Idiopathic retroperitoneal fibrosis and primary biliary cirrhosis. A new association?

We encountered a case of primary biliary cirrhosis in a nonalcoholic man who had been operated on for idiopathic retroperitoneal fibrosis 20 years previously. Chronic pancreatitis was also detected on endoscopic retrograde examination. After several episodes of digestive bleeding due to ruptured esophageal varixes, the patient died of massive hemorrhage. Postmortem examination showed stage 3 primary biliary cirrhosis and a thick retroperitoneal fibrous plaque, consisting of densely fibrotic areas of collagen with rare vessels and mononuclear cells. We suggest that idiopathic retroperitoneal fibrosis may be a new autoimmune disorder associated with primary biliary cirrhosis and that primary biliary cirrhosis is a potential cause of portal hypertension, cholestasis, or both in the course of idiopathic retroperitoneal fibrosis.

Fibrosing alveolitis and hepatitis B surface antigen-associated chronic active hepatitis in a patient with immunoglobulin A deficiency.

Fibrosing alveolitis is described in a 22 year old woman with immunoglobulin A (IgA) deficiency and hepatitis B surface antigen (HBsAg)-associated chronic active hepatitis. At lung biopsy HBsAg was detected by indirect immunofluorescence in the alveolar space but not in the septal fibrosis. We discuss the possible relationships between IgA deficiency on the one hand, and HBsAg-associated lung and liver diseases on the other hand.

Autovagotomy by esophageal carcinoma. An unusual cause of gastric retention without outlet obstruction.

A case of gastric retention without outlet obstruction in a 44-year-old patient with esophageal carcinoma is reported. Radiological (and particularly C.T.) examinations support the opinion that the gastric motility disorders were due to the autovagotomy secondary to malignant involvement of the intrathoracic vagus nerve.

Bleeding cecal typhoid ulcer. Value of emergency angiography for selective surgical hemostasis.

A case of bleeding cecal typhoid ulcer in a 22-year-old patient demonstrated by superior mesenteric arteriography is described. An attempt to stop hemorrhage using intraarterial vasopressin infusion, failed. However, arteriography proved helpful in locating the bleeding site, and in permitting conservative surgery, namely cecal ulcer suture to achieve hemostasis. On the basis of this case and a review of the literature, management of complicating hemorrhage resulting from typhoid fever is discussed, with particular reference to radiological procedures.

[Gardner's syndrome and epidermoid cyst of the thymus]. / Syndrome de Gardner et kyste épidermoïde du thymus.

The hitherto undescribed association of Gardner's syndrome (familial adenomatous polyposis coli, abdominal fibromas, osteoma, dental abnormality, cystic fundic gland polyposis) with a thymic epidermoid cyst in a 38 year old woman is reported. Thymic cyst was discovered on a routine chest roentgenogram, and diagnosis, suspected by CT findings, was established by histological examination of surgical material. The authors suggest that this association is not fortuitous.

[Hepatic brucelloma (pseudotumoral brucellar caseous necrosis of the liver). Study of a case and review of the literature]. / Brucellome hépatique. (Nécrose caséeuse brucellienne hépatique pseudo-tumorale). Etude d'un cas et revue de la littérature.

We report the case of a 43 year-old Portuguese woman, hospitalized for long-standing fever, fatigue and weight-loss. Biologic investigation demonstrated anicteric cholestasis. Abdominal plain film showed a single hepatic calcification; computerized tomography and ultrasonography of the liver led to the discovery of a large mass, centered by the calcification. C. T.-guided biopsy showed caseiform necrosis, surrounded by histiocytic and lymphocytic cells. Brucella agglutination tests were negative at the beginning of the illness but became positive secondarily. The germ was not isolated from the hepatic fragment. The melitine intradermo-reaction was positive. Outcome was rapidly favorable with antibiotic treatment. Analysis of the 14 previously published cases showed that the most constant features were the hepatic calcifications and the epidemiologic context.

[Natural history of glandulocystic fundic polyposis in Gardner's syndrome]. / Histoire naturelle de la polypose fundique glandulo-kystique dans le syndrome de Gardner.

