Biliary atresia: The profile, management and outcome of patients treated at a tertiary hospital in central South Africa


  • E Brits Department of Surgery, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa
  • S M le Grange Department of Surgery, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa



biliary atresia, developing country, Lesotho, South Africa


Background. Biliary atresia (BA) is an obstructive inflammatory disease of the bile ducts. Without intervention, the disease rapidly progresses to liver cirrhosis and fibrosis, with end-stage liver failure and death occurring within the first 3 years of life. It is the most common indication for liver transplantation (LT) in the paediatric population. The management of BA in South Africa (SA) faces multiple challenges, such as late referrals and socioeconomic burdens, with suboptimal outcomes.

Objectives. To determine risk factors and shortcomings that are detrimental to the outcome of the paediatric patient population by reviewing the profile, management and outcome of patients with BA treated at Universitas Academic Hospital Complex (UAHC), Bloemfontein, SA.

Methods. This was a retrospective analytical record review of all patients diagnosed with BA and treated at UAHC from 1 January 2009 to 31 December 2019.

Results. In total, 67 patients were included; 74.6% were female, and 86.6% were black Africans. Most (62.7%) had isolated BA. A Kasai portoenterostomy (KPE) was performed in 32 patients (47.8%). Of 5 patients referred for LT evaluation, 2 received a transplant. Of 55 patients with known outcomes, 5.5% (n=3) survived and 94.5% (n=52) died after receiving a KPE or palliative treatment. Of the 3 patients who were alive at the end of the study period, 1 had a KPE and 2 had LTs.

Conclusion. Late presentation, cholangitis and cessation of bile flow after an initial successful KPE, and socioeconomic challenges are issues of concern and had a detrimental influence on the outcome of BA in our study population. Implementing screening measures and education programmes at the primary healthcare level is essential to diagnose and refer BA patients timeously. Establishing support systems to assist socioeconomically disadvantaged patients will enable them to qualify for LT.

Author Biography

  • S M le Grange, Department of Surgery, Faculty of Health Sciences, University of the Free State, Bloemfontein, South Africa

    Consultant Paediatric Surgery, Department of Surgery, Faculty of Health Sciences


Davenport M. Biliary atresia. Semin Pediatr Surg 2005;14(1):42-48. sempedsurg.2004.10.024

Superina R. Biliary atresia and liver transplantation: Results and thoughts for primary liver transplantation in select patients. Pediatr Surg Int 2017;33(12):1297-1304. s00383-017-4174-4

Lin YC, Chang MH, Liao SF, et al. Decreasing rate of biliary atresia in Taiwan: A survey, 2004 - 2009. Pediatrics 2011;128(3):e530-e536.

Wada H, Muraji T, Yokoi A, et al. Insignificant seasonal and geographical variation in incidence of biliary atresia in Japan: A regional survey of over 20 years. J Pediatr Surg 2007;42(12):2090-2092.

Livesey E, Cortina Borja M, Sharif K, et al. Epidemiology of biliary atresia in England and Wales (1999 - 2006). Arch Dis Child Fetal Neonatal Ed 2009;94(6):F451-F455. adc.2009.159780

Chardot C, Carton M, Spire-Bendelac N, le Pommelet C, Golmard JL, Auvert B. Epidemiology of biliary atresia in France: A national study 1986 - 96. J Hepatol 1999;31(6):1006-1013. https://doi. org/10.1016/s0168-8278(99)80312-2

Jimenez-Rivera C, Jolin-Dahel KS, Fortinsky KJ, Gozdyra P, Benchimol EI. International incidence and outcomes of biliary atresia. J Pediatr Gastroenterol Nutr 2013;56(4):344-354. MPG.0b013e318282a913

Hopkins PC, Yazigi N, Nylund CM. Incidence of biliary atresia and timing of hepatoportoenterostomy in the United States. J Pediatr 2017;187:253-257.

Chiu CY, Chen PH, Chan CF, Chang MH, Wu TC; Taiwan Infant Stool Color Card Study Group. Biliary atresia in preterm infants in Taiwan: A nationwide survey. J Pediatr 2013;163(1):100-3.e1.

Van Wessel DB, Boere T, Hulzebos CV, et al. Preterm infants with biliary atresia: A nationwide cohort analysis from the Netherlands. J Pediatr Gastroenterol Nutr 2017;65(4):370-374. https://doi. org/10.1097/MPG.0000000000001692

Tam PKH, Yiu RS, Lendahl U, Andersson ER. Cholangiopathies – towards a molecular understanding [published correction appears in EBioMedicine 2018 Oct;36:564]. EBioMedicine 2018;35:381-393.

