Prenatal diagnosis of esophageal atresia with the pouch sign

A 19-year-old primipara was referred to our center for a routine scan at 33 + 4 weeks' gestation. A visible but small stomach bubble (18 mm) was detected in the fetal abdomen, associated with a dilation of the esophagus with a tapering distal part. Direct visualization of filling and emptying of the proximal esophagus suggested the presence of obstruction. A cystic pouch in the region of the esophagus was observed to be full and empty in accordance with fetal swallowing. No other fetal anomalies were detected. Fetal biometric measurements were on the 10th percentile and polyhydramnios was detected. Esophageal atresia type I was suspected. Fetal karyotyping was declined by the parents, no therapy was given and the pregnancy continued until week 37 when a female baby was delivered following spontaneous labor. Esophageal atresia type I was radiographically confirmed. Bougienage was used for 8 weeks and esophageal anastomosis was successfully performed when the infant was 11 months old. No other anomalies were found and the child is currently in excellent health. The present case shows that the upper neck pouch sign may be a delayed manifestation present only in certain types of esophageal atresia. However, when it occurs it should prompt careful fetal examination as it is an important step in the diagnosis of esophageal atresia.