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Differential Diagnosis: Infantile Stridor
Amy Stinson MS IV KCUMB
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Stridor An expression of partial respiratory tract obstruction 2° to external compression or partial occlusion within the airway1 Character & Intensity: Site & Degree of obstruction Airflow velocity & Pressure gradient Stridor
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Stridor Inspiratory – problem at or above vocal cords, usually high pitched at cords, low pitched above cords Laryngomalacia, unilateral cord paralysis Expiratory – problem is below cords – tracheobronchial tree, more prolonged Vascular compression Biphasic – usually subglottic Subglottic stenosis, subglottic hemangioma, bilateral cord paralysis
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Laryngomalacia Most common cause of stridor
Most common congenital laryngeal abnormality “Congenital flaccid larynx” or “Inspiratory laryngeal collapse” Inspiration: Prolapse of supraglottic structures Extreme infolding of “Omega-shaped” epiglottis and aryepiglottic folds Lateral margins of epiglottis fold inward; aryepiglottic folds are tall, foreshortend and thin; arytenoids are large with redundant tissue. Mucosal edema makes symptoms worse.
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Laryngomalacia Photographs show a case of laryngomalacia during expiration (A) and inspiration (B). Note the infolding of the aryepiglottic folds.
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Laryngomalacia Normal Vs Abnormal
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Laryngomalacia Signs & symptoms:
Inspiratory stridor within a few days of birth Initially mild more pronounced with a peak at 6 –9 mo4 Stridor is worse when supine and neck flexion: better when prone and neck extension1 Symptoms worse when sleeping, feeding, or on exertion Most commonly – mild stridor that is self limited Most cases spontaneously resolve by 2 yrs of age
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Laryngomalacia More Severe: Presumed etiologies:4
Severe stridor, apneic episodes, feeding problems, & FTT pulmonary HTN & cor pulmonale Presumed etiologies:4 Abnormally pliable supraglottic cartilage Neuromuscular abnormalities GER
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Laryngomalacia DX: Treatment: Endoscopy under local anesthesia
Laryngotracheobronchoscopy to R/O other path Polysomnography to detect desaturations w/hypoxia or hypercapnia Treatment: OBSERVE Temp trach in severe cases Sx for 10% supraglottoplasty which reduces amount of laryngeal mucosa Anti-reflux meds Supraglottoplasty – divide aryepiglotitc folds, excise wedge of folds with or with out trimming arytenoids on lateral borders of epiglottis, suture epiglottis to base of tongue Watch out for bleeding, aspiration and supraglottic scarring
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Laryngomalacia “State Dependent” Laryngomalacia6
Neurogenic factors would cause at states of awareness – often paradoxical. Direct stimulation resolves Discoordinate Pharyngolaryngomalacia6 Assoc. with severe collapse and poor outcomes Need CPAP & nasal stents and poss. trach
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Vocal Cord Paralysis Second most common congenital abnormality of larynx4 Congenital & Bilateral is most common presentation with stridor and is usually seen in males4 Unilateral paralysis on Left seems to be more common but less associated with stridor6 Check nucleus ambiguous & supranuclear tracts plus Vagus nerve & branches3
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Vocal Cord Paralysis Etiology: Idiopathic
CNS: Arnold-Chiari malformation CV: Congenital abnormalities of heart & great vessels of Sx correction of Trauma: Repair of TE fistula, birth trauma, head injury Inflammatory: Guillian-Barre PNS: myotonic dystrophy, myasthenia gravis
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Vocal Cord Paralysis Signs & Symptoms:
Asymp acute airway obstruction High pitched inspiratory stridor, apnea, cyanosis Hoarse, breathy cry Weak cough All more common with bilateral palsy
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Vocal Cord Paralysis Dx: Tx: Fiberoptic endoscopy
Laryngotracheobronchoscopy MRI Tx: Unilateral: Observe, Speech therapy Bilateral: Tracheotomy, frequent endoscopies, no Sx for at least a year – maybe longer CNS mcc in infancts, cords are usually adducted which can cause airway compromise
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Vocal Cord Paralysis
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Subglottic Stenosis 3rd most common congenital cause of stridor
Subglottis is the narrowest part of airway & the only complete ring (cricoid cartilage) Congenital & Acquired
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Subglottic Stenosis2,4,5 Congenital: Acquired:
Soft tissue stenosis or cartilaginous stenosis Severe: stridor at birth Mild: intermittent stridor & resp tract infections Acquired: Neonatal intubations, external trauma, high trach, infection, burns Repeated failure of attempted extubation Gradual onset of stridor after extubation Pressure necrosis of subglottic mucosa, duration of intubation is most determining factor Edema & ulceration follow perichondritis granulation & fibrous tissue formation
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Subglottic Stenosis1,2 Stenosis if < 4mm in full term infant; < 3mm in preterm infant Meyer-Cotton Grading I: 0-50% II: 51-70% III: 71-99% IV: no detectable lumen
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Subglottic Stenosis Decrease risk: Uncuffed, polyvinylchloride tubes
Smaller tubes Nasotracheal intubation = less friction Risks – larynx 1/3 size of adults; arytenoids make up more of the glottis; smaller subglottis; tissue is softer and more pliable
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Subglottic Stenosis Treatment:
Observe: Grade I, II, airway can increase with growth of child Tracheotomy: until reconstruction Endoscopic: Laser can decrease granulation tissue, can actually worsen with long term scarring Laryngotracheal reconstruction: requires cartilage grafts and stents, enlarges stenosed portion Cricotracheal resection: excises stenosed portion, higher success rate, but increased risk of recurrent laryngeal nerve damage
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Subglottic Hemangioma
A soft, compressible, bluish tumor below true vocal cords Female > male 2:1 50% have cutaneous hemanigioma Subglottis is most common location – usually unilateral Tend to proliferate from birth – 1 yr then involute. Usually resolved by 5 yrs.
