Recurrent Hepatocellular Carcinoma in an Adult With Alagille Syndrome Treated With Liver Resection Followed by Liver Transplantation: A Rare Case Report.

Abstract

Alagille syndrome (AGS) is a disorder that affects the liver, heart, kidneys, and skeleton. Development of hepatocellular carcinoma (HCC) is rare in AGS. A 41-y-old male with AGS presented with a 6 × 8-cm HCC and underwent transarterial chemoembolization (TACE) followed by right hepatic lobectomy. One year later, he developed HCC recurrence within Milan’s criteria and received a deceased donor liver transplant. An interposition donor iliac artery graft from the supraceliac aorta to the donor hepatic artery was needed due to celiac axis occlusion noted on TACE. He subsequently underwent a Roux-en-Y hepaticojejunostomy for a bile leak. Surveillance imaging for HCC revealed a 3-cm pseudoaneurysm of his aortoiliac vascular anastomosis, 3 mo posttransplant. An infrarenal aortic jump graft to the donor hepatic artery and ligation of supraceliac aortic conduit was performed, followed by aortic stent-graft placement to occlude the pseudoaneurysm. He received a deceased donor kidney transplant 13 mo after the liver transplant. He remains HCC free with excellent liver and renal allograft function. Adults with AGS undergoing liver transplantation for HCC need special consideration due to related vascular, cardiac, and renal anomalies.