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Pediatric Biliary Atresia Follow-up

  • Author: Steven M Schwarz, MD, FAAP, FACN, AGAF; Chief Editor: Carmen Cuffari, MD  more...
Updated: Jun 28, 2016

Further Outpatient Care

A meta-analysis reported that moderate high-dose steroid therapy improves jaundice clearance, especially for infants who undergo hepatoportoenterostomy by 70days of age. However, more randomized controlled trials with longer follow-up are necessary to demonstrate the effect of steroids on the long-term outcomes of biliary atresia.[17]



Complications following portoenterostomy in patients with biliary atresia include both acute and chronic problems.

  • In the early postoperative phase, an unsuccessful anastomosis with failure to achieve adequate bile drainage is the most common complication. In this case, adequacy of bile flow may be predicted by the preoperative liver histology and the caliber of bile ductular remnants in the porta hepatis. In one third of all patients, bile flow is inadequate following surgery, and these children succumb to complications of biliary cirrhosis in the first few years of life unless orthotopic liver transplantation is performed.
  • Later in the course, complications related to progressive liver disease and portal hypertension occur in more than 60% of infants who achieved initial surgical success.
  • Cholangitis develops in 50% of patients following portoenterostomy.
  • Hepatocellular carcinoma may be a risk for those patients with cirrhosis and no clinical evidence of portal hypertension. Progressive fibrosis and biliary cirrhosis develop in children who do not drain bile, and liver transplantation is the only option for long-term survival.
  • Detailed management of these complications is described in Histologic Findings, Medical Care, Consultations, Diet, and Medications.


See the list below:

  • Data regarding outcome from centers worldwide widely vary. The initial success rate of Kasai portoenterostomy (for achieving bile flow) is 60-80%. Clearly, the most critical determinant of outcome remains age at the time of operation. Although individual centers have reported favorable surgical results in some infants older than 3 months, patients are significantly less likely to require early liver transplantation if the portoenterostomy is performed when they are younger than 10 weeks. In the postoperative period, the rate of decline in serum bilirubin levels directly correlates with a positive prognosis.
  • One study evaluated 244 infants who were enrolled in the prospective Childhood Liver Disease Research and Education Network and underwent Kasai portoenterostomy (KPE). The results noted that at 1 and 2 years post-KPE, the transplant-free survival rate was 53.7% and 46.7%, respectively. Risk of transplant/death was significantly lower in patients who achieved bile drainage within 3 months post-KPE, while it increased in patients with porta hepatis atresia, nonpatent common bile duct, biliary atresia splenic malformation syndrome, nodular liver appearance compared with firm, and age at KPE. No association with outcome was noted with gestational age, sex, race, ethnicity, or extent of porta hepatis dissection.[18]
  • As discussed in Mortality/Morbidity, bile flow, even if achieved at surgery, may be inadequate in as many as one third of patients after the initial postoperative period. These children require early (< 2 y) liver transplantation. Practice guidelines for the evaluation of a patient for liver transplantation have been established by the American Association for the Study of Liver Diseases.[19] Factors that predict improved long-term outcome after Kasai portoenterostomy include the following:
    • Younger than 10 weeks (in some reports, 2 mo) at operation
    • Preoperative histology and ductal remnant size
    • Presence of bile in hepatic lobular zone 1
    • Absence of portal hypertension, cirrhosis, and associated anomalies
    • Experience of the surgical team
    • Postoperative clearing of jaundice
  • The following 3 categories of patients with extrahepatic biliary atresia should be considered for reexploration following a Kasai or modified Kasai portoenterostomy:
    • Infants who become jaundiced after an initial anicteric phase postoperatively
    • Infants with favorable hepatic and biliary duct remnant histology at initial operation, who do not successfully drain bile
    • Infants who may have had an inadequate initial surgery
  • Extrahepatic biliary atresia is the most common primary diagnosis in children requiring orthotopic liver transplantation (OLT), comprising more than 50% of patients with liver transplants in most series.[20]
    • Overall, a review demonstrated that 66% of infants undergoing the Kasai procedure ultimately required OLT, including more than 50% of patients who initially achieved bile drainage.
    • In most series reported to date, the primary indications for OLT are the symptoms of end-stage liver disease and/or hepatic failure, including progressive cholestasis, recurrent cholangitis, poorly controlled portal hypertension, intractable ascites, decreased hepatic synthetic function (eg, hypoalbuminemia, coagulopathy unresponsive to vitamin K), and growth failure.
    • As long-term outcomes following OLT in children continue to improve (along with increased living donor availability) using split-liver grafts, application of this surgical modality for early treatment of biliary atresia will likely increase, certainly in patients with inadequate bile flow following portoenterostomy.
Contributor Information and Disclosures

