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PEDIATRIC NOISY BREATHING

Overview
Noisy breathing can take several forms, and characterization of the sound is important to help develop a working differential diagnosis. Stertor can be used to describe any noisy breathing from vibration of tissues superior to the larynx, from the nasal cavity to the oropharynx. Stertor is generally low-pitched and often sounds similar to snoring. Stridor refers specifically to noise produced from laryngeal or tracheal obstruction and is described as high-pitched, musical, or harsh and may occasionally be confused with wheezing (which refers to expiratory noisy breathing originating from the lower airways). After determining the nature of the noisy breathing, it important to characterize its timing during the breathing cycle. Inspiratory stridor, or stridor that occurs while breathing in, usually originates from the supraglottis or glottis. Biphasic stridor, or stridor that occurs during both breathing in and out, generally occurs due to pathology in the subglottis or proximal trachea. Expiratory stridor, or stridor that occurs during breathing out, results from more distal obstruction in the distal trachea, carina, or bronchi. Edema or narrowing of the glottis, subglottis, or proximal trachea may also produce a “barky cough” as commonly seen with croup, also known as laryngotracheobronchitis. These are general guidelines, however, and there are many exceptions. Risk factors for noisy breathing in children include prematurity, history of intubation, uncontrolled gastroesophageal reflux, various anatomic variants, and syndromes including trisomy 21, CHARGE, VACTERL, Pierre Robin sequence, Treacher-Collins, and Beckwith-Wiedemann.

While this section will focus on the most common cause of stridor in infants, laryngomalacia, a broad differential diagnosis during evaluation of pediatric noisy breathing is critical, including pathology from the nasal airway down to the bronchi. Laryngomalacia most commonly presents with progressive inspiratory stridor in infants a few weeks or months old with symptomatic worsening when supine, agitated, or feeding. Hypotonia of the larynx, neurologic immaturity, collapsible redundant supraglottic tissue, and aryepiglottic fold shortening are all factors that may contribute to noisy breathing in laryngomalacia. Awake nasopharyngoscopy with visualization of supraglottic tissue collapse upon inspiration is diagnostic, although the possibility of multilevel airway obstruction should be considered, especially if symptoms are severe. Treatment is guided by the severity of laryngomalacia symptoms, as infants usually outgrow laryngomalacia by 1-2 years of age. Laryngomalacia frequently presents with concomitant acid reflux disease, which should be treated simultaneously in many cases. Mild cases may be treated conservatively with observation and repeat exams. Moderate cases often benefit from upright positioning and thickened feeds and consideration of acid suppression therapy. Severe cases may present with symptoms of failure to thrive, respiratory distress, cyanosis, BRUEs (brief resolved unexplained events), difficulty feeding, or cor pulmonale, which are general indications for surgical intervention with supraglottoplasty. Tracheostomy is almost never necessary for laryngomalacia alone.

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Key supplies for Noisy Breathing Consultation

  • Appropriate PPE including mask, eye protection, gloves, and gown

  • Headlight

  • Topical anesthetic/decongestant spray such as lidocaine/oxymetazoline combination (generally not necessary in children 4-5 years of age or younger)

  • Flexible endoscope (1.9 mm vs 2.5 mm dependent on patient size), preferably videoendoscopy via portable unit or tower to record exam

  • Antifog solution (Fred)

  • Equipment for fiberoptic bronchoscope awake intubation

 

Management

See all consultations for noisy breathing immediately as acute causes may worsen rapidly. Airway evaluation and potential treatment takes precedence. Signs of impending airway compromise include, but are not limited to, muffled cry; significant lip, tongue, or oral cavity edema; stridor (especially at rest); and distress, anxiety, altered mental status, tachypnea, retractions, cyanosis, tripod or sniffing positioning, and drooling or inability to manage secretions. A history of stridor that becomes less apparent while other signs of distress persist is particularly concerning. When time allows, a history including any prior similar events, witnessed foreign body ingestion, aspiration or recent choking spells, or recent upper respiratory tract illness should be elicited.

