Outcomes of late Kasai portoenterostomy in biliary atresia: a single-center experience
- PMID: 33947263
- PMCID: PMC8113946
- DOI: 10.1177/03000605211012596
Outcomes of late Kasai portoenterostomy in biliary atresia: a single-center experience
Abstract
Objectives: To describe the clinical, histopathologic, and outcomes data for a cohort of patients with biliary atresia (BA), and to identify the factors affecting survival.
Methods: This was a cross-sectional study of all BA patients diagnosed between 1999 and 2017. Clinical, biochemical, imaging, and histopathologic data were analyzed, and Kaplan-Meier survival rates were compared to identify potential prognostic factors.
Results: We evaluated 23 patients. The median age at the Kasai procedure was 77 ± 34 days, and the median overall survival was 12.5 ± 65 months. Thirteen (56%) patients survived with their native livers, 3 (13%) received a transplant, and 6 died (26%) while awaiting a transplant. Cholangitis and the use of ursodeoxycholic acid were associated with longer survival, while impaired synthetic function was associated with shorter survival.
Conclusions: Most patients presented late for the Kasai procedure. The survival rate with the native liver was comparable to other cohorts. Therefore, clinicians are encouraged to refer for the Kasai procedure even with late presentation (between 60 and 90 days), provided there is no hepatic decompensation.
Keywords: Kasai portoenterostomy; Saudi Arabia; biliary atresia; cholestasis; liver transplantation; pediatric.
Conflict of interest statement
Figures




Similar articles
-
Effect of repeat Kasai hepatic portoenterostomy on pediatric live-donor liver graft for biliary atresia.Exp Clin Transplant. 2013 Jun;11(3):259-63. doi: 10.6002/ect.2012.0188. Epub 2013 Mar 26. Exp Clin Transplant. 2013. PMID: 23530849
-
Outcome of biliary atresia among Saudi children: A tertiary care center experience.Saudi J Gastroenterol. 2019 May-Jun;25(3):176-180. doi: 10.4103/sjg.SJG_306_18. Saudi J Gastroenterol. 2019. PMID: 30479320 Free PMC article.
-
[Follow up for a cohort of patients with biliary atresia: late surgery and development of biliary cysts].Rev Chil Pediatr. 2017;88(5):629-634. doi: 10.4067/S0370-41062017000500009. Rev Chil Pediatr. 2017. PMID: 29546948 Spanish.
-
Surgical modifications, additions, and alternatives to Kasai hepato-portoenterostomy to improve the outcome in biliary atresia.Pediatr Surg Int. 2017 Dec;33(12):1275-1282. doi: 10.1007/s00383-017-4162-8. Epub 2017 Oct 4. Pediatr Surg Int. 2017. PMID: 28980051 Review.
-
Surgery of biliary atresia.Scand J Surg. 2011;100(1):49-53. doi: 10.1177/145749691110000109. Scand J Surg. 2011. PMID: 21482505 Review.
Cited by
-
Alterations of gut microbiota in infants with biliary atresia identified by 16S rRNA-sequencing.BMC Pediatr. 2024 Feb 14;24(1):117. doi: 10.1186/s12887-024-04582-9. BMC Pediatr. 2024. PMID: 38355416 Free PMC article.
-
Clinical Characteristics and Outcome of Infants with Biliary Atresia in Bahrain.Oman Med J. 2023 Mar 31;38(2):e485. doi: 10.5001/omj.2023.64. eCollection 2023 Mar. Oman Med J. 2023. PMID: 37122417 Free PMC article.
-
Early bile drainage improves native liver survival in biliary atresia without cholangitis.Front Pediatr. 2023 Jul 12;11:1189792. doi: 10.3389/fped.2023.1189792. eCollection 2023. Front Pediatr. 2023. PMID: 37502192 Free PMC article.
-
Relationship between hepatic function recovery and postoperative cholangitis in neonates undergoing hepaticojejunostomy for biliary atresia.Am J Transl Res. 2024 Aug 15;16(8):3822-3831. doi: 10.62347/CPFT4246. eCollection 2024. Am J Transl Res. 2024. PMID: 39262696 Free PMC article.
-
The outcome of Kasai portoenterostomy after day 70 of life.Front Pediatr. 2022 Oct 21;10:1015806. doi: 10.3389/fped.2022.1015806. eCollection 2022. Front Pediatr. 2022. PMID: 36340701 Free PMC article.
References
-
- Balistreri WF, Bezerra JA. Whatever happened to “neonatal hepatitis”? Clin Liver Dis 2006; 10: 27–53, v. DOI: 10.1016/j.cld.2005.10.008. - PubMed
-
- Kim WR, Lake JR, Smith JM, et al.. OPTN/SRTR 2017 Annual Data Report: Liver. Am J Transplant 2019; 19: 184–283. DOI: 10.1111/ajt.15276. - PubMed
-
- Al Sebayel M, Abaalkhail F, Al Abbad S, et al.. Liver transplantation in the Kingdom of Saudi Arabia. Liver Transpl 2017; 23: 1312–1317. DOI: 10.1002/lt.24803. - PubMed
-
- Landing BH. Considerations of the pathogenesis of neonatal hepatitis, biliary atresia and choledochal cyst–the concept of infantile obstructive cholangiopathy. Prog Pediatr Surg 1974; 6: 113–139. - PubMed
MeSH terms
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical