenital anomalies and died. These cases are reported with a review of the literature.     

Paraganglioma of the prostatic urethra. A report of three cases and a review of the literature

OBJECTIVE: To determine the incidence and clinical presentation of prostatic urethral paragangliomas and to compare these findings with paragangliomas occurring in the other sites of the lower urinary tract. PATIENTS AND METHODS: The clinical records of three patients with prostatic urethral paragangliomas were reviewed, as was the histology of each specimen. The tumours were stained immunocytochemically with neuron-specific enolase, chromogranin, S100 protein, protein gene product 9.5, prostate-specific acid phosphatase and prostate-specific antigen. RESULTS: The three tumours occurred in elderly men, two of whom presented with haematuria, one of whom also had a vesical transitional cell carcinoma, and the third patient had obstructive symptoms due to co-existent prostatic hyperplasia. The histological appearances and immunocytochemical findings were consistent with paraganglioma. Treatment consisted of local excision and there were no recurrences or metastases at 5 and 6 years in two patients for whom follow-up was available. CONCLUSION: Urethral paragangliomas are rare tumours arising in elderly men and are hormonally inactive. Although haematuria may be the presenting symptom, it is important to exclude additional more common and possibly more sinister lesions such as transitional cell carcinoma. Local excision appears to be curative.     

Three major coronary artery-to-left ventricular shunts: report of three cases and review of literature

The gallbladder is perforated and stones are spilled more frequently during laparoscopic cholecystectomy than during open cholecystectomy. Recent reports have implicated spilled gallstones as a source of infrequent but serious complications of laparoscopic of laparoscopic cholecystectomy. They can cause serious morbidity, and in most cases the patient will require open surgery for management of these complications. The authors report the case of a patient who was ill for 14 months after laparoscopic cholecystectomy when spilled stones formed a nidus for intra-abdominal abscess and colocutaneous fistula. Every effort must be made to prevent gallbladder perforation. When it does occur, all stones should be retrieved. Attempts at repairing gallbladder perforations are often unsatisfactory. A simple solution to this potential problem is to retrieve all stones immediately, place them in an intraperitoneal specimen bag, and "park" the bag on the liver. As soon as the gallbladder is dissected off the liver it should be placed in the specimen bag with the stones and removed through the umbilical port opening.     

Neuroblastoma: a review of 21 cases presenting with spinal cord compression

The records of 21 children with neuroblastoma presenting with spinal cord compression, encountered over 17 years, were reviewed. Thirteen patients (61%) survive, free of neuroblastoma, at intervals ranging from nine months to 192 months (median, 78 months) from the time of diagnosis. The explanation for this relatively high survival rate was sought in an analysis of the cases which took into account age, site, extent of disease, and histology. The most significant features to emerge were the unusually high proportion of children under 12 months of age at presentation (11 of 21) most of whom survive (9 of 11) and the low incidence (3 of 21) of detectable at the time of diagnosis. The absence of a paraspinal mass was an unfavourable prognostic features (1 of 6 survives) whilst if a paraspinal mass was present, its anatomical level did not influence survival. In particular, children with retroperitoneal tumors fared no worse (survival, 6 of 7) than those with primary tumors at other sites (survival, 6 of 8). Morbidity was high (6 of 13), principally in infants with spinal cord compression from birth. Survival was also related to the histologic maturity of the tumor, even in the presence of metastases. Recommendations for management are made.     

Ureterovaginal fistula: a report of 12 cases and review of the literature

Ureterovaginal fistula is an uncommon complication of pelvic operations, seen most often after Wertheim's hysterectomy. We report 12 cases of ureterovaginal fistulas seen during a 20-year period, all of which followed operations for benign gynecologic conditions. Most patients had no urinary symptoms until the sudden onset of incontinence 1 to 4 weeks postoperatively. Diagnosis was established readily by a combination of excretory urography, cystography, cystoscopy, retrograde pyeloureterography and dye studies. In our series only 1 patient was treated by primary nephrectomy, while 11 underwent ureteroneocystostomy: 2 with a Boari flap and 9 by a direct method. Reconstruction failed in 2 patients, 1 of whom required a secondary nephrectomy.     

Atypical ameloblastoma: report of 3 cases and a review of the literature

The aim of our study was to test the hypothesis that the better absorption of sorbitol when ingested with glucose could be related to a delayed gastric emptying. We tested the effect of the ingestion of glucose and lipids on the gastric emptying and intestinal absorption of sorbitol in six healthy volunteers, using gastric scintigraphy and hydrogen breath test. After an overnight fast, subjects ingested in random order, on 48-h test periods separated by at least one week, the following solutions: (a) 20 g sorbitol alone; (b) 20 g sorbitol and 20 g glucose; (c) 20 g sorbitol and 9 g lipids. Isotopic acquisitions were taken for 3 h following the ingestion of sorbitol labelled with 111Indium. Hydrogen concentration was measured in end-expiratory samples during 5 h, and the areas under the breath hydrogen curve, reflecting the amounts of sorbitol unabsorbed in the small bowel, were compared between periods. Mean area under the curve was 397 +/- 159 when sorbitol was ingested alone, and this was significantly lower when ingested with glucose or lipids (313 +/- 181 and 337 +/- 135, respectively; P < 0.05). The three curves of sorbitol gastric emptying differed significantly from each other, the gastric emptying being the slowest for sorbitol plus lipids, and the fastest for sorbitol taken alone. We found a positive correlation between the half-emptying time and the hydrogen areas under the curve (r = 0.46, P = 0.05). In conclusion, our study demonstrates that adding glucose or lipids to a solution of sorbitol slows the gastric emptying of sorbitol, resulting in a better intestinal absorption of sorbitol.     

