Cystic dystrophy in heterotopic pancreas

Cystic dystrophy in heterotopic pancreas, or paraduodenal pancreatitis, is a rare and complicated presentation involving heterotopic pancreatic tissue in the duodenal wall. This condition is present in 5% of the general population but disease mainly affects middle-aged alcoholic-smoking men with chronic pancreatitis (CP). It may be purely duodenal or segmental (pancreatico-duodenopathy). Its pathophysiology arises from alcohol toxicity with obstruction of small ducts of heterotopic pancreatic tissue present in the duodenal wall and the pancreatic-duodenal sulcus, leading to repeated episodes of pancreatitis. The symptomatology includes episodes of acute pancreatitis, weight loss, and vomiting due to duodenal obstruction. Imaging shows thickening of the wall of the second portion of the duodenum with multiple small cysts. A stepwise therapeutic approach is preferred. Conservative medical treatment is favored in first intention (analgesics, continuous enteral feeding, somatostatin analogues), which allows complete symptomatic regression in 57% of cases associated with a 5% rate of complications (arterial thrombosis and diabetes). Endoscopic treatment may also be associated with conservative measures. Surgery achieves a complete regression of symptoms in 79% of cases but with a 20% rate of complications. Surgery is indicated in case of therapeutic failure or in case of doubt about a malignant tumor. Pancreaticoduodenectomy and duodenal resection with pancreatic preservation (PPDR) seem to be the most effective treatments. PPDR has also been proposed as a first-line treatment for purely duodenal location of paraduodenal pancreatitis, thereby preventing progression to an extended segmental form.