Three patients with congenital hepatic fibrosis and childhood-type autosomal recessive polycystic kidney disease are reported. Portal hypertension in two of the children was decompressed surgically by lieno-renal shunting, and the renal failure in two children has been successfully treated with renal transplantation. Prophylactic porta-caval shunting followed by renal transplantation is ideally suited to the sequence of events occurring clinically in the intermediate form of this condition, preventing complications of bleeding from oesophageal varices and hyperplenism. The relationship of congenital hepatic fibrosis with the various forms of polycystic kidney disease is discussed and classified.
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McGonigle et al. (1981) studied this question.