Vertebral osteomyelitis due to salmonellae: report of two cases and review

A static or kinetic visual disturbance affects subjects' ability to estimate the direction of the gravitational vertical. This kind of error is increased by a head roll inclination. In two experiments, we combined head orientation with a static (Experiment 1: tilted frame) versus kinetic (Experiment 2: rotating disk) visual disturbance. The results showed that with a static visual disturbance, the increase of errors in the inclined head condition was mainly the consequence of a postural head effect like an Aubert effect. On the contrary, with a kinetic visual disturbance, it appears that the disk effect increases with head inclination. However, individual errors observed with the head inclined in front of a stationary disk were systematically correlated with the errors triggered by the same head inclination in front of a rotating disk. These observations confirm that the head axis spatial reference plays an important role in orientation perception, whatever the head position and the kind of visual display.     

Chest discomfort associated with liposomal amphotericin B: report of three cases and review of the literature

Liposomal formulations of amphotericin B are designed to maintain therapeutic efficacy of amphotericin B deoxycholate while reducing its associated toxicities. In three patients chest discomfort occurred during planned 1-hour infusions of liposomal amphotericin B (AmBisome) 3 mg/kg/day during an open-label trial. The first patient experienced chest tightness and difficulty breathing and the second had dyspnea and acute hypoxia, both within 10 minutes of the start of the infusion. The third patient complained of chest pain 5 minutes after the start of two infusions. All symptoms resolved on terminating therapy. Two patients were later rechallenged with slower infusions and tolerated the drug well. A review of the English-language literature revealed only two other case reports of infusion-related chest or pulmonary reactions with the drug, although similar reactions were noted in several reports of clinical trials. Further review of the literature revealed reports of chest and pulmonary adverse events with other liposomal formulations of amphotericin B, liposomal daunorubicin, liposomal doxorubicin, and liposomes. The pathophysiology of such reactions remains unclear, and premedication with diphenhydramine did not completely prevent this reaction in one of our patients. We recommend infusing liposomal amphotericin B over at least 2 hours with careful monitoring for adverse reactions.     

Hypersensitivity reactions to etoposide. A report of three cases and review of the literature

Etoposide (VP 16) is an antineoplastic agent that has major activity against a number of tumors, including germ cell neoplasms, small cell lung cancer, and malignant lymphoma. It is a derivative of podophyllin, and acts by inhibiting mitosis. Common side effects include bone marrow suppression, alopecia, and gastrointestinal symptoms. Hypersensitivity reactions are uncommon however, and the underlying mechanism is unclear. We report three cases of etoposide hypersensitivity and review the literature.     

The rim sign in hepatic abscess: case report and review of the literature

We studied a previously healthy patient who presented with a 3-wk history of fever, flu-like symptoms and abdominal pain. METHODS: Blood cultures were positive for Escherichia coli. A computed tomography (CT) scan revealed a 2-cm low-density focus in the right hepatic lobe. A technetium-99m-mebrofenin scan showed a photopenic area in the right hepatic lobe surrounded by a rim of activity greater than the adjacent parenchymal activity. RESULTS: Gallbladder visualization was normal and the diagnosis of hepatic abscess was made. CT-guided percutaneous drainage of the lesion yielded six cc of pus, the culture of which grew E. coli, Prevotella and Bacteroides fragilis. Drainage and a 6-wk course of intravenous antibiotics were followed by clinical improvement and resolution of the abscess by CT. CONCLUSION: The rim sign and its possible mechanism of causation in hepatic abscess are discussed in this report, together with a review of the literature.     

Unilateral amnesic stroke. Six new cases and a review of the literature

BACKGROUND AND PURPOSE: Although persistent amnesia due to bilateral limbic system infarction is well described, reports of amnesic syndromes due to unilateral stroke have appeared infrequently and unsystematically. We report six new cases and review previously published reports to expand and consolidate knowledge regarding amnesic stroke. CASE DESCRIPTIONS: Six patients developed acute amnesia associated with unilateral stroke. Brain computed tomography or magnetic resonance imaging revealed lesions in limbic structures, which accounted for the memory deficit. Based on these as well as similar cases in the literature, we delineate three distinct syndromes of unilateral amnesic stroke involving the territories of the posterior cerebral, anterior choroidal, and thalamic penetrating arteries. Eighty-five percent of reported cases have involved the left hemisphere. CONCLUSIONS: Persistent as well as transient amnesia may be the sole or primary manifestation of unilateral hemispheric stroke. The vascular mechanisms of amnesic stroke are diverse. Patients presenting with acute amnesia possibly related to cerebral ischemia should be classified by documented or presumed lesion site as well as the involved vascular territory. Left amygdalohippocampal or diencephalic dysfunction may produce a particular vulnerability to global amnesia.