Korean J Hepatol.  2001 Dec;7(4):485-490.

Two Cases of Congenital Hepatic Fibrosis with Polycystic Kidney Disease

• Lee DH
• Kong ON
• Kim JY
• Park CW
• Moon JH
• Nam DG
• Chu HJ
• Kang DH
• Song GA
• Cho M
• Yang US

Abstract

Congenital hepatic fibrosis (CHF) is a rare developemental abnormality, which is characterized pathologically by periportal fibrosis with irregularly shaped proliferating bile ducts. In most, if not all, cases CHF is associated with autosomal recessive polycystic kidney disease. Recently, we experienced two cases, confirmed by percutaneous needle liver biopsy, of CHF with polycystic kidney disease. The first patient was a 19-year-old man and presented with hematemesis and hepatosplenomegaly. Esophageal varix was noted by an endoscopic examination and an endoscopic variceal ligation was performed. Abdominal CT scanning revealed innumerable cysts of both kidneys. The pateint also had cystic dilation of subarchnoid space in the basal cistern and posterior fossa detected through brain MRI. The second patient was a 24-year-old man admitted for an evaluation of splenomegaly. He had no esophageal varix but, splenic varix and splenorenal shunt were detected through an abdominal CT scanning. Innumerable renal cysts were also present. The diagnosis of CHF was confirmed in both cases by its typical histologic features. We report these cases with a review of the relevant literatures.

Keyword

Congenital hepatic fibrosis; Portal hypertension; Polycystic kidney disease; Achacnoid cyst

MeSH Terms

Bile Ducts
Biopsy
Brain
Diagnosis
Esophageal and Gastric Varices
Fibrosis*
Hematemesis
Humans
Hypertension, Portal
Kidney
Ligation
Liver
Magnetic Resonance Imaging
Needles
Polycystic Kidney Diseases*
Polycystic Kidney, Autosomal Recessive
Splenomegaly
Splenorenal Shunt, Surgical
Tomography, X-Ray Computed
Varicose Veins
Young Adult