Short-Term Clinical Outcomes of Kasai Porto-Enterostomy for Biliary Atresia: A Seven-Year Single-Center Experience
DOI:
https://doi.org/10.52206/jsmc.2026.16.3.1219Abstract
Background: Biliary atresia is a rare but progressive fibro-obliterative cholangiopathy of infancy and represents the leading surgically correctable cause of neonatal cholestasis. Untreated disease progresses rapidly to biliary cirrhosis, liver failure, and death. Kasai Porto-Enterostomy (KPE) remains the primary surgical treatment for restoring bile flow and delaying or preventing liver transplantation. However, postoperative outcomes vary considerably among institutions, and local evidence from Pakistan remains limited.
Objective: To evaluate the clinical characteristics, perioperative complications, and short-term outcomes of infants undergoing Kasai Porto-Enterostomy for biliary atresia at a tertiary care referral center.
Methodology: This retrospective observational study was conducted in the Department of Pediatric Surgery, Saidu Group of Teaching Hospitals, Swat, Pakistan. Medical records of infants who underwent Kasai Porto-Enterostomy between January 2018 and January 2025 were reviewed. Patients undergoing surgery before 12 weeks of age and completing six months of follow-up were included. Demographic, clinical, laboratory, radiological, operative, histopathological, and follow-up data were extracted from hospital records.
Results: Thirty-six infants underwent Kasai Porto-Enterostomy during the study period. Two patients died during the immediate postoperative period, and one patient was lost to follow-up, leaving 33 patients for six-month outcome analysis. The mean age at surgery was 8.85 ± 1.00 weeks, and 54.5% of patients were female. Mean serum bilirubin decreased significantly from 5.89 ± 1.72 mg/dL preoperatively to 1.52 ± 0.24 mg/dL after surgery (p < 0.001). Clinical improvement with clearance of jaundice was observed in 78.8% of patients. Postoperative complications included bile leakage (21.2%), anastomotic leakage (12.1%), postoperative sepsis (9.1%), and surgical site infection (6.1%). Seven patients (21.2%) experienced postoperative cholangitis. No statistically significant association was observed between preoperative hepatic fibrosis and postoperative cholangitis (p = 0.341).
Conclusion: Kasai Porto-Enterostomy achieved significant short-term improvement in biliary drainage and clinical recovery in most infants with biliary atresia. Despite favorable biochemical and clinical outcomes, postoperative complications remained common, emphasizing the importance of meticulous perioperative care and structured postoperative follow-up. Larger prospective multicenter studies with long-term follow-up are required to evaluate native liver survival and identify factors influencing long-term outcomes.
Keywords: Biliary Atresia, Cholangitis, Kasai Porto-Enterostomy, Liver Fibrosis, Neonatal Cholestasis, Pediatric Surgery.
References
Quelhas P, Cerski C, dos Santos JL. Update on etiology and pathogenesis of biliary atresia. Curr Pedi Rev. 2023;19(1):48 67. https://doi.org/10.2S174/1573396318666220510130259
Tam PK, Wells RG, Tang CS, Lui VC, Hukkinen M, Luque CD, et al. Biliary atresia. Nat Rev Dis Primers. 2024;10(1):47. https://doi.org/10.1038/s41572-024-00533-x
Azhar S, Ikam F, Shirazi SZ, Altaf J, Pervaiz R. An Insight into Neonatal Cholestasis; A Tertiary Care Hospital Experience in Rawalpindi, Pakistan. Pak Armed Forces Med J. 2023;73(5):1284. https://doi.org/10.51253/pafmj.v73i5.9108
Kakos CD, Ziogas IA, Alexopoulos SP, Tsoulfas G. Management of biliary atresia: To transplant or not to transplant. World J Transplant. 2021;11(9):400. https://doi.org?10.5500/wjt.v11.i9.400
Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH. Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop. Hepatology. 2007;46:566–581. https://doi.org/10.1002/hep.21790
Du M, Wang J, Tang Y, Jiang J, Chen G, Huang Y, et al. Evaluation of perioperative complications in the management of biliary atresia. Front Pediatr. 2020; 8:460. https://doi.org/10.3389/fped.2020.00460
