In-utero gastric perforation from combined duodenal and esophageal atresia without consistent polyhydramnios
A 24-year-old G2P0010 woman was referred to our fetal care center at 21- and 2/7-weeks’ gestation following routine ultrasound that identified ascites, dilated small intestine and absent versus right-sided stomach. Fetal ultrasound performed at our institution demonstrated a dilated stomach with possible ‘double-bubble’ sign (). Fetal magnetic resonance imaging (MRI) confirmed dilation of the stomach and proximal duodenum without clear distinction between the two structures (), suspicious for duodenal atresia. Polyhydramnios was not seen on either ultrasound or MRI. She was scheduled for amniotic fluid index (AFI) evaluations () and biophysical profiles (BPP) every 2 weeks with monthly growth ultrasounds, and induction was planned at 39-weeks’ gestation. At 30- and 2/7-weeks’ gestation, her AFI was mildly elevated at 29.7 cm (normal 5–25 cm). The ‘double-bubble’ sign was not identifiable on any follow-up evaluation. At 34- and 5/7-weeks’ gestation, she presented with decreased fetal movement. Her BPP was abnormal with absent fetal breathing episodes and a non-reactive non-stress test, necessitating further surveillance and ultimately Cesarean section. The infant was initially apneic, cyanotic without grimace and bradycardic. There was no response to continuous positive airway pressure ventilation requiring intubation with improvement in hemodynamic status. Oro-gastric sump tube placement was attempted but could not be advanced past the proximal esophagus. Radiographs noted coiling of the sump tube within the proximal esophagus and a gasless abdomen. On day of life two, the infant was taken to the operating room for rigid bronchoscopy and laparoscopic gastrostomy tube placement. Bronchoscopy demonstrated normal tracheal and bronchial anatomy. The bronchoscope was used to evaluate the esophagus and confirmed a blind-ending esophageal pouch. A gastrostomy tube was placed laparoscopically without issue but upon insufflation of the stomach, froth was noted along the lesser curve just proximal to the pylorus. A supra-umbilical midline incision was made to further evaluate, and a gastric perforation was noted. A red rubber catheter was passed through the perforation and met obstruction within the second portion of the duodenum. The perforation was repaired, and a standard duodenoduodenostomy was performed.\nOn day of life 15, an esophageal gap study was performed, which demonstrated a proximal esophageal pouch ending at the T2 vertebral level and a 7-mm distal esophageal stump, with an estimated esophageal gap of 6 cm (). The infant remained in the neonatal intensive care unit (NICU) awaiting long-gap esophageal atresia repair until 4 months of age when repeat esophageal gap study demonstrated a gap of approximately six vertebral bodies. He then underwent gastric pull-up, pyloroplasty and jejunostomy tube placement. Post-operative esophagram demonstrated no leak, and the infant was discharged home 3 weeks post-operatively with close follow-up.