Adult to pediatric living donor liver transplantation for portal cavernoma

Abstract

Portal cavernoma (PC) is an important cause of non-cirrhotic portal hypertension with severe complications, such as variceal hemorrhage in pediatric patients. With the development of new surgical techniques, living donor liver transplantation (LDLT) has recently been recognized as a viable but challenging treatment option for PC. The purpose of the present study was to summarize the efficacy of LDLT in PC patients and to carry out a follow-up study of pediatric recipients. The primary indication for LDLT in our research was PC with severe variceal bleeding and liver function decompensation. Three patients were diagnosed with PC following evaluation with computed tomography angiography and abdominal color Doppler ultrasonography (CDU). Various surgical techniques, including jump bypass grafting for portal vein anastomosis, were carried out according to the range and degree of cavernous transformation within the splenic vein and superior mesenteric vein. Postoperative CDU confirmed the early integrity of the portal vein (PV) in each patient. PV rethrombosis occurred in one patient 7 days after LDLT, despite anticoagulation therapy with coumadin. Two of the three patients had no further episodes of variceal hemorrhage during the 2-year follow-up period. The present study is the first report of the successful use of LDLT to treat pediatric PC patients. We conclude that LDLT is effective for the majority of pediatric patients with PC.