 

If patient is showing signs of airway distress

  • While talking to the consulting team, recommend placement of IV (although avoid agitating the child if any concern for epiglottitis), oxygen, telemetry, NPO status, and placement of airway cart by patient’s room.

  • Immediately activate the OR, notify senior resident/attending, Anesthesia team, and OR team, and mobilize help in obtaining airway adjuncts and supplies for intubation or an emergent surgical airway if needed.

  • Consider oxygen via nasal cannula, nasal trumpet if not contraindicated, facemask, non-rebreather, or Heliox. If available, consider THRIVE (Transnasal Humidified Rapid-Insufflation Ventilatory Exchange).

  • In the patient with significant distress, nasopharyngoscopy may be reserved for the OR, although in all but the most extreme cases, fiberoptic airway evaluation may be completed in the Emergency Department and can be performed with a video bronchoscope to facilitate immediate transition to intubation if necessary.

  • Ideally the airway should be secured in the OR. Generally, intubation over a rod telescope is preferred. Rigid bronchoscopy is an excellent rescue strategy, and flexible bronchoscopic intubation may be helpful in selected cases. However, if the patient is not a good candidate or has acute decompensation, an emergent needle cricothyroidotomy or tracheostomy may rarely be required. In general, a surgical cricothyroidotomy is best avoided in younger children if possible, given the risk of long-term sequalae. Emergent surgical airways are rarely needed in pediatric patients, but their rapid decompensation and poor reserve suggest that it is best to be prepared for this possibility.

  • If the airway is controlled in the OR, consider following with direct laryngoscopy to confirm underlying etiology of airway compromise.

 

If patient is showing signs of a stable airway

  • Assess vitals including oxygen saturation and any oxygen requirement, assess the breathing rate, sound (stertor or stridor and phase of sound), work of breathing, nasal flaring, retractions or accessory muscle use, and positionality of the symptoms. Keep in mind that desaturation and bradycardia are late and concerning signs.

  • Complete history with attention to the timeline regarding onset and progression and any aggravating factors such as supine positioning, agitation, or feeding as well as any symptoms suggestive of significant respiratory compromise such as retractions, cyanosis, apneas, BRUEs (brief resolved unexplained events), need for CPR, weight loss, or failure to thrive.

  • Assess past airway history including prior intubations and any airway surgeries.

  • Complete past medical history including birth history, any syndromes or known chromosomal abnormalities, gastroesophageal reflux, medications, past surgical history including any cardiac surgeries, and family history.

  • Complete head and neck exam focusing on the nasal cavity (fog on mirror under each nostril, nasal airway patency, anterior septum) oral cavity (size of tongue, position of tongue in mouth, size/shape/position of mandible and midface) and oropharynx (tonsils, palatal elevation, size/shape of epiglottis); evaluate for cutaneous hemangiomas in the “beard distribution” as this may be associated with an airway hemangioma.

  • Assess overall appearance including child size (check growth chart) and skin color. In patients with darker skin tones, the lips and nail beds are useful places to check.

  • Consider flexible laryngoscopy: Often difficult due to crying, head movement, secretions, etc. Assistance from Nursing staff or parents in holding child still is very helpful. Setting expectations with parents is crucial. If able to record, can be very useful to view later when playback can be slowed or paused. Structures to assess include.

    • Patency of nasal airway bilaterally and nasopharynx, size and positioning of tongue base, vallecula, piriform sinus, epiglottis (noting size/shape/position), supraglottic structures and soft tissue (noting evidence of redundant tissue or collapse of arytenoids during inhalation), shortened aryepiglottic folds, interarytenoid area, false vocal folds, post cricoid area, glottic larynx (noting mobility of cords, appearance of cords including vascularity, tone, vibration, lesions, webs, stenosis, scarring), and subglottis (evaluate for masses or stenosis). View of the subglottis is often limited in children, and it is rare to get a clear view of the airway beyond this with flexible laryngoscopy alone.

