The name, “congenital hepatic fibrosis”, suggested by Kerr and colleagues in 1961 , 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  as a rare curiosity. Grumbach et al. related it to portal hypertension in 1954 . Six patients were discussed by Parker in 1956 , and Kerr et al. described 13 cases in 1961 . 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 . An entire issue of Revue Internationale d’Hepatologie in 1964 is devoted to various articles about the disorder . Excellent articles were published by Fauvert et al. in 1964  and by Murray-Lyon and colleagues in 1973 . Summerfield et al. described 27 patients from the records of the Royal Free Hospital in London in 1986 .
KeywordsBile Duct Portal Hypertension Hepatic Fibrosis Esophageal Varix Variceal Bleeding
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