Falchetti D, de Carvalho F B, Clapuyt P, de Ville de Goyet J, de Hemptinne B, Claus D, Otte J B
Department of Pediatric Surgery, University of Louvain Medical School, Brussels, Belgium.
J Pediatr Surg. 1991 May;26(5):528-31. doi: 10.1016/0022-3468(91)90698-s.
Biliary atresia is the most common indication for orthotopic liver transplantation (OLT) in children. The polysplenia syndrome anomalies, which occur in approximately 10% of children with biliary atresia, may represent special difficulties at liver transplantation. We have reviewed our experience with this syndrome in 116 children with biliary atresia who underwent liver transplantation between March 1984 and December 1989. The main features of the polysplenia syndrome, which included absence of the inferior vena cava, preduodenal portal vein, midgut malrotation, aberrant hepatic artery, and situs inversus, were encountered in 12 of the 116 children (10.3%). Severe portal vein hypoplasia (3.5 mm or smaller) was also present in 7 of these children. Eight patients received a complete and four received a reduced liver graft. The vascular anomalies increased the technical difficulty of OLT but could be surmounted, although they did contribute to the peroperative death of one child. The 1-month survival rate was 83% for the 12 children with features of the polysplenia syndrome and 88% for the other 92 children with biliary atresia alone.
胆道闭锁是儿童原位肝移植（OLT）最常见的适应证。多脾综合征异常在约10%的胆道闭锁患儿中出现，可能给肝移植带来特殊困难。我们回顾了1984年3月至1989年12月间116例接受肝移植的胆道闭锁患儿中该综合征的情况。116例患儿中有12例（10.3%）出现多脾综合征的主要特征，包括下腔静脉缺如、十二指肠前门静脉、中肠旋转不良、肝动脉异常和内脏反位。其中7例患儿还存在严重的门静脉发育不全（3.5毫米或更小）。8例患者接受了全肝移植，4例接受了减体积肝移植。血管异常增加了OLT的技术难度，但可以克服，尽管它们确实导致了1例患儿术中死亡。12例有多脾综合征特征的患儿1个月生存率为83%，另外92例单纯胆道闭锁患儿的1个月生存率为88%。
