Porto Sinusoidal Vascular Disorder Manifesting as Diffuse Arterio-Portal Fistulae and Portal Hypertension After Donation-After-Circulatory-Death Liver Transplantation

ABSTRACT

Ischemic cholangiopathy (ICP) is a rare complication of orthotopic liver transplantation (LT), with an increased incidence in donation-after-circulatory-death (DCD) recipients, particularly without machine perfusion. As DCD utilization increases, challenges arise. We report a rare case of DCD-LT resulting in ICP, complicated by diffuse intrahepatic arterio-portal fistulae (APF) leading to portal hypertension and refractory ascites without any other cause. The candidate was relisted for LT, and the explant revealed diffuse intrahepatic APF and porto-sinusoidal vascular disorder (PSVD) without cirrhosis. To the best of our knowledge, no similar reports exist of LT recipients developing PSVD or diffuse APF, and their concurrence with ICP after DCD-LT raises concern for an association. This case report highlights the importance of considering diffuse APF and PSVD in LT recipients with ICP who develop features of portal hypertension without a clear cirrhotic etiology.