The authors report 3 cases of fundic gland polyposis associated with Gardner's syndrome. Control endoscopies revealed fundic gland polyposis disappearance 14, 14, and 7 years respectively after discovery, and colectomy performed at ages 29, 30, and 32, respectively. This regression, described in autonomous fundic gland polyposis, is poorly documented in Gardner's syndrome. This phenomenon is not related to colectomy. However no explanation is currently available, and further studies are necessary to determine the factors related to the natural history of fundic gland polyposis in familial adenomatosis coli/Gardner's syndrome.

[Cavernous lymphangioma of the liver. A report of 2 cases and review of the literature]. / Lymphangiome caverneux du foie. Etude de 2 cas et revue de la littérature.

The authors report 2 cases of hepatic lymphangioma observed in a 54-year-old woman, and in a 39-year-old man. These tumors were discovered upon ultrasound examination performed for jaundice due to viral hepatitis, and for abdominal right upper quadrant pain respectively. Computed tomography and angiography showed hypervascularized tumors. Diagnosis was established through surgical biopsy specimens. The clinical course was uneventful, during respectively the 9- and 3-year follow-up periods, respectively. From these and the 40 previously reported cases, the authors describe the different features of this unusual tumor.

[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.

[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.

[Massive hepatic steatosis disclosing adult celiac disease. Study of a case and review of the literature]. / Stéatose hépatique massive révélatrice d'une maladie coeliaque de l'adulte. Etude d'un cas et revue de la littérature.

The authors report the case of a 32-year old woman admitted for hepatomegaly, weight loss, and moderate diarrhea. Liver function tests showed anicteric cholestasis with slight increase in serum level of transaminases. Liver biopsy demonstrated massive steatosis. Biological and radiological investigations of the small intestine showed a malabsorption pattern. Stool fat excretion was 54 g per day. Duodenal biopsies disclosed total villous atrophy. A ten-day treatment with metronidazole (1,5 g per day), followed by a gluten-free diet, resulted in rapid improvement of hepatic and intestinal symptoms. This case report shows that: 1) adult celiac disease may be the cause of severe steatosis; 2) anicteric cholestasis with or without hepatomegaly during the course of adult celiac disease may be secondary to steatosis, as well as primary biliary cirrhosis or malignant infiltration of the liver; 3) bacterial overgrowth should be searched and eventually treated in the case of massive fatty liver occurring in adult celiac disease.

[Xanthogranulomatous cholecystitis. X-ray computed tomographic study of a pseudotumoral form]. / Cholécystite xanthogranulomateuse. Etude scanographique d'une forme pseudo-tumorale.

The authors report a case of xanthogranulomatous cholecystitis in a 63-year-old woman hospitalized because of a right hypochondrium mass. Ultrasonography, computed tomography, and celiac arteriogram showed a gallbladder tumour. Surgical management allowed subtotal tumour resection. Diagnosis of xanthogranulomatous cholecystitis was established by histological examination. Twenty-two months later, patient is in good health. From this observation and a world-wide literature review (90 previously reported cases), the authors analyse the clinical, pathological, and pathophysiological features of this very unusual entity.

[Spontaneous contained perforation of a hiatal hernia. A new equivalent of Boerhaave's syndrome]. / Perforation "spontanée" cloisonnée d'une hernie hiatale. Un nouvel équivalent du syndrome de Boerhaave.

The authors report the case of an alcoholic 49-year-old man hospitalized because of epigastric pain and hematemesis. Upper endoscopy showed a tear in a hiatus hernia (interpreted as a Mallory-Weiss lesion) and a bleeding duodenal ulcer. Upper barium examination performed 16 days later revealed a mediastinal collection communicating with the hiatus hernia. Retrospective evaluation of chest roentgenograms obtained at admission showed a limited mediastinal gas collection. With medical management, the collection progressively disappeared. From a theoretical point of view, this case illustrates Watts' theory on the effect of hiatus hernia on the site of postemetic injury. From a practical point of view, it confirms that conservative management is indicated in contained spontaneous perforations.

