Left extended hepatectomy with biliary resection and reconstruction for hilar cholangiocarcinoma in patient with Osler-Rendu-Weber disease: a case report and review of literature

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Osler-Rendu-Weber syndrome is a genetic disease that involves organs, liver included, characterized by alterations in the vessel walls, making them more vulnerable to spontaneous rupture and bleeding indeed. Our aim is to report a case of patient with Osler-Rendu-Weber syndrome undergoing extended hepatectomy with biliary resection for hilar cholangiocarcinoma and a review of literature on liver resection performed in patients with this syndrome. Preoperative, intraoperative, postoperative, radiographic, and pathologic data of case report's patient were collected. Review of literature included studies from 2000 to 2024, searching them with following search keywords: (liver resection OR hepatectomy) AND (Osler-Rendu-Weber disease OR hereditary hemorrhagic telangiectasia). A 78-year-old woman with Osler-Rendu-Weber syndrome presented hilar lesion compatible with cholangiocarcinoma. Before surgery, the patient underwent embolization of an aneurysm in segment 6. A left extended hepatectomy with biliary resection was performed. Intraoperative blood loss was 500 cc. Post-operative course was uneventful and length of hospital stay was 10 days. 5 cases of liver resection in patient with this syndrome are reported in literature, including 2 cases of major hepatectomies. Major complications' rate was 60% (3 cases): two cases of post-operative bleeding and one case of ascites decompensation. In one case exitus, consequent to massive bleeding, was reported (20%). This is the first case of extended hepatectomy with biliary resection performed in patient with Osler-Rendu-Weber syndrome. This underlying condition makes surgical approach demanding and challenging also in high volume centers. Proper patient selection and management could allow treatment and execution of a safe liver resection in patients with this syndrome.

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