Caroli Syndrome in a Child: A Case Report

Caroli syndrome is a congenital disorder characterized by multiple segmental or saccular dilatations of the intrahepatic bile ducts associated with congenital hepatic fibrosis.

A 3-year-old boy with abdominal distention was referred to gastroentrology ward of Amiralmomenin hospital (Semnan, Iran) in summer 2018. In his abdominal sonography, multiple cysts were detected in the liver with hepatomegaly, and the portal vein pressure was 10 mm. Also, in liver biopsy, dilated portal bile ducts (trichrome stain) with inspissated bile and congenital hepatic fibrosis were reported. He was discharged after conservative therapy and followed up.

Definitive treatment, i.e surgery, should be offered to prevent future complications.