  • If anterior glottic web present: Genetic screening for 22q11.2 (DiGeorge Syndrome) and cardiac consultation.

  • If laryngeal cleft or laryngotracheoesophageal cleft: Screening for tracheoesophageal fistula, congenital heart disease, Opitz-Frias syndrome, and trisomy 21.

 

Imaging

  • Consider chest x-ray if any concern for pulmonary disease or foreign body.

  • Consider swallow study for evaluation of aspiration, laryngoesophageal clefts, tracheoesophageal fistula, etc.

  • Consider MRI brain in cases of bilateral vocal fold paralysis to rule out Arnold-Chiari malformation.

 

Treatment

  • If pending respiratory compromise, proceed down airway algorithm to ensure a secure airway and adequate oxygenation and ventilation.

  • Therapy is tailored to the individual diagnosis, but generally requires surgical correction of the airway obstruction in cases of redundant tissue, stenosis, lesions, clefts, or webs.

  • Laryngomalacia treatment can be subdivided by severity of disease:

    • Mild disease with inspiratory stridor, no significant feeding impairment or dysphagia, and no clinical or radiographic evidence of second airway lesion: Clinical observation and repeat examinations with optional acid suppression therapy.

    • Moderate disease with cough, choking, regurgitation, or feeding difficulty: Acid suppression therapy and consideration of swallow evaluation. If trending poorly on growth curve due to feeding difficulties and expending additional effort for respiration during feedings or experiencing aspiration or pulmonary compromise, consider supraglottoplasty.

    • Severe disease with apnea, cyanosis, failure to thrive, pulmonary hypertension, or cor pulmonale: Acid suppression therapy, swallow evaluation, and strong consideration for surgical intervention with microlaryngoscopy, bronchoscopy, and supraglottoplasty.

  • Functional endoscopic evaluation of swallow or video fluoroscopic swallow study can be useful adjuncts, especially if concern for aspiration or evidence of failure to thrive. Can consider SEES (static endoscopic evaluation of swallow) for toddlers unable to complete a functional endoscopic evaluation of swallow.

  • Consider ordering a polysomnogram (sleep study) as an outpatient if concern for obstructive sleep apnea. Keep in mind these studies may be difficult to interpret in young infants and premature babies.

 

Disposition and Follow-up

  • Disposition varies based on the underlying etiology and management but have a low threshold to admit if concerns for unsafe airway or rapidly progressive symptoms.

  • Follow-up usually within 1-2 weeks dependent on the severity of the symptoms and abnormality on exam.

  • Detailed counseling of return precautions prior to discharge.

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References
1. Carter, J., Rahbar, R., Brigger, M., et al. (2016). International Pediatric ORL Group (IPOG) laryngomalacia consensus recommendations. Int J Pediatr Otorhinolaryngol. 86:256-261.
2. Dohar, J.E., Anne, S. (2013). Stridor, Aspiration, and Cough. In Johnson, J.J., Rosen, C.A. (Eds.), Bailey’s Head and Neck Surgery-Otolaryngology 5e (pp. 1338-1355). Baltimore, MD: Lippincott Williams & Wilkins.
3. Hartl, T.T., Chadha, N.K. (2012). A systematic review of laryngomalacia and acid reflux. Otolaryngol Head Neck Surg. 147(4):619-626.
4. Klinginsmith, M., Goldman, J. (2021). Laryngomalacia. In: StatPearls. Treasure Island, FL: StatPearls Publishing; June 11.
5. Myer, C.M., O’Connor, D.M., Cotton, R.T. (1994). Proposed grading system for subglottic stenosis based on endotracheal tube sizes. Ann Otol Rhinol Laryngol. 103(4 Pt 1):319-323.
6. Sidell, D.R., Messner, A.H. (2020). Evaluation and Management of the Pediatric Airway. In Flint, P.W., et al. (Eds.), Cummings Otolaryngology Head and Neck Surgery 7e (pp. 3053-3067). Philadelphia, PA: Elsevier.