Congenital Hepatic Fibrosis with Caroli's Disease.
- Author:
Yoon Jung KIM
;
Soon Ae OAK
;
In Chul LEE
- Publication Type:Case Report
- Keywords:
Congenital hepatic fibrosis;
Caroli's disease
- MeSH:
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
- From:Korean Journal of Pathology
1997;31(3):275-279
- CountryRepublic of Korea
- Language:Korean
-
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.
