Combined Predictors of Long-Term Outcomes of Kasai Surgery in Infants with Biliary Atresia

Overview

Journal Pediatr Gastroenterol Hepatol Nutr

Date 2024 Jul 22

PMID 39035404

Authors

Anna Degtyareva

Medan Isaeva

Elena Tumanova

Elena Filippova

Anna Sugak

Alexander Razumovsky

Nadezhda Kulikova

Marina Albegova

Denis Rebrikov

Affiliations

Soon will be listed here.

Abstract

Purpose: Biliary atresia (BA) is the leading cause of neonatal cholestasis (25-45%). The primary treatment is hepatic portoenterostomy (Kasai procedure), but only 20-40% provide long-term benefits. This study aimed to develop a predictive model for surgical efficacy by comparing preoperative and early postoperative indicators in infants with different outcomes.

Methods: We enrolled 166 infants with BA (93 girls, 73 boys) who underwent the Kasai procedure between September 2002 and December 2021, dividing them into favorable or adverse outcome groups. Over 40 parameters were measured, and the diagnostic significance of the prognostic model was evaluated.

Results: Kasai surgery was efficacious in 69 patients (42%) and non-efficacious in 97 (58%). Our model assesses efficacy by day 14 after surgery, improving on the <34 µmol/L direct bilirubin threshold established for 3-6 months after the procedure. Including the Desmet fibrosis score refined the model.

Conclusion: Blood cholesterol below 5.41 mmol/L, direct bilirubin below 56.3 µmol/L on postoperative days 14±3, and a low Desmet score indicate a high probability of efficacious Kasai surgery in infants with BA.

Citing Articles

Laparoscopic vs. open portoenterostomy for biliary atresia: a meta-analysis of pediatric surgical outcomes.

Zhu J, Wu B, Cai P, Pan J, Zhu Z Front Pediatr. 2024; 12:1476195.

PMID: 39717191 PMC: 11663641. DOI: 10.3389/fped.2024.1476195.

References

Shneider B, Brown M, Haber B, Whitington P, Schwarz K, Squires R . A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr. 2006; 148(4):467-474. DOI: 10.1016/j.jpeds.2005.12.054. View

Yassin N, El-Tagy G, Abdelhakeem O, Asem N, El-Karaksy H . Predictors of Short-Term Outcome of Kasai Portoenterostomy for Biliary Atresia in Infants: a Single-Center Study. Pediatr Gastroenterol Hepatol Nutr. 2020; 23(3):266-275. PMC: 7231743. DOI: 10.5223/pghn.2020.23.3.266. View

Ryon E, Parreco J, Sussman M, Quiroz H, Willobee B, Perez E . Drivers of Hospital Readmission and Early Liver Transplant after Kasai Portoenterostomy. J Surg Res. 2020; 256:48-55. DOI: 10.1016/j.jss.2020.06.019. View

Chiang L, Lee C, Krishnaswamy G, Nah S, Kader A, Ong C . Seventeen years of Kasai portoenterostomy for biliary atresia in a single Southeast Asian paediatric centre. J Paediatr Child Health. 2016; 53(4):412-415. DOI: 10.1111/jpc.13379. View

Nguyen A, Pham Y, Vu G, Nguyen M, Hoang T, Holterman A . Biliary atresia liver histopathological determinants of early post-Kasai outcome. J Pediatr Surg. 2021; 56(7):1169-1173. DOI: 10.1016/j.jpedsurg.2021.03.039. View

Wong C, Chung P, Tam P, Wong K . Long-term Results and Quality of Life Assessment in Biliary Atresia Patients: A 35-Year Experience in a Tertiary Hospital. J Pediatr Gastroenterol Nutr. 2017; 66(4):570-574. DOI: 10.1097/MPG.0000000000001854. View

Goda T, Kawahara H, Kubota A, Hirano K, Umeda S, Tani G . The most reliable early predictors of outcome in patients with biliary atresia after Kasai's operation. J Pediatr Surg. 2013; 48(12):2373-7. DOI: 10.1016/j.jpedsurg.2013.08.009. View

