Presentation on theme: "Current Concepts in Diagnosis and anagement of Laryngomalacia"— Presentation transcript:
1 Current Concepts in Diagnosis and anagement of Laryngomalacia Shraddha Mukerji, MDHarold Pine, MDDepartment of OtolaryngologyUniversity of Texas Medical Branch, GalvestonMarch 31, 2009
2 Overview Discuss Pathogenesis Clinical presentation Laryngomalacia and GERDDiagnosisMedical and surgical managementParental counseling
3 What is laryngomalacia? Laryngomalacia (LM) is the commonest congenital laryngeal anomaly of the newborn characterized by flaccid laryngeal tissue and inward collapse of the supraglottic structures leading to upper airway obstructionJackson C, Jackson C. Diseases and injuries of the larynx. New York: MacMillan; p.63–9
5 Cartilage immaturityFirst proposed by Sutherland and Lack in the late 19th centuryDelayed development of the cartilageneous support of the larynxTheory has been disprovedNo histological evidence of chondropathyIncidence not different in premature infants
6 Anatomic abnormalityLM 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’Congenital laryngeal stridor (laryngomalacia): etiologic factors and associated disorders. Belmont JR, Grundfast K. Ann Otol Rhinol Laryngol Sep-Oct;93(5 Pt 1):430-7.
7 Neuromuscular immaturity There is a high prevalance of neurologic disorders with LMSome believe that neuromuscular immaturity leads to laryngeal hypotonia and LMMay be one of the several components of LM
8 Laryngomalacia and GERD 80-100% of infants with LM have GERDIt is not clear whether GERD is a cause or an effect of LMEMPIRIC REFLUX THERAPYchoking,frequent emesis,regurgitationor feeding difficulty
9 Proposed pathogenesis of GERD in LM Respiration against afixed obstructionIncreased obstructionLarge –ve intrathoracicpressureIncreased prolapseReflux into esophagus andLPRLaryngeal edema
10 Proposed pathogenesis of GERD in LM Disruption of effective vagal tone to LESRelative decreased LESGERDThis pathogenesis leads credence to “NEUROLOGICAL IMMATURITY” theory of LM
12 Clinical presentation Stridor is the hallmark of congenital LMHigh pitched, inspiratory, worsens with agitation, crying, feeding or in the supine positionFeeding symptomsChoking, coughing, prolonged feeding time, recurrent emesis, dysphagia, weight lossGERD symptomsComplications
13 Complications of LM 10-20% of patients present with complications Life threatening airway obstructionFailure to thriveCyanosisSleep apneaPulmonary hypertension, developmental delay and cardiac failure
14 Classification schemes Based on symptomatology/flexible laryngoscopyMildModerateSevereBased on mechanism of collapseAnterior: epiglottisPosterior: large arytenoidsLaterally: AE folds
15 Classification scheme based on symptoms, flexible laryngoscopy MILDSEVERE
16 Classification scheme based on mechanism of LM ANTERIORLATERALPOSTERIOR
18 Direct laryngoscopy video You may have to click or double-click to see the movie
19 Complementary studies Chest X-ray to r/o aspirationEsophagramExtent and degree of refluxr/o concomitant GI disorderpH study if Nissen’s surgery is necessarySleep Study to document severity of apnea in severe LM and in surgical failures
20 Management Medical Surgical Empiric reflux acid suppression Feeding modificationsPosture repositioningSurgicalSupraglottoplastyEpiglottopexyTracheostomyThompson 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.
21 Empiric reflux acid suppression 80-100% of patients with LM have GERDH2 receptor antagonist (RA) or Proton pump inhibitor (PPI)H2RA: ranitidine 3mg/kg three times dailyPPI: 1mg/kg dailyIf symptoms worsen6mg/kg of ranitidine at night + 1mg/kg of PPI dailyThompson DM. Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia: a new theory of etiology. Laryngoscope 2007;117:1–33.
22 Feeding modifications PacingThickening formula feedsUpright feeding positionSmall, frequent feeds
23 Evolving concepts in surgical management of LM Variot was the first to suggest removal of excess of AE tissue as treatment of LM1920Re-introduction of concept of removal of SG tissue for treatment of LMSporadic reports of endoscopic trimming, partial epiglottopexy, wedge resection but no definite technique1980Endoscopic techniques revisited and definedEndoscopic supraglottoplastyEpiglottopexyCurrent
24 Indications for surgery Absolute indicationsRelative indicationsCor pulmonaleAspirationPulmonary hypertensionDifficult-to-feed child who has failed medical interventionHypoxiaWeight loss with feeding difficultyApneaRecurrent cyanosisFailure to thrivePectus excavatumStridor with respiratory compromiseStridor with significant retractionsRichter GT, Thompson DM. The surgical management of laryngomalacia. Otolaryngol Clin North Am Oct;41(5):837-64, vii.
