All issues > Volume 45(7); 2002

Case Report

J Korean Pediatr Soc. 2002;45(7):923-927. Published online July 15, 2002.

A Case of Congenital Hepatic Fibrosis Accompanied by Renal Tubular Ectasia, Caroli Syndrome and Choledochal Cyst

Bong Seok BS Choi¹, Sang Nam SN Bae¹, Yong Tak YT Im¹, Jae Hong JH Park¹, Chang Hoon CH Lee², Jun Woo JW Lee³

¹Department of Pediatrics, Collage of Medicine, Pusan National University, Pusan, Korea
²Department of Pathology, Collage of Medicine, Pusan National University, Pusan, Korea
³Department of Diagnostic Radiology, Collage of Medicine, Pusan National University, Pusan, Korea

Correspondence Jae Hong JH Park ,Email: minambong@hanmail.net

Abstract

Congenital hepatic fibrosis is a relatively rare disease, characterized by bile ductular proliferation and prominent fibrosis in the portal area of liver resulting in portal hypertension. It is frequently associated with other abnormalities such as polycystic kidney, Caroli syndrome, cystic dysplasia of pancreas, intestinal lymphangiectasia, pulmonary emphysema, hemangioma, and cleft palate. We report here a case of congenital hepatic fibrosis associated with renal tubular ectasia in a 3-year- old girl, whose chief complaint was abdominal distension. Her liver function test did not reveal any abnormal findings. Hepatosplenomegaly and multiple dilated bile ducts were seen in the abdominal CT scaning. Esophageal varix was not detected by an endoscopic examination. Microscopically, diffuse portal fibrosis and widening with proliferation of blie ductules in the liver specimen and tubular ectasia in renal cortex were seen.

Keywords :Congenital hepatic fibrosis, Renal tubular ectasia