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The Korean Journal of Pathology 1997;31(3): 275-279.
Congenital Hepatic Fibrosis with Caroli's Disease.
Yoon Jung Kim, Soon Ae Oak, In Chul Lee
Departmemt of Pathology, Asan Medical Center College of Medicine, University of Ulsan, Seoul 138-040, Korea.
Congenital hepatic fibrosis is an inherited, congenital disorder of the liver, and is occasionally associated with cystic disease of the liver and kidney. We present a case of congenital hepatic fibrosis with Caroli's disease. A 21-year-old woman had suffered from an episodic fever with headaches for 3 years. In laboratory examination, the liver function test was within the normal limits. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and multiple dilated bile ducts were seen by abdominal CT scanning. An orthotopic whole liver transplantation was done. The liver was fibrotic and enlarged. Multiple cystically dilated intrahepatic ducts were noted. Microscopically, diffuse portal fibrosis and widening with proliferation of bile ductules were seen. Intrahepatic bile ducts were markedly dilated and tortuous. The liver cell cords were well preserved.
Key Words: Congenital hepatic fibrosis; Caroli's disease
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