Native Liver Survivors of Portoenterostomy for Biliary Atresia with Excellent Outcome: Redefining "successful" Portoenterostomy

Overview

Journal Pediatr Surg Int

Specialties General Surgery
Pediatrics

Date 2022 Dec 1

PMID 36454515

Authors

Koichi Tsuboi

Hiroko Watayo

Takafumi Tsukui

Kazuto Suda

Eri Abe

Takamori Fujimoto

Takanori Ochi

Geoffrey J Lane

Hiroyuki Koga

Atsuyuki Yamataka

Affiliations

Soon will be listed here.

Abstract

Purpose: Native liver survivors (NLS) after portoenterostomy (PE) for biliary atresia (BA) with normal biomarkers defined as total bilirubin (T-Bil), aspartate aminotransferase (AST)/alanine aminotransferase (ALT) for liver function (LF), cholinesterase (ChE), platelet count (PC), and absence of portal hypertension (PHT) were reviewed to redefine "successful" PE.

Methods: 92 post-PE BA patients were classified as NLS-1: normal biomarkers, PHT (-); NLS-2: at least one abnormal biomarker, PHT (-); NLS-3: normal biomarkers, PHT ( +); NLS-4: abnormal biomarkers, PHT ( +) and reviewed for a maximum 32 years.

Results: As of June 2022, 55/92 (59.8%) had received liver transplants and 37/92 (40.2%) were NLS. NLS patients were classified as excellent outcome (EO): NLS-1 (n = 10; 27.0%) or non-EO: NLS-2: (n = 8; 21.6%), NLS-3: (n = 6; 16.2%), and NLS-4: (n = 13; 35.1%). Compared with non-EO, EO had PE earlier (50.5 versus 65 days; not significant; p = 0.08), significantly earlier onset of symptoms (13 days versus 32 days; p = 0.01) and significantly shorter jaundice-clearance (JC; 34.5 days versus 56.0 days; p < 0.001). Durations of follow-up were similar: 13 years in EO, 18.5 years in NLS-2, 20 years in NLS-3, and 15 years in NLS-4.

Conclusion: Incidence of "successful" PE or EO is low and correlated with early onset of symptoms and quicker JC.

Citing Articles

Comparison of different Kasai portoenterostomy techniques in the outcomes of biliary atresia: a systematic review and network meta-analysis.

Zhang Y, Liu S, Yang Q, Sun R, Liu J, Meng Y Pediatr Surg Int. 2024; 41(1):6.

PMID: 39592482 DOI: 10.1007/s00383-024-05920-9.

Bile acid profiles in adult patients with biliary atresia who achieve native liver survival after portoenterostomy.

Takeda M, Takei H, Suzuki M, Tsukui T, Tsuboi K, Watayo H Sci Rep. 2024; 14(1):2492.

PMID: 38291117 PMC: 10827714. DOI: 10.1038/s41598-024-52969-6.

Robotic-assisted Kasai portoenterostomy for biliary atresia.

Zhang M, Cao G, Li X, Zhang X, Li Y, Chi S Surg Endosc. 2023; 37(5):3540-3547.

PMID: 36602550 DOI: 10.1007/s00464-022-09855-x.

References

Nio M . Japanese Biliary Atresia Registry. Pediatr Surg Int. 2017; 33(12):1319-1325. DOI: 10.1007/s00383-017-4160-x. View

Hopkins P, Yazigi N, Nylund C . Incidence of Biliary Atresia and Timing of Hepatoportoenterostomy in the United States. J Pediatr. 2017; 187:253-257. DOI: 10.1016/j.jpeds.2017.05.006. View

Sharma S, Gupta D . Surgical modifications, additions, and alternatives to Kasai hepato-portoenterostomy to improve the outcome in biliary atresia. Pediatr Surg Int. 2017; 33(12):1275-1282. DOI: 10.1007/s00383-017-4162-8. View

Nakamura H, Koga H, Wada M, Miyano G, Dizon R, Kato Y . Reappraising the portoenterostomy procedure according to sound physiologic/anatomic principles enhances postoperative jaundice clearance in biliary atresia. Pediatr Surg Int. 2011; 28(2):205-9. DOI: 10.1007/s00383-011-3019-9. View

Zhang M, Xun P, He K, Cai W . Adjuvant steroid treatment following Kasai portoenterostomy and clinical outcomes of biliary atresia patients: an updated meta-analysis. World J Pediatr. 2016; 13(1):20-26. DOI: 10.1007/s12519-016-0052-8. View

