Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

ERCP for biliary disease in children

Moises Guelrud, MD
Section Editors
Elizabeth B Rand, MD
Douglas A Howell, MD, FASGE, FACG
Melvin B Heyman, MD, MPH
Deputy Editor
Alison G Hoppin, MD


Endoscopic retrograde cholangiopancreatography (ERCP) has an important role in the care of children with a variety of biliary tract disorders, which can be divided broadly as congenital or acquired (table 1). This topic review will summarize indications for ERCP in children with biliary diseases, focusing on some of the more common disorders. Most of these disorders are discussed in further detail in their corresponding topic reviews. The technical aspects of performing ERCP in children, including complications, and for the use of ERCP for pancreatic disorders, are presented separately. (See "ERCP in children: Technique, success and complications" and "ERCP for pancreatic disease in children".)


Endoscopic retrograde cholangiopancreatography (ERCP) is an important diagnostic and therapeutic tool for biliary disease in infants and children, as it is in adults. With the advent of new diagnostic imaging techniques such as magnetic resonance cholangiopancreatography (MRCP), ERCP is evolving into a predominantly therapeutic procedure. The usefulness and safety of ERCP in the pediatric age group depends in large part on the experience of the endoscopist. If the necessary experience and equipment are available at an institution, the following are appropriate biliary indications for ERCP:

Diagnostic indications

Neonatal cholestasis (for biliary cysts; and at some institutions as part of the evaluation for suspected biliary atresia)

Investigation of inconclusive abnormal findings on other examinations (eg, MRCP or CT scan)

Therapeutic indications

Biliary obstruction (eg, due to known or suspected choledocholithiasis, bile plug syndrome, parasitic infestation, biliary strictures, or primary sclerosing cholangitis)


Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Sep 2016. | This topic last updated: Oct 6, 2015.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
  1. Liu QY, Nguyen V. Endoscopic approach to the patient with congenital anomalies of the biliary tract. Gastrointest Endosc Clin N Am 2013; 23:505.
  2. Schreiber RA, Kleinman RE. Biliary atresia. J Pediatr Gastroenterol Nutr 2002; 35 Suppl 1:S11.
  3. Derkx HH, Huibregtse K, Taminiau JA. The role of endoscopic retrograde cholangiopancreatography in cholestatic infants. Endoscopy 1994; 26:724.
  4. Iinuma Y, Narisawa R, Iwafuchi M, et al. The role of endoscopic retrograde cholangiopancreatography in infants with cholestasis. J Pediatr Surg 2000; 35:545.
  5. Mitchell SA, Wilkinson ML. The role of ERCP in the diagnosis of neonatal conjugated hyperbilirubinemia. Gastrointest Endosc 1994; 40:A55.
  6. Ohnuma N, Takahashi T, Tanabe M, et al. The role of ERCP in biliary atresia. Gastrointest Endosc 1997; 45:365.
  7. Guelrud M, Carrl Locke DL, Fox VL. ERCP in Pediatric Practice: Diagnosis and Treatment, Isis Medical Media Ltd, Oxford 1997.
  8. Aabakken L, Aagenaes I, Sanengen T, et al. Utility of ERCP in neonatal and infant cholestasis. J Laparoendosc Adv Surg Tech A 2009; 19:431.
  9. Shanmugam NP, Harrison PM, Devlin J, et al. Selective use of endoscopic retrograde cholangiopancreatography in the diagnosis of biliary atresia in infants younger than 100 days. J Pediatr Gastroenterol Nutr 2009; 49:435.
  10. Petersen C, Meier PN, Schneider A, et al. Endoscopic retrograde cholangiopancreaticography prior to explorative laparotomy avoids unnecessary surgery in patients suspected for biliary atresia. J Hepatol 2009; 51:1055.
  11. Keil R, Snajdauf J, Rygl M, et al. Diagnostic efficacy of ERCP in cholestatic infants and neonates--a retrospective study on a large series. Endoscopy 2010; 42:121.
  12. Heyman MB, Shapiro HA, Thaler MM. Endoscopic retrograde cholangiography in the diagnosis of biliary malformations in infants. Gastrointest Endosc 1988; 34:449.
  13. Shteyer E, Wengrower D, Benuri-Silbiger I, et al. Endoscopic retrograde cholangiopancreatography in neonatal cholestasis. J Pediatr Gastroenterol Nutr 2012; 55:142.
  14. Guelrud M, Jaen D, Mendoza S, et al. ERCP in the diagnosis of extrahepatic biliary atresia. Gastrointest Endosc 1991; 37:522.
  15. Morelli A, Pelli MA, Vedovelli A, et al. Endoscopic retrograde cholangiopancreatography study in Alagille's syndrome: first report. Am J Gastroenterol 1983; 78:241.
  16. Guelrud M, Morera C, Rodriguez M, et al. Normal and anomalous pancreaticobiliary union in children and adolescents. Gastrointest Endosc 1999; 50:189.
  17. Misra SP, Dwivedi M. Pancreaticobiliary ductal union. Gut 1990; 31:1144.
  18. Ng WD, Liu K, Wong MK, et al. Endoscopic sphincterotomy in young patients with choledochal dilatation and a long common channel: a preliminary report. Br J Surg 1992; 79:550.
  19. Ito T, Ando H, Nagaya M, Sugito T. Congenital dilatation of the common bile duct in children.--The etiologic significance of the narrow segment distal to the dilated common bile duct. Z Kinderchir 1984; 39:40.
  20. Yamaguchi M. Congenital choledochal cyst. Analysis of 1,433 patients in the Japanese literature. Am J Surg 1980; 140:653.
  21. Leblanc A, Hadchouel M, Jehan P, et al. Obstructive jaundice in children with histiocytosis X. Gastroenterology 1981; 80:134.
  22. Debray D, Pariente D, Urvoas E, et al. Sclerosing cholangitis in children. J Pediatr 1994; 124:49.
  23. DiPalma JA, Strobel CT, Farrow JG. Primary sclerosing cholangitis associated with hyperimmunoglobulin M immunodeficiency (dysgammaglobulinemia). Gastroenterology 1986; 91:464.
  24. Vergani D, Mieli-Vergani G. Autoimmune Hepatitis and PSC Connection. Clin Liver Dis 2008; 12:187.
  25. Miloh T, Arnon R, Shneider B, et al. A retrospective single-center review of primary sclerosing cholangitis in children. Clin Gastroenterol Hepatol 2009; 7:239.
  26. Hiejima E, Komatsu H, Sogo T, et al. Utility of simplified criteria for the diagnosis of autoimmune hepatitis in children. J Pediatr Gastroenterol Nutr 2011; 52:470.
  27. Alpert LI, Jindrak K. Idiopathic retroperitoneal fibrosis and sclerosing cholangitis associated with a reticulum cell sarcoma. Report of a case. Gastroenterology 1972; 62:111.
  28. Werlin SL, Glicklich M, Jona J, Starshak RJ. Sclerosing cholangitis in childhood. J Pediatr 1980; 96:433.
  29. Mieli-Vergani G, Vergani D. Sclerosing cholangitis in the paediatric patient. Best Pract Res Clin Gastroenterol 2001; 15:681.
  30. Deneau M, Jensen MK, Holmen J, et al. Primary sclerosing cholangitis, autoimmune hepatitis, and overlap in Utah children: epidemiology and natural history. Hepatology 2013; 58:1392.
  31. Vo HD, Xu J, Rabinowitz SS, et al. The liver in pediatric gastrointestinal disease. J Pediatr Gastroenterol Nutr 2014; 59:288.
  32. Faubion WA Jr, Loftus EV, Sandborn WJ, et al. Pediatric "PSC-IBD": a descriptive report of associated inflammatory bowel disease among pediatric patients with psc. J Pediatr Gastroenterol Nutr 2001; 33:296.
  33. Ferrara C, Valeri G, Salvolini L, Giovagnoni A. Magnetic resonance cholangiopancreatography in primary sclerosing cholangitis in children. Pediatr Radiol 2002; 32:413.
  34. Chavhan GB, Roberts E, Moineddin R, et al. Primary sclerosing cholangitis in children: utility of magnetic resonance cholangiopancreatography. Pediatr Radiol 2008; 38:868.
