Current Gastroenterology Reports

, Volume 13, Issue 2, pp 193–201 | Cite as

Biliary Disease in Children



Biliary diseases in children are infrequent; however, they can be associated with high morbidity and mortality if an accurate diagnosis is not made and adequate treatment provided in a timely fashion. Biliary atresia, choledochal cysts, gallbladder disease, and Alagille syndrome can be associated with similar clinical symptoms, laboratory findings, and radiographic findings, which makes accurate diagnosis difficult. The correct treatment for each of these clinical entities is different and can significantly reduce morbidity and mortality from these diseases. In this article, we discuss the epidemiology, approach to diagnosis, prognosis, and treatment modalities for these four disease processes.


Biliary atresia Choledochal cyst Cholelithiasis Biliary dyskinesia Kasai Alagille syndrome Hyperbilirubinemia Hepatoportoenterostomy Hepatic iminodiacetic acid scan Magnetic resonance cholangiopancreatography Cholecystectomy Endoscopic retrograde cholangiopancreatography Cholestasis Liver transplantation 



Conflicts of interest: M. Goldman—none; T. Pranikoff—none.


Papers of particular interest, published recently, have been highlighted as: •• Of major importance

  1. 1.
    McEvoy CF, Suchy FJ: Biliary tract disease in children. Pediatr. Clin. North Am. 1996;43(1):75–98.CrossRefPubMedGoogle Scholar
  2. 2.
    Ashcraft KW, Holcomb GW, Murphy JP: Pediatric Surgery. Edited by Holcomb GW. 4th ed. Saunders; 2004.Google Scholar
  3. 3.
    •• Makin E, Quaglia A, Kvist N, et al.: Congenital biliary atresia: liver injury begins at birth. J. Pediatr. Surg. 2009;44(3):630–633. In this retrospective study, patients who were diagnosed with biliary atresia during infancy were evaluated for the timing of liver injury in biliary atresia. Three infants studied had undergone surgical intervention for other presumed diagnoses with concomitant liver biopsies. Diagnosis of biliary atresia was later confirmed in each of these infants, although their liver biopsies within the first week of life were histologically normal. CrossRefPubMedGoogle Scholar
  4. 4.
    Bezerra JA: Biliary atresia–translational research on key molecular processes regulating biliary injury and obstruction. Chang Gung Med J. 2006;29(3):222–230.PubMedGoogle Scholar
  5. 5.
    Hays DM, Snyder WH: Life-span in untreated biliary atresia. Surgery. 1963;54:373–375.PubMedGoogle Scholar
  6. 6.
    Chang M: Screening for biliary atresia. Chang Gung Med J. 2006;29(3):231–233.PubMedGoogle Scholar
  7. 7.
    •• Lee WS, Chai PF: Clinical features differentiating biliary atresia from other causes of neonatal cholestasis. Ann. Acad. Med. Singap. 2010;39(8):648–654. In this prospective trial from Malaysia from 1994 to 2004, the authors attempted to find clinical features that help to differentiate biliary atresia from other causes of neonatal cholestasis in 146 patients who presented with jaundice in the first 4 months of life. They found no specific clinical features with adequate sensitivity or specificity to differentiate biliary atresia from many other causes of neonatal cholestasis. PubMedGoogle Scholar
  8. 8.
    Kelly DA, Stanton A: Jaundice in babies: implications for community screening for biliary atresia. BMJ. 1995;310(6988):1172–1173.PubMedGoogle Scholar
  9. 9.
    Matsui A, Ishikawa T: Identification of infants with biliary atresia in Japan. Lancet. 1994;343(8902):925.CrossRefPubMedGoogle Scholar
  10. 10.
    Rozel C, Garel L, Rypens F, et al.: Imaging of biliary disorders in children. Pediatr Radiol. 2010.Google Scholar
  11. 11.
    Lee H, Lee S, Park W, Choi S: Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology. 2003;229(2):395–400.CrossRefPubMedGoogle Scholar
  12. 12.
    •• Raval MV, Dzakovic A, Bentrem DJ, et al.: Trends in age for hepatoportoenterostomy in the United States. Surgery. 2010;148(4):785–791; discussion 791–792. This retrospective study evaluated the age (in days) at which 192 patients underwent Kasai hepatoportoenterostomy for biliary atresia. It showed that, although early operative intervention is associated with better outcomes, the age at operation has not improved over the past two decades. The investigators showed that the age at operation is significantly better at larger institutions, and noted that socioeconomic factors play a role in diagnosis and early treatment. CrossRefPubMedGoogle Scholar
