[Extrahepatic cholestasis secondary to Castleman's disease]. / Cholestase extrahépatique secondaire à une maladie de Castleman.

Castleman's disease is a rare lymphoproliferative disorder of unknown etiology with different clinical manifestations. A 76-year-old man presented with pruritus and jaundice, a cholestasis and an increase of acute phase reactants. Peroperative investigations evidenced a 3-cm lymphadenopathy that compressed the biliary duct. Diagnosis of multicentric Castleman's disease was confirmed by the histologic examination of the lymphadenopathy. HIV testing was negative. Corticosteroid therapy was started and partially controlled the disease.

[Esophageal leiomyomatosis revealing an Alport syndrome]. / Léiomyomatose oesophagienne révélatrice d'un syndrome d'Alport.

Alport syndrome is a rare progressive hematuric nephropathy associated with sensorineural deafness. Leiomyomatosis associated with Alport syndrome is quite rare. We report a particular case of Alport syndrome which was diagnosed in the setting of an oesophageal leiomyomatosis. Alport syndrome and leiomyomatosis are caused by mutation of the genes encoding for the alpha chain of type IV collagen. In view of the important clinical and genetic implications, renal function and urinary status should be controlled in any patient with oesophageal leiomyomatosis.

[Sacral chordoma: a rare tumor of the buttocks]. / Chordome sacré : une tumeur fessière rare.

INTRODUCTION: Described and recognized for the first time as a pathological entity by Virchow in 1857, chordoma is a tumour of embryonic origin secondary to an attack of the notochord. In most cases it is asymptomatic, resulting in fairly late diagnosis. PATIENTS AND METHODS: We report the case of a 62-year-old patient presenting a bulky tumefaction, nodular in places, not very painful, and extending towards the anal area, scrotum and the posterior aspect of the upper left thigh. Histopathological examination of a macrobiopsy sample of this tumefaction pointed to chordoma. On magnetic resonance imaging (MRI), the tumour presented multiple ramifications extending towards the scrotal area, the sciatic area and the posterior aspect of the left thigh. Palliative tumorectomy was performed. Given the very slow progression of the tumour and the risk of adverse effects in such a large tumoral exposure field, radiotherapy was ruled out. DISCUSSION: This is a typical observation of a rare tumour that dermatologists may encounter.

[Sarcomas of the small intestine]. / Sarcomes du grêle.

Sarcomas of the small intestine are rare, clearly differentiated, malignant, mesenchymatous tumours that can be of smooth muscle, Schwann cell or fibroblastic origin. From a clinical point of view, the pain and abdominal mass are the 2 types of symptoms that frequently reveal the disease. In rare cases, sarcomas of the small intestine are manifested by an acute complication. No imaging method can clearly confirm the diagnosis. Before immunohistochemistry, differential diagnosis was made on undifferentiated mesenchymatous "stromal" tumours, which are also rare. Exeresis must be complete and without perforation of the tumour because of the risk of locoregional relapse. The benefits provided by chemotherapy and radiotherapy are limited because of the low mitotic activity of the tumour cells and its weak vascularisation. Long-term survival is limited by poor prognosis criteria: high grade malignancy, size greater than 5 cm, tumour extension, perforation of the tumour, quality of surgical resection and histological type.

[Role of laparoscopy in the diagnosis of peritoneal tuberculosis in endemic areas]. / Intérêt de la laparoscopie dans le diagnostic de la tuberculose péritonéale en zones endémiques.

The aim of our study is to demonstrate the difficulties of diagnosis of peritoneal tuberculosis in endemic areas and the value of laparoscopy for diagnostic confirmation. We report a retrospective study of 60 cases of peritoneal tuberculosis, collected from January 2004 to December 2009. The patients' median age was 41 years, and the sex ratio 0.4. The dominant reasons for consultation were pain and abdominal distension. Abdominal ultrasound found ascites in all patients. Computed tomography (CT) was performed in 30 patients and confirmed the ultrasound data. Paracentesis of the ascites took place in all patients, collecting exudative fluid in all cases; lymphocytes predominance in 83%. Screening for Mycobacterium tuberculosis in the ascites on direct examination and after culture was performed in 43 cases and negative in all of them. Surgical biopsy was performed laparoscopically in 29 patients and by laparotomy in 31 patients. Histological examination of the peritoneal biopsies confirmed the diagnosis of peritoneal tuberculosis in all cases.

[Internal trans-Oddi's sphincter fistula drainage of hydatid cysts communicating with the bile ducts]. / Drainage interne trans-fistulo-oddien des kystes hydatiques ouverts dans les voies biliaires.

