Andreani P, Srinivasan P, Ball C S, Heaton N D, Rela M
Liver Transplant Surgical Service, King's College Hospital, Denmark Hill, London SE5 9RS, UK.
Int J Surg Investig. 2000;2(1):81-4.
Extrahepatic biliary atresia is the most common indication for liver transplantation in children. Coexistent congenital anomalies are common (25%), but the combination of polysplenia syndrome (10%) and absence of portal vein is rare.
We report a case of successful reduced size liver transplantation on a 13-month-old girl with extrahepatic biliary atresia, polysplenia syndrome and congenital absence of the portal vein.
Technical and post-operative problems related to absence of the portal vein and polysplenia are described and the literature reviewed.
The donor portal vein was successfully anastomosed to the confluence of the superior mesenteric vein and splenic vein. The child is well with good graft function at a follow-up of 20 months.
Absence of the portal vein and polysplenia syndrome is not a contraindication for liver transplantation although an increased post-operative morbidity may be expected.
肝外胆管闭锁是儿童肝移植最常见的适应症。并存的先天性异常很常见（25%），但多脾综合征（10%）合并门静脉缺如则很罕见。
我们报告一例成功为一名13个月大患有肝外胆管闭锁、多脾综合征和先天性门静脉缺如的女孩进行减体积肝移植的病例。
描述了与门静脉缺如和多脾相关的技术及术后问题，并对文献进行了综述。
供体门静脉成功吻合至肠系膜上静脉和脾静脉汇合处。患儿在20个月的随访中情况良好，移植肝功能正常。
门静脉缺如和多脾综合征并非肝移植的禁忌症，尽管术后发病率可能会增加。
