Duodenal atresia and stenosis: reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up.

Duodenal atresia and stenosis was observed in 103 infants and children from 1972 to 1991. There were 59 girls and 44 boys. Atresia was noted in 79 instances and stenosis in 24. Maternal hydramnios was detected in 33 cases, 46 babies were premature, and 31 had Down's syndrome. Fifty-four infants had significant associated anomalies including 35 with cardiac defects. Diagnosis was achieved by prenatal ultrasound examination in 14 cases, observation of a double-bubble sign on abdominal radiograph in 73, and contrast studies in 30 infants including 24 with stenosis. At operation annular pancreas was noted in 37 cases, malrotation in 37 cases, anterior portal vein in 4, and a second web in 3. Surgical treatment included duodenoduodenostomy in 85, duodenotomy and web excision in 8, and duodenojejunostomy in 10. Operative survival was 95%. Deaths were related to complex cardiac defects. Despite antenatal diagnosis, prompt intervention, and apparent early surgical success (95% survival), late deaths (5%) and late complications including motility disorders, megaduodenum, gastroesophageal reflux, duodenal-gastric reflux, gastritis, peptic ulcer disease, blind loop syndrome, and biliary-pancreatic conditions may be observed months to years after management during the neonatal period. Modifications in surgical technique including early tapering duodenoplasty may be useful, and close long-term follow-up is an essential component of patient care.