Clinical Features Of A Few Rare Pancreas Diseases (Report Of 8 Cases)

BACKGROUND & AIM: A few congenital pancreas diseases, such as heterotopic pancreas, annular pancreas and pancreas divisum, are rare in clinic. The clinical manifestations of these diseases are usually atypical, which is easy to lead misdiagnoses. This article has explored the clinical characteristics of these congenital pancreas diseases with the clinic cases .MATERIAL & METHODS: The clinical data of 8 cases of congenital pancreas diseases, included 5 cases of heterotopic pancreas, 2 cases of annular pancreas and 1 case of pancreas divisum. All the cases were reviewed and analyzed retrospectively, which were from the 2nd affiliated hospital of Zhejiang University during Jan 2002 to Dec 2006.RESULT: Two cases of heterotopic pancreas located in body of stomach, two cases in sinus ventriculi and one case in duodenal bulb. Three cases of heterotopic pancreas patients had upper abdominal pain, sour regurgitation and belching. During these five cases, one case was misdiagnosised as leiomyoma of stomach, one case was misdiagnosised as tumor of stomach, two cases were diasnosised as protrusion lesion, only one case was diagnosised as heterotopic pancreas before surgery. Both of two annular pancreas patients suffered from long term upper abdominal pain, one case was diagnosised by ERCP and the other was diagnosised by surgery. The case of pancreas divisum patient had the history of acute Relapsing Pancreatitis and diagnosised byERCP.CONCLUSIONS: Heterotopic pancreas is a congenital anomaly consisting of the presence of pancreatic tissue outside its normal Location, should be considered in the differential diagnosis of extramucosal gastric masses. Patients with upper abdominal pain and vomiting associated with protrusion lesion located in the digestive tract may consider of heterotopic pancreas. Annular pancreas is rare in the adults, the symptoms of annular pancreas in adults include cramping epigastric pain, postprandial fullness and relief by vomiting. The diagnosis of unknown bowel obstruction or pancreatitis should never be accepted without further investigations. Surgery is the radical cure for symptomatic adult annular pancreas. Pancreas divisum is a congenital anatomic variant consisting of a separate pancreatic ductal system, the relationship between Pancreas divisum and pancreatic disease is still been discussed. When patients suffer from acute relapsing pancreatitis, we should consider the possibility of congenital variants of the pancreas, such as pancreas divisum.