Congenital hepatic fibrosis in Jamaican children.

In 1961, Kerr, Harrison, Sherlock, and Walker described a series of children, all but one under 10 years ofage, whose main features were hepatosplenomegaly or bleeding oesophageal varices. They reviewed the published material fully, and differentiated their cases from 'juvenile cirrhosis' following neonatal hepatitis, blood group incompatibility, Wilson's disease, and fibrocystic disease of the pancreas. They credited MacMahon (1929) with being the first to have recognized a fibrotic change in the liver, distinct from cirrhosis and not usually associated with disease of the pancreas. The condition accounted for a significant proportion of portal hypertension seen in children and young adults. The clinical course was more benign than that of cirrhosis, there was no jaundice, and liver function tests were normal. They proposed the name 'congenital hepatic fibrosis', subscribing to the views of MacMahon (1955) and Parker (1956), that the condition was congenital and that the fundamental anomaly was an excess of fibrous tissue associated with an abnormality of portal veins. Other authors who have been mainly concerned with the condition in adults (Moschcowitz, 1906; Norris and Tyson, 1947; Comfort, Gray, Dahlin, and Whitesell, 1952; Melnick, 1955) had emphasized the presence of cystic elements, and the association with polycystic kidneys and other congenital abnormalities. Since 1961, we have diagnosed this condition either clinically or at necropsy, in 8 young children. Since they presented features not much emphasized previously and we have been able to perform chromosome studies in 3, we here present the clinicopathological data on these cases.