[Liver transplantation for intrahepatic Rendu-Osler-Weber's disease: the Paul Brousse hospital experience].
Nine centers have reported 15 cases of liver transplantation for Rendu-Osler-Weber's disease with liver involvement. Six cases are reported to analyse the specific technical and hemodynamical aspects.


METHODS
Five women and 1 man were transplanted for Rendu-Osler-Weber's disease. The clinical presentation was biliary disease in 3 cases, portal hypertension in 2 cases, cardiac failure in 1 case. Systemic hemodynamics were assessed at the beginning and at the end of the transplantation procedure.


RESULTS
The procedure lasted from 11 to 15 hours (median=13 hours and 15 minutes). Blood transfusion during the procedure varied from 16 to 88 blood units (median=59 blood units). Six patients had hyperkinetic syndrome at the beginning of the procedure. At the end of transplantation, mean arterial pressure significantly increased (from 66 +/- 2 to 72 +/- 6 mmHg, p<0.05), whereas cardiac output (from 9.2 +/- 3.0 à 5.7 +/- 0.5 L/mn, p<0.05) significantly decreased. Two patients died at D2 and D11 and 4 are alive 3 to 7.5 years (median=4 years 9 months) after transplantation with a normal liver function and without any cardiac symptoms.


CONCLUSIONS
Liver transplantation for Rendu-Osler-Weber's disease is a difficult procedure. When successful, liver transplantation is curative of both the liver disease and the hyperkinetic state.
Azoulay
Daniel
D
Département de Chirurgie Hépato-Biliaire, Hôpital Paul Brousse, Université Paris-Sud, UPRES 1596, IFR 89.9, Villejuif, France. daniel.azoulay@pbr.ap-hop-paris.fr
Precetti
Sophie
S
Emile
Jean-François
JF
Ichai
Philippe
P
Gillon
Marie-Christine
MC
Duclos-Vallée
Jean-Charles
JC
Visda
Selim
S
Adam
René
R
Castaing
Denis
D
Samuel
Didier
D
Bismuth
Henri
H
D000328:Adult; D000368:Aged; D002761:Cholangitis / Q000209:etiology / Q000601:surgery*; D017809:Fatal Outcome; D005260:Female; D005602:France; D006333:Heart Failure / Q000209:etiology / Q000601:surgery*; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D008875:Middle Aged; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000601:surgery*; D016896:Treatment Outcome