Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 6, 2019; 7(9): 1073-1079
Published online May 6, 2019. doi: 10.12998/wjcc.v7.i9.1073
Rare variant of pancreaticobiliary maljunction associated with pancreas divisum in a child diagnosed and treated by endoscopic retrograde cholangiopancreatography: A case report
Guang-Xing Cui, Hai-Tao Huang, Jian-Feng Yang, Xiao-Feng Zhang
Guang-Xing Cui, Hai-Tao Huang, Jian-Feng Yang, Xiao-Feng Zhang, Department of Gastroenterology, Affiliated Hangzhou First People’s Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China
Author contributions: Zhang XF is an advanced endoscopist who performed the ERCP procedure; Cui GX wrote the paper; Yang JF helped to revise the paper; Zhang XF and Huang HT analyzed the special part of the case.
Informed consent statement: Informed consent to publish was obtained from the patient.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Xiao-Feng Zhang, MD, PhD, Chief Doctor, Director, Department of Gastroenterology, Affiliated Hangzhou First People’s Hospital, Zhejiang University School of Medicine, No. 261, Huansha Road, Shangcheng District, Hangzhou 310006, Zhejiang Province, China. 18258855475@163.com
Telephone: +86-182-58855475
Received: December 26, 2018
Peer-review started: December 27, 2018
First decision: January 19, 2019
Revised: February 23, 2019
Accepted: March 16, 2019
Article in press: March 16, 2019
Published online: May 6, 2019
Abstract
BACKGROUND

Pancreaticobiliary maljunction (PBM) is an uncommon congenital anomaly of the pancreatic and biliary ductal system, defined as a union of the pancreatic and biliary ducts located outside the duodenal wall. According to the Komi classification of PBM, the common bile duct (CBD) directly fuses with the ventral pancreatic duct in all types. Pancreas divisum (PD) occurs when the ventral and dorsal ducts of the embryonic pancreas fail to fuse during the second month of fetal development. The coexistence of PBM and PD is an infrequent condition. Here, we report an unusual variant of PBM associated with PD in a pediatric patient, in whom an anomalous communication existed between the CBD and dorsal pancreatic duct.

CASE SUMMARY

A boy aged 4 years and 2 mo was hospitalized for abdominal pain with nausea and jaundice for 5 d. Abdominal ultrasound showed cholecystitis with cholestasis in the gallbladder, dilated middle-upper CBD, and a strong echo in the lower CBD, indicating biliary stones. The diagnosis was extrahepatic biliary obstruction caused by biliary stones, which is an indication for endoscopic retrograde cholangiopancreatography (ERCP). ERCP was performed to remove biliary stones. During the ERCP, we found a rare communication between the CBD and dorsal pancreatic duct. After clearing the CBD with a balloon, an 8.5 Fr 4-cm pigtail plastic pancreatic stent was placed in the biliary duct through the major papilla. Six months later, his biliary stent was removed after he had no symptoms and normal laboratory tests. In the following 4-year period, the child grew up normally with no more attacks of abdominal pain.

CONCLUSION

We consider that ERCP is effective and safe in pediatric patients with PBM combined with PD, and can be the initial therapy to manage such cases, especially when it is combined with aberrant communication between the CBD and dorsal pancreatic duct.

Keywords: Pancreaticobiliary maljunction, Pancreas divisum, Endoscopic retrograde cholangiopancreatography, Variant, Communication, Children, Case report

Core tip: The coexistence of pancreaticobiliary maljunction (PBM) and pancreas divisum is an infrequent condition. According to the Komi classification of PBM, the common bile duct (CBD) directly fuses with the ventral pancreatic duct in all types. However, we present a case who had an anomalous communication existing between the CBD and dorsal pancreatic duct. There is lack of therapeutic experience for such a case. We successfully diagnosed and treated the little child by endoscopic retrograde cholangiopancreatography. The child remained asymptomatic during 4 yr of follow-up.