Soejima Yuji, Taguchi Tomoaki, Ogita Keiko, Taketomi Akinobu, Yoshizumi Tomoharu, Uchiyama Hideaki, Ohno Takuro, Shimada Mitsuo, Maehara Yoshihiko
Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Liver Transpl. 2006 May;12(5):845-9. doi: 10.1002/lt.20692.
Congenital absence of the portal vein (CAPV) is a rare malformation of the mesenteric vasculature in which visceral venous blood bypasses the liver, completely draining into the systemic circulation through a congenital porto-systemic shunt. Liver transplantation has rarely been indicated for patients with this disease. We present a child with CAPV who was managed successfully by living donor auxiliary partial orthotopic liver transplantation (APOLT), while preserving the right lobe of the native liver. In conclusion, APOLT for patients with CAPV is a feasible and ideal procedure because portal vein (PV) diversion is not necessary.
先天性门静脉缺如（CAPV）是一种罕见的肠系膜血管系统畸形，其中内脏静脉血绕过肝脏，通过先天性门体分流完全排入体循环。对于患有这种疾病的患者，很少有肝移植的指征。我们报告一例患有CAPV的儿童，通过活体供体辅助部分原位肝移植（APOLT）成功治疗，同时保留了原肝的右叶。总之，对于CAPV患者，APOLT是一种可行且理想的手术，因为无需进行门静脉（PV）分流。