Steven M Schwarz, MD, FAAP, FACN, AGAF Professor of Pediatrics, Children's Hospital at Downstate, State University of New York Downstate Medical Center

Steven M Schwarz, MD, FAAP, FACN, AGAF is a member of the following medical societies: American Academy of Pediatrics, American College of Nutrition, American Association for Physician Leadership, New York Academy of Medicine, Gastroenterology Research Group, American Gastroenterological Association, American Pediatric Society, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Stefano Guandalini, MD Founder and Medical Director, Celiac Disease Center, Chief, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Chicago Medical Center; Professor, Department of Pediatrics, Section of Gastroenterology, Hepatology and Nutrition, University of Chicago Division of the Biological Sciences, The Pritzker School of Medicine

Stefano Guandalini, MD is a member of the following medical societies: American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, European Society for Paediatric Gastroenterology, Hepatology & Nutrition, North American Society for the Study of Celiac Disease

Disclosure: Received consulting fee from AbbVie for consulting.

Chief Editor

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers' bureau.

Additional Contributors

Jorge H Vargas, MD Professor of Pediatrics and Clinical Professor of Pediatric Gastroenterology, University of California, Los Angeles, David Geffen School of Medicine; Consulting Physician, Department of Pediatrics, University of California at Los Angeles Health System

Jorge H Vargas, MD is a member of the following medical societies: American Liver Foundation, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

  1. Haber BA, Erlichman J, Loomes KM. Recent advances in biliary atresia: prospects for novel therapies. Expert Opin Investig Drugs. 2008 Dec. 17(12):1911-24. [Medline].

  2. Bassett MD, Murray KF. Biliary atresia: recent progress. J Clin Gastroenterol. 2008 Jul. 42(6):720-9. [Medline].

  3. Mogul D, Zhou M, Intihar P, Schwarz K, Frick K. Cost-Effective Analysis of Screening for Biliary Atresia With The Stool Color Card. J Pediatr Gastroenterol Nutr. 2014 Sep 11. [Medline].

  4. Fischler B, Ehrnst A, Forsgren M, et al. The viral association of neonatal cholestasis in Sweden: a possible link between cytomegalovirus infection and extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr. 1998 Jul. 27(1):57-64. [Medline].

  5. Chang MH, Huang HH, Huang ES, et al. Polymerase chain reaction to detect human cytomegalovirus in livers of infants with neonatal hepatitis. Gastroenterology. 1992 Sep. 103(3):1022-5. [Medline].

  6. Wilson GA, Morrison LA, Fields BN. Association of the reovirus S1 gene with serotype 3-induced biliary atresia in mice. J Virol. 1994 Oct. 68(10):6458-65. [Medline]. [Full Text].

  7. Steele MI, Marshall CM, Lloyd RE, Randolph VE. Reovirus 3 not detected by reverse transcriptase-mediated polymerase chain reaction analysis of preserved tissue from infants with cholestatic liver disease. Hepatology. 1995 Mar. 21(3):697-702. [Medline].

  8. Wang W, Donnelly B, Bondoc A, Mohanty SK, McNeal M, Ward R, et al. The rhesus rotavirus gene encoding VP4 is a major determinant in the pathogenesis of biliary atresia in newborn mice. J Virol. 2011 Jun 22. EPub ahead of print. [Medline]. [Full Text].

