Congenital hepatic fibrosis: the long-term prognosis.

Abstract

SUMMARY The long-term prognosis in congenital hepatic fibrosis has been assessed in 30 patients, 13 treated at Newcastle and 17 from other British centres. Twenty-four patients had been followed-up for more than five years from diagnosis. Shunt operations, performed in 18 patients, were successful in controlling haemorrhage with alow mortality (1/18), low incidence of recurrent haemorrhage (3/18) and portal-systemic encephalopathy (3/18) but with a higher incidence of postoperative jaundice (7/18). About a third of the survivors had some evidence of hepatic dysfunction but none had chronic portal systemic encephalopathy. Intelligence quotient was close to normal but educational achievement and job status were low. Serum albumin fell after shunt surgery and a few patients developed ascites: anaemia and hypersplenism however, were corrected by surgery. Blood ammonia is raised in survivors with congenital hepatic fibrosis and rises further after a normal meal; further observations are needed on cerebral function after several decades of survival.

Correlation of Kidney Function, Volume and Imaging Findings, and PKHD1 Mutations in 73 Patients with Autosomal Recessive Polycystic Kidney Disease

“…All ARPKD patients manifest some degree of congenital hepatic fibrosis (CHF) caused by ductal plate malformation of the developing portobiliary system; some patients also have macroscopic dilations of the intrahepatic bile ducts, a combination termed Caroli's syndrome (7,13,14). Portal hypertension complicates CHF and often results in esophageal varices and hypersplenism (15)(16)(17)(18). Early-onset severe hypertension, often requiring multiagent therapy, occurs in most ARPKD patients (5).…”

Congenital hepatic fibrosis

“…With the progression of portal hypertension, the spleen increases and the platelet count decreases due to hypersplenism, developing portosystemic collateral circulation and creating oesophageal and gastric varices. The risk of bleeding increases with the increase in varice size [4,5,6]. Variceal bleeding can occur at any age, but is more common in older children and adults.…”

Congenital Hepatic Fibrosis: A Study of a Saudi Family and Review

“…In 1978, Kerr et al reported on the long-term prognosis of 30 patients with CHF from the United Kingdom, 24 of whom had been followed up for more than five years from diagnosis [22]. Shunt operations, performed on 18 patients, were successful in controlling hemorrhage and reducing recurrences with low incidence of PSE.…”

“…Surgical portacaval shunt can also be considered. Portasystemic encephalopathy (PSE) and liver failure following a shunt procedure are usually less common in CHF than in those with cirrhosis [21,22].…”