Current Concepts in Current Concepts in Diagnosis and Diagnosis and anagementanagement

of of LaryngomalaciaLaryngomalacia

ShraddhaShraddha

Mukerji, MDMukerji, MDHarold Pine, MDHarold Pine, MD

Department of OtolaryngologyDepartment of OtolaryngologyUniversity of Texas Medical Branch, GalvestonUniversity of Texas Medical Branch, Galveston

March 31, 2009March 31, 2009

OverviewOverview

Discuss PathogenesisDiscuss PathogenesisClinical presentationClinical presentationLaryngomalaciaLaryngomalacia

and GERDand GERD

DiagnosisDiagnosisMedical and surgical managementMedical and surgical managementParental counselingParental counseling

What is What is laryngomalacialaryngomalacia??

LaryngomalaciaLaryngomalacia

(LM) is the commonest (LM) is the commonest congenital laryngeal anomaly of the congenital laryngeal anomaly of the newborn characterized by newborn characterized by flaccidflaccid laryngeal tissue and inward collapse of the laryngeal tissue and inward collapse of the supraglotticsupraglottic structures leading to structures leading to upper upper airway obstructionairway obstruction

Jackson C, Jackson C. Diseases and injuries of the larynx. New York: MacMillan; 1942. p.639

EtiopathogenesisEtiopathogenesis

Cartilage immaturityCartilage immaturity

Anatomic abnormalityAnatomic abnormality

Neuromuscular immaturityNeuromuscular immaturity

Cartilage immaturityCartilage immaturity

First proposed by Sutherland and Lack in First proposed by Sutherland and Lack in the late 19the late 19thth

centurycentury

Delayed development of the Delayed development of the cartilageneouscartilageneous

support of the larynxsupport of the larynx

Theory has been disprovedTheory has been disprovedNo histological evidence of No histological evidence of chondropathychondropathy

Incidence not different in premature infantsIncidence not different in premature infants

Anatomic abnormalityAnatomic abnormalityLM is a result of the exaggeration of an infantile LM is a result of the exaggeration of an infantile larynx (Iglauer1922)larynx (Iglauer1922)

May or may not be an important factor since May or may not be an important factor since stridorstridor

is not seen in all infants with is not seen in all infants with omega omega

epiglottisepiglottis

Congenital laryngeal stridor (laryngomalacia): etiologic factors and associated disorders. Belmont JR, Grundfast K. Ann Otol Rhinol Laryngol. 1984 Sep-Oct;93(5 Pt 1):430-7.

Neuromuscular immaturityNeuromuscular immaturity

There is a high There is a high prevalanceprevalance

of neurologic of neurologic disorders with LMdisorders with LM

Some believe that neuromuscular Some believe that neuromuscular immaturity leads to laryngeal immaturity leads to laryngeal hypotoniahypotonia

and LMand LM

May be one of the several components of May be one of the several components of LMLM

LaryngomalaciaLaryngomalacia

and GERDand GERD

8080--100% of infants with LM have GERD100% of infants with LM have GERDIt is not clear whether GERD is a cause or It is not clear whether GERD is a cause or an effect of LMan effect of LMEMPIRIC REFLUX THERAPYEMPIRIC REFLUX THERAPY

choking,choking,frequent emesis,frequent emesis,regurgitationregurgitationor feeding difficultyor feeding difficulty

Proposed pathogenesis of GERD in LMProposed pathogenesis of GERD in LM

Respiration against a fixed obstruction

Large ve

intrathoracicpressure

Reflux into esophagus andLPR

Laryngeal edema

Increased

prolapse

Increased

obstruction

Proposed pathogenesis of GERD in LMProposed pathogenesis of GERD in LM

Disruption of effective vagal

tone to LES

Relative decreased LES

GERD

This pathogenesis leads credence to NEUROLOGICAL IMMATURITY

theory of LM

Conditions that worsen LMConditions that worsen LM

PrematurityPrematurityNeuromuscular disorders: higher Neuromuscular disorders: higher incidence, increased severityincidence, increased severitySynchronous airway lesionSynchronous airway lesion

20% incidence20% incidenceTracheomalaciaTracheomalacia, , sublglotticsublglottic stenosis,stenosis,bronchomalaciabronchomalacia, , pharyngomalaciapharyngomalacia, , vallecularvallecular cystcystPotentiates GERDPotentiates GERDSurgical failuresSurgical failures

Toynton SC, SaundersMW, Bailey CM. Aryepiglottoplasty for laryngomalacia: 100 consecutive cases. J Laryngol Otol 2001;115:358.

