Wickramasekara Nipun, Ignatius Jenosha, Lamahewage Ananda
Lady Ridgeway Hospital for Children, Colombo, Sri Lanka.
Pediatr Surg Int. 2023 Feb 28;39(1):142. doi: 10.1007/s00383-023-05424-y.
Outcome data after Kasai portoenterostomy (KPE) reported worldwide show considerable regional and institutional variation. It is not known whether the same standards of outcomes reported in western world can be replicated in resource-poor countries.
We reviewed 79 patients of which 43 had completed a 2-year minimum follow-up. Two cohorts were based on age at KPE. The median age at surgery was 60 days.
Clearance of jaundice (COJ) at 3 months was 20.93% and was not affected by age at surgery (p = 0.295). Four patients (9.3%) received liver transplant and 16 patients (37.21%) were recorded dead at a median age of 7 months. Native liver survival (NLS) was 53.49% and overall survival (OS) was 62.79%. Kaplan-Meier estimated 4- and 6-year NLS were 55.8% and 49.6%, respectively. There was a significant difference in the NLS between early and late surgery groups.
While causes for low COJ need to be explored, these data reaffirm that early surgery has a significant favorable effect on survival. NLS was comparable with data from the developed world, whereas low OS is explained by limited access to transplant. Thus, where the survival depends on native liver longevity, emphasis should be on as early KPE as possible.
全球范围内报道的Kasai肝门空肠吻合术（KPE）后的结果数据显示出显著的地区和机构差异。尚不清楚西方世界所报道的相同结果标准能否在资源匮乏国家得以复制。
我们回顾了79例患者，其中43例完成了至少2年的随访。两个队列基于KPE时的年龄。手术时的中位年龄为60天。
3个月时黄疸清除率（COJ）为20.93%，且不受手术时年龄的影响（p = 0.295）。4例患者（9.3%）接受了肝移植，16例患者（37.21%）被记录死亡，中位年龄为7个月。自体肝生存率（NLS）为53.49%，总生存率（OS）为62.79%。Kaplan-Meier法估计4年和6年的NLS分别为55.8%和49.6%。早期和晚期手术组的NLS存在显著差异。
虽然需要探究COJ较低的原因，但这些数据再次证实早期手术对生存率有显著的有利影响。NLS与发达国家的数据相当，而较低的OS是由于肝移植机会有限所致。因此，如果生存取决于自体肝的寿命，应强调尽早进行KPE。
