Symptoms and signs of concern The Neonatal Airway:  Stertor, Stridor and  Respiratory Distress   Dysphonia (hoarse cry)  Nasal flaring  Anthony E Magit, MD, MPH Rady Children’s Hospital  Retractions University of California, San Diego  Feeding difficulties

Historical factors of The sounds say a lot importance

 Stertor  Traumatic labor  Responsible for 20% of TVC palsy  Nasal or pharyngeal source  Intubation  Ease, size of tube, duration, re-intubation, failure of  Inspiratory stridor extubation  Supraglottis, rarely high  Aggravating/alleviating factors trachea  Agitation, positioning, feeding, crying  Biphasic stridor  Reflux (overt vs “silent”)  Glottis/subglottis  Neurologic impairment  Expiratory stridor  Cardiac disease or surgery  Intrathoracic obstruction  Birthmarks  Syndromes

Neonatal anatomic and physiologic Physical exam considerations

 Nasal/pyriform aperture/choana  Vocal cord length 6-8mm  Masses, edema, septal deviation, stenosis, atresia  Oral cavity/oropharyngeal  Transverse length post glottis  Masses, tongue size, mandibular position, tonsils, 4mm tone  Subglottic diameter 5-7mm  Neck  Masses, vertebral anomalies, vessels  Tracheal length 4cm  Listen to qualify abnormal sounds  1mm of edema  airway  Stertor caliber  Stridor  Breath sounds, , murmurs  In the glottis by 35%  In the subglottis by 44%  Watch neonate feed if possible

1 Neonatal anatomic and physiologic considerations Neonatal Nasal Obstruction  Neonates are obligate nasal breathers until 3-6  Obligate nasal breathers months of age (some variability) x 2-5 mos  Epiglottis touches soft  Stertor  A low-pitched inspiratory sound produced by nasal or palate nasopharyngeal obstruction: snorting  Tongue occupies all space in mouth  Oral a reflex that doesn’t develop until several weeks after birth  Nasopharyngeal airway narrower due to Waldeyer’s ring

Management Strategies Neonatal Nasal Obstruction  Observation  Neonatal rhinitis  Medical treatment to reduce inflammation  Choanal atresia/stenosis

 Supplemental O2 or CPAP  Nasolacrimal duct cysts  Bypass the obstruction  Pyriform aperture stenosis  Oral airway  Oral intubation  Nasopharyngeal mass  Surgery  Repair the defect  Septal Deviation  Tracheotomy

Physical Examination-Neonate Neonatal Nasal Obstruction: Rhinitis of Infancy  What is it?  Mirror for nasal airflow  Constellation of conditions that generates nasal mucosal edema with airway obstruction  Headless  Not uncommon but not well recognized and not well described in literature  Catheter  Presentation  Traumatic  Stertor  Everyone does this  Respiratory difficulty  Flexible endoscope  Feeding difficulty, failure to thrive

2 Rhinitis of Infancy Rhinitis of Infancy  Etiology unclear  Diagnosis  GERD  Milk allergy  Boggy, swollen  Meconium aspiration turbinates  Iatrogenic  Clear secretions  Aggressive nasal suctioning  Stertor improves  Maternal medications / drug abuse (transiently) with  Antihypertensives, narcotics, -blockers, antidepressants decongestants  Infection  Chlamydia, Strep, Staph, Viral, Syphilis

Rhinitis of Infancy Choanal Atresia

 Treatment  Bilateral Atresia  Unilateral Atresia  Reduce nasal suctioning  Present immediately after birth  No acute respiratory distress  Respiratory distress with  Chronic unilateral nasal  Saline drops cyanosis discharge  Oxymetazoline drops x 3 days  Relieved by crying  Presents later in life  Decadron ophthalmic 0.1% bid x 5-7 days  Feeding difficulty  GERD precautions / treatment  Culture of nasal secretions  Occasionally need oral steroids  If persists, CT scan