OBJECTIVE: The purpose of this study was to examine the contribution of internal trans-fistulary drainage without opening the cystic cavity for the treatment of hydatic cyst of the liver. PATIENTS AND METHODS: Between 1985 and 1990, 14 patients with hydatid cyst of the liver communicating with the bile duct were treated by internal trans-fistulary drainage without opening the hydatic cavity. Choledocoscopy demonstrated good bile flow without a remaining cystic fragment in all cases. The residual cavity was drained via an external drain in the common bile duct. A second hydatic cyst of the liver was also treated in 4 patients. RESULTS: Internal trans-fistulary drainage was achieved easily. There were two treatment-related postoperative complications: bilary leakage in one patient and infection of the residual cavity leading to septicemia and death in another. CONCLUSION: Internal trans-fistulary drainage without opening the hydatic cavity is indicated for central hydatic cyst of the liver measuring less than 15 cm with a flexible pericyst and a large biliocystic fistula measuring more than 5 mm. Peroperative choledochoscopy of the hydatic cavity is necessary.

[Fatal postoperative crisis in acute hereditary porphyria]. / Crise post-operatoire fatale d'une porphyrie aiguë héréditaire.

The acute porphyria is an autosomal dominant disorder of the héme biosynthesis enzyme. The aim of this work is to determin a diagnostic step in order to anticipate porphyric crises. We report the observation of a child which was hospitalized in the pédiatric service three years ago for peripheric arthralgies and myalgies where the diagnosis of an acute porphyria was not posed. He wase admitted in our service for appendicitis, he was operated, the anesthesie was carried out by the thiopenthal and the succinylcholine. At the first post-operative day, a respiratory insufficiency was the cause of death. The rate of coproporphyrines and of uroporphyrines in the urine had confirmed the diagnosis of acute porphyria. The positive diagnostic of acute porphyria is difficult, the association of abdominal, psychological and neurologic signs must suggest the diagnostic, all the more, if the urines have a dark colour. The presence of uroporphyrines and coproporphyrines and certain precursors in the urines or in the high-rated stools confirm the diagnostic. The family survey and dosage of porphyric compounds in the sickness-bearing patient's family members allow to reveal certain asymptomatic forms of the heriditary acute porphyria, it is caused by certain products used in neuropsychiatry, in aneasthesic or in certain circunstances, such as, the infection or the trauma. The treatment of the acute porphyria is an emergency wich requires the transfert of the patient to a unit of intensive care.

Duodenal diverticulum associated with annular pancreas: a rare cause of severe cholangitis.

Duodenal diverticulum is a common occurrence but most are asymptomatic. However, in some cases, they can cause mechanical biliary compression. We report the case of a duodenal diverticulum in a 64-year-old woman revealed by severe cholangitis with septic shock and a liver abscess. Associated annular pancreas was found. We discuss the various investigations to diagnose these two entities as well as the therapeutic strategy in this unique combination of disease.

Peritoneal tuberculosis.

The peritoneum is one of the locations outside the most common pulmonary tuberculosis. Peritoneal tuberculosis poses a public health problem in endemic regions of the world. The phenomenon of migration, the increased use of immunosuppressive therapy and the epidemic of AIDS have contributed to a resurgence of this disease in regions where it was previously controlled. The aim of this review is to expose the clinical, biologic end radiologic futures of the peritoneal tuberculosis and to present the methods of diagnosis and treatment. The diagnosis of this disease is difficult and still remains a challenge because of its insidious nature, the variability of presentation and limitations of available diagnostic tests. The disease usually presents a picture of lymphocytic exudative ascites. There are many complementary tests with variable sensitivities and specificities to confirm the diagnosis of peritoneal tuberculosis. Isolation of mycobacteria by culture of ascitic fluid or histological examination of peritoneal biopsy ideally performed by laparoscopy remains the investigation of choice. The role of PCR, ascitic adenosine deaminase, interferon gamma and the radiometric BACTEC system can improve the diagnostic yield. An antituberculous treatment with group 1 of the WHO for 6 months is sufficient in most cases.

Unusual cause of intestinal obstruction: ileal endometriosis.

Endometriosis is a common condition affecting the female genital tract, but involvement of the ileum is very rare. Its symptoms are vague and are similar to other benign and malignant disorders, and radiographic findings lack specificity. We report the case of a 23-year-old woman presenting with acute intestinal obstruction for whom preoperative diagnosis favoured acute appendicitis. Laparotomy revealed ileal stenosis. A partial small bowel resection was performed. Pathological examination diagnosed ileal endometriosis. Endometriosis may be a cause acute abdominal pain in women, and should be considered in differential diagnosis. Difficulties in establishing its diagnosis are discussed.