Caroli's disease: an ultrasonic diagnosis

Abstract

Congenital dilatation of the intrahepatic bile ducts (Caroli's disease) was first reported in 1958 (Caroli et al.) and is usually diagnosed late in the patient's illness and seldom before operation (Watts et al., 1974). Goddard (1974) could only find 16 previous case reports of the disease which attests to its rarity. Caroli (1968) has classified congenital intrahepatic cystic dilatation into two groups: first, a pure form not asociated with hepatic cirrhosis or portal hypertension and second, a type associated with congenital hepatic fibrosis. There is probably some overlap but Watts et al. (1974) consider most cases belong to the first group. The radiological diagnosis of Caroli's disease has, in the past, proven difficult. None of the 15 cases collected by Mujahed et al. (1971) were diagnosed before surgery. With improved modern techniques in organ visualization, the pre-operative diagnosis has become easier and we report the following case which was confidently diagnosed by ultrasonography and confirmed by transhepatic cholangiography, thus rendering invaluable assistance to the surgeon. A female of 19 years was first admitted to hospital in December 1975 because of increasing jaundice and rigors. Her clinical history was unremarkable except for her occupation as an assistant in a hospital sterilizing department and handling liver biopsy packs. Her total bilirubin was 5 mg% (conjugated level 3.8 mg%) and her alkaline phosphatase was 132 international units.