Enteric duplication cyst is one of the rarest forms of cystic lesion of the pancreas. We report a unique case of an enteric duplication cyst of the pancreas that was communicating with a duplicated pancreatic duct. A 7-year-old girl with severe acute abdominal pain was found to have a large cyst that was smoothly communicating with the dilated pancreatic duct in the pancreatic tail. Analysis of cyst fluid showed elevated levels of amylase, carcinoembryonic antigen (CEA) and CA 19-9, and no epithelial cells. Intraoperative cyst pancreatography revealed that the pancreatic duct was duplicated in the tail: 1 duct was communicating with the cyst, and the other was dilated within the pancreatic tail. The patient underwent spleen-preserving distal pancreatectomy and complete cyst excision without complication. Because preoperative diagnosis of duplication cyst of the pancreas is difficult, this condition should be considered during differential diagnosis of atypical cystic lesions of the pancreas. Complete excision is desirable for the management of duplication cyst of the pancreas.
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