Laryngomalacia

1. DEAPCIT apporach to Laryngomalacia (LM) Hamilton registrar conference 2012 Angus Shao

2. Definition Congenital laryngeal anomaly of the newborn characterised by flaccid laryngeal tissue and inward collapse of the supraglottic structure leading to upper airway obstruction Jackson C, Jackson C. Diseases and injuries of the larynx. New York: MacMillan; 1942. p.63–9

3. E pidemiology commonest cause (~ 65%) of stridor in infants ( 17% have another intercurrent airway lesion) may occur in older children & adults more common in male and term baby Association with other syndromes and neurologically-impaired (e.g. cerebral palsy )

4. Aetiology ?Cartilage immaturity ?Anatomic abnormality ?Neuromuscular immaturity ?Inflammatory

5. Anatomic abnormality LM is a result of the exaggeration of an infantile larynx (Iglauer1922) May or may not be an important factor since stridor is not seen in all infants with ‘ omega epiglottis ’ Belmont JR, Grundfast K .. Congenital laryngeal stridor ( laryngomalacia ): etiologic factors and associated disorders. Ann Otol Rhinol Laryngol . 1984 Sep-Oct;93(5 Pt 1):430-7.

6. Anatomic abnormality Shortening of aryepiglottic folds and anterior collapse of cuneiform and corniculate cartilage Prospective case-control by Manning et al created a ratio of aryepiglottic fold length to glottic length Severe laryngomalacia = 0.380 Control = 0.535 Manning SC, Inglis AF, Mouzakes J, Carron J, Perkins JA. Laryngeal anatomic differences in pediatric patients with severe laryngomalacia . Archives of Otolaryngology Head and Neck Surgery. 2005 Apr; 131 (4): 340-3.

7. Neurologic immaturity Immature neuromuscular control and movement result in neuromuscular hypotonia LAR (laryngeal adductor reflex) Vagal mediated SLN Receptors at aryepiglottic fold Altered laryngeal tone and sensorimotor integrative function  weak tone Dana M. Thompson. Laryngoalacia : factors that influence disease severity and outcome of management. Current opinion in Otolaryngology&Head and Neck Surg. 2010, 18: 546-570.

8. inflammatory Reflux can induce posterior supraglottic oedema and secondarily LM 65-100% of infants with LM have GORD Not clear whether GORD is a cause or an effect of laryngomalacia Dana M. Thompson. Laryngoalacia : factors that influence disease severity and outcome of management. Current opinion in Otolaryngology&Head and Neck Surg. 2010, 18: 546-570.

9. Clinical Stridor is the hallmark of congenital LM lower pitched, inspiratory, worsens with agitation, crying, feeding or in the supine position median time to spontaneous resolution of stridor is 9 months of age, and 75% will have no stridor by 18 months of age Feeding symptoms Choking, coughing, prolonged feeding time, recurrent emesis, dysphagia, weight loss FTT, Aspiration, Apnoea , Hypoxia, Recurrent cyanosis, Cor-pulmonale

10. Classification Several classification systems have been proposed with none being predominant at this time Olney DR,  Greinwald   Jr  JH, Smith RJ, et al:  Laryngomalacia and its treatment.  Laryngoscope  1999; 109:1770-1775 . Chen JC,  Holinger  LD: Congenital laryngeal lesions: pathology study using serial macrosections and review of the literature.   Pediatr Pathol   1994; 14:301-325 . Shah UK, Wetmore RF:  Laryngomalacia : a proposed classification form.   Int J Pediatr Otorhinolaryngol   1998; 46:21-26.

11. Based on mechanism of collapse Anterior: epiglottis Posterior: arytenoid Lateral: AE fold Based on symptomatology/flexible laryngoscopy Mild Moderate Severe

12. Investigation Flexible fibreoptic laryngoscopy Age range Good for dynamic assessment May avoid needing formal endoscopy/GA Unable to assess lower airway Microlaryngoscopy and Bronchoscopy Gold standard GA required (Rigid endoscopes) Dynamic assessment can be more difficult Allows complete structural & dynamic view Better control of airway cf flexible endoscopy

13. Investigation Other Adjuncts: FEES ( videofluroscopy ) Chest X-ray to r/o aspiration Oesophagram Extent and degree of reflux r/o concomitant GI disorder pH study if Nissen ’ s surgery is necessary Sleep s tudy to document severity of apnea in severe LM and in surgical failures

14. Treatment Observation Medical Empiric reflux acid suppression Feeding modifications Posture repositioning Surgical Supraglottoplasty Epiglottopexy Tracheostomy Thompson DM. Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia : a new theory of etiology. Laryngoscope 2007;117:1–33. Giannoni C, Sulek M, Friedman EM, et al. Gastroesophageal reflux association with laryngomalacia:a prospective study. Int J Pediatr Otorhinolaryngol 1998;43:11–20.

15. Surgical intervention

16. Indication Absolute Cor pulmonale Hypoxia Apnea Recurrent cyanosis Failure to thrive Pectus excavaium Stridor with respiratory compromise Stridor with significant retractions Relative Aspiration Difficult-to- feed child who has failed medical intervention Weight loss with feeding difficulty Richter GT, Thompson DM. The surgical management of laryngomalacia . Otolaryngol Clin North Am. 2008 Oct;41(5):837-64, vii.

17. Supraglottoplasty

18. Unilateral Supraglottoplasty Endoscopic Aryepiglottoplasty CO2 Laser Supraglottoplasty Microdebrider Supraglottoplasty Aryepiglottic Fold Division Epiglottoplasty Epiglottopexy Tracheostomy Supraglottoplasty

19. Thank you