Pancreaticobiliary maljunction in Turkish patients: a multicenter case series

Area of Science:

• Gastroenterology and Hepatology
• Surgical Oncology
• Pediatric Surgery

Background:

• Pancreaticobiliary maljunction (PBM) is a congenital anomaly where pancreatic and bile ducts join outside the duodenal wall.
• PBM is linked to biliary and pancreatic diseases and carries a significant risk of biliary or gallbladder carcinoma.
• Multicenter studies on PBM are crucial for understanding its diverse clinical and epidemiological characteristics.

Purpose of the Study:

• To present a multicenter case series of Pancreaticobiliary maljunction (PBM) from Turkey.
• To analyze the clinical presentations, PBM types, ERCP findings, and surgical outcomes in adult and pediatric patients.
• To compare the PBM characteristics and management strategies between adult and pediatric populations in Turkey.

Main Methods:

• Retrospective review of adult and pediatric PBM patients undergoing ERCP at three Turkish tertiary centers (July 2007 - May 2020).
• Data collected included clinical presentations, PBM types, ERCP findings, surgical history, and postoperative course, including malignancy development.
• Statistical analysis was performed to compare characteristics between adult and pediatric groups.

Main Results:

• The study included 47 patients (31 adults, 16 children).
• Type D PBM was more frequent (27.7%) than reported in Eastern studies. Type A was more common in adults, while Type C was more prevalent in pediatric patients.
• Adults showed more cystic dilatation and larger common bile duct diameters. Pediatric patients underwent more resective surgeries, while adults had more cholecystectomies.

Conclusions:

• Findings are consistent with Eastern studies but highlight a higher prevalence of Type D PBM in the Turkish population.
• Significant differences in PBM types, anatomical variations, and surgical management exist between adult and pediatric patients.
• Further research is needed to elucidate the long-term outcomes and optimal management strategies for PBM in different age groups.