VATER: esophageal atresia, tracheo-esophageal fistula and radial ray anomalies

Case contributed by Maulik S Patel
Diagnosis almost certain

There is dextroposition of the heart and additional right upper lobe collapse.  The feeding tube is coiled in the upper esophagus and the upper pouch is gas-filled and distended. Features here are suggestive of esophageal atresia and there is also an absent radius on the right.

Cropped, zoomed images of the tube coiled in the upper esophageal pouch, right forearm, dextrocardia, and normal spine.

Case Discussion

The heart is in the right hemithorax and a cause is needed.  There is right upper lobe collapse and mediastinal shift, but the apex is pointing right and features here are highly suggestive of dextrocardia.

In addition, there is a feeding tube coiled in a distended esophageal pouch highly suggestive of esophageal atresia. Since there is gas in the bowel, there must also be a tracheoesophageal fistula.

Where there is TOF/OA, go searching for vertebral anomalies (there are not any) and other anomalies. There is no evidence of distal bowel obstruction, but if you look at the right arm, there is an absent radius and absent thumb, which is part of the VATER association. 

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