Caroli's syndrome: a heterogeneous entity.

We report our experience with nine adult patients (six females, three males) with Caroli's syndrome. Most of these patients were young (mean age 27.0 yr), and had presented at a mean interval of 12.1 yr after the onset of symptoms. Recurrent abdominal pain was the most common symptom, present in eight of nine patients. Mild jaundice (serum bilirubin 1.8-4.2 mg/dl) and raised serum alkaline phosphatase (18-36 King-Armstrong units) were the only other significant findings. Ultrasound proved to be a good screening test. However, the diagnosis was confirmed on cholangiography. Six of nine patients had associated extrahepatic biliary cysts (type IVa). The operative management was directed mainly at excising the extrahepatic cysts and draining the biliary tree, and was met with satisfactory results.