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

Laryngomalacia

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  • 1. DEAPCIT apporach toLaryngomalacia (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 obstructionJackson C, Jackson C. Diseases and injuries of the larynx. New York: MacMillan; 1942. p.639 3. Epidemiology 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 epiglottisBelmont 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 = 0.535Manning 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 laryngomalaciaDana 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 Feeding symptoms 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 Choking, coughing, prolonged feeding time, recurrent emesis, dysphagia, weight lossFTT, 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 symptomatology/flexible laryngoscopy Mild Moderate SevereBased on mechanism of collapse Anterior: epiglottis Posterior: arytenoid Lateral: AE fold 12. Investigation Flexible fibreoptic laryngoscopy Age range Good for dynamic assessment May avoid needing formal endoscopy/GA Unable to assess lower airwayMicrolaryngoscopy 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 Nissens surgery is necessarySleep study to document severity of apnea in severe LM and in surgical failures 14. Treatment Observation Medical Empiric reflux acid suppression Feeding modifications Posture repositioningSurgicalSupraglottoplasty Epiglottopexy Tracheostomy Thompson DM. Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia: a new theory of etiology. Laryngoscope 2007;117:133. Giannoni C, Sulek M, Friedman EM, et al. Gastroesophageal reflux association with laryngomalacia:a prospective study. Int J Pediatr Otorhinolaryngol 1998;43:1120. 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 difficultyRichter GT, Thompson DM. The surgical management of laryngomalacia. Otolaryngol Clin North Am. 2008 Oct;41(5):837-64, vii. 17. Supraglottoplasty 18. Thank you