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Background  Pancreaticopleural fistula (PPF) is a rare complication of chronic pancreatitis (CP) that requires a high index of clinical suspicion in the patient who presents with a pleural effusion. Visualizing the fistula tract from the pancreatic duct to the pleural space by radiological imaging provides confirmation of this complication.    Case presentation  A 9-year-old boy who presented with massive right pleural effusion secondary to PPF, a complication of CP from a genetic mutation involving two mutations of  SPINK1 . We successfully managed the case with by endoscopic pancreatic duct stent placement after failure of conservative treatment approaches.    Conclusions  PPF is a rare but serious complication of CP in all ages. The diagnosis of PPF in children requires a high index of clinical suspicion and should be considered in the differential diagnosis of massive pleural effusion where pancreatic pathology is present. A high level of pleural fluid amylase and the results from radiological imaging when the patients have symptoms play essential roles in the diagnosis of PPF. Currently, Magnetic resonance cholangiopancreatigraphy (MRCP) is the imaging modality of choice. Endoscopic therapy and surgery are treatment options for patients who do not respond to conservative therapy.

Pancreaticopleural fistula in a child with chronic pancreatitis harboring compound SPINK1 variants