Cystic dystrophy of the duodenal wall in a heterotopic pancreas

Cystic dystrophy of the duodenal wall in heterotopic pancreas is a rare disease affecting younger men in particular. On account of its difficult diagnosis and nonspecific signs, it was first described in 1970. A 51-year-old male patient was admitted for clarification of several months of severe pain in the upper abdomen accompanied by weight-loss, and due to ultrasonically established suspicion of carcinoma in the head of the pancreas. Clinical examination and laboratory tests were nonsignificant. Barium meal and esophagogastroduodenoscopy revealed a severe deformation of the duodenum. Biopsy of the duodenal mucosa detected moderate inflammatory changes. Ultrasound examination showed cystic changes in the duodenal wall and in the vicinity of the head of the pancreas. Only with the aid of endoscopic ultrasound, could bizarre cystic changes in the submucosa of the duodenal wall be detected, which was also confirmed by computer tomography. The signs of disease are nonspecific. Duodenal biopsy also does not reveal its cause since the typical lesions lie deeper in the submucosa or muscularis propria. Cystic changes with fibrosis, pancreatic ducts and lobi without anatomic or vascular connection to the pancreas are found. The anomaly involves, above all, the stomach and duodenum. Prior to the introduction of imaging techniques such as endoscopic ultrasonography, computer tomography and nuclear magnetic resonance, the diagnosis could only be made by means of an operative procedure. Treatment is primarily surgical; smaller cysts can be treated by endoscopic fenestration. Pharmacological treatment with somatostatin is rarely performed.