Successful Treatment of Caroliʼs Disease by Hepatic Resection

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Abstract
Caroli''s disease is a congenital disease of cystic or saccular dilatation of the intrahepatic bile ducts. There are 2 disease entities: a simple type and a periportal fibrosis type. Frequent complications with the simple type are recurrent cholangitis, liver abscess, intraductal lithiasis, abdominal pain and fever that often lead to fatal sepsis. Development of portal hypertension and esophageal varices is usually a final feature of the periportal fibrosis type. Malignancies are also possible complications with Caroli''s disease. During the recent 13 yr, the author had experiences with 8 patients with Caroli''s disease of the simple type; 6 of these 8 underwent hepatic resection: right lobectomy in 2, left lobectomy in 3, and left lateral segmentectomy in 1. The other 2 patients died of sepsis and cholangiocellular carcinoma, respectively. All 6 patients with hepatic resections were relieved from the disabling symptoms after surgery and have had no recurrent hepatobiliary problems for 3 mo. to 13 yr. Hepatic resection may be indicated for more patients than previously assumed in the treatment of Caroli''s disease of the simple type.

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