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Pancreaticobiliary maljunction in Turkish patients: a multicenter case series
Erkan Parlak1, Aydın Şeref Köksal2, Ahmet Tarık Eminler3
1Department of Gastroenterology, Faculty of Medicine, Hacettepe University, 06100, Altindag, Ankara, Turkey. drerkanparlak@gmail.com.
Insights
Pancreaticobiliary maljunction (PBM) is a rare ductal anomaly. This Turkish study found Type D PBM more frequent than in Eastern studies, particularly in children, and noted differences in anatomy and surgical approaches between adult and pediatric patients.
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.
Background And Aims:
Pancreaticobiliary maljunction (PBM) is a malformation in which the pancreatic and bile ducts join outside the duodenal wall. It is associated with various biliary and pancreatic diseases. In addition, patients with PBM carry a substantial lifetime risk of developing biliary or gallbladder carcinoma. We aimed to present a multicenter case series of PBM from Turkey.
Methods:
This study was conducted in adult and pediatric PBM patients who were referred to three tertiary reference centers of Turkey for endoscopic retrograde cholangiopancreatography (ERCP) between July 2007 and May 2020. The clinical presentations, types of PBM, ERCP findings, surgical histories, and the postoperative courses, including the development of biliary malignancies, were retrospectively reviewed.
Results:
The study group included 47 (31 adult and 16 children) patients. Type D PBM was more frequent (13/41: 27.7%) than that reported in Eastern studies. Type A PBM was more common in the adults (51.6% vs. 12.5%, p < 0.05), whereas type C was more common in pediatric patients (31.3% vs. 13.2%, p < 0.05). Although fusiform anatomy was predominant in both of the groups, cystic dilatation was more common (25.8% vs. 12.5%) in adults and the common bile duct diameter was greater [22 mm (range 11-58) vs. 12 mm (range 5-33)] in adult patients compared to pediatric patients. Resective surgeries were more frequently done in pediatric patients (73.3% vs. 53.6%), whereas cholecystectomy was more frequently performed in adult patients (21.4% vs. 6.7%).
Conclusion:
Although our findings were compatible with Eastern studies, type D PBM (associated with pancreas divisum) was more frequent in our study population.
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