title: [Current therapy of bile duct cysts. II. Intrahepatic cysts (Caroli syndrome)].
abstract: 17 patients with a Caroli's syndrome are reviewed with emphasis on clinical features and late results (follow-up: 2-10 years). The group consists of 9 women and 8 men ranging in age from 17 to 80 years (mean of 42.6). 14 patients had a diffuse form, 5 of whom had periportal fibrosis with secondary biliary cirrhosis, which in 3 cases resulted in a portal hypertension with hepatic failure. In only 3 cases the dilatation was limited to the left lobe of the liver. 9 of the patients had previously undergone surgery in the form of cholecystectomy and choledocholithotomy elsewhere. The disease was complicated by lithiasis (14 intrahepatic, 9 extrahepatic). Curative treatment was only possible in the 3 patients with unilobar Caroli's syndrome (partial liver resection). In 5 patients we performed a cholecystectomy and choledocholithotomy combined in 2 cases with a transduodenal sphincterotomy. There was no operative death. Endoscopic treatment consisted in removal of stones and decompression of the biliary tree by sphincterotomy in 9 cases and endoscopic retrograde biliary drainage (endoprosthesis) in 5 cases. Excepting the 3 curative operated patients who are asymptomatic respectively 2, 4 and 5 years after surgery, the remaining cases had repeated bouts of acute pains accompanied by recurrent episodes of cholangitis. A late mortality of 23.5% (4 patients) is proof of the poor prognosis of this disease.
authors.affiliation: klinik und Poliklinik f[r Chirurgie, Universitätsklinikums Charité der Humboldt-Universität Berlin.
authors.forename: T
authors.initials: T
authors.lastname: Benhidjeb
authors.forename: J M
authors.initials: JM
authors.lastname: Müller
authors.forename: K
authors.initials: K
authors.lastname: Gellert
authors.forename: J
authors.initials: J
authors.lastname: Zanow
authors.forename: B
authors.initials: B
authors.lastname: Rudolph
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016767:Caroli Disease / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D002763:Cholecystectomy; D005260:Female; D005500:Follow-Up Studies; D042882:Gallstones / Q000601:surgery; D006498:Hepatectomy; D006801:Humans; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000209:etiology; D016717:Sphincterotomy, Endoscopic; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
vernacular_title: Aktuelle Therapie der Gallengangscysten. II. Intrahepatische Cysten (Caroli-Syndrom)