[size=20px]A two-month old boy was born full term at three kg. He has had two pneumonias and often sputters and becomes cyanotic when fed orally. He has had at least one blue spell resulting in an emergency call by his parents. An upper gastrointestinal series and an oral pharyngeal motility study (OPMS) are normal but contrast appears in the airway. The next step in the evaluation of this patient with recurrent aspiration should be

 

A pH probe.

 

B repeat OPMS.

 

C cardiac echocadiography.

 

D bronchoscopy.

 

E esophagoscopy.[/size]

D

D

correct answer

D bronchoscopy.

 

H type tracheoesophageal fistulas (TEFs) occur in the 1:100,000 incidence range - occurring about four percent of the time in series of TEFs that are reported. Every reference on this topic discusses the difficulties encountered in diagnosing this lesion and the frequency in which multiple studies are performed without a diagnosis. Bronchoscopy and esophagography are complimentary in the work up.

 

Recurrent pneumonias, episodic cyanosis and coughing while eating are common presentations. Most patient series report several upper gastrointestinal series (UGI) being performed before a diagnosis was made. Traditional UGIs are often not diagnostic but seem to be a necessary step in the timeline of the diagnosis. Swallowing studies are not described as the diagnostic methodology of choice but can occasionally help in the diagnosis. Prone esophagrams with a tube placed in the distal esophagus and water soluble contrast being injected while the tube is pulled up toward the cervical esophagus, a pull back esophagram, is one method described for diagnosis. Another strategy is to place a tube near the cervical esophagus and inject contrast under pressure to distend the esophagus. This method seems to produce more distress with aspiration from the patient prompting recommendations by the authors to have resuscitation equipment available.

 

Most series describe some patients with negative bronchoscopies before the diagnosis was made. However, bronchoscopy is required prior to neck exploration to demonstrate the fistula and place a catheter for localization during the operation.

 

The lesion has a higher origin on the trachea relative to its insertion on the esophagus facilitating the placement of a catheter at the time of bronchoscopy during operative repair. Esophagoscopy may be helpful to withdraw the end of the catheter through the oropharynx to aid intraoperative localization.

 

Cervical exploration from the right neck is adequate to reach most fistulas. These are usually identified between C5 and T2 on the esophagram. Some authors have described a thoracoscopic approach for repair as well. The most common complication is recurrent laryngeal nerve neuropraxia. This occurs commonly (50%) and usually resolves over time.