TY - JOUR
T1 - Orthotopic liver transplantation for idiopathic portal hypertension
T2 - Indications and outcome
AU - Dumortier, J.
AU - Bizollon, T.
AU - Scoazec, J. Y.
AU - Chevallier, M.
AU - Bancel, B.
AU - Berger, F.
AU - Ducerf, C.
AU - Claudel-Bonvoisin, S.
AU - Paliard, P.
AU - Boillot, O.
AU - Trepo, C.
PY - 2001/1/1
Y1 - 2001/1/1
N2 - Background: Idiopathic portal hypertension is a rare clinical syndrome which may be associated with a spectrum of histological lesions, including nodular regenerative hyperplasia and incomplete septal cirrhosis. Here, we report 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. Our aims are: (a) to stress the distinctive clinical presentation of these patients, (b) to describe their biological and histopathological features, and (c) to evaluate the results of orthotopic liver transplantation in this rare indication. Methods: Complete clinical charts and histological data were 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 and portosystemic shunt in three. Results: The development of progressive hepatic failure led to the indication of liver transplantation after a delay ranging from 3 to 10 years. Explanted livers showed pure nodular regenerative hyperplasia in three patients and incomplete septal cirrhosis in five. Recovery was uneventful. All patients are alive, without recurrence of the disease. 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.
AB - Background: Idiopathic portal hypertension is a rare clinical syndrome which may be associated with a spectrum of histological lesions, including nodular regenerative hyperplasia and incomplete septal cirrhosis. Here, we report 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. Our aims are: (a) to stress the distinctive clinical presentation of these patients, (b) to describe their biological and histopathological features, and (c) to evaluate the results of orthotopic liver transplantation in this rare indication. Methods: Complete clinical charts and histological data were 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 and portosystemic shunt in three. Results: The development of progressive hepatic failure led to the indication of liver transplantation after a delay ranging from 3 to 10 years. Explanted livers showed pure nodular regenerative hyperplasia in three patients and incomplete septal cirrhosis in five. Recovery was uneventful. All patients are alive, without recurrence of the disease. 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.
KW - Idiopathic portal hypertension
KW - Incomplete septal cirrhosis
KW - Liver transplantation
KW - Nodular regenerative hyperplasia
UR - http://www.scopus.com/inward/record.url?scp=0035078732&partnerID=8YFLogxK
U2 - 10.1080/003655201300051298
DO - 10.1080/003655201300051298
M3 - Article
C2 - 11336168
AN - SCOPUS:0035078732
SN - 0036-5521
VL - 36
SP - 417
EP - 422
JO - Scandinavian Journal of Gastroenterology
JF - Scandinavian Journal of Gastroenterology
IS - 4
ER -