Losty P, Guiney E
Dept of Paediatric Surgery, Children's Research Centre, Our Lady's Hospital for Sick Children, Crumlin, Dublin.
Ir Med J. 1992 Dec;85(4):144-7.
A retrospective study analysing the outcome of 26 infants with extrahepatic biliary atresia (EHBA) is reported. Following surgery (mean age 54 days) 13 patients (50%) achieved 'total' biliary drainage, five (19%) 'partial' drainage and eight (31%) were 'failures'. Patients who developed hepatic decompensation (1985 onwards) were evaluated for liver transplantation (LTX). Eight patients (31%) underwent LTX six of whom were > two years (mean age 3.5 yrs). Overall 12 patients are alive (46%)--seven (27%) have had the Kasai operation, one (4%) hepaticojejunostomy. The other four patients are alive following LTX (survival 50%). Seven infants (27%) were referred beyond the optimal age for portoenterostomy, four (15%) of these infants are now dead. The importance of early referral of infants with EHBA is highlighted in this study. Furthermore portoenterostomy and LTX were shown to be complimentary rather than competitive options for these infants. The proposal that LTX is the primary treatment for EHBA has not been supported by this study.
本文报道了一项回顾性研究，分析了26例肝外胆管闭锁（EHBA）婴儿的治疗结果。手术后（平均年龄54天），13例患者（50%）实现了“完全”胆管引流，5例（19%）为“部分”引流，8例（31%）为“失败”。对出现肝失代偿（1985年起）的患者进行了肝移植（LTX）评估。8例患者（31%）接受了肝移植，其中6例年龄超过2岁（平均年龄3.5岁）。总体而言，12例患者存活（46%）——7例（27%）接受了Kasai手术，1例（4%）接受了肝空肠吻合术。另外4例患者在肝移植后存活（生存率50%）。7例婴儿（27%）在超过最佳肠造口术年龄后转诊，其中4例（15%）婴儿现已死亡。本研究强调了EHBA婴儿早期转诊的重要性。此外，肠造口术和肝移植对这些婴儿来说是互补而非竞争的选择。本研究不支持肝移植是EHBA主要治疗方法的提议。
