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
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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
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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.
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Evaluation of Possible Aspiration
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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
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Level B Recommendations
Recommendation 30: BAL is indicated in cases of suspected
aspiration
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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
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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.
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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
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Level B Recommendations
Recommendation 37: Chest CT with contrast should be performed
when a vascular anomaly is suspected.
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