Caroli's disease: A case report

Caroli's disease, also known as congenital communicating cavernous ectasia of the biliary tree, is a rare autosomal recessive developmental abnormality characterized by saccular dilatation of the intrahepatic bile ducts (1). Caroli's disease is a rare congenital disorder, and occasional cases have been reported from Japan and other parts of Asia (2). We present our experience with an unusual case of Caroli's disease. INTRODUCTION: Jacques Carole in 1958 first described a rare disease with multifocal, segmental, saccular dilatation of the large intrahepatic bile ducts, which causes stagnation of bile formation of biliary sludge and stones .(3) Etiology- unknown, Occurrence- sporadic, Caroli's disease results in recurrent cholangitis, abdominal pain and hepatic abscesses. Diagnosis- confirmed by ERCP and percutaneous transhepatic cholangiography. Purpose of treatment is to restore normal bile flow. Prognosis is poor despite drainage of bile and patients die from sepsis, hepatic abscess, hepatic failure or portal hypertension. Mortality is indirect and caused by complications. After cholangitis occurs, patients typically die within approximately 5-10 yrs . (4) We report a case of 30 yr female who was diagnosed as Caroli's disease. Most of the reported cases in literature are in pediatric age group. HISTORY: A 30 year old female presented with chief complaints of intermittent fever since 2 months, decreased appetite and vomiting also of same duration. She gave no history of hematemeses, malena or abdominal distension. She had not received any blood transfusion in the preceding months. Patient was diagnosed with Rheumatoid arthritis 11 years ago, but she was asymptomatic at presentation. There was no past history of diabetes mellitus, IHD or bronchial asthma. No one else in the family had similar illness