Annular pancreas combined with distal stenosis. A report of four cases and review of the literature.

BACKGROUND: Congenital duodenal obstruction (CDO) in combination with more distal duodenal obstructions is a rare anomaly occurring in 4% of neonates with duodenal atresia. The experience of two European Pediatric Centers in treatment of congenital double duodenal obstruction (CDDO) is reported and the pertinent literature is reviewed. MATERIALS AND METHODS: During the last 15 years a total of 86 neonates were operated upon for CDO at the department of pediatric surgery of "St. Sophia" Children's Hospital of Athens in Greece and the department of pediatric surgery of "Federico II" Children's University Hospital of Naples in Italy; four of them had a CDDO. These ones presented with nonbilious vomiting and the plain film of the abdomen showed the typical "double bubble". Our cases with CDDO presented annular pancreas causing complete obstruction of the second part of the duodenum and dilatation of the duodenum distal to this obstruction due to an additional congenital stenosis (two cases) or a membranous web (two cases). A diamond shaped duodeno-duodenal (DDD) anastomosis was carried out to relieve the proximal obstruction and a Heinecke-Mikulicz plasty was used to relieve the distal stenosis. RESULTS: All patients with double obstruction underwent successful surgery with no complications. The postoperative course was uneventfuL An upper gastrointestinal barium study at one month postoperatively showed no blind loop, megaduodenum, anastomotic stenosis or malfunction. CONCLUSIONS: a) The combination of duodenal atresia with annular pancreas and distal duodenal stenosis or web is extremely rare. b) Patency of the duodenum distal to the usual obstruction should always be checked in order to avoid misdiagnosis of this combination.