  35. Kerkar N, Miloh T. Sclerosing cholangitis: pediatric perspective. Curr Gastroenterol Rep 2010; 12:195.
  36. Classen M, Götze H, Richter HJ, Bender S. Primary sclerosing cholangitis in children. J Pediatr Gastroenterol Nutr 1987; 6:197.
  37. Stoker J, Laméris JS, Robben SG, et al. Primary sclerosing cholangitis in a child treated by nonsurgical balloon dilatation and stenting. J Pediatr Gastroenterol Nutr 1993; 17:303.
  38. Siegel JH, Guelrud M. Endoscopic cholangiopancreatoplasty: hydrostatic balloon dilation in the bile duct and pancreas. Gastrointest Endosc 1983; 29:99.
  39. Guelrud M, Mendoza S, Gelrud A. A tapered balloon with hydrophilic coating to dilate difficult hilar biliary strictures. Gastrointest Endosc 1995; 41:246.
  40. Malik AH, Saima BD, Wani MY. Management of hepatobiliary and pancreatic ascariasis in children of an endemic area. Pediatr Surg Int 2006; 22:164.
  41. Baba AA, Shera AH, Bhat MA, et al. Management of biliary ascariasis in children living in an endemic area. Eur J Pediatr Surg 2010; 20:187.
  42. Shaw PJ, Spitz L, Watson JG. Extrahepatic biliary obstruction due to stone. Arch Dis Child 1984; 59:896.
  43. Guelrud M, Mendoza S, Jaen D, et al. ERCP and endoscopic sphincterotomy in infants and children with jaundice due to common bile duct stones. Gastrointest Endosc 1992; 38:450.
  44. 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; 50:303.
  45. Nilsson S. Gallbladder disease and sex hormones. A statistical study. Acta Chir Scand 1966; 132:275.
  46. Arcement CM, Meza MP, Arumanla S, Towbin RB. MRCP in the evaluation of pancreaticobiliary disease in children. Pediatr Radiol 2001; 31:92.
  47. Topal B, Van de Moortel M, Fieuws S, et al. The value of magnetic resonance cholangiopancreatography in predicting common bile duct stones in patients with gallstone disease. Br J Surg 2003; 90:42.
  48. Scheers I, Ergun M, Aouattah T, et al. Diagnostic and Therapeutic Roles of Endoscopic Ultrasound in Pediatric Pancreaticobiliary Disorders. J Pediatr Gastroenterol Nutr 2015; 61:238.
  49. Guelrud M, Daoud G, Mendoza S, et al. Endoscopic sphincterotomy in a 6-month-old infant with choledocholithiasis and double gallbladder. Am J Gastroenterol 1994; 89:1587.
  50. Buckley A, Connon JJ. The role of ERCP in children and adolescents. Gastrointest Endosc 1990; 36:369.
  51. Brown K. Use of ERCP with pancreatic and biliary sphincterotomy for treatment of familial pancreatitis in a 2 year old pediatric patient. Gastrointest Endosc 1993; 39:A309.
  52. Man DW, Spitz L. Choledocholithiasis in infancy. J Pediatr Surg 1985; 20:65.
  53. Tarnasky PR, Tagge EP, Hebra A, et al. Minimally invasive therapy for choledocholithiasis in children. Gastrointest Endosc 1998; 47:189.
  54. Sandoval C, Stringel G, Ozkaynak MF, et al. Perioperative management in children with sickle cell disease undergoing laparoscopic surgery. JSLS 2002; 6:29.
  55. Komatsu Y, Kawabe T, Toda N, et al. Endoscopic papillary balloon dilation for the management of common bile duct stones: experience of 226 cases. Endoscopy 1998; 30:12.
  56. Wesdorp I, Bosman D, de Graaff A, et al. Clinical presentations and predisposing factors of cholelithiasis and sludge in children. J Pediatr Gastroenterol Nutr 2000; 31:411.
  57. Vrochides DV, Sorrells DL Jr, Kurkchubasche AG, et al. Is there a role for routine preoperative endoscopic retrograde cholangiopancreatography for suspected choledocholithiasis in children? Arch Surg 2005; 140:359.
  58. Bickerstaff KI, Britton BJ, Gough MH. Endoscopic palliation of malignant biliary obstruction in a child. Br J Surg 1989; 76:1092.
  59. Guelrud M, Mendoza S, Zager A, Noguera C. Biliary stenting in an infant with malignant obstructive jaundice. Gastrointest Endosc 1989; 35:259.
  60. Paganelli M, Beaunoyer M, Samson Y, et al. A child with unresectable biliary rhabdomyosarcoma: 48-month disease-free survival after liver transplantation. Pediatr Transplant 2014; 18:E146.
  61. Zhan J, Bao G, Hu X, et al. Carcinoid tumor of the common bile duct in children: a case report. J Pediatr Surg 2010; 45:2061.
  62. Feier FH, Chapchap P, Pugliese R, et al. Diagnosis and management of biliary complications in pediatric living donor liver transplant recipients. Liver Transpl 2014; 20:882.
  63. Darius T, Rivera J, Fusaro F, et al. Risk factors and surgical management of anastomotic biliary complications after pediatric liver transplantation. Liver Transpl 2014; 20:893.
  64. Pfau PR, Kochman ML, Lewis JD, et al. Endoscopic management of postoperative biliary complications in orthotopic liver transplantation. Gastrointest Endosc 2000; 52:55.
  65. Rerknimitr R, Sherman S, Fogel EL, et al. Biliary tract complications after orthotopic liver transplantation with choledochocholedochostomy anastomosis: endoscopic findings and results of therapy. Gastrointest Endosc 2002; 55:224.
  66. Otto AK, Neal MD, Mazariegos GV, et al. Endoscopic retrograde cholangiopancreatography is safe and effective for the diagnosis and treatment of pancreaticobiliary disease following abdominal organ transplant in children. Pediatr Transplant 2012; 16:829.
  67. Ulitsky A, Werlin S, Dua KS. Role of ERCP in the management of non-iatrogenic traumatic bile duct injuries in the pediatric population. Gastrointest Endosc 2011; 73:823.
  68. Kulaylat AN, Stokes AL, Engbrecht BW, et al. Traumatic bile leaks from blunt liver injury in children: a multidisciplinary and minimally invasive approach to management. J Pediatr Surg 2014; 49:424.