Volume 23
Number 2 July 2014Kamal A H M1 , Sarker M A2 , Molla K H3 , Khuda K4
Abstract
Segmental, saccular or fusifonn non-obstructive dilatation of intrahepatic biliary channels is referred to as Caroli's disease (CD). Two forms of this rare congenital disorder are described. The 'pure'form which involves only the large intrahepatic bile ducts is less common. The more common 'complex'form, which is associated with congenital hepatic fibrosis (CHF) is designated as Caroli's syndrome (C5)1. The inheritance of Caroli's disease is uncertain but in majority of cases it is transmitted in autosomal recessive fashion23. CD is associated with renal involvement is about 60%, and it includes medullary sponge kidney disease or polycystic kidney disease°. Review of the literature suggests that there are no large series of this disorders.
Although the disease present from birth, it usually remains asymptomatic during the first 20 years, and may also remain so throughout the life. However when becomes symptomatic, a significant number of patients present significant loss of quality in their life and the clinical course of the disease frequently worsen
Keywords:
- Professor, Department of Medicine
- Professor, Department of Radiology and Imaging
- Assistant Professor Department of Medicine
- Resident Physician, Department of Medicine