Factors Associated with Survival Rate in Biliary Atresia Patients Following Kasai Surgery

Authors

  • Agung Aji Prasetyo LecturerDivision Pediatric Surgery, Department of Surgery, Kariadi Hospital/Faculty of Medicine Diponegoro University, Semarang, Indonesia
  • Edwin Basyar Division Pediatric Surgery, Department of Surgery, Kariadi Hospital/Faculty of Medicine Diponegoro University, Semarang, Indonesia
  • Agoes Wibisono Division Pediatric Surgery, Department of Surgery, Kariadi Hospital/Faculty of Medicine Diponegoro University, Semarang, Indonesia
  • Avriana Pety Wardhani Division Pediatric Surgery, Department of Surgery, Kariadi Hospital/Faculty of Medicine Diponegoro University, Semarang, Indonesia
  • Banundari Rachmawati Department of Clinical Pathology, Kariadi Hospital/ Faculty of Medicine Diponegoro University, Semarang, Indonesia
  • Ignatius Riwanto Division Digestive Surgery, Department of Surgery, Kariadi Hospital/ Faculty of Medicine Diponegoro University, Semarang, Indonesia

DOI:

https://doi.org/10.36408/mhjcm.v11i2.1059

Keywords:

Biliary atresia, Kasai procedure, Ascites

Abstract

Introduction: Biliary atresia is the most frequent cause of obstructive jaundice leading to liver fibrosis, end-stage liver disease, and death. Kasai surgery offers a bridge to attenuate liver fibrosis progression through reconstruction of the biliary system. The success of the Kasai procedure depends on the presence of jaundice, age at the time of surgery, clay-colored stool, and bilirubin counts.

Aim: This study aimed to investigate and predict the death incidence of Biliary atresia patients following Kasai Surgery in our institution.

Method: We conducted a case-control study from March 2020 to January 2022 at Kariadi General Hospital, Semarang, Indonesia. We collected data related to gender, age at surgery, albumin level pre and post-surgery, total and direct bilirubin before and after surgery, and the presence of ascites. Bivariate analysis using the Chi-Square test with OR (95% CI) was performed to analyze the risk factors in BA patients following the Kasai procedure.

Results: 19 patients with biliary atresia underwent the Kasai procedure with a survival rate of 68.4%. Bilirubin levels ≥10 mg/dL before (p=0.033, OR 11.25) and after (p=0.025, OR 11.00) the Kasai procedure, and the presence of ascites (p=0.005) were significant factors associated with mortality cases. However, a combined multivariate analysis of these factors did not show any significant relationship with outcomes.

Conclusion: Bilirubin exceeding 10 mg/dL before and after the Kasai procedure and the presence of Ascites was a marker for poor outcomes for biliary atresia patients following the Kasai procedure

Downloads

Download data is not yet available.

References

1. Wildhaber, B.E. 2012. Biliary Atresia: 50 years after the first Kasai. ISRN Surg. Article ID 132089.

2. Peterson, C. 2006. Pathogenesis and treatment opportunities for biliary atresia. Clin Liver Dis 10(1):73–88

3. Schneider RA, Barker CC, Roberts EA, et al. Biliary atresia: The Canadian experience. J Pediatr. 2007; 151:659–65. 665.e.1.

4. Nio M, Ohi R, Miyano T, et al. Five- and 10-year survival rates after surgery for biliary atresia: A report from the Japanese Biliary Atresia Registry. J Pediatr Surg. 2003; 38:997–1000. [PubMed] [Google Scholar]

5. Lin YC, Chang MH, Liao SF, et al. Decreasing rate of biliary atresia in Taiwan: A survey, 2004–2009. Pediatrics. 2011; 128:e530–6.

6. Gunadi, Gunawan, T.A., Widiyanto, G., Yuanita, A., Mulyani, N.S., and Makhmudi, A. 2018. Liver transplant score for prediction of biliary atresia patients' survival following Kasai procedure. BMC Res Notes 11: 381

7. Chusilp, S., Sookpotarom, P., Tepmalai, K., Rajatapiti, P., Chongsrisawat, V., Poovorawan, Y., and Vejchapipat, P. 2016. Prognostic serum bilirubin at 7th-day post-Kasai for survival with native livers in patients with biliary atresia. Pediatr Surg Int 32(10): 927–31.

8. Zhong ZH, Chen HD, Huang LE, et al. 20-year transplant-free survival of biliary atresia after Kasai operation. Chin J Pediatr Surg. 2014; 35(4):265–268. doi: 10.3760/cma.j.issn.0253-3006.2014.04.007

9. Chusilp, S., Sookpotarom, P., Tepmalai, K., Rajatapiti, P., Chongsrisawat, V., Poovorawan, Y., and Vejchapipat, P. 2016. Prognostic values of serum bilirubin at 7th-day post-Kasai for survival with native livers in patients with biliary atresia. Pediatr Surg Int 32(10): 927–31.

10. Shneider, B.L., Magee, J.C., Karpen, S.J., Rand, E.B., Narkewicz, M.R., Bass, L.M., Schwarz, K., Whitington, P.F., Bezerra, J.A., Kerkar, N., Haber, B., Rosenthal, P., Turmelle, Y.P., Molleston, J.P., Murray, K.F., Ng, V.L., Wang, K.S., Romero, R., Squires, R.H., Arnon, R., Sherker, A.H., Moore, J., Ye, W., and Sokol, R.J. 2016. Total serum bilirubin within 3 months of hepatoportoenterostomy predicts short-term outcomes in biliary atresia. J Pediatr 170: 211–7.

