Woodle E S, Thistlethwaite J R, Emond J C, Whitington P F, Vogelbach P, Yousefzadeh D K, Broelsch C E
Department of Surgery, University of Chicago Pritzker School of Medicine, Ill.
Surgery. 1990 Apr;107(4):475-9.
The first case of hepatic transplantation in a patient with congenital absence of the portal vein (CAPV) is reported. A 10-year-old girl with biliary atresia and CAPV underwent successful hepatic transplantation and has normal liver function 9 months after transplantation. This case is only the seventh reported case of CAPV. Patients with CAPV commonly have additional liver anomalies, cardiac and inferior vena cava anomalies, and polysplenia. Surprisingly, hepatic encephalopathy is not a prominent feature in patients with CAPV despite systemic drainage of mesenteric venous blood. CAPV should not be considered a contraindication to hepatic transplantation.
报道了首例门静脉先天性缺如（CAPV）患者的肝移植病例。一名患有胆道闭锁和CAPV的10岁女孩接受了成功的肝移植，移植后9个月肝功能正常。该病例是报道的第7例CAPV病例。CAPV患者通常还伴有其他肝脏异常、心脏和下腔静脉异常以及多脾。令人惊讶的是，尽管肠系膜静脉血进行了体循环引流，但肝性脑病在CAPV患者中并非突出特征。CAPV不应被视为肝移植的禁忌证。
