Valayer J, Odièvre M, Alagille D
Chir Pediatr. 1982 May-Jun;23(3):202-4.
The study of portal pressure during surgical exploration for biliary atresia in the series of 260 children operated at the Hospital Saint-Vincent de Paul since 1968 showed that portal hypertension could be demonstrated in 61.8% of the cases before the age of 3 months. Among the 54 children surviving without jaundice, splenomegaly was found in 35 cases; esophageal varices were seen in 14 children, more frequently after 5 years of age. Bleeding from esophageal varices occurred only in cases with recurrence of jaundice or moderate biliary retention, i.e. for 6 children. A portocaval shunt was constructed in 7 cases; all had bled except one. One child died two and a half years later from pneumococcal sepsis after a splenorenal shunt with splenectomy; another child had numerous recurrence of bleeding episodes. Five children have a functional shunt and have not rebled. When biliary atresia is cured by hepatoportoenterostomy, with complete disappearance of biliary retention, the risk of bleeding from esophageal varices is unlikely, in spite of persistent cirrhotic alterations of the liver; in these children, there should very seldom be an indication for a portocaval shunt.
自1968年以来,在圣文森特·德·保罗医院接受手术的260例胆道闭锁患儿中,对手术探查期间门静脉压力的研究表明,61.8%的病例在3个月龄前可证实存在门静脉高压。在54例无黄疸存活的患儿中,35例发现脾肿大;14例患儿出现食管静脉曲张,5岁以后更为常见。食管静脉曲张出血仅发生在黄疸复发或中度胆汁潴留的病例中,即6例患儿。7例行门腔分流术;除1例之外均发生过出血。1例患儿在脾肾分流加脾切除术后两年半死于肺炎球菌败血症;另1例患儿多次出现出血发作。5例患儿有功能性分流且未再出血。当通过肝门肠吻合术治愈胆道闭锁,胆汁潴留完全消失时,尽管肝脏持续存在肝硬化改变,但食管静脉曲张出血的风险不大;对于这些患儿,很少有行门腔分流术的指征。