Intrahepatic cystic bile duct dilatations and stone formation: a new case of Caroli's disease
Mathias, K.; Waldmann, D.; Daikeler, G.; Kauffmann, G.
Acta Hepato-Gastroenterologica 25(1): 30-34
1978
ISSN/ISBN: 0300-970X PMID: 636738 Document Number: 127263
Caroli's disease is a rare familial anomaly with circumscribed saccular dilatations of enlarged intrahepatic bile ducts coated by regular or pseudo-papillomatous epithelium. The disease may be combined with additional malformations of the extrahepatic biliary tract as well as with medullary sponge kidney. Infection and stone formation are frequent complications. Surgery is indicated if the disease is limited to 1 lobe which should be resected or if biliary obstruction in diffuse disease necessitates biliodigestive anastomosis. Antibodies and CDCA -mediation are essential for symptomatic therapy. The findings in 40 cases were analyzed.