Ryckman F C, Alonso M H, Bucuvalas J C, Balistreri W F
Children's Hospital Medical Center, Liver Care Center, University of Cincinnati, OH 45229, USA.
Liver Transpl Surg. 1998 Sep;4(5 Suppl 1):S24-33.
Results show that the use of sequential surgical treatment, employing Kasai portoenterostomy in infancy, followed by selective liver transplantation for children with progressive hepatic deterioration yields improved overall survival. All children with successful Kasai portoenterostomy procedures who do not require OLT are survivors. Using newer transplant techniques, the 5-year survival rate for children who receive transplants with a primary diagnosis of biliary atresia was 82%. This yields an overall survival rate of 86% in this entire study population. Limited donor availability and increased complications after liver transplantation in infants less than 1 year of age mitigate against the use of primary liver transplantation without prior portoenterostomy for infants with biliary atresia. At present, these two operative procedures should be used as sequential and complementary modes of treatment rather than as competitive procedures. When biliary atresia is not recognized in infancy and established cirrhosis has resulted, primary transplantation should be offered as the initial surgical treatment.
结果显示，采用序贯手术治疗，婴儿期行肝门空肠吻合术，随后对进行性肝恶化的儿童进行选择性肝移植，可提高总体生存率。所有成功进行肝门空肠吻合术且不需要肝移植的儿童均存活。采用更新的移植技术，原发性诊断为胆道闭锁的儿童接受移植后的5年生存率为82%。在整个研究人群中，这使得总体生存率达到86%。供体来源有限以及1岁以下婴儿肝移植后并发症增加，不利于对胆道闭锁婴儿在未先行肝门空肠吻合术的情况下直接进行肝移植。目前，这两种手术应作为序贯和互补的治疗方式，而非相互竞争的手术。当婴儿期未识别出胆道闭锁并已导致肝硬化时，应将原发性移植作为初始手术治疗。