Chan KW, Lee KH, Tsui SY, Wong YS, Pang KY, Mou JW, et al. Laparoscopic versus open Kasai portoenterostomy in infant with biliary atresia: a retrospective review on the 5-year native liver survival. Pediatr Surg Int. 2012 Nov;28(11):1109-13. https://doi.org/10.1007/s00383-012-3172-9
Uecker M, Kuebler JF, Schukfeh N, Pfister ED, Baumann U, Petersen C, et al. Kasai procedure in patients older than 90 days: worth a cut. Eur J Pediatr Surg. 2022;32(01):080-4. https://doi.org/10.1055/s-0041-1740556
Ge L, Zhan J, Gao W, Zhao S, Xu X, Dou R. Relevant factors for early liver transplantation after Kasai portoenterostomy. BMC Pediatr. (2020):484. https://doi.org/10.1186/s12887-020-02355-8
Decharun K, Leys CM, West KW, Finnell SM. Prophylactic Antibiotics for Prevention of Cholangitis in Patients With Biliary Atresia Status Post-Kasai Portoenterostomy: A Systematic Review. Clin. Pediatr. 2016;55:66–72. https://doi.org/10.1177/0009922815594760
Townsend MR, Jaber A, Abi Nader H, Eid SM, Schwarz K. Factors associated with timing and adverse outcomes in patients with biliary atresia undergoing Kasai hepatoportoenterostomy. J Pediatr. 2018;199:237–42.e2. https://doi.org/10.1016/j.jpeds.2018.04.001
Zhou RJ, Ming AX, Diao M, Li L. The short-and mid-term prognosis and influencing factors of Kasai portoenterostomy for 220 cases of type III biliary atresia. Chin J Gen Surg. 2019;08:659–62. https://doi.org/10.3760/cma.j.issn.1007-631X.2019.08.004
Andrade WD, Silva MM, Tannuri AC, Santos MM, Gibelli NE, Tannuri U. Current management of biliary atresia based on 35 years of experience at a single center. Clinics. 2018;73:e289. https://doi.org/10.6061/clinics/2018/e289
Liu F, Yeung F, Chung PH. The outcome of Kasai portoenterostomy after day 70 of life. Front Pediatr. 2022;10:1015806. https://doi.org/10.3389/fped.2022.1015806
Wickramasekara N, Ignatius J, Lamahewage A. Prognostic factors and outcomes of Kasai portoenterostomy (KPE): nine-year experience from a lower-middle income country. Pediatr Surg Int. 2023;39(1):142. https://doi.org/10.1007/s00383-023-05424-y
Redkar R, Karkera PJ, Raj V, Bangar A, Hathiramani V, Krishnan J. Outcome of biliary atresia after Kasai’s portoenterostomy: a 15-year experience. Indian Ped. 2017 Apr;54(4):291-4.2017. https://doi.org/10.1007/s13312-017-1091-5
Nio M. Japanese biliary atresia registry. Pediatr Surg Int. 2017 Dec;33(12):1319-25. https://doi.org/10.1007/s00383-017-4160-x
Schoen B, Lee H, Sullivan K, Ricketts R. The kasai portoenteros- tomy: when is it too late? J Pediatr Surg.2001;36:97–99. https://doi.org/10.1053/jpsu.2001.20020
Song Z, Dong R, Shen Z, Chen G, Yang Y, Zheng S. Surgical outcome and etiologic heterogeneity of infants with biliary atresia who received kasai operation less than 60 days after birth: a retrospective study. Medicine (Baltimore). 2017;96:7267. https://doi.org/10.1097/MD.000000000000726
Chung PHY, Chan EKW, Yeung F, Chan ACY, Mou JWC, Lee KH, et al. Lifelong follow up and management strategies of patients living with native livers after Kasai portoenterostomy. Sci Rep. 2021;11:11207. https://doi.org/10.1038/s41598-021-90860-w
Li D, Chen X, Fu K, Yang J, Feng J. Preoperative nutritional status and its impact on cholangitis after Kasai portoenterostomy in biliary atresia patients. J Pediatr Surg. 2017;33:901-6. https://doi.org/10.1007/s00383-017-4118-z
Liu J, Dong R, Chen G, Dong K, Zheng S. Risk factors and prognostic effects of cholangitis after Kasai procedure in biliary atresia patients: A retrospective clinical study. J Pediatr Surg. 2019;54(12):2559- 2564. https://doi.org/10.1016/j.jpedsurg.2019.08.026
Gustomi S, Kalim AS, Dwiantama K, Amadeus VC, Seta A. Prognostic Factors for Cholangitis in Biliary Atresia Patients Following Kasai Procedure. Age (days). 2024;86:86.
Li D, Chen X, Fu K, Yang J, Feng J. Preoperative nutritional status and its impact on cholangitis after Kasai portoenterostomy in biliary atresia patients. Pediatr Surg Int. 2017;33:901-6. https://doi.org/10.1007/s00383-017-4118-z
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