[Diagnosis and surgical therapy in congenital ectasias of the intra- and extrahepatic bile ducts].

: 31 patients with congenital ectasias of extra- and intrahepatic bile ducts have been diagnosed and treated between 1980 and 1991 at the Charite. 7 ectasias belonged to type I, two to type II, three to type III, four to type IV and fifteen to type V of classification by Alonso-Lej/Todani. The age of affection was between 8 and 80 years. Symptoms are not specified and directed to affection of liver or bile ducts. Main symptoms of type I--III were complaints in the upper abdomen and jaundice, of type IV--especially after previous surgery--fever and also jaundice. Complaints in the upper abdomen and fever dominated in type V. The right diagnosis succeeds at 27 out of 31 patients with direct cholangiography. Sonography and computerized tomography were not always in a position to attach the clinical picture to congenital ectasias. In extrahepatic ectasias is the resection of bile duct cysts--carried out in 2/3 of all cases--the therapy of choice. Once was found a carcinoma in a resected bile duct cyst 23 years after a choledochocysto-jejunostomy. By Caroli syndrome the therapy must be fixed individually. 6 patients were supplied operative (liver resection, choledochotomy, papillotomy, extraction of lithiasis). Instrumentally were carried out 9 endoscopic papillotomies with extraction of concrements and 6 lay ins of internal drainage catheters were done.