Annular Pancreas: Dramatic Differences Between Children and Adults

Background: Annular pancreas is rare; only 737 cases have been reported in the English literature. In addition, no large analysis has compared children and adults. Recently, prenatal diagnosis and advances in imaging have led to increased experience with this condition. Study Design: Data from 103 patients (48 children, 55 adults) with annular pancreas, managed from 1992 to 2006, were reviewed. Patients with isolated duodenal atresia, stenosis, or webs were excluded. Results: Median ages at diagnosis were 1 day in children and 47 years in adults. Annular pancreas was more common in girls and women (children, 58%; adults, 69%). Congenital anomalies were more frequent (p < 0.01) in children (71%) than in adults (16%); Down syndrome, cardiac, and intestinal anomalies were most common. Prenatal diagnosis was suspected in 56% of infants, and adults presented with pain (75%), vomiting (24%), pancreatitis (22%), or abnormal liver tests (11%). All children were managed with duodenal bypass. Children were more likely (p < 0.01) to require surgery for associated anomalies. In contrast, adults had fewer duodenal bypass procedures (24%) but more often required endoscopic pancreatobiliary procedures (67%), cholecystectomy (56%), and other pancreatobiliary surgery (20%; p < 0.01). Adults more commonly (p < 0.01) had pancreas divisum (29%) and pancreatobiliary neoplasia (11%). Five children (6%) with multiple anomalies died; all adults survived their operations. Late deaths occurred in 2 children (4%) with multiple anomalies and 3 adults (5%) with pancreatobiliary cancer. Conclusions: Annular pancreas is associated with a spectrum of disease that differs in children and adults. Congenital anomalies are more common in children with annular pancreas; complex pancreatobiliary disorders and malignancy are more frequent in adults.