Liver Transplantation Using a Whole-Liver Graft Donated from a Brain-Dead Donor Complicated with Rendu–Osler–Weber Syndrome: A Case Report

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INTRODUCTION: Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia (HHT), is a rare autosomal dominant genetic vascular disease characterized by arteriovenous malformations, vascular wall fragility, dilatation, and rupture of the vessels with hepatic symptoms. As HHT with hepatic symptoms is recognized as the primary etiology for liver transplantation, liver transplantation with liver grafts from donors affected by HHT is extremely rare. Herein, we report a successful liver transplantation in a patient with biliary atresia who received a whole-liver graft from a young brain-dead donor with HHT. CASE PRESENTATION: The patient was a 15-year-old girl with decompensated liver cirrhosis who underwent Kasai surgery for biliary atresia at 3 months of age. The donor was a female in her teens, diagnosed with brain death due to cerebral hemorrhage. Although the donor was diagnosed with Osler disease, she had no hepatic symptoms and normal liver function. CT did not reveal any apparent vascular malformations in the liver. A whole-liver transplant was performed using the donated liver. The patient recovered well in terms of liver function, without any hepatic-related symptoms. CONCLUSIONS: Our experience with this patient may have important implications for liver transplantations from donors with HHT.

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