Children born prematurely commonly experience pulmonary disease and thus require close clinical monitoring on an outpatient basis. A summary of the 2021 updated American Thoracic Society (ATS) guidelines for what are currently referred to as “post-prematurity respiratory diseases” (PPRD) was recently published in the Annals of the American Thoracic Society.1,2 This set of guidelines on the outpatient respiratory management of infants, children, and adolescents with PPRD includes 7 recommendations (some of which are multi-part), based on available evidence and expert opinion, that address treatment and diagnostics.

Definitions

The recent ATS guideline uses the term “PPRD” to include patients with respiratory disease directly associated with premature birth (ie, <37 weeks post-menstrual age [PMA]), authors of the recent guideline summary explained. They noted that this also includes patients born prematurely but whose condition does not meet the definition for bronchopulmonary dysplasia. “Use of this term intentionally captured all premature infants with respiratory sequelae who would benefit from consensus recommendations for longitudinal management,” the authors wrote.1


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Recommendations for Management

The authors stated that patients with PPRD have an increased risk of cough and wheeze, and that up to 25% receive an asthma diagnosis, suggesting short-acting bronchodilators may hold utility for these patients. The ATS guideline makes the following suggestions for bronchodilators:

  • Short-acting inhaled bronchodilator therapy should not be routinely prescribed in infants, children, and adolescents with PPRD who do not have recurrent respiratory symptoms (Recommendation 1a; conditional recommendation, very low-certainty evidence).
  • Initiate a trial of short-acting inhaled bronchodilator with monitoring to assess for clinical improvement in symptoms in patients with PPRD who have recurrent respiratory symptoms such as cough or wheeze (Recommendation 1b; conditional recommendation, very low-certainty evidence).

Given that airway inflammation is a key feature of PPRD, clinicians often attempt a trial of inhaled corticosteroids in patients with persistent respiratory symptoms. The ATS guideline makes the following suggestions for inhaled corticosteroids:

  • Inhaled corticosteroids should not be routinely prescribed in infants, children, and adolescents with PPRD who do not have chronic cough and recurrent wheezing (Recommendation 2a; conditional recommendation, very low-certainty evidence).
  • Initiate a trial of inhaled corticosteroids with monitoring to assess for clinical improvement of symptoms in infants, children, and adolescents with PPRD who have chronic cough or recurrent wheezing (Recommendation 2b; conditional recommendation, very low-certainty evidence).

The ATS guideline summary authors wrote that the utility of diuretics in post-NICU care is uncertain, even though many infants are still discharged from the NICU on these medications. The ATS guideline:

  • Suggests against the routine use of diuretics in infants, children, and adolescents with PPRD (Recommendation 3a; conditional recommendation, very low-certainty evidence); and
  • Suggests discontinuation of diuretics “in a judicious manner” in infants with PPRD who have been discharged from the NICU on chronic diuretic therapy (Recommendation 3b; conditional recommendation, very low-certainty evidence).

Recommendations for Diagnostics

The guideline authors note that infants who are born preterm are at an increased risk of sleep disordered breathing (SDB), which may also increase the risk of neurological, cardiac, growth, and behavioral issues. Additionally, the authors explained that polysomnography (PSG) testing to diagnose sleep disorders “is resource-intensive, and may lead to unnecessary interventions,” yet the guideline makes specific suggestions for PSG in distinct clinical contexts:

  • Consider PSG for infants with PPRD who are otherwise ready to be discharged from the NICU who have persistent apnea, intermittent desaturation, or bradycardia at >40 weeks post-menstrual age (Recommendation 4a; conditional recommendation, very low-certainty evidence).
  • Consider PSG and/or sleep medicine referral for infants, children, and adolescents with PPRD with symptoms of SDB, including persistent snoring, failure to thrive, or persistent need for supplemental oxygen at 2 years of age (Recommendation 4b; conditional recommendation, very low-certainty evidence).

Additionally, the guideline committee recommends obtaining an overnight or 24-hour oximetry to screen for SDB, when a PSG is indicated but not available, followed by a PSG and/or sleep medicine referral for further as-needed evaluation (Recommendation 4c; conditional recommendation, very low-certainty evidence).

The authors of the guideline summary wrote that premature infants with pulmonary disease may have an increased risk for swallow dysfunction. The guideline committee makes the following suggestion for swallow evaluations in infants, children, and adolescents with PPRD:

  • Perform a swallow evaluation (videofluoroscopic swallow study) for patients who are eating by mouth and have cough or persistent oxygen desaturation during feeding, suspected or confirmed vocal cord paralysis, or other airway anomalies, failure to wean from oxygen therapy or ventilatory support as expected, persistent or worsening pulmonary hypertension, failure to thrive, or chronic pulmonary symptoms out of proportion to viral respiratory infections (Recommendation 5; conditional recommendation, very low-certainty evidence).

Given that infants with PPRD have a higher risk for airway abnormalities, airway endoscopy is considered an optimal test to identify airway pathology “in the absence of a gold standard,” the authors wrote. The guideline suggests airway endoscopy for infants, children, and adolescents with PPRD with unexplained symptoms such as chronic cough, wheezing, ventilator dependence, persistent hypoxemia, or a history of patent ductus arteriosus ligation with stridor and weak cry (Recommendation 6; conditional recommendation, very low-certainty evidence).

Airway endoscopy is invasive, however, and may not be safe in critically ill neonates. The authors note that a dynamic computed tomography (CT) scan may be an alternative in spontaneously breathing infants. The ATS guideline makes the following suggestions for dynamic airway imaging:

  • Dynamic airway imaging (CT or magnetic resonance imaging [MRI]) should not be used as a screening test for the routine diagnosis of tracheobronchomalacia (TBM) in infants, children, and adolescents with PPRD who do not have symptoms suggestive of airway malacia (Recommendation 7a; conditional recommendation, very low-certainty evidence).
  • Use unsedated, dynamic airway imaging (CT or MRI) to diagnose or re-evaluate TBM in patients with PPRD with recurrent symptoms suggestive of airway malacia as an alternative to bronchoscopy when risk of anesthesia for bronchoscopy is deemed higher than risks from radiation or if bronchoscopy is not available or feasible (Recommendation 7b; conditional recommendation, very low-certainty evidence).

According to the authors of the guideline summary, more research is needed, particularly in the outpatient setting, to identify the optimal management of infants who are born prematurely. “Moreover, the PPRD population contains heterogeneous clinical phenotypes, and patients would likely benefit from individualized approaches,” the authors wrote.

References

1. Eldredge LC, Levin JC, Tracy MC, et al. Summary for clinicians: Clinical practice guidelines for outpatient respiratory management of infants, children, and adolescents with post-prematurity respiratory disease. Ann Am Thorac Soc. Published online March 3, 2022. doi:10.1513/AnnalsATS.202201-007CME

2. Cristea AI, Ren CL, Amin R, et al. Outpatient Respiratory Management of Infants, Children, and Adolescents with Post-Prematurity Respiratory Disease: An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med. 2021;204(12):e115-e133. doi:10.1164/rccm.202110-2269ST