[Carney's syndrome disclosed by hemoperitoneum]. / Syndrome de Carney révélé par un hémopéritoine.

The authors report a case of Carney's triad in a blood-group O woman who successively sustained a) at age 13, a multicentric gastric leiomyoblastoma revealed by an hemoperitoneum; b) 6 years later, a pulmonary chondroma diagnosed by chest standard roentgenogram and CT scan, associated with leiomyoblastoma recurrence and hepatic metastases; c) 10 years later, a juxta-carotid tumor of unknown (paraganglion?) origin. This patient is presently in good clinical condition, in spite of hepatic metastases known for 5 years. Based on this case and the 33 previously reported cases, the main features of this very unusual entity are analyzed.

[Increased fasting gallbladder volume: a new ultrasonic sign of cirrhosis?]. / L'augmentation du volume vésiculaire à jeun: un nouveau signe échographique de cirrhose?

Ultrasound was used to calculate fasting gallbladder volume in three groups of patients: 90 with cirrhosis (alcoholic in 75 cases), 41 with non cirrhotic liver disease (alcoholic in 14 cases), and 38 controls. Gallbladder volume was evaluated according to sex, age, alcoholism, presence of gallstones, time of diagnosis, and biological tests of hepatocellular function. Mean fasting gallbladder volume was significantly higher in cirrhotic patients (45.89 +/- 32.65 ml, m +/- 1 SD) than in patients with non cirrhotic liver disease (25.31 +/- 14.08 ml) and in control subjects (21.28 +/- 10.30 ml) (P less than 0.001), but there was a great overlap between individual results in each group. No relationship was found between gallbladder volume and all clinical and biological tested parameters, except for decreased prothrombin time (P less than 0.02). Further studies are necessary to consider this ultrasound sign as an useful diagnostic tool in cirrhosis.

[Portal venous system calcifications. Study of 3 cases and review of the literature]. / Calcifications du système porte. Etude de 3 cas et revue de la littérature.

Three cases of calcifications of the portal system are reported in men aged 42, 53 and 40 years old. Two patients had alcoholic cirrhosis, and one had familial congenital hepatic fibrosis, respectively. Calcifications were discovered fortuitously on plain abdominal films. Ultrasound and computed tomography studies, and in two cases, angiography, confirmed that calcifications were located within the vein walls. The veins involved were the portal vein in 3 cases, the splenic vein in 2 cases, and the superior mesenteric and left gastric veins in one case each. Investigations demonstrated splenoportal and splenic thrombosis in 2 cases, whereas the portal vein system was patent in the other. In two cases, the splenic artery was aneurysmal, associated with several intrasplenic arterial aneurysms in one. Based on these three cases, combined with a review of the literature of 123 previous reports, the features of this rare entity are described with emphasis placed on the value of computed tomography studies.

[Portal hypertension and neutrocytic ascites in POEMS syndrome]. / Hypertension portale et ascite neutrocytique au cours du syndrome POEMS.

We report a case of portal hypertension and neutrocytic ascites in a 52 year old man with POEMS syndrome. POEMS syndrome is an association of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy and skin changes. Portal hypertension is rare in POEMS syndrome and this is the first time that culture negative neutrocytic ascites has been described with this syndrome.

[Endoscopic sclerotherapy of hemorrhagic esophageal varices. 86 cases]. / Sclérose endoscopique des varices oesophagiennes hémorragiques. 86 observations.