Luo Y, Zheng S . Current concept about postoperative cholangitis in biliary atresia. World J Pediatr. 2008; 4(1):14-9. DOI: 10.1007/s12519-008-0003-0. View

Sundaram S, Mack C, Feldman A, Sokol R . Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care. Liver Transpl. 2016; 23(1):96-109. PMC: 5177506. DOI: 10.1002/lt.24640. View

Antala S, Taylor S . Biliary Atresia in Children: Update on Disease Mechanism, Therapies, and Patient Outcomes. Clin Liver Dis. 2022; 26(3):341-354. PMC: 9309872. DOI: 10.1016/j.cld.2022.03.001. View

Okubo R, Nio M, Sasaki H . Impacts of Early Kasai Portoenterostomy on Short-Term and Long-Term Outcomes of Biliary Atresia. Hepatol Commun. 2021; 5(2):234-243. PMC: 7850309. DOI: 10.1002/hep4.1615. View

10.

Chan K, Lee K, Wong H, Tsui S, Mou J, Tam Y . Ten-Year Native Liver Survival Rate After Laparoscopic and Open Kasai Portoenterostomy for Biliary Atresia. J Laparoendosc Adv Surg Tech A. 2018; 29(1):121-125. DOI: 10.1089/lap.2018.0350. View

11.

Nio M, Ohi R, Shimaoka S, Iwami D, Sano N . The outcome of surgery for biliary atresia and the current status of long-term survivors. Tohoku J Exp Med. 1997; 181(1):235-44. DOI: 10.1620/tjem.181.235. View

12.

Bijl E, Bharwani K, Houwen R, de Man R . The long-term outcome of the Kasai operation in patients with biliary atresia: a systematic review. Neth J Med. 2013; 71(4):170-3. View

13.

Parolini F, Boroni G, Milianti S, Tonegatti L, Armellini A, Garcia Magne M . Biliary atresia: 20-40-year follow-up with native liver in an Italian centre. J Pediatr Surg. 2018; 54(7):1440-1444. DOI: 10.1016/j.jpedsurg.2018.10.060. View

14.

Jain V, Burford C, Alexander E, Dhawan A, Joshi D, Davenport M . Adult Liver Disease Prognostic Modelling for Long-term Outcomes in Biliary Atresia: An Observational Cohort Study. J Pediatr Gastroenterol Nutr. 2021; 73(1):93-98. DOI: 10.1097/MPG.0000000000003116. View

15.

Sundaram S, Alonso E, Haber B, Magee J, Fredericks E, Kamath B . Health related quality of life in patients with biliary atresia surviving with their native liver. J Pediatr. 2013; 163(4):1052-7.e2. PMC: 4014354. DOI: 10.1016/j.jpeds.2013.04.037. View

16.

Koga H, Miyano G, Takahashi T, Shimotakahara A, Kato Y, Lane G . Laparoscopic portoenterostomy for uncorrectable biliary atresia using Kasai's original technique. J Laparoendosc Adv Surg Tech A. 2011; 21(3):291-4. DOI: 10.1089/lap.2010.0162. View

17.

Nakajima H, Koga H, Okawada M, Nakamura H, Lane G, Yamataka A . Does time taken to achieve jaundice-clearance influence survival of the native liver in post-Kasai biliary atresia?. World J Pediatr. 2018; 14(2):191-196. DOI: 10.1007/s12519-018-0139-5. View

18.

Nakamura H, Ara M, Koga H, Miyano G, Okawada M, Doi T . Duration from the first pale stool to portoenterostomy is prognostic in biliary atresia. Comparison with age at portoenterostomy. Clin Res Hepatol Gastroenterol. 2021; 45(5):101584. DOI: 10.1016/j.clinre.2020.11.014. View

19.

Wiesner R, McDiarmid S, Kamath P, Edwards E, Malinchoc M, Kremers W . MELD and PELD: application of survival models to liver allocation. Liver Transpl. 2001; 7(7):567-80. DOI: 10.1053/jlts.2001.25879. View

20.

Malinchoc M, Kamath P, Gordon F, Peine C, Rank J, ter Borg P . A model to predict poor survival in patients undergoing transjugular intrahepatic portosystemic shunts. Hepatology. 2000; 31(4):864-71. DOI: 10.1053/he.2000.5852. View