Respiratory failure due to retained esophagus: A complication of esophageal replacement

Recurrent fistulas occur in about 10% of infants treated for esophageal atresia with distal tracheosophageal fistula. Failed repair of a recurrent fistula rarely requires esophageal replacement and removal or diversion of the native esophagus. We present a patient who underwent multiple operations for recurrent tracheosophageal fistula whose native esophagus was eventually replaced with a colonic interposition graft. Over the subsequent 9 years he experienced failure to thrive, respiratory distress, and repeated pulmonary infections attributed to chronic aspiration. Eventually, he developed respiratory failure and required endotracheal intubation and mechanical ventilation. He became increasingly difficult to ventilate and, in spite of aggressive efforts, suffered a cardiac arrest from which he could not be resuscitated. At postmortem, a ditated blind segment of native esophagus, which was compressing and obstructing the malacic trachea, was found in the posterior mediastinum. Death was caused by massive air embolus, which was in turn attributed to the high airway pressures needed to ventilate the patient. Tracheal compression by a remnant of native esophagus should be considered in the differential diagnosis of respiratory failure after esophageal replacement.