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Subglottic Hemangioma
Signs & Symptoms: Intermittent stridor that progresses to biphasic stridor with dyspnea and cyanosis Originally dx as croup
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Subglottic Hemangioma
Treatment Observe: if small Tracheotomy until involution Steroids – possible estrogen receptor involution Laser therapy – good for hemostasis Surgical excision – becoming more common because of stenosis from trach Interferon – alfa-2a has antiangiogenic activity when hemangioma in proliferate phase Trach & Laser stenosis Steroids HTN Cushings growth retardation IFN – can get rebound growth and effects in kids not totally known
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Less Common Causes of Stridor
Dysphagia lusoria Laryngeal cysts Congenital laryngeal webs Laryngeal foreign bodies Respiratory papillomatosis
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Dysphagia Lusoria “Dysphagia of unclear etiology”
Congenital anomalies of aortic arch: Double aortic arch Anomalous origin of R or L subclavian artery Kommerell’s Diverticulum – saccular aneurysmal dilation at of ARSA or ALSA If LA or ductus present between subclavian and pulmonary complete vascular tracheobronchial ring Presents as respiratory distress, dysphagia & stridor
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Dysphagia Lusoria Dx: Tx: CXR Barium swallow w/ esophogram
Sx repair through lateral thoracotomy with lung separation Embryo – distsal end of interrupted 4th aortic arch btw carotid and subclavian
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Laryngeal Cysts2,4 Rare cause of Stridor More superficial Fluid filled
Ductal: MC, originate from obstruction of submucous gland Saccular: in laryngeal ventricles, usually congenital
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Congenital Laryngeal Webs1,2,4
Embryology: Failure of complete recanalization Most common in anterior glottis (fusion of ant portion of vocal cords) Abnormal cry & stridor Incise thin webs Excise and stent severe webs
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Laryngeal Foreign Bodies4
MC in kids 1-3 yrs Most inhaled objects pass through larynx and lodge distally If lodged in larynx & partially obstructed Stridor, hoarseness, and cough Confirm w/X-ray Remove in OR
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Respiratory Papillomatosis1,2,4
Most common neoplasm of larynx in children Dx: most common btw 2 –5 yrs Increased risk: First born, vaginal delivery, teenage mother HPV 6, 11 Gradual progression of dyspnea and stridor Tx: surgical ablation w/CO2 laser webs & scarring Larynx mc involved, thought to be b/c of amniotic fluid aspiration, can lead to life threatening airway obsruction
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References 1. Rowe, LD. Pediatric Airway Obstruction. Otolaryngology – Head and Neck Surgery. Current Surgery. Chap 38. 2. Kirby, GS. et al. Respiratory Tract and Mediastinum. Current Pediatrics. Chap 18. 3. Gormley, PK. et al. Congenital vascular anomalies and persistent respiratory symptoms in children. International Pediatric Journal of Otorhinolaryngology. Nov 1999: 51:23-31. 4. Lange, et al. Current Opinion in Otolaryngology and Head and Neck Surgery. Lippincott, Wilkins and Williams. Dec p 349. 5. Mossad, E. et al. Diverticulum of Kommerell: A review of a Series and a Report of a Case with Tracheal Deviation Compromising Single Lung Ventilation. Anesth Analog. 2002:94:1462-4 6. Bent, J. Pediatric Laryngotracheal Obstruction: Current Perspectives on Stridor. Laryngoscope. 2006: 116: 7. Sisk, EA. et al. Tracheotomy in Very Low Birth Weight Neonates: Indications and Outcomes. Laryngoscope. 2006: 116:
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