Emre Sukru, Arnon Ronen, Cohen Emil, Morotti Raffaella A, Vaysman Dmitriy, Shneider Benjamin L
Department of Pediatrics, Mount Sinai Medical Center, Mount Sinai School of Medicine, New York, NY, USA.
Liver Transpl. 2007 Dec;13(12):1662-8. doi: 10.1002/lt.21349.
Congenital absence of portal vein and extrahepatic portocaval shunt, also referred to as an Abernethy type 1, is a rare malformation of the mesenteric vasculature. A 9-year-old girl presented with dyspnea on exertion and orthodeoxia. A diagnosis of an Abernethy malformation type 1b and hepatopulmonary syndrome (HPS) was made, and she underwent auxiliary partial orthotopic liver transplantation (APOLT). Symptoms and signs of HPS resolved 2 months after the operation. HPS in Abernethy syndrome is a manifestation of normal portal blood flow bypassing the liver and can be ameliorated by redirecting blood flow to a segment of liver with normal portal anatomy. APOLT is a feasible and successful surgical procedure for patients with Abernethy malformation and HPS.
先天性门静脉缺如和肝外门腔分流，也称为阿伯内西1型，是一种罕见的肠系膜血管系统畸形。一名9岁女孩出现劳力性呼吸困难和直立性低氧血症。诊断为阿伯内西1b型畸形和肝肺综合征（HPS），并接受了辅助性部分原位肝移植（APOLT）。术后2个月，HPS的症状和体征消失。阿伯内西综合征中的HPS是正常门静脉血流绕过肝脏的一种表现，通过将血流重新导向门静脉解剖结构正常的肝段可使其得到改善。APOLT对于患有阿伯内西畸形和HPS的患者是一种可行且成功的外科手术。
