Evaluation of Bronchiectasis
Level A Recommendations Recommendation 23: A negative CXR does not rule out the presence of bronchiectasis Recommendation 24: The presence of bronchiectasis can be best evaluated by low radiation chest CT without contrast Recommendation 25: Chest CT should be considered in cases of chronic irreversible changes on CXR
Level C Recommendations Recommendation26: CXR should be obtained annually in all children with TEF regardless of their clinical status Recommendation27: Chest CT should be obtained annually in all children with TEF regardless of their clinical status
Bronchiectasis is defined as an abnormal dilatation of the bronchial tree and it is one of the more serious pulmonary complications in patients with EA-TEF65,66,109. A ratio >0.8 of the bronchial & arterial lumens in a chest CT is considered diagnostic50. The finding of “tramlines” and/or of “signet ring” sign opacities in plain radiographs are typical of bronchiectasis but their absence does not rule out the diagnosis110,111. Monitoring of disease progression is best achieved with a low-dose high resolution chest CT because it may reveal airway changes that can be prevented from progressing if treated early and aggressively9,50,65,107. Radiation exposure from chest CT evaluations remains a concern, especially for young children. High-resolution ultrashort echo time (UTE) magnetic resonance imaging (MRI) completely eliminates radiation exposure112, whereas modifications in the technique of the dual multidetector CT (MDCT) allow scanning of the pediatric lungs in less than 1 sec and with markedly lower dose of radiation than a regular CT scan113. Neither technique requires sedation. However, the techniques are not readily available in every hospital and therefore they are not recommended at this point.
Evaluation of Possible Aspiration
Level A Recommendations Recommendation 29: Multiple episodes of lobar pneumonia should be evaluated for aspiration or possible underlying anatomical abnormalities Recommendation 29: Rigid bronchoscopy is indicated for the evaluation of a possible laryngeal cleft
Level B Recommendations Recommendation 30: BAL is indicated in cases of suspected aspiration
Level C Recommendations Recommendation 31: Asymptomatic children with EA/TEF who have positive cultures in BAL should be evaluated for possible aspiration Recommendation 32: In infants with TEF, the CXR may show characteristic changes consistent with aspiration Recommendation 33: Chest CT should be considered in cases of known or highly suspected chronic aspiration Recommendation 34: Presence of lipid laden macrophages in BAL of children without acute or chronic lung disease is suggestive of aspiration Recommendation 35: Modified Barium Swallow should be performed in all patients prior to initiating oral feedings
Patients with EA-TEF are predisposed to aspiration and the possibility should be investigated in cases of recurrent pneumonia. Conditions to be ruled out include undiagnosed occult communications (e.g. laryngeal cleft and/or undiagnosed fistulae)18,59–61,74–76,114 or functional abnormalities such as impaired swallowing, vocal cord dysfunction and esophageal dysmotility that predispose to aspiration. Laryngeal clefts in particular can be found in almost 20% of the cases of EA-TEF, and rigid airway endoscopy with probing of the posterior glottis is the gold standard for its diagnosis115,116. Presence of contrast material in the tracheobronchial tree after a contrast study (e.g. upper GI series) or foodstuff seen in the trachea of a patient with tracheostomy are definitive of the diagnosis but rather “accidental” findings. Direct visualization of the swallowing with videofluoroscopy or flexible laryngoscopy are the most commonly used clinical modalities that can provide a definitive diagnosis when positive but do not rule out the diagnosis when negative52,117,118. Chest radiographs and especially CT scans of the chest are useful in documenting the presence and extent of the lung damage but they are not pathognomonic of aspiration. Bronchoalveolar lavage is widely used for the diagnosis of aspiration on the basis of the presence of lipid laden macrophages (LLM) in the BAL119. The assumption is that normally there are no lipid molecules in the BAL, therefore their presence is evidence of aspiration of exogenous lipids119,120. However, lipid molecules are also released as part of cell death. Therefore lipid-laden macrophages can be found in conditions causing acute or chronic damage to the airways independent of aspiration (e.g. pneumonia, Cystic Fibrosis)121,122. Of other potential biologic markers, the most promising is pepsin, but its use has not yet been standardized123,124. A detailed approach to the investigation of gastroesophageal abnormalities predisposing EA-TEF patients is provided in the INoEA gastroesophageal guidelines statement19.
VI. Evaluation of Cyanosis
Level A Recommendations Recommendation 36: An echocardiogram should be performed in infants with EA-TEF who develop episodes of severe cyanosis in order to rule out anatomical shunts
Level B Recommendations Recommendation 37: Chest CT with contrast should be performed when a vascular anomaly is suspected.