First living donor liver transplantation for congenital hepatic fi brosis in Azerbaijan

Abstract

Congenital hepatic fibrosis is a rare autosomal recessive disease. Only a few such cases have been described worldwide, but the exact incidence of the disease is unknown. The diagnosis is sometimes difficult to establish and one of the main diagnostic method is the histological evaluation. The management and prognosis of congenital hepatic fibrosis is dependent on alimentary tract bleeding secondary to portal hypertension. In late childhood, the abdominal pain, cholangitis, and features of hypersplenism complicate the problem. Herein we present the case report of patient with congenital hepatic fibrosis. Our choice of treatment was the living donor liver transplantation. This procedure is very difficult but it is the only life-saving option for the patients with congenital hepatic fibrosis.