Liver resection in patients with hepatic hereditary hemorrhagic telangiectasia.
BACKGROUND
Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Rendu-Weber disease, is a rare genetic disorder characterized by widespread telangiectasia and vascular malformations involving the liver in most of the cases. The consequences of this pathologically underlying parenchyma on liver resection have been poorly described.


METHODS
More than 2,000 liver resections were performed at our institution over a 14-year period, whereby 2 major hepatectomies for malignancy were performed on patients with HHT with liver involvement. In addition, a systematic search was performed in the PubMed database to identify all original articles on hepatectomy in patients with HHT.


RESULTS
The first patient underwent a left hepatectomy for cholangiocarcinoma with an uneventful postoperative course. The second patient underwent right hepatectomy and segment 3 resection for colorectal liver metastases. The postoperative course was marked by ascites without liver failure. For both patients, 90-day mortality was nil.


CONCLUSIONS
In selected HHT patients with liver involvement, liver resections, including major hepatectomies, can be safely performed. Specific attention should be paid to postoperative liver function and ascites.
Department of Hepato-Pancreato-Biliary Surgery, Pôle des Maladies de l'Appareil Digestif (PMAD), AP-HP, Beaujon Hospital, Clichy, France.
Sébastien
S
Gaujoux
Margot
M
Bucau
Maxime
M
Ronot
Valerie
V
Paradis
Valerie
V
Vilgrain
Jacques
J
Belghiti
D000328:Adult; D006528:Carcinoma, Hepatocellular / Q000150:complications / Q000473:pathology* / Q000601:surgery; D018281:Cholangiocarcinoma / Q000150:complications / Q000473:pathology* / Q000601:surgery; D015179:Colorectal Neoplasms / Q000150:complications / Q000473:pathology; D005260:Female; D006498:Hepatectomy / Q000379:methods*; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications / Q000473:pathology* / Q000556:secondary* / Q000601:surgery; D008875:Middle Aged; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000473:pathology / Q000601:surgery*