Ramachandran Priya, Safwan Mohamed, Srinivas Sankaranarayanan, Shanmugam Naresh, Vij Mukul, Rela Mohamed
Institute of Liver Disease and Transplantation, Global Health City, Chennai, Tamil Nadu, India; Department of Pediatric Surgery, Kanchi Kamakoti CHILDS Trust Hospital, CHILDS Trust Medical Research Foundation, Chennai, Tamil Nadu, India; National Foundation for Liver Research, Chennai, Tamil Nadu, India.
Institute of Liver Disease and Transplantation, Global Health City, Chennai, Tamil Nadu, India.
J Indian Assoc Pediatr Surg. 2016 Apr-Jun;21(2):66-71. doi: 10.4103/0971-9261.176941.
In 1955, Professor Morio Kasai first performed a hepatic portoenterostomy. Since then, the procedure has changed the lives of children with biliary atresia (BA). We report our initial experience in performing "extended" Kasai portoenterostomy (KPE), a modification of the original procedure.
Since 2013, we have used the technique of "extended KPE" and prospectively recorded data on all children undergoing this operation. Data on demographics, clinical features, liver function tests, and perioperative cholangiogram findings were collected. Outcome of KPE was measured by Jaundice Disappearance Rate (JDR) and Native Liver Survival Rate (NLSR). We present our preliminary results from a 30-month period (February 2013 to May 2015).
Thirty-one children underwent KPE during this period (19 males) and only 1 child had biliary atresia splenic malformation (BASM). The mean age at KPE was 73 ± 24 days. Five (16.1%) children were more than 90 days old at the time of KPE. Fourteen children cleared jaundice (JDR 45.2%). Eleven (35.5%) children developed episodes of cholangitis, of whom 8 had early cholangitis (within 3 months of the operation). The proportion of children who survived with their own liver 6 months after KPE (NLSR) was 84.2%. Of those children older than 90 days, 2 cleared jaundice and have survived with their native livers for more than 16 months.
In our preliminary report of 31 children, we conclude that the extended KPE leads to increased jaundice clearance and improved NLSR in children with BA.
1955年，笠井守夫教授首次实施了肝门空肠吻合术。自那时起，该手术改变了胆道闭锁（BA）患儿的生活。我们报告了我们实施“扩大”肝门空肠吻合术（KPE）的初步经验，这是对原始手术的一种改良。
自2013年以来，我们采用了“扩大KPE”技术，并前瞻性记录了所有接受该手术患儿的数据。收集了人口统计学、临床特征、肝功能检查和围手术期胆管造影结果的数据。KPE的结果通过黄疸消失率（JDR）和自体肝生存率（NLSR）来衡量。我们展示了30个月期间（2013年2月至2015年5月）的初步结果。
在此期间，31名儿童接受了KPE（19名男性），仅有1名儿童患有胆道闭锁脾畸形（BASM）。KPE时的平均年龄为73±24天。5名（16.1%）儿童在KPE时年龄超过90天。14名儿童黄疸消退（JDR为45.2%）。11名（35.5%）儿童发生胆管炎发作，其中8名患有早期胆管炎（在手术后3个月内）。KPE后6个月自体肝存活的儿童比例（NLSR）为84.2%。在那些年龄超过90天的儿童中，2名黄疸消退，自体肝存活超过16个月。
在我们对31名儿童的初步报告中，我们得出结论，扩大KPE可提高BA患儿的黄疸清除率并改善NLSR。