Choanal Atresia Choanal Atresia  CT Scan  Diagnosis  Nasal  Suction nose immediately prior to scan  Mirror exam, cotton  All with bony component test  Lateral nasal wall and pterygoid  Mucoid cast  Passage of a 5-6 Fr catheter  Flexible endoscopy  CT scan-mandatory

3 Choanal Atresia Choanal Atresia-Surgical repair  Medical emergency  Transnasal  Stimulate to cry  Transpalatal  Insert finger  Establish an airway  Tools:  Microdebrider  McGovern, cross-cut nipple  Drill  Oral airway  Ureteral sounds  Intubation  120 degree telescope  Backbiters  Work up for associated anomalies  Stenting  Cardiac 50%  Mitomycin  CHARGE Syndrome  Ciprodex

Nasolacrimal Duct Cysts Anterior pyriform aperture stenosis  Presentation  Isolated anomaly versus midline defect  Nasal obstruction  Respiratory distress with stertor  Microform of holoprosencephaly but some nasal air flow  Pituitary axis abnormalities (different than choanal atresia)  Epiphora  Mega-incisor  PE and CT-Cystic mass in inferior meatus  Management  Remove endoscopically  Presentation:  Ophthalmology consult for NLD obstruction  Respiratory difficulty  Bilateral nasal airflow  Difficulty passing catheters or flexible endoscope  Narrow nasal vestibule

Anterior pyriform aperture nasal Anterior pyriform aperture stenosis stenosis

 Systemic evaluation  CT scan  Observation  Surgical repair involving a sub-labial drill-out of the pyriform aperture

Visvanathan V, Wynne DM. Congenital nasal pyriform aperture stenosis: a report of 10 cases and literature review. Int J Pediatr Otorhinolaryngol 2012; 76: 28-30

4 Nasopharyngeal Mass Encephalocele  Neoplasm (Synovial cell carcinoma) Intra-or extranasal  Nasofrontal angle  Adenoidal hypertrophy  Medial in nasal cavity  Rare in neonate Don’t biopsy!  Encephalocele Bluish, compressible Enlarge with crying Meningitis, CSF leak

Septal Deviation  Result of pressure due to intrauterine position  Occasionally due to birth trauma  Visible with otoscope  CT for other anomalies  Closed reduction for significant deformity-at bedside  Most milder forms self correct by one month

Maternal medication history Stridor  Harsh sound caused by turbulent airflow  Implies partial airway obstruction

 Laryngeal stridor-inspiratory, biphasic

5 Physical Examination Physical Examination Let parent hold child Let parent hold chilMirror for nasal airflow irror for nasal airflow“Headless” stethoscope “Headless” stethoscope Positional changes Mandibular thrust

Where to perform the procedures? Awake flexible laryngoscopy  Flexible laryngoscopy  Direct laryngoscopy can be done at the and bronchoscopy  Assess dynamic status of larynx (*) bedside need to be performed  Less controlled than under GA but safe  Topical or no anesthetic in OR with experienced endoscopist required  General anesthesia  No need for NPO status  Routine preoperative  Lesions below the glottis not well unless not protecting NPO orders visualized airway  Exception: ECMO pts  Can be present up to 5% of time  May also be done in OR can be done in unit  Thus more of a screening procedure

Awake flexible laryngoscopy technique Rigid laryngotracheobronchoscopy

 Afrin and/or ponticaine to nare for decongestion  Investigation of choice for all complex airway as needed lesions  Local is often not used as it can induce apneas  Highly technical and requires an experienced  Endoscope is advanced through the nares and/or team familiar with procedure and neonates mouth (surgeon, anesthesiologist, scrub tech)  Suction may be needed to clear secretions  Spontaneous ventilation allows dynamic  Want to see TVC motion, rule out , assessment of airway look for masses or other anatomic anomalies, and assess subglottis if possible  Topicalization decreases risk of spasm  Intubation is avoided if possible

