[Cystic dilatation of the intrahepatic bile ducts (Caroli's disease)]

Congenital cystic dilatation of the intrahepatic biliary ducts, known as Caroli's disease, is rarely recognized in general surgical practice and often overlooked. Until now little more than a hundred cases have been described although modern diagnostic procedures disclose more frequently new ones. Three patients with this anomaly have been treated by the authors during the past years and the different aspects concerning diagnosis and treatment are discussed and compared with existing information. In all three patients colic pain in the upper abdomen was the first symptom reported in the first years of life. In one patient jaundice and fever were associated with the abdominal pain and cholangitis developed a secondary biliary cirrhosis. Somatic underdevelopment, consequence of the cirrhosis in this child was observed. In two patients an intrahepatic hepaticojejunostomy was performed and a left hepatectomy in the third. In the two older children intrahepatic gallstones were found; in the younger one no stones were found inside the dilated ducts.