2368 A Rare Case of Segmental Arterial Mediolysis in a Patient With Alpha-1 Antitrypsin Deficiency and Alcoholic Cirrhosis

Abstract

INTRODUCTION: Alpha-1 antitrypsin deficiency (AAT deficiency) is a systemic disease characterized by lack of the protease inhibitor, alpha-1 antitrypsin and typically manifests as severe emphysema and/or liver disease. There are several case reports describing vascular compromise in patients with AAT deficiency, however mostly in the context of systemic vasculitis and connective tissue disease. Segemental arterial medioloysis (SAM) is a rare non-vasculitis, non-atherosclerotic arteriopathy of middle and small arteries that can lead to aneurysmal dilation or arterial stenosis. We report the first case of a patient with cirrhosis secondary to AAT deficiency noted to have arteriopathy due to SAM. CASE DESCRIPTION/METHODS: A 30 year old patient with end stage renal disease (ESRD) on hemodialysis, alcoholic cirrhosis and underlying AAT deficiency presented to our tertiary care center for liver transplant evaluation with a presenting MELD score of 39. Evaluation for other causes of cirrhosis revealed AAT deficiency with a homozygous ZZ phenotype and undetectable serum levels. As part of his pre-transplantation workup at our institution, he underwent computed tomography angiography (CTA) of the abdomen and pelvis which revealed severe irregular narrowing of the celiac axis. Similar narrowing was noted of the renal arteries and splenic artery and focal aneurysmal dilation of the hepatic and gastroduodenal arteries was additionally seen (Image 1). Autoimmune serologies were negative. Patient proceeded to orthotropic liver transplantation during his hospitalization. Intraoperatively, hepatic artery appeared visually vasculitic but histopathologic report was consistent with diagnosis of SAM. Due to concern for potential graft compromise that could result from the recipient hepatic artery aneurysm, an aortic conduit was performed using the donor vessels with anastomosis to the recipient supraceliac aorta. DISCUSSION: To our knowledge, there is only one other published report describing SAM in a patient with co-existing AAT deficiency. SAM in this case resulted in hepatic aneurysm which complicated liver transplantation, necessitating alternative graft revascularization via an aortic conduit. Although doppler evaluation 3 days after transplantation showed hepatic artery anastomosis was still patent, the rate of complications related to aorto-hepatic bypass grafts is high, and close follow up remains prudent.