6 Indications for operative endoscopy Laryngoscopy

 To establish diagnosis  Parson’s ventilating laryngoscope used to assess for TVC motion, masses, cysts, webs, and  Severe or progressive stridor posterior laryngeal clefts  Allows for improved visualization, ability to ventilate  Cyanosis or concerns patient  Radiologic abnormalities  Rigid telescope helps to magnify findings and allows for photodocumentation  Parental or physician anxiety  Arytenoid motion checked passively if TVC paralysis is present

Tracheobronchoscopy

 Telescope can be advanced through TVC to assess subglottis, trachea, and mainstem bronchi  Significant subglottic stenosis would preclude use of bronchoscope Common pathologies  Ventilating bronchoscope can be utilized to evaluate to the level of the bronchi  Assess for , vascular rings, complete tracheal rings, stenosis, masses

Laryngomalacia Laryngomalacia

 #1 cause of neonatal stridor  Endoscopic appearance  Inward collapse into laryngeal inlet during inspiration:  A-E folds  cuneiform cartilage  Epiglottis  Short A-E folds  Omega shaped epiglottis

7 Laryngomalacia Laryngomalacia

 Intermittent inspiratory  Treat GERD,  Vast majority are mild stridor observation in most  Do these really need operative endoscopy?  Worse with feeds, cases, rarely crying, supine supraglottoplasty  Parental reassurance & education  Better prone  Transient worsening, gradual improvement  Indications for OR  Weight gain issues  15% associated with  Respiratory difficulty  GERD issues secondary airway  Feeding difficulty lesions  Failure to thrive

Discoordinate Severe Laryngomalacia pharyngo-laryngomalacia

 Absence of classical laryngomalacia  Respiratory difficulty findings  Feeding difficulty  Prolapse of pharyngeal tissues  Failure to thrive  Neurologic abnormalities

 GERD  Often older infants

 CNS abnormalities  Treatment with trial BiPAP, consider tracheostomy  A-E fold division may make airway obstruction worse

Vocal cord paralysis Vocal cord paralysis

 #2 most common congenital laryngeal anomaly  In the NICU population, cardiac surgery is an  Unilateral usually from birth trauma enormous risk factor  Often goes unnoticed  Weak cry, mild stridor, aspiration  Paresis means partial paralysis  Bilateral usually from CNS abnormality  Indicates nerve is likely intact  Generally an airway emergency with better prognosis  Normal cry, high pitched insp stridor  May need to wait years for  Other causes: C-A joint fixation, posterior glottic return of function web/stenosis, infiltrative lesions  Swallow study critical to ensure no aspiration

8 Posterior laryngeal cleft  Dx with DL and  Posterior cricoid esophagram lamina doesn’t fuse  Tx with endoscopic vs  Associated with T-E open repair Uncommon Pathologies fistulas, laryngomalacia, congenital cardiac dz, CLP, Trisomy 21  Inspiratory stridor, pneumonia, aspiration

Laryngeal Cleft Laryngeal Clefts  High index of suspicion needed  Types 1-4  Stridor & aspiration problems  Assess with microlaryngoscopy  1-observation  2 handed distraction technique  2-endoscopic repair  Palpation of interarytenoid region  3-Laryngofissure with buttress and multilayer closure  4-Thoracotomy

Tracheomalacia Tracheomalacia

 Tracheal walls are soft and collapse during  May be found in conjunction with compressive respiration, causing wheezing, stridor, or both lesions, such as mediastinal masses, vascular  May or may not improve with age slings, and vascular rings.  Also occurs in some children with chronic  If extrathoracic, primarily inspiratory collapse and inflammation of the proximal airways airway sounds  Chronic lung disease of infancy, GERD, or other forms of chronic aspiration.  If intrathoracic, predominately expiratory collapse and airway sounds (more common)  Frequently found after repair of a T-E fistula  Diagnose with bronchoscopy  Anterior wall collapses posteriorly  Rule out vascular ring/sling  Assess for tracheal stenosis, foreign bodies

