Miyano T, Fujimoto T, Ohya T, Shimomura H
Department of Pediatric Surgery, Juntendo University, School of Medicine, Tokyo, Japan.
World J Surg. 1993 May-Jun;17(3):332-6. doi: 10.1007/BF01658700.
Hepatic portoenterostomy (Kasai operation) for the patient with biliary atresia (BA) can restore the bile flow in approximately 80% of children operated on before 60 days of life [1]. However, in terms of long-term survival, according to a recent nationwide survey among the major pediatric centers in Japan, only 325 of 2013 patients had more than 10 years' survival, and only 157 patients (7.8%) remained jaundice-free with normal liver function [2]. About 20% of BA cases without jaundice are generally able to survive for long periods; and most of those patients have portal hypertension or abnormal liver function [3-5]. As the results of liver transplantation have improved, controversy has arisen over the optimal care of these children [4, 6, 7]. Some investigators have claimed that transplantation is the favored primary therapy for most patients with BA [8]. We are thus at a turning point concerning the primary therapy of BA, which makes it necessary to determine the exact indications for the Kasai portoenterostomy and the timing of liver transplantation. This paper describes our strategy for the optimal treatment of BA patients based on our 117 patients who have had various form of portoenterostomy.
对于患有胆道闭锁（BA）的患者，肝门肠吻合术（Kasai手术）可使约80%在出生60天前接受手术的儿童恢复胆汁流动[1]。然而，就长期生存而言，根据日本主要儿科中心最近的一项全国性调查，2013例患者中只有325例存活超过10年，只有157例患者（7.8%）保持无黄疸且肝功能正常[2]。一般来说，约20%无黄疸的BA病例能够长期存活；这些患者中的大多数患有门静脉高压或肝功能异常[3 - 5]。随着肝移植结果的改善，对于这些儿童的最佳治疗出现了争议[4, 6, 7]。一些研究者声称，移植是大多数BA患者首选的初始治疗方法[8]。因此，我们正处于BA初始治疗的转折点，这使得有必要确定Kasai肝门肠吻合术的确切适应证以及肝移植的时机。本文基于我们117例接受了各种形式肝门肠吻合术的患者，描述了我们对BA患者的最佳治疗策略。