OBJECTIVES: Haemorrhage due to rupture of oesophageal varices is a major cause of death in patients with cirrhosis. We evaluated retrospectively our results with endoscopic sclerosis in order to evaluate recurrence, compliance and long-term survival. METHODS: Endoscopic sclerosis was performed with a flexible endoscope in 86 patients from 1986 to 1989. Ninety-nine percent of the patients had cirrhosis; they were equally distributed in the 3 Child-Pugh classes. Sclerosis was performed once a week for three weeks then once every 3 weeks until eradication. Sessions was performed during an episode of haemorrhage in 17 patients and begun after bleeding had stopped in 69 others. Mean clinical follow-up was 24 +/- 14 months and mean endoscopic follow-up, 19 +/- 15 months. RESULTS: The mean number of sessions was 5.3 +/- 3.5 (range 1-17) per patient. Haemostasis was obtained in 4 out of 17 patients treated in emergency situations. Eradication was attained in 50 patients (58%). Recurrent varices were observed in 28 of these 50 and recurrent bleeding in 13, leading to 2 deaths. Global mortality over the period studied was 36%: 24 patients died before eradication, 7 after and 2 due to extra-hepatic causes. More than two-thirds of the deaths occurred during the first 2 months, mainly after recurrent bleeding. Global actuarial survival was 70% at 12 months, 62% at 24 months and 56% at 36 months. There was a significant difference in actuarial survival between Child-Pugh classes A and B patients and Child-Pugh class C patients. CONCLUSION: Our experience and the data in the literature indicate that sclerosis can be beneficial in patients with haemorrhagic oesophageal varices, but must be carried out within the framework of comprehensive care and follow-up.

Gastric leiomyoblastoma: diagnosis by CT and ultrasonography.

A case of gastric leiomyoblastoma is reported in a 57-year-old woman complaining of upper abdominal discomfort. Ultrasound and CT examinations discovered an epigastric mass, and ultrasound manoeuvers by multiple positioning allowed to refer it to a gastric origin. Definitive diagnosis was established by histological examination of the surgically removed tumor. The authors emphasize the interest of the radiological examinations, particularly ultrasound and CT, in this rare condition.

Spontaneous Yersinia enterocolitica peritonitis in idiopathic hemochromatosis.

We report a case of spontaneous Yersinia enterocolitica peritonitis revealing idiopathic hemochromatosis in a 62-yr-old man with fever and diarrhea. Blood and stool cultures were repeatedly negative, but Yersinia enterocolitica serotype 9 was isolated from ascitic fluid and serum agglutinins against this organism were positive at a dilution of 1:2560. Moreover, a serum Brucella agglutination test was positive at a dilution of 1:320. Antibiotic therapy with doxycycline led to a rapid clinical improvement, with disappearance of the organism in the ascitic fluid. This case suggests that Yersinia enterocolitica infection must be suspected in any febrile abdominal syndrome occurring in patients with iron overload, such as thalassemia major or idiopathic hemochromatosis.

Occult hepatocellular carcinoma in cirrhosis: value of ultrasound-guided biopsy of portal vein system thrombus.

We report two cases of occult hepatocellular carcinoma associated with alcoholic cirrhosis, in which the diagnosis of malignancy was respectively established by ultrasound-guided biopsy of umbilical vein and portal vein branch thrombus. When measurement of serum alpha-foetoprotein, and liver ultrasonography and/or computed tomography failed to clearly demonstrate hepatocellular carcinoma, the presence of a thrombosis of the portal vein system is highly suggestive of malignancy, which may be easily confirmed by venous biopsy.

[Accidents of circumcision and excision in the African environment]. / Les accidents de la circoncision et de l'excision en mileu africain

PIP: The authors report on 15 cases of complications of male circumcision and female excision of the clitoris observed between April 1972 and January 1975 at the Lome and Dakar, Senegal, hospitals. Male and female circumcision is still widely practiced in most North-West African countries; unfortunately, these procedures are done mostly in totally unsanitary conditions, and at the hands of inexperienced people. Among the complications observed in this series there were, for male circumcision, 1 case of hemorrhage, 4 cases of partial or total amputation of the penis, 1 case of tetanus, 5 cases of urethral fistula, 1 deformation of the glans, and 3 cases of lymphoedema of the penis. For female excision the major complications are the coalescence of the labia majora with consequent partial closing of the vaginal orifice, and retention of the urine. Treatment of such lesions depends on the severity of each case; if it is often possible to open a vagina or to suture back an amputated penis it is not always possible to prevent infection or death by tetanus and/or hemorrhage. Since circumcision, female and male, is part of the North-West African cultural background care should be taken that every operation be performed in a hospital if possible, or at least at the hands of experienced personnel.^ieng