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Differential Diagnosis: Infantile Stridor

Differential Diagnosis: Infantile Stridor. Amy Stinson MS IV KCUMB. Stridor. An expression of partial respiratory tract obstruction 2 ° to external compression or partial occlusion within the airway 1 Character & Intensity: Site & Degree of obstruction

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Differential Diagnosis: Infantile Stridor

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  1. Differential Diagnosis: Infantile Stridor Amy Stinson MS IV KCUMB

  2. 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

  3. 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

  4. 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

  5. Laryngomalacia • Photographs show a case of laryngomalacia during expiration (A) and inspiration (B). Note the infolding of the aryepiglottic folds. • www.entjournal.com/htmlDocs/Images/ped-0209.jpg

  6. Laryngomalacia • Normal Vs Abnormal • www.meei.harvard.edu/.../images/laryngomal.jpg

  7. 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

  8. Laryngomalacia • More Severe: • Severe stridor, apneic episodes, feeding problems, & FTT •  pulmonary HTN & cor pulmonale • Presumed etiologies:4 • Abnormally pliable supraglottic cartilage • Neuromuscular abnormalities • GER

  9. Laryngomalacia • DX: • 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

  10. 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

  11. 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 www.meei.harvard.edu/.../images/laryngomal.jpg

  12. 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

  13. 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 • www.meei.harvard.edu/.../images/laryngomal.jpg

  14. Vocal Cord Paralysis • Dx: • Fiberoptic endoscopy • Laryngotracheobronchoscopy • MRI • Tx: • Unilateral: Observe, Speech therapy • Bilateral: Tracheotomy, frequent endoscopies, no Sx for at least a year – maybe longer

  15. Vocal Cord Paralysis www.meei.harvard.edu/.../images/laryngomal.jpg

  16. Subglottic Stenosis • 3rd most common congenital cause of stridor • Subglottis is the narrowest part of airway & the only complete ring (cricoid cartilage) • Congenital & Acquired • www.meei.harvard.edu/.../images/laryngomal.jpg

  17. Subglottic Stenosis2,4,5 • Congenital: • 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

  18. 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 • www.meei.harvard.edu/.../images/laryngomal.jpg

  19. Subglottic Stenosis • Decrease risk: • Uncuffed, polyvinylchloride tubes • Smaller tubes • Nasotracheal intubation = less friction • www.meei.harvard.edu/.../images/laryngomal.jpg

  20. 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

  21. 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. • www.childrensenthouston.com/images/laryngomal

  22. Subglottic Hemangioma • Signs & Symptoms: • Intermittent stridor that progresses to biphasic stridor with dyspnea and cyanosis • Originally dx as croup www.meei.harvard.edu/.../images/laryngomal.jpg

  23. 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

  24. Less Common Causes of Stridor • Dysphagia lusoria • Laryngeal cysts • Congenital laryngeal webs • Laryngeal foreign bodies • Respiratory papillomatosis

  25. 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

  26. Dysphagia Lusoria • Dx: • CXR • Barium swallow w/ esophogram • Tx: • Sx repair through lateral thoracotomy with lung separation

  27. 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 • www.meei.harvard.edu/.../images/laryngomal.jpg

  28. 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 • www.meei.harvard.edu/.../images/laryngomal.jpg

  29. 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

  30. 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

  31. 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 1999. 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: 1059-1070 • 7. Sisk, EA. et al. Tracheotomy in Very Low Birth Weight Neonates: Indications and Outcomes. Laryngoscope. 2006: 116: 928-933

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