Burns J, Davenport M. Adjuvant treatments for biliary atresia. Transl Pediatr 2020;9(3):253-265.

Davenport M. Biliary atresia: From Australia to the zebrafish. J Pediatr Surg 2016;51(2):200-205.

Sokol RJ, Mack C, Narkewicz MR, Karrer FM. Pathogenesis and outcome of biliary atresia: Current concepts. J Pediatr Gastroenterol Nutr 2003;37(1):4-21. 200307000-00003

Harpavat S, Finegold MJ, Karpen SJ. Patients with biliary atresia have elevated direct/conjugated bilirubin levels shortly after birth. Pediatrics 2011;128(6):e1428-e1433. peds.2011-1869

Davenport M, Savage M, Mowat AP, Howard ER. Biliary atresia splenic malformation syndrome: An etiologic and prognostic subgroup. Surgery 1993;113(6):662-668.

Asai A, Miethke A, Bezerra JA. Pathogenesis of biliary atresia: Defining biology to understand clinical phenotypes. Nat Rev Gastroenterol Hepatol 2015;12(6):342-352. nrgastro.2015.74

Schwarz KB, Haber BH, Rosenthal P, et al. Extrahepatic anomalies in infants with biliary atresia: Results of a large prospective North American multicenter study. Hepatology 2013;58(5):1724-1731.

Zani A, Quaglia A, Hadzić N, Zuckerman M, Davenport M. Cytomegalovirus-associated biliary atresia: An aetiological and prognostic subgroup. J Pediatr Surg 2015;50(10):1739-1745. https://doi. org/10.1016/j.jpedsurg.2015.03.001

Parolini F, Hadzic N, Davenport M. Adjuvant therapy of cytomegalovirus IgM + ve associated biliary atresia: Prima facie evidence of effect. J Pediatr Surg 2019;54(9):1941-1945. jpedsurg.2018.12.014

Lakshminarayanan B, Davenport M. Biliary atresia: A comprehensive review. J Autoimmun 2016;73:1- 9.

Kaur N, Goyal G, Garg R, Tapasvi C, Chawla S, Kaur R. Potential role of noninvasive biomarkers during liver fibrosis. World J Hepatol 2021;13(12):1919-1935.

Bravo AA, Sheth SG, Chopra S. Liver biopsy. N Engl J Med 2001;344(7):495-500. https://doi. org/10.1056/NEJM20010215344070

Capparelli MA, Ayarzabal VH, Halac ET, et al. Preoperative risk factors for the early failure of the Kasai portoenterostomy in patients with biliary atresia. Pediatr Surg Int 2021;37(9):1183-1189. https://doi. org/10.1007/s00383-021-04933-y

El-Araby HA, Saber MA, Radwan NM, Taie DM, Adawy NM, Sira AM. Temporal histopathological changes in biliary atresia: A perspective for rapid fibrosis progression. Ann Hepatol 2021;21:100263.

Chardot C, Buet C, Serinet MO, et al. Improving outcomes of biliary atresia: French national series 1986 - 2009. J Hepatol 2013;58(6):1209-1217.

Bondoc AJ, Taylor JA, Alonso MH, et al. The beneficial impact of revision of Kasai portoenterostomy for biliary atresia: An institutional study. Ann Surg 2012;255(3):570-576. SLA.0b013e318243a46e

KakosCD,ZiogasIA,AlexopoulosSP,TsoulfasG.Managementofbiliaryatresia:Totransplantornot to transplant. World J Transplant 2021;11(9):400-409.

Scottoni F, Davenport M. Biliary atresia: Potential for a new decade. Semin Pediatr Surg 2020;29(4):150940.

Serinet MO, Wildhaber BE, Broué P, et al. Impact of age at Kasai operation on its results in late childhood and adolescence: A rational basis for biliary atresia screening. Pediatrics 2009;123(5):1280- 1286.

Chung PHY, Zheng S, Tam PKH. Biliary atresia: East versus West. Semin Pediatr Surg 2020;29(4):150950. SA, Venkat V, Arnon R, et al. Improved outcomes for liver transplantation in patients with biliary atresia since pediatric end- stage liver disease implementation: Analysis of the Society of Pediatric Liver Transplantation Registry. J Pediatr 2020;219:89-97.