  9. Uemura M, Ozawa A, Nagata T, et al. Sox17 haploinsufficiency results in perinatal biliary atresia and hepatitis in C57BL/6 background mice. Development. 2013 Feb. 140(3):639-48. [Medline].

  10. Shneider BL, Magee JC, Karpen SJ, et al. Total Serum Bilirubin within 3 Months of Hepatoportoenterostomy Predicts Short-Term Outcomes in Biliary Atresia. J Pediatr. 2016 Mar. 170:211-217.e2. [Medline].

  11. Zhou L, Shan Q, Tian W, Wang Z, Liang J, Xie X. Ultrasound for the Diagnosis of Biliary Atresia: A Meta-Analysis. AJR Am J Roentgenol. 2016 May. 206 (5):W73-82. [Medline].

  12. Shteyer E, Wengrower D, Benuri-Silbiger I, Gozal D, Wilschanski M, Goldin E. Endoscopic retrograde cholangiopancreatography in neonatal cholestasis. J Pediatr Gastroenterol Nutr. 2012 Aug. 55(2):142-5. [Medline].

  13. Ng VL, Haber BH, Magee JC, Miethke A, Murray KF, Michail S, et al. Medical status of 219 children with biliary atresia surviving long-term with their native livers: results from a North American multicenter consortium. J Pediatr. 2014 Sep. 165 (3):539-546.e2. [Medline].

  14. Willot S, Uhlen S, Michaud L, Briand G, Bonnevalle M, Sfeir R, et al. Effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia. Pediatrics. 2008 Dec. 122(6):e1236-41. [Medline].

  15. Lindor KD, Kowdley KV, Luketic VA, et al. High-dose ursodeoxycholic acid for the treatment of primary sclerosing cholangitis. Hepatology. 2009 Sep. 50(3):808-14. [Medline]. [Full Text].

  16. Bezerra JA, Spino C, Magee JC, Shneider BL, Rosenthal P, Wang KS, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA. 2014 May 7. 311 (17):1750-9. [Medline].

  17. Chen Y, Nah SA, Chiang L, Krishnaswamy G, Low Y. Postoperative steroid therapy for biliary atresia: Systematic review and meta-analysis. J Pediatr Surg. 2015 Jun 5. [Medline].

  18. Superina R, Magee JC, Brandt ML, et al. 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 Oct. 254(4):577-585. [Medline].

  19. [Guideline] Murray KF, Carithers RL Jr. AASLD practice guidelines: Evaluation of the patient for liver transplantation. Hepatology. 2005 Jun. 41(6):1407-32. [Medline].

  20. Tessier ME, Harpavat S, Shepherd RW, Hiremath GS, Brandt ML, Fisher A, et al. Beyond the Pediatric end-stage liver disease system: solutions for infants with biliary atresia requiring liver transplant. World J Gastroenterol. 2014 Aug 28. 20(32):11062-8. [Medline]. [Full Text].

  21. Balistreri WF, Grand R, Hoofnagle JH, et al. Biliary atresia: current concepts and research directions. Summary of a symposium. Hepatology. 1996 Jun. 23(6):1682-92. [Medline].

  22. Barshes NR, Lee TC, Balkrishnan R, et al. Orthotopic liver transplantation for biliary atresia: the U.S. experience. Liver Transpl. 2005 Oct. 11(10):1193-200. [Medline].

  23. [Guideline] Bates MD, Bucuvalas JC, Alonso MH, Ryckman FC. Biliary atresia: pathogenesis and treatment. Semin Liver Dis. 1998. 18(3):281-93. [Medline].

  24. Bittmann S. Surgical experience in children with biliary atresia treated with portoenterostomy. Curr Surg. 2005 Jul-Aug. 62(4):439-43. [Medline].

  25. Chin LT, D'Alessandro AM, Knechtle SJ, et al. Liver transplantation for biliary atresia: 19-year, single-center experience. Exp Clin Transplant. 2004 Jun. 2(1):178-82. [Medline].

