What is a heterotopic pancreas?

A heterotopic pancreas is shown by the presence of ectopic pancreatic tissue in the duodenal wall. The most frequent locations of the heterotopic pancreas are the duodenum and the stomach. It is the consequence of anomalies in embryonic development.

What is cystic dystrophy in heterotopic pancreas?

Also known as paraduodenal pancreatitis or pancreatitis of the pancreatic-duodenal groove, cystic dystrophy in heterotopic pancreas (CDHP) is a benign disease corresponding to an inflammatory and fibrotic thickening of the duodenal wall, in which one or more cysts are found. Chronic tobacco or alcohol use plays a crucial role in its pathogenesis, probably owing to direct toxicity in the ectopic pancreatic tissue (which is more sensitive to alcohol) and an increase in the viscosity of pancreatic secretions leading to obstruction of the minor papilla. Duodenal stenosis may occur. Association with chronic pancreatitis is present in two out of three cases.

How is CDHP diagnosed?

It is a rare pathology, and its incidence is not known.

CDHP occurs mostly in men in their 40s with chronic alcoholism and tobacco use. Pain is the most frequent symptom, sometimes associated with weight loss, vomiting (in relation with upper intestinal obstruction), obstructive jaundice (owing to compression of the main biliary duct) and episodes of acute pancreatitis.

Positive diagnosis is based on the combination of endoscopic exams and imaging, which also enable exclusion of a pancreatic or ampullary tumour as possible cause. 

Endoscopy of the upper digestive tract may reveal muscosal oedema of the genu superius, which may be complicated by duodenal stenosis. CT scan, magnetic resonance imaging (MRI) and endoscopic ultrasound show duodenal thickening, the presence of one or more cysts in the duodenal wall, densification of peripancreatic fat in the cephalic portion, chronic pancreatic lesions (calcifications, dilation of the Wirsung duct, etc.), gastric distension in relation with duodenal stenosis.

What is its specific treatment?

Care is based on treating the addictions. In the acute phase, pain relievers and artificial nutrition are necessary. In case of failure, an endoscopic treatment by endoscopic retrograde cholangiopancreatography (ERCP) or endoscopic ultrasound is possible. Surgery may be undertaken in case of failure in medical treatment. 

Source: Reference Centre for Rare Pancreatic Diseases and Disorders

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