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(7):997-1000. s0022-3468(03)00178-7

DavenportM,OngE,SharifK,etal.BiliaryatresiainEnglandandWales:Resultsofcentralisationand new benchmark. J Pediatr Surg 2011;46(9):1689-1694. 34. Davenport M, Grieve A. Maximizing Kasai portoenterostomy in the treatment of biliary atresia:

Medical and surgical options. S Afr Med J 2012;102(11 Pt 2):865-867.


Levin LN. Biliary atresia at Red Cross War Memorial Children’s Hospital: A retrospective descriptive study reviewing the age of presentation, clinical course and outcome of infants presenting to RCWMCH with biliary atresia. Master’s thesis. Cape Town: Department of Paediatrics and Child Health, Faculty of Health Sciences, University of Cape Town, 2016. (accessed 30 October 2019).

Moore SW, Zabiegaj-Zwick C, Nel E. Problems related to CMV infection and biliary atresia. S Afr Med J 2012;102(11 Pt 2):890-892.

Spearman CW, McCulloch M, Millar AJ, et al. Liver transplantation at Red Cross War Memorial Children’s Hospital. S Afr Med J 2006;96(9 Pt 2):960-963.

Lala SG, Britz R, Botha J, Loveland J. Paediatric liver transplantation for children treated at public health facilities in South Africa: Time for change. S Afr Med J 2014;104(11):829-832. https://doi. org/10.7196/SAMJ.8624

Van der Schyff F, Terblanche AJ, Botha JF. Improving poor outcomes of children with biliary atresia in South Africa by early referral to centralised units. JPGN Rep 2021;2(2):e073 PG9.0000000000000073

Zuckerman M, Hajinicolaou C. Incidence and outcome of biliary atresia in black infants in Soweto (South Africa): Review of cases from 1993 - 1996. J Pediatr Gastroenterol Nutr 1998;26(5):587.

The French METAVIR Cooperative Study Group, Bedossa P. Intraobserver and interobserver variations in liver biopsy interpretation in patients with chronic hepatitis C. Hepatology 1994;20(1):15- 20.

Davenport M, Caponcelli E, Livesey E, Hadzic N, Howard E. Surgical outcome in biliary atresia: Etiology affects the influence of age at surgery. Ann Surg 2008;247(4):694-698. SLA.0b013e3181638627

Lien TH, Chang MH, Wu JF, et al. Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan. Hepatology 2011;53(1):202-208. hep.24023

Alonso EM, Ye W, Hawthorne K, et al. Impact of steroid therapy on early growth in infants with biliary atresia: The Multicenter Steroids in Biliary Atresia Randomised Trial. J Pediatr 2018;202:179-185.e4.

Karangwa OR. A retrospective review of the outcome of children presenting to Tygerberg Children’s Hospital with biliary atresia. PhD thesis. Cape Town: Stellenbosch University, 2016. https:// bfff8b317d009a9a79a438126be21887ba3b4989 (accessed 4 October 2023).

Human Sciences Research Council. The Fifth South African National HIV Prevalence, Incidence, Behaviour and Communication Survey, 2017. Pretoria: HSRC, 2018. pageContent/9234/SABSSMV_Impact_Assessment_Summary_ZA_ADS_cleared_PDFA4.pdf (accessed 29 May 2019).

Xu Y, Yu J, Zhang R, et al. The perinatal infection of cytomegalovirus is an important etiology for biliary atresia in China. Clin Pediatr (Phila) 2012;51(2):109-113. 48. Davenport M, Puricelli V, Farrant P, et al. The outcome of the older (≥100 days) infant with biliary

atresia. J Pediatr Surg 2004;39(4):575-581.

Sanghai SR, Shah I, Bhatnagar S, Murthy A. Incidence and prognostic factors associated with biliary

atresia in western India. Ann Hepatol 2009;8(2):120-122.

Koga H, Wada M, Nakamura H, et al. Factors influencing jaundice-free survival with the native liver

in post-portoenterostomy biliary atresia patients: Results from a single institution. J Pediatr Surg 2013;48(12):2368-2372.







How to Cite

Brits E, le Grange SM. Biliary atresia: The profile, management and outcome of patients treated at a tertiary hospital in central South Africa. S Afr Med J [Internet]. 2023 Nov. 6 [cited 2024 May 21];113(11):57-62. Available from:

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