[Hepatoid adenocarcinoma of the stomach]. / Adénocarcinome hépatoïde de l'estomac.

One year after gastric resection for cancer, a 67 year old patient was hospitalized because of a large hepatic tumor with extremely high serum alpha-foetoprotein levels (13,245 ng/ml). Histological and immunohistochemical studies of the gastric tumor revealed hepatoid foci with alpha-foetoprotein and protease inhibitor-producing cells. Histopathological and immunohistochemical features of this and the 8 previously reported cases of hepatoid adenocarcinoma of the stomach are analyzed.

[Hydrocele of pancreatic origin. X-ray computed tomographic study of an intrascrotal collection in an acute outbreak of chronic pancreatitis]. / Hydrocèle d'origine pancréatique. Etude scanographique d'une collection intrascrotale lors d'une poussée aiguë de pancréatite chronique.

In an alcoholic 46-year-old man complaining of painful scrotum enlargement, CT examination allowed to refer the intrascrotal fluid collection to its pancreatic origin.

[Cecal hernia through Winslow's foramen. Radiographic study of a case and review of the literature]. / Hernie caecale par l'hiatus de Winslow. Etude radiographique d'un cas et revue de la littérature.

A case of herniation of the cecum through the foramen of Winslow is reported in a 49-year-old man, admitted for acute abdominal pain. Diagnosis, suspected by abdominal plain films, and established by water-soluble enema, was confirmed by emergency surgical management. Herniation through Winslow's foramen represents the least common variety of internal hernias. Only 136 observations were previously reported, the cecum being involved in 25-30% of cases. Radiological diagnosis was made in only one patient out of ten. Although rare in occurrence, it carries a high mortality risk when diagnosis and treatment are delayed. A better appreciation of the classic radiological features will allow for earlier recognition and treatment. Therefore, the authors emphasize the interest of radiologic findings in this condition.

[Multiple duodenal lipomas. A x-ray computed tomographic diagnosis]. / Lipomes duodénaux multiples. Un diagnostic scanographique.

In a 65-year-old man, upper endoscopy revealed several polypoid lesions into the duodenum, for which histologic examination of the biopsy specimens showed normal mucosa. CT studies demonstrated homogeneous fat density of these nodules, and thus were diagnostic of duodenal lipomas. The diagnosis was ultimately histologically confirmed by deeper peri-endoscopic biopsies. About this case and the some previously reported ones, the authors emphasize the interest of CT examination in this small bowel tumor.

[Real-time ultrasonographic study of the parathyroid glands. Apropos of 100 explorations]. / Etude ultrasonographique temps réel des parathyroïdes. A propos de 100 explorations.

The authors using high resolution (8 MHz) real-time echotomography have explored 100 patients. They certify that the presence of little hypoechogenic spicules at the periphery of a mass is strongly in relation with a parathyroid. They found in 17 patients signs of hypertrophic parathyroid; 8 of these had surgery with very good results for echotomography in 7 cases. Localizing parathyroid masses before surgery did not shorter time of exploration in these 8 cases. Ultrasonography, if positive, may relate biological troubles to a parathyroid etiology. It appears, that echotomography is a method of choice to survey chronic hemodialysis.

[Gallbladder atony and celiac disease in adults. Radiographic and echographic study of 15 cases]. / Atonie vésiculaire et maladie coeliaque de l'adulte. Etude radiographique et échographique de 15 cas.

Gallbladder emptying was evaluated in 15 adult celiac disease patients by oral cholecystography or ultrasonography, after fatty meal or cholecystokinin stimulation. Gallbladder inertia was found in 13 cases. Our study agrees with previously reported results; however it is the first one in which this abnormality was demonstrated by ultrasonography. Our findings may suggest that gallbladder inertia is due to duodenal release of an inactive endogenous cholecystokinin. Control studies demonstrates that gallbladder inertia is reversible after gluten-free diet, recurs with relapse, and thus represents a true celiac disease sign, and not an associated condition. Finally, our cases suggest that gallbladder inertia may be radiologically evident before clinical features of malabsorption become apparent. Awareness of this possibility may persuade the radiologist to research in this condition an occult celiac disease.

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.