Complete Agenesis of Dorsal Pancreas with Type 1 Choledochal Cyst- A Rare Association and its Surgical Implications: A Case Report
Authors
Keywords:
- Pancreatic agenesis, obstructive jaundice, anomalous pancreaticobiliary junction, congenital anomaly
Abstract
Introduction: Choledochal cysts (CDC) are common congenital biliary anomalies frequently associated with anomalous pancreaticobiliary junctions. Complete agenesis of the dorsal pancreas (ADP), resulting from failed development of the dorsal pancreatic bud, is a rare developmental anomaly and its coexistence with CDC is exceptionally uncommon, with only two cases previously reported.
Case Presentation: An 11-year-old girl presented with one-week history of upper abdominal pain, jaundice and vomiting. Laboratory evaluation suggested obstructive jaundice. Magnetic resonance cholangiopancreatography demonstrated a type I choledochal cyst with a long common channel and complete dorsal pancreatic agenesis. Following treatment of cholangitis, she underwent complete cyst excision with Roux-en-Y hepaticojejunostomy. Histopathology showed inflammatory changes without dysplasia. Recovery was uneventful and at 18-month follow-up she remained asymptomatic with normal liver function tests and no clinical or biochemical evidence of endocrine or exocrine pancreatic insufficiency.
Conclusion: This is the third reported case of choledochal cyst associated with complete dorsal pancreatic agenesis and the first reported in a female child. Preoperative recognition of this rare association on MRCP is important because it facilitates meticulous surgical planning, enables safe intrapancreatic dissection and identifies patients who require long-term surveillance for pancreatic endocrine and exocrine dysfunction.
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References
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