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Annular pancreas associated with pancreaticobiliary maljunction in an infant
1Department of Pediatric Surgery, Kurume University School of Medicine, Fukuoka, Japan.
Insights
This study details the first infant case of annular pancreas with pancreaticobiliary maljunction in Japan. Surgical intervention was successful, highlighting a rare pediatric gastrointestinal anomaly.
Area of Science:
- Gastroenterology
- Pediatric Surgery
- Medical Imaging
Background:
- Annular pancreas is a rare congenital anomaly where pancreatic tissue encircles the duodenum.
- Pancreaticobiliary maljunction (PBM) involves abnormal junction of the pancreatic and bile ducts, increasing risks of biliary and pancreatic diseases.
- PBM without cholangiectasis is less common, particularly in pediatric cases.
Observation:
- A 2-year-old infant presented with abdominal pain, vomiting, and elevated amylase, diagnosed with annular pancreas and PBM.
- Endoscopic retrograde cholangiopancreatography (ERCP) was unsuccessful due to duodenal stenosis.
- Intraoperative cholangiopancreatography confirmed PBM and a complete annular pancreas without bile duct dilation.
Findings:
- The infant had Millbourn's 2a pancreatic duct pattern and Yumura's type I annular portion duct.
- Surgical repair included duodeno-duodenostomy and portal jejunostomy (Roux en Y).
- The patient recovered well, discharged 13 days post-operation.
Implications:
- This case supports Lecco's hypothesis on ventral pancreas origin of annular pancreas.
- Suggests ventral pancreas dysgenesis may contribute to PBM development.
- Highlights the importance of surgical management for this rare pediatric condition.
Abstract:
We report the first known case of an annular pancreas associated with pancreaticobiliary maljunction without cholangiectasis in an infant, aged 2 years and 5 months in Japan. Only two other cases have been reported in Japan both of which were in adults. In our case, the main clinical features were abdominal pain, vomiting and an increasing level of plasma amylase. Endoscopic retrograde cholangiopancreatography (ERCP) was not successful in demonstrating the pancreaticobiliary maljunction due to duodenal stenosis. At operation, a complete type of annular pancreas was found with no enlargement of the common bile duct. We could visualize the pancreaticobiliary maljunction using cholangiopancreatography from the gallbladder during the operation. We then performed duodeno-duodenostomy (side-to-side anastomosis, diamond anastomosis) and portal jejunostomy (Roux en Y anastomosis). The infant was discharged in a good condition at 13 days after the operation. The pattern of the pancreatic ducts was Millbourn's 2a and the type of the duct in the annular portion was Yumura's type I. These results correspond to Lecco's hypothesis that the ring formation originates from the ventral pancreas. It has been further suggested that the cacogenesis and/or dysplasia of the ventral pancreas plays a role in the development at the stage of the pancreaticobiliary maljunction.