Affiliation:
1. Lady Ridgeway Hospital for Children
Abstract
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.
Publisher
Research Square Platform LLC
Reference26 articles.
1. Five- and 10-year survival rates after surgery for biliary atresia: A report from the Japanese biliary atresia registry;Nio M;Journal of pediatric surgery,2003
2. Petersen, C., Harder, D., Abola, Z., Becker, T., Chardot, C., Davenport, M., Deutschmann, A., Khelif, K., Kobayashi, H., Kvist, N., Leonhardt, J., Melter, M., Pakarinen, M., Pawlowska, J., Petersons, A., Pfister, E. D., Rygl, M., Schreiber, R., Kelly, D.: European biliary atresia registries: Summary of a symposium. European journal of pediatric surgery: official journal of Austrian Association of Pediatric Surgery … et al] = Zeitschrift fu¨r Kinderchirurgie 18, 111–6 (2008). https://doi.org/10.1055/s-2008-1038479
3. A study on cholestasis in infants less than six months of age presenting to lady ridgeway hospital for children, Colombo;Luthufdeen I;Sri Lanka Journal of Child Health,2016
4. Biliary atresia;Hartley J;Lancet,2009
5. Biliary atresia: Swiss national study, 1994–2004;Wildhaber B;Journal of pediatric gastroenterology and nutrition,2008