11. Kasai M, Suzuki S. A new operation for "non-correctable" biliary atresia: hepatic porto-enterostomy. Shuiyutsu. 1959; 13: 733–739

12. Otte JB, de Ville de Goyet J, Reding R, et al. Sequential treatment of biliary atresia with Kasai portoenterostomy and liver transplantation: a review. Hepatology. 1994; 20(1 pt 2):S41–S48

13. Howard ER, MacLean G, Nio M, Donaldson N, Singer J, Ohi R. Survival patterns in biliary atresia and comparison of quality of life of long-term survivors in Japan and England. J Pediatr Surg. 2001; 36(6):892–897

14. Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O. Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology. 2005; 41(2):366–371

15. Qisthi, S. A., Saragih, D. S. P., Sutowo, D. W., Sirait, D. N., Imelda, P., Kencana, S. M. S., Makhmudi, A., & Gunadi (2020). Prognostic Factors for Survival of Patients with Biliary Atresia Following Kasai Surgery. The Kobe journal of medical sciences, 66(2), E56–E60.

16. Nightingale, S., Stormon, M.O., O'Loughlin, E.V., Shun, A., Thomas, G., Benchimol, E.I., Day, A.S., Adams, S., Shi, E., Ooi, C.Y., Kamath, B.M., Fecteau, A., Langer, J.C., Roberts, E.A., Ling, S.C., and Ng, V.L. 2017. Early posthepatoportoenterostomy predictors of native liver survival in biliary atresia. J Pediatric Gastroenterol Nutr 64(2): 203–9.

17. Gad, E. H., Kamel, Y., Salem, T. A., Ali, M. A., & Sallam, A. N. (2021). Short- and long-term outcomes after Kasai operation for type III biliary atresia: Twenty years of experience in a single tertiary Egyptian center-A retrospective cohort study. Annals of medicine and surgery (2012), 62, 302–314. https://doi.org/10.1016/j.amsu.2021.01.052

18. Ramos-Gonzalez, G., Elisofon, S., Dee, E.C., Staffa, S.J., Medford, S., Lillehei, C., and Kim, H.B. 2019. Predictors of need for liver transplantation in children undergoing hepatoportoenterostomy for biliary atresia. J Pediatr Surg. 54: 1127–31

19. Sundaram, S. S., Mack, C. L., Feldman, A. G., & Sokol, R. J. (2017). Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care. Liver transplantation: official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society, 23(1), 96–109. https://doi.org/10.1002/lt.24640

20. Serinet, M.O., Wildhaber, B.E., Broué, P., Lachaux, A., Sarles, J., Jacquemin, E., Gauthier, F., and Chardot, C. 2009. Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics 123: 1280–6.

21. Nio, M., Ohi, R., Miyano, T., Saeki, M., Shiraki., and Tanaka, K. 2003. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese biliary atresia registry. J Pediatr Surg 38(7): 997–1000

22. Wong, K.K., Chung, P.H., Chan, I.H., Lan, L.C., and Tam, P.K. 2010. Performing Kasai portoenterostomy beyond 60 days of life is not necessarily associated with a worse outcome. J Pediatric Gastroenterol Nutr 51(5): 631–4.

23. Schoen, B.T., Lee, H., Sullivan, K., and Ricketts, R.R. 2001. The Kasai portoenterostomy: when is it too late? J Pediatr Surg 36: 97–99.

24. 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; 199:237–242.e2

25. Nio M, Sasaki H, Wada M, Kazama T, Nishi K, Tanaka H. Impact of age at Kasai operation on short- and long–term outcomes of type III biliary atresia at a single institution. J Pediatr Surg 2010; 45(12):2361–3.

26. Qiao G, Li L, Cheng W, Zhang Z, Ge J, Wang C. Conditional probability of sur- survival in patients with biliary atresia after Kasai portoenterostomy: a Chinese population-based study. J Pediatr Surg 2015; 50(8):1310–15

27. Karrer FM. Long-term results with the Kasai operation for biliary atresia. Arch Surg 1996; 131(5):493.

28. Yang, C. et al. (2022) "Impact of early Kasai portoenterostomy on short-term outcomes of biliary atresia: A systematic review and meta-analysis," Frontiers in Surgery, 9. Available at: https://doi.org/10.3389/fsurg.2022.924506.

29. Sasaki, H., Tanaka, H., Wada, M., Kazama, T., Nakamura, M., Kudo, H. Okubo, R., Sakurai, T., and Nio, M. 2016. Analysis of the prognostic factors of long-term native liver survival in survivors of biliary atresia. Pediatr Surg Int 32(9): 839–43.

Additional Files

Published

2024-07-31

How to Cite

1.
Prasetyo AA, Basyar E, Wibisono A, Wardhani AP, Rachmawati B, Riwanto I. Factors Associated with Survival Rate in Biliary Atresia Patients Following Kasai Surgery. Medica Hospitalia J. Clin. Med. [Internet]. 2024 Jul. 31 [cited 2024 Dec. 21];11(2):204-8. Available from: http://medicahospitalia.rskariadi.co.id/medicahospitalia/index.php/mh/article/view/1059

Issue

Section

Original Article

Citation Check