CONGENITAL CYSTIC DILATATION OF THE INTRA-HEPATIC BILE-DUCTS (CAROLIS DISEASE) - REPORT OF A CASE AND REVIEW OF THE LITERATURE

  • 1 January 1979
    • journal article
    • research article
    • Vol. 85  (5) , 589-592
Abstract
A case of congenital cystic dilatation of the intrahepatic bile ducts (Caroli''s disease) followed for more than 7 yr is reported. This patient presented also with congenital hepatic fibrosis, gallstones, and biliary hypersecretion of more than 3000 ml in 24 h. An analysis of the literature relating to Caroli''s disease disclosed 46 well-documented cases of both hepatic histopathology and biliary tree studies. Six cases (13%) were isolated forms of intrahepatic cystic dilatations; 16 (34.7%) were associated with congenital hepatic fibrosis; and 10 (21.7%) presented with either a choledochal cyst or nonobstructive extrahepatic biliary tree dilatation. In 14 cases (30%), the 3 anomalies were found together in the same patient. Congenital hepatic fibrosis, congenital cystic dilatations of the intrahepatic bile ducts (Caroli''s disease), choledochal cyst and other nonobstructive dilatations of the extrahepatic biliary tree are possibly the same congenital disease with different levels of involvement.

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