Combined Duodenal Atresia and Pure Esophageal Atresia

Combined duodenal atresia and pure esophageal atresia is a rare combination which has been reported only 13 times previously in the literature. Although this pair of congenital anomalies is amenable to current treatment strategies, it is often associated with additional defects which lead to the baby's demise. We present a newborn prenatally diagnosed with trisomy 21, duodenal atresia and an AV canal defect. Postnatally, these diagnoses were confirmed, and the baby was also found to have pure esophageal atresia. Following a successful, staged repair of his duodenal atresia and pure esophageal atresia, the baby developed severe tracheobronchomalacia. At that point, the parents decided to withdraw care rather than embark on airway stent placement followed by cardiac surgery. This report highlights two of the most challenging aspects of pediatric surgery: 1) severe newborn anomalies requiring sound planning strategies and technical expertise and 2) an intimate relationship with the parents, so that the care can be individualized for the wishes of the family