eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology

Biliary Atresia: Follow-up

Author: Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, Children's Hospital at Downstate, SUNY-Downstate Medical Center
Contributor Information and Disclosures

Updated: Apr 28, 2009

Follow-up

Complications

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.

Prognosis

  • 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.
  • 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.8 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.
    • 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.

Miscellaneous

Medicolegal Pitfalls

  • Although not strictly a medical-legal issue, whether portoenterostomy or orthotopic liver transplantation (OLT) is the best initial therapy for extrahepatic biliary atresia remains controversial.
  • Transplantation has certainly been suggested as the initial procedure of choice because of its excellent long-term survival rate and the fact that more than 60% of infants undergoing the Kasai procedure ultimately require OLT. However, a careful review of available data indicates that overall survival statistics are not significantly altered by primary transplantation.
  • In addition, at a time when organ procurement problems remain the most important obstacles to survival for patients requiring OLT, portoenterostomy represents a therapeutic option that secures favorable long-term outcomes in a significant number of patients with biliary atresia.
 


More on Biliary Atresia

Overview: Biliary Atresia
Differential Diagnoses & Workup: Biliary Atresia
Treatment & Medication: Biliary Atresia
Follow-up: Biliary Atresia
Multimedia: Biliary Atresia
References
[ CLOSE WINDOW ]

References

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

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

  3. 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. Jul 1998;27(1):57-64. [Medline].

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

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

  6. 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. Mar 1995;21(3):697-702. [Medline].

  7. 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. Dec 2008;122(6):e1236-41. [Medline].

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

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

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

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

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

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

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

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

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

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

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

  19. 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. Jun 1998;8(3):142-5. [Medline].

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

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

  22. 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. Jan 1997;181(1):235-44. [Medline].

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

  24. 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. Jul 1994;20(1 Pt 2):41S-48S. [Medline].

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

  26. 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. Nov 1994;29(11):1459-64. [Medline].

  27. 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. Nov 1998;30(7):3326-7. [Medline].

  28. 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. Aug 2005;147(2):180-5. [Medline].

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

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

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

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

biliary atresia, extrahepatic biliary atresia, cholestasis, jaundice, obliteration of the extrahepatic biliary system, discontinuity of the extrahepatic biliary system, obstruction of bile flow, bile obstruction, biliary obstruction, secondary biliary cirrhosis, isolated biliary atresia, postnatal biliary atresia, situs inversus, polysplenia, asplenia, liver transplantation, end-stage liver disease, cholangitis, portal hypertension, hepatocellular carcinoma, hepatomegaly, splenomegaly, diagnosis, treatment

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, Children's Hospital at Downstate, SUNY-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 College of Physician Executives, American Gastroenterological Association, American Pediatric Society, Gastroenterology Research Group, New York Academy of Medicine, North American Society for Pediatric Gastroenterology and Nutrition, and Society for Pediatric Research
Disclosure: TAP Pharmaceuticals Honoraria Speaking and teaching; Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor

Medical Editor

Jorge H Vargas, MD, Professor of Pediatrics and Clinical Professor of Pediatric Gastroenterology, David Geffen School of Medicine, University of California at Los Angeles; 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, American Society for Gastrointestinal Endoscopy, American Society for Parenteral and Enteral Nutrition, Latin American Society of Pediatric Gastroenterology, Hepatology & Nutrition, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Stefano Guandalini, MD, Director, University of Chicago Celiac Disease Program, Section Chief of Gastroenterology, Hepatology and Nutrition; Professor, Department of Pediatrics, University of Chicago Comer Children's Hospital
Stefano Guandalini, MD is a member of the following medical societies: American Gastroenterological Association, European Society for Paediatric Gastroenterology, Hepatology & Nutrition, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

CME Editor

David Pallares, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville
David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology
Disclosure: Nothing to disclose.

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, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.