Orthotopic liver transplantation for idiopathic portal hypertension: Indications and outcome

Background: Idiopathic portal hypertension is a rare clinical syndrome whic h may be associated with a spectrum of histological lesions, including nodu lar regenerative hyperplasia and incomplete septal cirrhosis. Here, we repo rt eight adult patients with idiopathic portal hypertension who experienced an unusually severe clinical evolution characterized by the development of progressive hepatic failure requiring orthotopic liver transplantation. Ou r aims are: (a) to stress the distinctive clinical presentation of these pa tients, (b) to describe their biological and histopathological features, an d (c) to evaluate the results of orthotopic liver transplantation in this r are indication. Methods: Complete clinical charts and histological data wer e available in all patients. All patients were male. Their age at diagnosis ranged from 17 to 59 years. Complications of portal hypertension revealed the disease in all cases. Medical treatment was performed in all patients a nd portosystemic shunt in three. Results: The development of progressive he patic failure led to the indication of liver transplantation after a delay ranging from 3 to 10 years. Explanted livers showed pure nodular regenerati ve hyperplasia in three patients and incomplete septal cirrhosis in five. R ecovery was uneventful. All patients are alive, without recurrence of the d isease. Conclusions: This report points to the existence of severe cases of idiopathic portal hypertension occurring without underlying or associated systemic disease and characterized by a poor clinical course and requiring liver transplantation.