Caroli's Disease in Two Sisters

Abstract

Caroli's disease is a rare congenital disease with multiple segmental dilatations of the bile ducts. It may present in two forms: one (the simple form) with segmental dilatations only, and the other with dilatations combined with fibroangioadenomatosis, frequently complicated by portal hypertension. The disease is thought to be recessively inherited, but few familial cases have been reported. We have observed two sisters with the disease, one with the simple form, the other with fibroangioadenomatosis. Their case histories and diagnostic findings, with special reference to ultrasonography and computed tomography, are presented, and the potential for diagnosis by different imaging modalities is discussed.