Cystic Dystrophy of the Duodenal Wall Developing in Heterotopic Pancreas

Abstract

Cystic dystrophy in heterotopic pancreas (CDHP), an uncommon but serious complication of dilatation of the ectopic pancreatic ducts of heterotopic pancreatic tissue, occurs most often in the second part of the duodenum. This disorder is difficult to diagnose and raises various therapeutic questions. The aim of this study was to evaluate the most useful procedures for investigating suspected CDHP and to consider late results after surgical treatment by pancreaticoduodenectomy (PD). This retrospective study concerned 9 patients who developed CDHP of the duodenal wall during an 18-year period (1983-2001). All patients were symptomatic and underwent PD (no postoperative deaths). Clinical presentation, imaging findings, pathological features, and follow-up were analyzed. All patients but one were male alcoholics (mean age, 48 years; range, 37-63 years). The clinical presentation was abdominal pain (n = 9) associated with vomiting (n = 6), jaundice (n = 2), and weight loss (n = 9). Upper endoscopy (n = 9) showed nonspecific inflammatory lesions with duodenal stenosis (n = 4). Retrospective analysis of computed tomographic findings (n = 9) studied specific signs of CDHP and nonspecific inflammatory changes in periduodenal space. Endoscopic ultrasonography (n = 4) and magnetic resonance cholangiopancreatography (n = 2) were the most useful investigations. Pathological findings in surgical specimens were multiple cysts located in the thickened duodenal wall (n = 9), ectopic pancreatic tissue (n = 7), fibrosis of the normal gland (n = 4), and adenocarcinoma in ectopic tissue (n = 1). During the follow-up period, 4 patients died 12, 16, 48, and 72 months after PD. The cause of death was independent of CDHP or PD, but related to persistent alcoholism in 2 cases involving chronic pancreatitis. In 1 case, the patient died of disseminated adenocarcinoma. The other 5 patients had a good result. Endoscopic ultrasonography and magnetic resonance pancreatography are the most useful investigations for the diagnosis of CDHP. Pancreaticoduodenectomy is justified after failure of medical treatment or endoscopic drainage. Late results after PD are dependent on alcoholic consumption when CDHP is associated with chronic pancreatitis.