Esophageal Atresia

  • Etiology: congenital
  • Types: Esophageal atresia with distal tracheo-esophageal fistula – 85%, Esophageal atresia without tracheo-esophageal fistula (isolated esophageal atresia) – 10%, Esophageal atresia with H type tracheo-esophageal fistula – 4%, Esophageal atresia with proximal tracheo-esophageal fistula – 1%, Esophageal atresia with proximal and distal tracheo-esophageal fistulas – 1%
  • Imaging: in current practice, there is no indication for doing an upper GI with positive contrast material, if necessary an upper GI can be performed by injecting air into the proximal pouch
  • Complications: pre-op – aspiration pneumonia in H-type, long gap between atretic segments, tracheomalacia, post-op – anastomotic leak can develop immediately after surgery, stricture can develop at anastomosis and worsen over time with +/- impacted food at anastomosis, recurrent fistula can develop any time after surgery, dysmotility of distal esophagus causes delayed esophageal emptying, distal esophageal stricture due to chronic gastroesophageal reflux
  • Clinical: VACTERL syndrome seen in 66% – Vertebral anomaly in 25% / Anal atresia in 10% / Cardiac (VSD) in 25% / Tracheo-Esophageal fistula / Renal anomaly in 40% / Limb (radial ray) hypoplasia in 10%, can be associated with duodenal atresia

Cases of Esophageal Atresia

AP image from an upper GI performed with a bougie dilator placed into the proximal esophageal pouch through the oropharynx and a bougie dilator placed through a gastrostomy tube site into the distal esophagus with both dilators held under tension shows a 2 cm gap between the proximal and distal esophageal segments.
Upper GI of esophageal atresia with distal tracheo-esophageal fistula
CXR AP (left) shows a nasogastric tube that cannot be advanced further in the esophagus. Vintage upper GI lateral image (right) shows a dilated proximal esophageal pouch compressing the airway. In current practice, there is no indication for doing an upper GI with positive contrast material.
AP image from an upper GI shows ~ 75% luminal narrowing at the esophageal anastomosis between the dilated proximal esophageal pouch and the distal esophagus. This narrowing was subsequently successfully treated with dilation.
Upper GI of esophageal atresia
AP image from an upper GI shows marked luminal narrowing at the esophageal anastomosis between the dilated proximal esophageal pouch and the distal esophagus with a large filling defect in the proximal pouch. At endoscopy a large piece of meat was removed from above the anastomosis and the anastomosis was successfully dilated.
Upper GI of esophageal atresia with recurrent fistula
Lateral image from an upper GI exam shows a contrast filled esophagus (posteriorly) connecting to the trachea (anteriorly) through a superiorly oriented fistula (in the middle).