Korean J Pediatr Gastroenterol Nutr.  2004 Mar;7(1):98-101.

A Case of Congenital Hepatic Fibrosis with Variceal Bleeding

• Shin DS
• Lim SH

Abstract

Congenital hepatic fibrosis is an inherited, congenital disorder of the liver characterized by portal hypertension and hepatic fibrosis. We experienced a case of congenital hepatic fibrosis with esophageal varix in a 9-year-old male. He complained hematemesis, hematochezia, dizziness. In laboratory examination, AST/ALT was slightly increased. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and hypoechoic lesion of periportal area were seen by abdominal CT scanning. Histologic finding of liver biopsy showed fibrous tracts containing dilated bile ductules connecting adjacent portal spaces that were widened by mature fibrosis. Endocopic sclerotherpy and ligation was done. We summarized a case with review of literatures.

Keyword

Congenital hepatic fibrosis; Esophageal varix

MeSH Terms

Bile
Biopsy
Child
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Dizziness
Esophageal and Gastric Varices*
Fibrosis*
Gastrointestinal Hemorrhage
Hematemesis
Humans
Hypertension, Portal
Ligation
Liver
Male
Tomography, X-Ray Computed