Liver transplantation using a whole graft from a deceased donor affected with Osler–Weber–Rendu syndrome: a first ever

Abstract

Dear Editors-in-Chief, The critical use of extended criteria donor (ECD) grafts has allowed patients on the waiting-list for liver transplantation (LT) to have a greater chance of being transplanted for end-stage liver disease and/or primary hepatic tumor. Osler–Weber–Rendu syndrome, or hereditary hemorrhagic telangiectasia (HHT), is a rare autosomal dominant genetic vascular disease characterized by arteriovenous malformations (AVMs) and pathological angiogenesis causing a fibro-vascular systemic dysplasia, fragility of the vascular wall, and dilatation and consequent rupture of the vessels. We report the first LT recipient who received a whole liver graft from a young brain-dead donor affected with HHT to introduce a broader concept in this field of medicine in which HHT is usually a primary etiology for liver transplant [1–3]. The organ donor was a 30-year-old, blood group A male, who suffered from a spontaneous brain hemorrhage due to cerebral AVM rupture 2 weeks before the evidence of brain death. The donor after brain death (DBD) was affected with HHT, with evidence of mucocutaneous telangiectasias, recurrence of epistaxis, and family history of HHT in a firstdegree relative. Except for this congenital disorder, there was no other relevant past medical history, and he was on no medications. During the intensive care unit hospitalization, the DBD underwent a digital angiographic evaluation of the brain and a diagnosis of cerebral AVM near the splenium of the corpus callosum with radiological evidence of a pathological blush with the presence of a plexiform nidus of about 3 cm of maximum diameter and a single ectatic venous collector near the straight sinus. Computed tomography total body scan and hepatic Doppler ultrasonography excluded any signs of diffuse small telangiectasias or large AVMs of the liver [2]. Following an attempt at decompressive hemicraniectomy, further clinical deterioration led to confirmation of brain death; organ procurement was performed on January 18th, 2019. The procurement resulted in three solid organ transplants: two kidneys and liver. No macroscopic anomalies were detected during the surgical procedures, nor did histological examination revealed any microscopic pathologic features of the hepatic parenchyma (Fig. 1Panel a). The recipient of the donated liver was a 63-year-old male with end-stage liver disease secondary to non-alcoholic steatohepatitis, and previous history of recurrent gastrointestinal bleeding secondary to F3 esophageal varices already treated with transjugular intrahepatic portosystemic shunt in February, 2017. Before LT, the clinical status was characterized by a Model of End-Stage Liver Disease Score of 16, and a Child–Turcotte–Pugh Score of B8 due to refractory and Electronic supplementary material The online version of this article (https ://doi.org/10.1007/s1330 4-019-00676 -7) contains supplementary material, which is available to authorized users.