Congenital choledochal malformations in children: management strategies

Jiledar Rawat, Sudhir Singh, Digamber Chaubey


Background: A Congenital Choledochal Malformations (CCM) is common congenital defect of biliary tree in Asian subcontinent. Presentations of CCM are vague from asymptomatic to life threatening cholangitis or pancreatitis. Complete cyst excision and bilioenteric anastomosis is now accepted surgical treatment.

Methods: This is a retrospective study of five years duration. In this study the clinical presentation, haematological, biochemical and radiological findings, operative procedure and outcome were studied from case records.

Results: Total of 20 patients were studied, with clinical presentation of recurrent abdominal pain in eight cases, previous history of cholangitis in five cases, acute cholangitis two cases, biliary peritonitis in four cases and previous history of pancreatitis in one case. In 13 cases Roux-en-Y hepaticojejunostomy (RYHJ) and in 4 cases hepaticoduodenostomy (HD) done after cyst excision. One case of type II CCM managed with only cyst excision, in two cases Lilly’s procedure, one case requires temporary external drainage and in two cases temporary internal drainage done in view on cholangitis and jaundice.

Conclusions: However, the definitive treatment of CCM is complete cyst excision and bilioenteric anastomosis, though lot of other temporary majors are also required at different stages of disease.


Congenital Choledochal Malformations (CCM), Hepaticoduodenostomy (HD), Roux en Y Hepaticojejunostomy (RYHJ)

Full Text:



Soares KC, Arnaoutakis DJ, Kamel I, Rastegar N, Anders R, Maithel S, et al. Choledochal cysts: presentation, clinical differentiation, and management. J Am Coll Surg. 2014;219:1167-80.

Alonso-Lej F, Rever WB, Pessagno DJ. Congenital choledochal cyst, with a report of 2, and an analysis of 94, cases. Int Abstr Surg. 1959;108:1-30.

Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K. Congenital bile duct cysts: Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am J Surg. 1977;134:263-9.

Lilly JR, Stellin GP, Karrer FM. Forme fruste choledochal cyst. J Pediatr Surg. 1985;20:449-51.

Sarin YK, Sengar M, Puri AS. Forme fruste choledochal cyst. Indian Pediatr. 2005;42:1153-5.

Lilly JR. The surgical treatment of choledochal cyst. Surg Gynecol Obstet. 1979;149:36-42.

Babbit DP. Congenital choledochal cyst: New etiological concept based on anomalous relationships of common bile duct and pancreatic bulb. Ann Radiol. 1969;12:231-40.

Mc Loughlin M, Mitchell R. Sphincter of Oddi dysfunction and pancreatitis. World J Gastroenterol. WJG. 2007;13(47):6333-43.

Arah R, Lone AH, Khursheed O, Baasit S, Rashid S, Omar, Shah J, et al. To study the clinicopathological status, role of various diagnostic and treatment modalities and outcome of patients with choledochal cyst in Kashmir valley. Int J Health Sci Res. 2015;5(1):39-50.

Edil BH, Cameron JL, Reddy S, Lum Y, Lipsett PA, Nathan H, et al. Choledochal cyst disease in children and adults: a 30-year single-institution experience. Am Coll Surg. 2008;206:1000-5.

Weber FP. Cystic dilatation of the common bile duct. Br J Child Dis. 1934;31:27-9.

Sarin YK, Singh VP. Biliary ascites caused by perforation of choledochal cyst. Indian Pediatrics. 1995;32(7):815-7.

Meschino M, García-Ochoa C, Hernandez-Alejandro R. Ruptured choledochal cyst: a rare presentation and unique approach to management. Hepatobiliary Surg Nutrition. 2015;4(1):E8-12.

Goel P, Jain V, Manchanda V, Sengar M, Gupta CR, Mohta A. Spontaneous Biliary Perforations: An Uncommon yet Important Entity in Children. J Clin Diagnostic Res JCDR. 2013;7(6):1201-6.

Chauhan S, Pandit A, Jacob M, Kumar P. Spontaneously ruptured choledochal cyst: Rare diagnosis on hepatic scintigraphy. Ind J Nuclear Med: IJNM. 2011;26(3):157-8.

Lal R, Agrawal S, Shivhare R, Kumar A, Sikora S, Kapoor V, et al. Management of complicated choledochal cysts. Dig Surg. 2007;24:456-62.

Suzuki M, Sai JK, Shimizu T. Acute pancreatitis in children and adolescents. World J Gastrointest Pathophysiol. 2014;5:416-26.

Fujishiro J, Masumoto K, Urita Y, Shinkai T, Gotoh C. Pancreatic complications in pediatric choledochal cysts. J Pediatric Surg. 2013;48:(9);1897-902.

Banks PA, Conwell DL, Toskes PP. The management of acute and chronic pancreatitis. Gastroenterol Hepatol (NY). 2010;6:1-16.

Shimotakahara A, Yamataka A, Yanai T, Kobayashi H, Okazaki T, Lane GJ, 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;21:5-7.

Elhalaby E, Hashish A, Elbarbary M, Elwagih M. Roux -En-Y. hepaticojejunostomy versus hepaticoduodenostmy for biliary reconstruction after excision of choledochal cysts in children. Ann Paediatr Surg. 2005;1:79-85.

Silva-Baeza H, Coello-Ramírezb P, MizraímIxtabalán-Escalanteb E, Sotelo-Anayac E, Gallo-Moralesc M, et al. Treatment of choledochal cyst in a pediatric population. A single institution experience of 15-years. Case series. Ann Med Surg. 2016;5:81-5.

Mukhopadhyay B, Shukla RM, Mukhopadhyay M, Mandal KC, Mukherjee PP, Roy D, et al. Choledochal cyst: a review of 79 cases and the role of hepaticodochoduodenostomy. Journal of Indian Association Pediatric Surgeons. 2011;16(2):54-7.

Narayanan SK, Chen Y, Narasimhan KL, Cohen RC. Hepaticoduodenostomy versus hepaticojejunostomy after resection of choledochal cyst: A systematic review and meta-analysis. J Pediatr Surg. 2013;48:2336-42.