  13. 13.
    Haber BA, Erlichman J, Loomes KM: Recent advances in biliary atresia: prospects for novel therapies. Expert Opin Investig Drugs. 2008;17(12):1911–1924.CrossRefPubMedGoogle Scholar
  14. 14.
    Shneider BL, Brown MB, Haber B, et al.: A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J. Pediatr. 2006;148(4):467–474.CrossRefPubMedGoogle Scholar
  15. 15.
    •• Shinkai M, Ohhama Y, Take H, et al.: Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: >20-year experience at a children's hospital. J. Pediatr. Gastroenterol. Nutr. 2009;48(4):443–450. This retrospective chart review examined 80 patients who received surgical intervention for biliary atresia from 1970 to 1986. The study focused on long-term outcomes in those patients who did not subsequently require liver transplantation. It reveals that age at surgery and postoperative bilirubin are associated with development of liver failure. Several of the long-term medical comorbidities in patients 20 years after undergoing surgical intervention are discussed. CrossRefPubMedGoogle Scholar
  16. 16.
    Chung HY, Kak Yuen Wong K, Cheun Leung Lan L, Kwong Hang Tam P: Evaluation of a standardized protocol in the use of steroids after Kasai operation. Pediatr. Surg. Int. 2008;24(9):1001–1004.CrossRefPubMedGoogle Scholar
  17. 17.
    Davenport M, Stringer MD, Tizzard SA, et al.: Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia. Hepatology. 2007;46(6):1821–1827.CrossRefPubMedGoogle Scholar
  18. 18.
    •• Lao OB, Larison C, Garrison M, et al.: Steroid use after the Kasai procedure for biliary atresia. Am. J. Surg. 2010;199(5):680–684. This study examined 516 children at 42 institutions from 2003 to 2008 who underwent surgical intervention for biliary atresia, and studied outcomes of those who received perioperative steroids versus those who did not. Although this study was not able to find statistically significant reductions in cholangitis and mortality, it showed that steroids in the perioperative setting are associated with shorter hospital stay postoperatively. As mentioned above, the steroid debate continues in the treatment of biliary atresia. CrossRefPubMedGoogle Scholar
  19. 19.
    Willot S, Uhlen S, Michaud L, et al.: Effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia. Pediatrics. 2008;122(6):e1236–1241.CrossRefPubMedGoogle Scholar
  20. 20.
    •• Kamath BM, Loomes KM, Piccoli DA: Medical management of Alagille syndrome. J. Pediatr. Gastroenterol. Nutr. 2010;50(6):580–586. This recently published review article provides an in-depth view of clinical evaluation and management of patients with Alagille syndrome. It focuses on management of the liver disease and the associated comorbidities in Alagille syndrome. CrossRefPubMedGoogle Scholar
  21. 21.
    Emerick KM, Rand EB, Goldmuntz E, et al.: Features of Alagille syndrome in 92 patients: frequency and relation to prognosis. Hepatology. 1999;29(3):822–829.CrossRefPubMedGoogle Scholar
  22. 22.
    Krantz ID, Piccoli DA, Spinner NB: Clinical and molecular genetics of Alagille syndrome. Curr. Opin. Pediatr. 1999;11(6):558–564.CrossRefPubMedGoogle Scholar
  23. 23.
    Kamath BM, Bason L, Piccoli DA, et al.: Consequences of JAG1 mutations. J. Med. Genet. 2003;40(12):891–895.CrossRefPubMedGoogle Scholar
  24. 24.
    •• Kaye AJ, Rand EB, Munoz PS, et al.: Effect of Kasai procedure on hepatic outcome in Alagille syndrome. J. Pediatr. Gastroenterol. Nutr. 2010;51(3):319–321. This retrospective review of the clinical database at Children's Hospital of Philadelphia evaluates 430 patients with a diagnosis of Alagille syndrome, focusing on outcomes of those patients who underwent hepatoportenterostomy (19) versus those who did not receive surgical intervention. PubMedGoogle Scholar
  25. 25.
    Kamath BM, Schwarz KB, Hadzić N: Alagille syndrome and liver transplantation. J. Pediatr. Gastroenterol. Nutr. 2010;50(1):11–15.CrossRefPubMedGoogle Scholar
  26. 26.
    Ling SC: Congenital cholestatic syndromes: what happens when children grow up? Can. J. Gastroenterol. 2007;21(11):743–751.PubMedGoogle Scholar
