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Cystic dystrophy in heterotopic pancreas: a rare indication for pancreaticoduodenectomy.

BACKGROUND
Cystic dystrophy in heterotopic pancreas (CDHP) is a rare benign condition characterized by the presence of cysts in the wall of the digestive tract associated with inflammation and fibrosis, intermingled with heterotopic pancreatic tissue. Treatment options for CDHP are poorly defined.


METHOD
We report a case of CDHP, and review the literature focusing on the diagnosis and management.


RESULTS
CDHP is mainly encountered in men in the fifth decade of life in association with chronic pancreatitis secondary to alcohol ingestion. Alcohol and mechanical obstruction of heterotopic pancreatic ducts have been implicated in its pathogenesis. Clinical presentation is varied and current imaging provides the diagnosis. Treatment options include somatostatin analogue injections, endoscopic cyst fenestration and surgical resection (pancreaticoduodenectomy or gastrointestinal bypass).


CONCLUSION
CDHP is rare and presents a diagnostic and therapeutic challenge. The long term efficacy and indications for different treatment options need to be refined.