Prognostic Factors and Outcomes of Kasai Portoenterostomy (KPE): Nine Year Experience from a Lower Middle-Income Country

Abstract

Purpose Outcome data after Kasai Portoenterostomy (KPE) reported worldwide shows 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. Methods We reviewed 79 patients of which 43 had completed a 2-year minimum follow-up. Two cohorts were based on age at KPE. Median age at surgery was 60 days. Results 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. Conclusion Whilst causes for low COJ need to be explored, this 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.

Paediatric liver transplantation in Sri Lanka; From an impossible dream to a lifesaving reality.

“…These are difficult cases for LT and are not the ideal cases for a PLT programme at its initiation 2,13 . In addition, these infants often present with additional complications such as portal vein thrombosis, portal vein hypoplasia, prior portoenterostomy, cholangitis and malnutrition 8,9 . Managing transplantation and recovery in these patients poses significant challenges 13 .…”

“…Overcoming the challenges related to living donors, it is important to educate the parents of children with diagnosed chronic liver disease, to lose weight in advance and to maintain a healthy lifestyle. Lifestyle change counselling of parents of children especially with BA, immediately following Kasai is helpful, as this group of children would require LT at a later stage in life 8,9 .…”

“…Till recently, due to lack of PLT facilities in SL, most children with liver failure died, only a handful being able to afford treatment overseas. In 2016, Shanmugam NP, et al 8 reported the non-availability of PLT facilities in SL and Wickramasekera N, et al 9 stated that the low survival of Sri Lankan children with biliary atresia (BA) was due to limited access to LT. Since the first PLT in 2020, the PLT programme in SL has been evolving steadily amidst challenging circumstances [1][2][3] .…”