Anatomic observations and etiologic and surgical considerations in choledochal cyst
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Anomalous pancreatico-biliary ductal junction in children with choledochal cysts allows pancreatic reflux, causing chronic cholangitis and cyst formation. Surgical cyst excision is recommended to prevent complications like malignancy.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Surgical Pathology
Background:
- Choledochal cysts are congenital bile duct dilatations.
- Anomalous pancreatico-biliary ductal junction (APBDJ) is a recognized association.
- The exact pathophysiology linking APBDJ to choledochal cysts requires further elucidation.
Purpose of the Study:
- To investigate the relationship between APBDJ and choledochal cysts in pediatric patients.
- To understand the mechanism by which APBDJ contributes to choledochal cyst formation.
- To evaluate the implications of pancreatic reflux into the biliary tree.
Main Methods:
- Review of 25 operative and postoperative cholangiograms in children with choledochal cysts.
- Assessment of the pancreatico-biliary ductal anatomy and the sphincter of Oddi mechanism.
- Correlation of ductal anatomy with clinical presentation and presumed etiology.
Main Results:
- All 25 patients exhibited an intimate relationship between choledochal cysts and APBDJ.
- The distal main pancreatic duct (MPD) was consistently excluded from the sphincter of Oddi.
- Free reflux of pancreatic juice into the biliary tree was observed, leading to chronic cholangitis.
Conclusions:
- APBDJ leads to pancreatic reflux, causing chronic cholangitis and subsequent choledochal cyst formation.
- Pancreatic reflux poses risks including perforation, bile peritonitis, and malignant transformation.
- Total cyst excision and separation from pancreatic reflux is the suggested treatment, though long-term outcomes are pending.
Abstract:
Twenty-five operative and postoperative cholangiograms in children with choledochal cysts indicated an intimate relationship between these cysts and anomalous pancreatico-biliary ductal junction. In all of these patients the distal main pancreatic duct (MPD) is excluded from the sphinctor of Oddi mechanism and free reflux of pancreatic juice up the biliary tree occurs. This leads to chronic cholangitis, which is presumed to be responsible for the formation of the choledochal cysts. Abrupt and intense exposure of the common bile duct (CBD) to pancreatic reflux may cause perforation and bile peritonitis. Long-standing exposure may induce malignant transformation in the choledochal cyst. Total cyst excision and separation of the biliary system from exposure to pancreatic reflux is suggested as the procedure of choice. Long-term results and metabolic and digestive consequences of this operation are yet to be determined.