International Surgery Journal | November 2017 | Vol 4 | Issue 11 Page 3705 International Surgery Journal Rawat J et al. Int Surg J. 2017 Nov;4(11):3705-3710 http://www.ijsurgery.com pISSN 2349-3305 | eISSN 2349-2902 Original Research Article Congenital choledochal malformations in children: management strategies Jiledar Rawat, Sudhir Singh*, Digamber Chaubey INTRODUCTION The incidence of Congenital Choledochal Malformations (CCM) in Asia is 1/1000 live birth. 1 The exact incidence in India is not known. CCM is more common in females with a ratio of 3:1. Presentation in children is very nonspecific and vague. The classical presentation is triad of symptoms pain abdomen, palpable mass in right upper abdomen and jaundice. Although abdominal ultrasound scan (USG) is the first and easily available diagnostic modality but Magnetic resonance cholangiopancreatography (MRCP) is the “gold standard” for the diagnosis and planning the management. Contrast enhanced computed tomography (CECT) abdomen is also helpful in cases present with abdominal mass. Endoscopic retrograde cholangiopancreatography (ERCP) is an invasive investigation and require expertise in children. The most commonly followed classification for CCM was initially given by Alonso-Lej et al. in 1959 and later Todani et al. in 1977 modified it. 2,3 Lilly et al. described and Sarin et al. give clinical significance of an entity called “forme frusta. 4,5 The most commonly proposed theory for CCM is supposed to be caused by an anomalous pancreatic-biliary junction. Complications of CCM include pancreatitis, cholangitis, secondary biliary ABSTRACT 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. Keywords: Congenital Choledochal Malformations (CCM), Hepaticoduodenostomy (HD), Roux en Y Hepaticojejunostomy (RYHJ) Department of Pediatric Surgery, King George’s Medical University, Lucknow-226003, India Received: 28 August 2017 Accepted: 28 September 2017 *Correspondence: Dr. Sudhir Singh, E-mail: drsudhir_singh25@yahoo.in Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. DOI: http://dx.doi.org/10.18203/2349-2902.isj20174890