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: nasogastric tube curled in dilated proximal esophageal pouch, 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 due to tracheal compression from dilated proximal pouch
    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

Radiology 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.
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 a post-operative upper GI exam shows a contrast filled esophagus (posteriorly) connecting to the trachea (anteriorly) through a superiorly oriented fistula (in the middle).

Gross Pathology Cases of Esophageal Atresia

Pathologic image of esophageal atresia
Pathological image taken from the posterior aspect of the specimen shows a dilated proximal esophageal pouch superiorly that abruptly transitions to a more narrow in diameter distal esophageal segment.