Discussion
This is the first international database to examine complications of
SARS-CoV-2 infection in children with EA-TEF. Our dataset was
representative of a normal EA-TEF cohort with 81% of patients having
Type C, EA-TEF, comparative to 78-90% rates in the
literature11, 15, 16, cardiovascular anomalies in 33%
, comparative to 29% reported in the literature17.
19% of our cohort were on inhaled bronchodilators, inhaled
corticosteroids or montelukast, consistent with reported rates of asthma
of 10-30%18, 19 and 25% of our cohort had
tracheomalacia, comparative to rates of 17%-78%20.
Recurrent strictures were noted in 33% of our cohort and third of
patients were on proton pump inhibitor (PPI), again, comparative to
rates of 18-50%21 and gastroesophageal reflux disease
(GERD) rates of 15-66%22-24 respectively.
Our population had a high rate of admission to hospital comparative to a
pediatric population, where rates of hospitalization have been
documented at 1.2-2.9%25. This high rate is likely
reflective of the fact that patients with EA-TEF are more susceptible to
respiratory deterioration given high rates of comorbidities such as
chronic lung disease (a known risk factor for severe SARS-CoV-2
infection26) secondary to airway anomalies, reactive
airway disease or chronic aspiration; and be reflective of decreased
respiratory reserve.
It is paramount for the clinician and carers to be mindful of this
increased acute morbidity with respiratory tract infections in those
with EA-TEF, and longer-term morbidity with recurrent lower respiratory
tract infections leading to bronchiectasis, poor lung function and lower
quality of life in adulthood14. Concomitant infections
may also impact on oral intake and nutrition. For these reasons, it is
important that EA-TEF patients continue to receive multidisciplinary
care, for early detection and management of risk factors for chronic
lung disease as well as advocacy for access to primary prevention
mechanisms, such as vaccination.
It is interesting to note that medication use for any reason was
associated with an increased risk of hospitalization, however that
individual medication use was not. This may be due to that fact that
those on medications are likely to have more severe comorbidities and
therefore risk factors for chronic lung disease than those not on
medications.
We did not see an association with PPI use and hospitalization. PPI use
has been postulated to be associated with more severe
infection8, 27, possibly secondary to increased virus
survival in those with higher gastric pH, although this has not been
demonstrated in children.
Our study had several limitations. As EA-TEF is a rare disease, only 42
patients were able to be included in this cohort. There is the
possibility that patients may have under-reported infections if
asymptomatic or well, thereby skewing this dataset towards patients with
more severe SARS-CoV-2. Demographic data such as birthweight,
gestational age at birth – traditionally associated with more severe
morbidity were not included to focus on other modifiable risk factors.
Finally, there was an increased rate of hospitalization in the first 12
months of the database, which may represent worse outcomes before
widespread pediatric vaccination or increased caution early in the
pandemic from clinicians.
Future avenues for research include long term pulmonary and
extra-pulmonary outcomes for those infected with SARS-CoV-2 as well as
risk factors for ‘long covid’ or PIMS-TS in the EA/TEF population.