Clinical characteristics and endoscopic treatment of pancreatitis caused by pancreaticobiliary malformation in Chinese children

Objectives: We aimed to describe the clinical characteristics of pediatric patients with pancreatitis caused by pancreaticobiliary malformation and to evaluate the efficacy and safety of magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis of the disease.

Methods: Medical records of pediatric patients with pancreatitis related to pancreaticobiliary malformation diagnosed by ERCP and treated in our hospital between April 2008 and December 2020 were retrospectively reviewed. Clinical manifestations, laboratory indicators, genetic testing results and imaging findings including MRCP were collected.

Results: Of the 148 patients with pancreaticobiliary malformation-related pancreatitis, 90 (60.8%) had pancreaticobiliary maljunction (PBM), 52 (35.1%) had pancreatic divisum (PD), and six (4.1%) had annular pancreas (AnnP). Compared with the PD group, patients with PBM were younger (P < 0.001), and were more likely to have jaundice (P < 0.001) and fever (P = 0.034). Genetic mutation was found in 51.6% of patients with PD, 50.0% with AnnP, and 15.0% with PBM. Diagnostic rate of PBM, PD, and AnnP using MRCP was 46.7%, 15.4%, and 100%, respectively. In total, 87.8% of patients had symptomatic improvement after endoscopic treatment. ERCP-related complications were observed in 28 out of the 260 procedures, including post-ERCP pancreatitis (7.7%), infection (2.3%), and gastrointestinal bleeding (0.8%).

Conclusions: PBM should be considered when jaundice and fever occur in pediatric patients. Genetic testing is recommended for those with PD and AnnP. The role of MRCP is limited in identifying pancreaticobiliary malformation in children. ERCP is effective and safe for the diagnosis and treatment for pediatric pancreatitis caused by pancreaticobiliary malformation.