Ramachandran P, Safwan M, Srinivas S, Shanmugam N, Vij M, Rela M . The extended Kasai portoenterostomy for biliary atresia: A preliminary report. J Indian Assoc Pediatr Surg. 2016; 21(2):66-71. PMC: 4790131. DOI: 10.4103/0971-9261.176941. View

Shneider B, Magee J, Karpen S, Rand E, Narkewicz M, Bass L . Total Serum Bilirubin within 3 Months of Hepatoportoenterostomy Predicts Short-Term Outcomes in Biliary Atresia. J Pediatr. 2016; 170:211-7.e1-2. PMC: 4826612. DOI: 10.1016/j.jpeds.2015.11.058. View

10.

Sun S, Zheng S, Lu X, Chen G, Ma Y, Chen L . Clinical and pathological features of patients with biliary atresia who survived for more than 5 years with native liver. Pediatr Surg Int. 2018; 34(4):381-386. DOI: 10.1007/s00383-018-4231-7. View

11.

Jain V, Burford C, Alexander E, Sutton H, Dhawan A, Joshi D . Prognostic markers at adolescence in patients requiring liver transplantation for biliary atresia in adulthood. J Hepatol. 2019; 71(1):71-77. DOI: 10.1016/j.jhep.2019.03.005. View

12.

Khanna K, Bhatnagar V, Agarwala S, Srinivas M, Gupta S . Ratio of Preoperative and Postoperative Serum Bilirubin Levels Predicts Early Outcome Following Biliary Atresia Surgery. J Indian Assoc Pediatr Surg. 2018; 23(2):81-86. PMC: 5898209. DOI: 10.4103/jiaps.JIAPS_192_17. View

13.

Liu L, Wei L, Qu W, Liu Y, Zeng Z, Zhang H . Impact of the Kasai Procedure and the Length of Native Liver Survival Time on Outcomes of Liver Transplantation for Biliary Atresia. Liver Transpl. 2021; 28(2):224-235. DOI: 10.1002/lt.26287. View

14.

Razumovskiy A, Degtyareva A, Kulikova N, Ratnikov S . [Advantages of Kasai procedure through minimally invasive approach in children with biliary atresia (in Russian only)]. Khirurgiia (Mosk). 2019; (3):48-59. DOI: 10.17116/hirurgia201903148. View

15.

Tam P, Chung P, St Peter S, Gayer C, Ford H, Tam G . Advances in paediatric gastroenterology. Lancet. 2017; 390(10099):1072-1082. DOI: 10.1016/S0140-6736(17)32284-5. View

16.

Fanna M, Masson G, Capito C, Girard M, Guerin F, Hermeziu B . Management of Biliary Atresia in France 1986 to 2015: Long-term Results. J Pediatr Gastroenterol Nutr. 2019; 69(4):416-424. DOI: 10.1097/MPG.0000000000002446. View

17.

Chung P, Wong K, Tam P . Predictors for failure after Kasai operation. J Pediatr Surg. 2015; 50(2):293-6. DOI: 10.1016/j.jpedsurg.2014.11.015. View

18.

Abdel-Aziz S, Sira M, Gad E, Ayoub I, Soltan M . Preoperative alkaline phosphatase is a potential predictor of short-term outcome of surgery in infants with biliary atresia. Clin Exp Hepatol. 2019; 5(2):155-160. PMC: 6728868. DOI: 10.5114/ceh.2019.85072. View

19.

Ramos-Gonzalez G, Elisofon S, Dee E, Staffa S, Medford S, Lillehei C . Predictors of Need for Liver Transplantation in Children Undergoing Hepatoportoenterostomy for Biliary Atresia. J Pediatr Surg. 2019; 54(6):1127-1131. DOI: 10.1016/j.jpedsurg.2019.02.051. View

20.

Superina R, Magee J, Brandt M, Healey P, Tiao G, Ryckman F . The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. Ann Surg. 2011; 254(4):577-85. PMC: 3460800. DOI: 10.1097/SLA.0b013e3182300950. View