  27. 27.
    Singhavejsakul J, Ukarapol N: Choledochal cysts in children: epidemiology and outcomes. World J Surg. 2008;32(7):1385–1388.CrossRefPubMedGoogle Scholar
  28. 28.
    Singham J, Yoshida EM, Scudamore CH: Choledochal cysts: part 1 of 3: classification and pathogenesis. Can J Surg. 2009;52(5):434–440.PubMedGoogle Scholar
  29. 29.
    •• Huang CS, Huang CC, Chen DF: Choledochal cysts: differences between pediatric and adult patients. J. Gastrointest. Surg. 2010;14(7):1105–1110. This study aims to differentiate clinical presentation and pathologic differences in children versus adults at the time of diagnosis of choledochal cyst disease. Although the classic presentation of jaundice, abdominal pain, and a palpable abdominal mass is described in many studies, this retrospective study finds that children tend to present with a palpable abdominal mass, whereas adults are more likely to present with abdominal pain and no palpable mass. Both groups were found to present with jaundice without any statistical difference. CrossRefPubMedGoogle Scholar
  30. 30.
    Singham J, Yoshida EM, Scudamore CH: Choledochal cysts: part 2 of 3: Diagnosis. Can J Surg. 2009;52(6):506–511.PubMedGoogle Scholar
  31. 31.
    Lipsett PA, Pitt HA: Surgical treatment of choledochal cysts. J Hepatobiliary Pancreat Surg. 2003;10(5):352–359.CrossRefPubMedGoogle Scholar
  32. 32.
    Dabbas N, Davenport M: Congenital choledochal malformation: not just a problem for children. Ann R Coll Surg Engl. 2009;91(2):100–105.CrossRefPubMedGoogle Scholar
  33. 33.
    Kim M, Han SJ, Yoon CS, et al.: Using MR cholangiopancreatography to reveal anomalous pancreaticobiliary ductal union in infants and children with choledochal cysts. AJR Am J Roentgenol. 2002;179(1):209–214.PubMedGoogle Scholar
  34. 34.
    Park DH, Kim M, Lee SK, et al.: Can MRCP replace the diagnostic role of ERCP for patients with choledochal cysts? Gastrointest. Endosc. 2005;62(3):360–366.Google Scholar
  35. 35.
    Tao K, Lu Y, Wang T, Dou K: Procedures for congenital choledochal cysts and curative effect analysis in adults. HBPD INT. 2002;1(3):442–445.PubMedGoogle Scholar
  36. 36.
    Shi LB, Peng SY, Meng XK, et al.: Diagnosis and treatment of congenital choledochal cyst: 20 years' experience in China. World J. Gastroenterol. 2001;7(5):732–734.PubMedGoogle Scholar
  37. 37.
    Shimotakahara A, Yamataka A, Yanai T, et al.: Roux-en-Y hepaticojejunostomy or hepaticoduodenostomy for biliary reconstruction during the surgical treatment of choledochal cyst: which is better? Pediatr. Surg. Int. 2005;21(1):5–7.CrossRefPubMedGoogle Scholar
  38. 38.
    Watanabe Y, Toki A, Todani T: Bile duct cancer developed after cyst excision for choledochal cyst. J Hepatobiliary Pancreat Surg. 1999;6(3):207–212.CrossRefPubMedGoogle Scholar
  39. 39.
    Miltenburg DM, Schaffer R, Breslin T, Brandt ML: Changing indications for pediatric cholecystectomy. Pediatrics. 2000;105(6):1250–1253.CrossRefPubMedGoogle Scholar
  40. 40.
    Hofeldt M, Richmond B, Huffman K, et al.: Laparoscopic cholecystectomy for treatment of biliary dyskinesia is safe and effective in the pediatric population. Am Surg. 2008;74(11):1069–1072.PubMedGoogle Scholar
  41. 41.
    Lugo-Vicente HL: Gallbladder dyskinesia in children. JSLS. 1997;1(1):61–64.PubMedGoogle Scholar
  42. 42.
    Kaye AJ, Jatla M, Mattei P, et al.: Use of laparoscopic cholecystectomy for biliary dyskinesia in the child. J. Pediatr. Surg. 2008;43(6):1057–1059.CrossRefPubMedGoogle Scholar
  43. 43.
    Punia R, Garg S, Bisht B, et al.: Clinico-pathological spectrum of gallbladder disease in children. Acta Paediatr. 2010.Google Scholar
  44. 44.
    Chan S, Currie J, Malik AI, Mahomed AA: Paediatric cholecystectomy: Shifting goalposts in the laparoscopic era. Surg Endosc. 2008;22(5):1392–1395.CrossRefPubMedGoogle Scholar
  45. 45.
    Bogue CO, Murphy AJ, Gerstle JT, et al.: Risk factors, complications, and outcomes of gallstones in children: a single-center review. J. Pediatr. Gastroenterol. Nutr. 2010;50(3):303–308.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of Surgery, Section of Pediatric SurgeryWake Forest University School of MedicineWinston SalemUSA

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