25 Contraindications Relatively uncommon Proceed with caution Patients with comorbiditiesPatients with multiple levels of airway obstructionPostpone surgery till resolution of URIWEIGHT AND AGE ARE NOT CI TO SURGERY
26 Pre-operative counseling Overnight hospitalization in the ICUThe stridor will improve, but NOT DISAPPEARExpect feeding improvementReflux precautions and medications to be continuedRisk of revision surgery
27 Anesthetic considerations Spontaneous breathing analgesiaETT in the nasopharynx, mouthSpray (1% lidocaine <2 years, 2% ≥ 2yrs)0.5 mg/kg of decadronIntubation only for unstable patients or patients with poor pulmonary reserveGOOD COMMUNICATION WITH THE ANESTHESIOLOGISTS
28 Surgical Set-up Rigid bronchoscopy Visualize subglottis, trachea and bronchiR/O synchronous airway lesionAssess VC mobility if not assessed previously
29 What is Supraglottoplasty It is a surgery designed to treat LM that aims to trim the aryepiglottic folds and remove soft tissue, overriding the arytenoids
30 Surgical Steps of Supraglottoplasty 1Pharyngoepiglottic fold2ArytenoidsAE foldsExtent of AE fold dissection
33 Removal of redundant arytenoid mucosa Achieve hemostasis using Afrin pledgetsLaser precautionsCO2 laser to remove redundant soft tissue over both arytenoidsPreserve inter-arytenoid mucosa
34 How much supra-arytenoid mucosa is to be removed? Suction testPolonovski JM, Contencin P, Francois M, et al. Aryepiglottic fold excision for the treatment of severe laryngomalacia. Ann Otol Rhinol Laryngol 1990;99:625–7.Zalzal GH, Collins WO. Microdebrider-assisted supraglottoplasty Int J Pediatr Otorhinolaryngol Mar;69(3): Epub 2004 Dec 8.
37 Post-operative care Intubation versus immediate extubation Feeding may be started when infant is awakeOne or two doses of post-operative steroidsAggressive empiric reflux therapyFollow-up in 2-4 weeksMonitor airway symptoms, apneic spells and feeding adequacy
38 Complications after surgery 8%, relatively uncommonIncreases with multiple comorbiditesSite-specific complications include bleeding, infection, web formation, granulation tissueTechnical complications include supraglottic stenosis – difficult to treat, so best is prevention
39 EpiglottopexyIndicated if the primary level of obstruction is a retroflexed epiglottisCommonly seen in infants with global delay, hypotonia & neurological disordersTell parents that tracheostomy may be necessaryMain risks are aspiration, supraglottic stenosis
40 Epiglottopexy: Surgical technique Suspension of the patientMucosa of the epiglottis is denuded with CO2 laser (1-10W) under microscopic guidanceAdditionally the epiglottis can be secured to the tongue base with 4.0 vicrylWhymark AD, Clement WA, Kubba H, et al. Laser epiglottopexy for laryngomalacia:10 years’ experience in the west of Scotland. Arch Otolaryngol Head Neck Surg 2006;132:978–82.
41 Indications for tracheotomy Presence of > 3 comorbiditiesSevere sleep apneaWorsening symptoms after revision supraglottoplasty
42 Proposed algorithm for the treatment of mild and moderate laryngomalacia Moderate LMMild LM1m FU + FL2m FU + FLAcid suppression+Feeding modification3m FU + FLSymp worsen, persistSURGERYtill resolutionComplications
43 Proposed algorithm for treatment of severe LM Consider tracheotomySevere LMConsider PSGMaximum acid suppression and SGPSymptoms worsenSymptoms worsenRevision SGPFU 2-4 weeks post oppH study and Nissen’s fundocplicationFU as recommended for mild/moderate LM
44 So what did we learn?LM is the commonest congenital anomaly of the newborn larynx.80-90% of patients have a benign courseHigh pitched inspiratory stridor is the hallmark clinical presentationFeeding difficulties and GERD are seen in % of patients with LMHistory, PE and Flexible laryngoscopy aid diagnosis
45 Learning pearls contd… Identifying patients who will benefit most from surgery is of paramount importance“Less is More” when performing surgery on the infant larynxStrict FU and reflux therapy