J Korean Pediatr Soc.
2002 Jul;45(7):923-927.
A Case of Congenital Hepatic Fibrosis Accompanied by Renal Tubular Ectasia, Caroli Syndrome and Choledochal Cyst
- Affiliations
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- 1Department of Pediatrics, Collage of Medicine, Pusan National University, Pusan, Korea. minambong@hanmail.net
- 2Department of Pathology, Collage of Medicine, Pusan National University, Pusan, Korea.
- 3Department of Diagnostic Radiology, Collage of Medicine, Pusan National University, Pusan, Korea.
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.
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