[Liver transplantation for intrahepatic Rendu-Osler-Weber's disease: the Paul Brousse hospital experience]

Abstract

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.

Patients and 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.

Conclusion: 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.