  26. el-Youssef M, Whitington PF. Diagnostic approach to the child with hepatobiliary disease. Semin Liver Dis. 1998. 18(3):195-202. [Medline].

  27. Karrer FM, Price MR, Bensard DD, et al. Long-term results with the Kasai operation for biliary atresia. Arch Surg. 1996 May. 131(5):493-6. [Medline].

  28. Kasai M. Treatment of biliary atresia with special reference to hepatic porto- enterostomy and its modifications. Prog Pediatr Surg. 1974. 6:5-52. [Medline].

  29. Lai MW, Chang MH, Hsu SC, et al. Differential diagnosis of extrahepatic biliary atresia from neonatal hepatitis: a prospective study. J Pediatr Gastroenterol Nutr. 1994 Feb. 18(2):121-7. [Medline].

  30. Mack CL, Sokol RJ. Unraveling the pathogenesis and etiology of biliary atresia. Pediatr Res. 2005 May. 57(5 Pt 2):87R-94R. [Medline]. [Full Text].

  31. Matsuo S, Suita S, Kubota M, Shono K. Long-term results and clinical problems after portoenterostomy in patients with biliary atresia. Eur J Pediatr Surg. 1998 Jun. 8(3):142-5. [Medline].

  32. Mowat AP. Biliary atresia into the 21st century: a historical perspective. Hepatology. 1996 Jun. 23(6):1693-5. [Medline].

  33. Muraji T, Higashimoto Y. The improved outlook for biliary atresia with corticosteroid therapy. J Pediatr Surg. 1997 Jul. 32(7):1103-6; discussion 1106-7. [Medline].

  34. Nio M, Ohi R, Shimaoka S, et al. The outcome of surgery for biliary atresia and the current status of long-term survivors. Tohoku J Exp Med. 1997 Jan. 181(1):235-44. [Medline].

  35. Okazaki T, Kobayashi H, Yamataka A, et al. Long-term postsurgical outcome of biliary atresia. J Pediatr Surg. 1999 Feb. 34(2):312-5. [Medline].

  36. 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 Jul. 20(1 Pt 2):41S-48S. [Medline].

  37. [Guideline] Ryckman FC, Alonso MH, Bucuvalas JC, Balistreri WF. Biliary atresia--surgical management and treatment options as they relate to outcome. Liver Transpl Surg. 1998 Sep. 4(5 Suppl 1):S24-33. [Medline].

  38. Tan CE, Davenport M, Driver M, Howard ER. Does the morphology of the extrahepatic biliary remnants in biliary atresia influence survival? A review of 205 cases. J Pediatr Surg. 1994 Nov. 29(11):1459-64. [Medline].

  39. Tanaka H, Kita Y, Kawarasaki H, et al. Beneficial effect of ursodeoxycholic acid on serum gamma-GTP in patients with biliary atresia following living related liver transplantation. Transplant Proc. 1998 Nov. 30(7):3326-7. [Medline].

  40. [Guideline] Utterson EC, Shepherd RW, Sokol RJ, et al. Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation. J Pediatr. 2005 Aug. 147(2):180-5. [Medline].

  41. Valayer J. Conventional treatment of biliary atresia: long-term results. J Pediatr Surg. 1996 Nov. 31(11):1546-51. [Medline].

  42. Visser BC, Suh I, Hirose S, et al. The influence of portoenterostomy on transplantation for biliary atresia. Liver Transpl. 2004 Oct. 10(10):1279-86. [Medline].

  43. Yoon PW, Bresee JS, Olney RS, et al. Epidemiology of biliary atresia: a population-based study. Pediatrics. 1997 Mar. 99(3):376-82. [Medline]. [Full Text].

Biliary atresia.
Bile ductular proliferation in liver biopsy specimen (hematoxylin and eosin stain) from patient with biliary atresia. Also note hepatocellular bile staining as a consequence of cholestasis.
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