Taoube K A, Alonso Calderón J L, Yandza T, St Vil D, Blanchard H
Hopital Sainte-Justine, Montréal, Quebec, Canadá.
Cir Pediatr. 1999 Jan;12(1):38-40.
The congenital absence of the portal vein is a rare malformation, which has been generally discovered in association with another anomalies like a cardiac, gastrointestinal or genitourinary defects. With portal hypertension and hepatic cirrhosis, this anomaly is similar to spontaneous porto-systemic derivation, and for that not collateral venous drainage is present. In these patients, total interruption of mesentaric venous drainage during procurement of liver transplant produce a very important bowel and mesenteric edema, which can promote an injert fatal evolution. The authors present the first paediatric liver transplant, in a patient with portal venous agenesia, with de piggy-back technique was done and this complication was obviated, and a review of the literature about this issue is done.
门静脉先天性缺如是一种罕见的畸形，通常与其他异常情况如心脏、胃肠道或泌尿生殖系统缺陷相关联被发现。伴有门静脉高压和肝硬化时，这种异常类似于自发性门体分流，因此不存在侧支静脉引流。在这些患者中，肝移植获取过程中肠系膜静脉引流完全中断会导致非常严重的肠和肠系膜水肿，这可能促使病情发展至致命结局。作者介绍了首例为门静脉发育不全患儿实施的肝移植手术，采用背驮式技术完成手术，避免了这一并发症，并对有关该问题的文献进行了综述。
