Korean J Transplant.  2021 Sep;35(3):177-182. 10.4285/kjt.21.0018.

Indications and outcomes of liver transplantation for post-Kasai biliary atresia in young adults

• Namgoong JM
• Hwang S
• Ahn CS
• Moon DB
• Ha TY
• Song GW
• Jung DH
• Park GC
• Yoon YI
• Kim KM
• Lee SG

• KMID: 2520680
• DOI: http://doi.org/10.4285/kjt.21.0018

Abstract

Background
Some young adults have a long survival period with native liver after Kasai portoenterostomy (KPE) for biliary atresia (BA). However, a considerable proportion of these patients require liver transplantation (LT). This study aimed to analyze the indications and outcomes of LT in young adults after a long survival period with native liver after KPE.
Methods
We selected seven patients who were 18 years or older at the time of LT out of 116 BA patients who underwent primary LT from 2008 to 2019 at Asan Medical Center.
Results
The mean ages at KPE and LT were 2.1±0.9 months and 22.0±5.1 years, respectively. Mean serum total bilirubin level and model for end-stage liver disease score at LT were 7.91±7.22 mg/dL and 15.3±6.0, respectively. The main reasons for LT were liver cirrhosis with portal hypertension-associated complications in five patients and intractable cholangitis in two patients. There were five cases of living donor LT and two cases of deceased donor LT. All the seven patients are currently alive during the mean follow-up period of 74.7±40.9 months. One patient suffered from outflow graft vein obstruction requiring endovascular stenting. Another patient showed core antibody-positivity-induced de novo hepatitis B virus infection, which was well managed with antiviral therapy.
Conclusions
Young adult patients with BA are a unique group of patients requiring specialist care regarding transition from pediatric to adult services. The outcomes of LT in young adult BA patients were excellent. Therefore, LT should be considered in patients showing serious BA-associated complications.

Keyword

Portal hypertension; Cholangitis; Liver cirrhosis; Portoenterostomy; Adolescence

Figure

• 

  Fig. 1 Distribution of recipient age at the time of liver transplantation for biliary atresia.

• 

  Fig. 2 Peritransplant computed tomography findings of the seven young adult recipients with biliary atresia. The numbers indicate case numbers.

Reference

1. Livesey E, Cortina Borja M, Sharif K, Alizai N, McClean P, Kelly D, et al. 2009; Epidemiology of biliary atresia in England and Wales (1999-2006). Arch Dis Child Fetal Neonatal Ed. 94:F451–5. DOI: 10.1136/adc.2009.159780. PMID: 19457876.

2. Davenport M, Ong E, Sharif K, Alizai N, McClean P, Hadzic N, et al. 2011; Biliary atresia in England and Wales: results of centralization and new benchmark. J Pediatr Surg. 46:1689–94. DOI: 10.1016/j.jpedsurg.2011.04.013. PMID: 21929975.

3. Bijl EJ, Bharwani KD, Houwen RH, de Man RA. 2013; The long-term outcome of the Kasai operation in patients with biliary atresia: a systematic review. Neth J Med. 71:170–3.

4. Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O. 2005; Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology. 41:366–71. DOI: 10.1002/hep.20547. PMID: 15660386.

5. Hsu EK, Mazariegos GV. 2017; Global lessons in graft type and pediatric liver allocation: a path toward improving outcomes and eliminating wait-list mortality. Liver Transpl. 23:86–95. DOI: 10.1002/lt.24646. PMID: 27706890. PMCID: PMC6767049.

6. Sasaki H, Tanaka H, Wada M, Kazama T, Nakamura M, Kudo H, et al. 2016; Analysis of the prognostic factors of long-term native liver survival in survivors of biliary atresia. Pediatr Surg Int. 32:839–43. DOI: 10.1007/s00383-016-3934-x. PMID: 27464487.

7. Samyn M, Davenport M, Jain V, Hadzic N, Joshi D, Heneghan M, et al. 2019; Young people with biliary atresia requiring liver transplantation: a distinct population requiring specialist care. Transplantation. 103:e99–107. DOI: 10.1097/TP.0000000000002553. PMID: 30461724.

8. Nio M, Sano N, Ishii T, Sasaki H, Hayashi Y, Ohi R. 2004; Cholangitis as a late complication in long-term survivors after surgery for biliary atresia. J Pediatr Surg. 39:1797–9. DOI: 10.1016/j.jpedsurg.2004.08.021. PMID: 15616933.

9. Kyoden Y, Tamura S, Sugawara Y, Yamashiki N, Matsui Y, Togashi J, et al. 2008; Outcome of living donor liver transplantation for post-Kasai biliary atresia in adults. Liver Transpl. 14:186–92. DOI: 10.1002/lt.21344. PMID: 18236393.

10. Kumagi T, Drenth JP, Guttman O, Ng V, Lilly L, Therapondos G, et al. 2012; Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review. Liver Int. 32:510–8. DOI: 10.1111/j.1478-3231.2011.02668.x. PMID: 22098694.

11. Uchida Y, Kasahara M, Egawa H, Takada Y, Ogawa K, Ogura Y, et al. 2006; Long-term outcome of adult-to-adult living donor liver transplantation for post-Kasai biliary atresia. Am J Transplant. 6:2443–8. DOI: 10.1111/j.1600-6143.2006.01487.x. PMID: 16889600.

12. Kang SH, Hwang S, Ahn CS, Kim KH, Moon DB, Ha TY, et al. 2020; Changes in the indications for living donor liver transplantation: single-institution experience of 3,145 cases over 10 years. Korean J Transplant. 34:47–54.

13. Ha H, Hong J, Kim I, Lee S, Lee A, Ha T, et al. 2019; Deceased donor liver transplantation under the Korean model for end-stage liver disease score-based liver allocation system: 2-year allocation results at a high-volume transplantation center. Korean J Transplant. 33:112–117. DOI: 10.4285/jkstn.2019.33.4.112.

14. Namgoong J, Hwang S, Kim D, Ha T, Song G, Jung D, et al. 2020; Whole liver deceased donor liver transplantation for pediatric recipients: single-center experience for 20 years. Korean J Transplant. 34:249–56. DOI: 10.4285/kjt.20.0036.

15. Bae SK, Akamatsu N, Togashi J, Ichida A, Kawahara T, Maki H, et al. 2019; Hepatitis B virus recurrence after living donor liver transplantation of anti-HBc-positive grafts: a 22-year experience at a single center. Biosci Trends. 13:448–55. DOI: 10.5582/bst.2019.01283. PMID: 31666441.

16. Cholongitas E, Papatheodoridis GV, Burroughs AK. 2010; Liver grafts from anti-hepatitis B core positive donors: a systematic review. J Hepatol. 52:272–9. DOI: 10.1016/j.jhep.2009.11.009. PMID: 20034693.

17. Wong TC, Fung JY, Cui TY, Lam AH, Dai JW, Chan AC, et al. 2019; Liver transplantation using hepatitis B core positive grafts with antiviral monotherapy prophylaxis. J Hepatol. 70:1114–22. DOI: 10.1016/j.jhep.2019.03.003. PMID: 30871981.

18. Suehiro T, Shimada M, Kishikawa K, Shimura T, Soejima Y, Yoshizumi T, et al. 2005; Prevention of hepatitis B virus infection from hepatitis B core antibody-positive donor graft using hepatitis B immune globulin and lamivudine in living donor liver transplantation. Liver Int. 25:1169–74. DOI: 10.1111/j.1478-3231.2005.01165.x. PMID: 16343068.