9 Vascular rings Congenital subglottic stenosis  #3 most common congenital laryngeal anomaly  Biphasic stridor, barking , dyspnea with feeding  Diameter of <4mm at the cricoid in a full-term infant,  Double aortic arch (most common causing stridor) <3mm in a premature infant  Right aortic arch  Anomalous innominate artery  May present as recurrent croup, or if more severe  Anomalous L common carotid can cause biphasic stridor  Pulmonary artery sling  Retroesophageal R subclavian A (most common  Rigid endoscopy to establish dx compressing aerodigestive tract)  Most improve with age, some require trach and  CT, MRI, barium swallow +/- angio laryngotracheal reconstruction  Tx with pexy or reimplant of vessel if symptomatic

Congenital Airway Acquired Subglottic stenosis Abnormalities  Intubation trauma  Glottic webs remains a significant risk factor for acquired  Tend to be thick and extend to subglottis stenosis  Laryngeal atresia variant  22 q deletion association  Reflux management is  Rare familial pattern critical  Surgical Options  Endoscopic vs. Open  Keel placement

Subglottic hemangioma Subglottic hemangioma  Benign congenital vascular tumor  Endoscopic dx without bx:  Cellular hyperplasia of endothelial cells, mast cells, lesion is a compressible fibroblasts, and macrophages asymmetric submucosal mass  Grow rapidly over the 1st year of life, generally resolve in posterolateral subglottis, with by age 5 bluish or reddish discoloration  Typical symptomatology of insp or biphasic stridor +/-  Many tx options inc trach barky cough +/- dysphonia (avoid), steroids, laser, endoscopic vs open excision, INF-2a  Propranolol

10 Post Intubation Injuries Post Intubation Injuries  Anterior commissure web  Weak, hoarse cry  History  Mild-moderate respiratory distress  Rx: endoscopic division Intubation--even transient   Short term post-op intubation  NICU Graduate  Mitomycin C  Stridor with URI  History of recurrent croup or  Poor response to standard therapy

Post Intubation Injuries Post Intubation Injuries  Posterior glottic injury: progressive changes  Interarytenoid web  Granulation  Difficult problem  Ulceration  Mistaken for vocal cord paralysis  Furrow  Assess with MSL using 2 handed distraction technique Posterior   Repair: endoscopic division alone  Interarytenoid scar rarely successful  Mitomycin C  Mucosal flap interposition  Posterior cricoid expansion

Post Intubation Injuries Post Intubation Injuries Subglottic Cysts  Often multiple  Subglottic Stenosis  Removal  Assess entire airway  Forceps

 Size & grade stenosis  Laser (CO2 / KTP)  Laryngotracheal reconstruction  Microdebrider  Treat for GERD

11 Concerns in Airway Surgery Management Approach  Surgical Debate Postoperative edema, infection  Open vs Endoscopic Long term treatment with steroids  Pediatric laryngology  All techniques with advantages and disadvantages Extended hospitalizations, intensive care  Facility with all techniques  Development of newer instruments/techniques Complications: subglottic stenosis, glottic webs  Don’t abandon traditional “cold steel” instruments Voice abnormalities  Goals  Safe and effective means of achieving resolution  Reduce pitfalls of current techniques  Minimal number of interventions

Synchronous lesions Tracheotomy  Not uncommon  Not a bad word!  Laryngomalacia  Avoid a trach, create a crisis!  GERD issue  Genetic / Syndromic

Pediatric Stridor: Conclusions Conclusion Assess urgency of intervention  Small airways can make for big problems Thorough evaluation of the situation: age, history,  History and exam often can make diagnosis clinical presentation Anticipate risks and benefit of examination  Bedside and/or operative laryngoscopy/bronchoscopy give critical information Relationship with anesthesia critical-spontaneous  Flexible nasopharyngoscope, Parsons laryngoscope and ventilation ventilating bronchoscope crucial tools Pediatric endoscopic equipment  Many entities do not require ultimate surgical Consider all possibilities, co-morbidities and intervention synchronous lesions

12 Thank you for your attention

 Any questions?

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