Korean J Pathol.  1997 Mar;31(3):275-279.

Congenital Hepatic Fibrosis with Caroli's Disease

• Kim YJ
• Oak SA
• Lee IC

Abstract

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.

Keyword

Congenital hepatic fibrosis; Caroli's disease

MeSH Terms

Bile
Bile Ducts
Bile Ducts, Intrahepatic
Caroli Disease*
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Esophageal and Gastric Varices
Female
Fever
Fibrosis*
Headache
Humans
Kidney
Liver
Liver Function Tests
Liver Transplantation
Tomography, X-Ray Computed
Young Adult