Congenital Hepatic Fibrosis

Abstract

The name, “congenital hepatic fibrosis”, suggested by Kerr and colleagues in 1961 [1], describes the condition well but fails to indicate its relationship to cystic disease of the liver and kidney. The lesion was first described in 1929 by MacMahon [2] as a rare curiosity. Grumbach et al. related it to portal hypertension in 1954 [3]. Six patients were discussed by Parker in 1956 [4], and Kerr et al. described 13 cases in 1961 [1]. By 1965, McCarthy et al. were able to collect 84 cases from the literature and added ten of their own from the files of the Mayo Clinic [5]. An entire issue of Revue Internationale d’Hepatologie in 1964 is devoted to various articles about the disorder [6]. Excellent articles were published by Fauvert et al. in 1964 [7] and by Murray-Lyon and colleagues in 1973 [8]. Summerfield et al. described 27 patients from the records of the Royal Free Hospital in London in 1986 [9].