Clinical presentationClinical presentationStridorStridor

is the hallmark of congenital LMis the hallmark of congenital LM

High pitched, High pitched, inspiratoryinspiratory, worsens with agitation, crying, feeding , worsens with agitation, crying, feeding or in the supine positionor in the supine position

Feeding symptomsFeeding symptomsChoking, coughing, prolonged feeding time, recurrent emesis, Choking, coughing, prolonged feeding time, recurrent emesis, dysphagiadysphagia, weight loss, weight loss

GERD symptomsGERD symptoms

ComplicationsComplications

Complications of LMComplications of LM1010--20% of patients present with complications20% of patients present with complications

Life threatening airway obstructionLife threatening airway obstruction

Failure to thriveFailure to thrive

CyanosisCyanosis

Sleep apneaSleep apnea

Pulmonary hypertension, developmental delay Pulmonary hypertension, developmental delay and cardiac failureand cardiac failure

Classification schemesClassification schemes

Based on Based on symptomatologysymptomatology/flexible /flexible laryngoscopylaryngoscopy

MildMildModerateModerateSevereSevere

Based on mechanism of collapseBased on mechanism of collapseAnterior: epiglottisAnterior: epiglottisPosterior: large arytenoidsPosterior: large arytenoidsLaterally: AE foldsLaterally: AE folds

Classification scheme based on Classification scheme based on symptoms, flexible symptoms, flexible laryngoscopylaryngoscopy

MILD SEVERE

Classification scheme based on Classification scheme based on mechanism of LMmechanism of LM

ANTERIOR

POSTERIOR

LATERAL

DiagnosisDiagnosis

HistoryHistory

Physical examinationPhysical examination

Flexible Flexible laryngoscopylaryngoscopy

Direct Direct laryngoscopylaryngoscopy

videovideo

You may have to click or double-click to see the movie

Complementary studiesComplementary studies

Chest XChest X--ray to ray to r/or/o

aspirationaspirationEsophagramEsophagram

Extent and degree of refluxExtent and degree of refluxr/or/o concomitant GI disorderconcomitant GI disorder

pH study if pH study if NissenNissenss

surgery is necessarysurgery is necessarySleep Study to document severity of Sleep Study to document severity of apnea in severe LM and in surgical apnea in severe LM and in surgical failuresfailures

ManagementManagement

Medical Medical Empiric reflux acid suppressionEmpiric reflux acid suppressionFeeding modificationsFeeding modificationsPosture repositioningPosture repositioning

SurgicalSurgicalSupraglottoplastySupraglottoplastyEpiglottopexyEpiglottopexyTracheostomyTracheostomy

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.

Empiric reflux acid suppressionEmpiric reflux acid suppression

8080--100% of patients with LM have GERD100% of patients with LM have GERDH2 receptor antagonist (RA) or Proton H2 receptor antagonist (RA) or Proton pump inhibitor (PPI)pump inhibitor (PPI)H2RA: ranitidine 3mg/kg three times dailyH2RA: ranitidine 3mg/kg three times dailyPPI: 1mg/kg dailyPPI: 1mg/kg dailyIf symptoms worsenIf symptoms worsen 6mg/kg of ranitidine 6mg/kg of ranitidine at night + 1mg/kg of PPI dailyat night + 1mg/kg of PPI daily

Thompson DM. Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia: a new theory of etiology. Laryngoscope 2007;117:133.

Feeding modificationsFeeding modifications

PacingPacing

Thickening formula feedsThickening formula feeds

Upright feeding positionUpright feeding position

Small, frequent feedsSmall, frequent feeds

Evolving concepts in surgical Evolving concepts in surgical management of LMmanagement of LM

1920

1980

Current

Variot

was the first to suggest removal of excess of AE tissue as treatment of LM

Re-introduction of concept of removal of SG tissue for treatment of

LM

Sporadic reports of endoscopic trimming, partial epiglottopexy, wedge resection but no definite technique

Endoscopic techniques revisited and defined

Endoscopic supraglottoplasty

Epiglottopexy

Indications for surgeryIndications for surgeryAbsolute indicationsAbsolute indications Relative indicationsRelative indications

CorCor

pulmonalepulmonale AspirationAspiration

Pulmonary hypertensionPulmonary hypertension DifficultDifficult--toto--feed child who has feed child who has failed medical interventionfailed medical intervention

HypoxiaHypoxia Weight loss with feeding difficultyWeight loss with feeding difficulty

ApneaApnea

Recurrent cyanosisRecurrent cyanosis

Failure to thriveFailure to thrive

PectusPectus

excavatumexcavatum

StridorStridor

with respiratory with respiratory compromisecompromiseStridorStridor

with significant retractionswith significant retractions

Richter GT, Thompson DM. The surgical management of laryngomalacia. Otolaryngol Clin North Am. 2008 Oct;41(5):837-64, vii.

ContraindicationsContraindications

Relatively uncommonRelatively uncommonProceed with cautionProceed with caution

Patients with comorbiditiesPatients with comorbiditiesPatients with multiple levels of airway Patients with multiple levels of airway obstructionobstruction

Postpone surgery till resolution of URIPostpone surgery till resolution of URIWEIGHT AND AGE ARE NOT CI TO WEIGHT AND AGE ARE NOT CI TO SURGERYSURGERY

PrePre--operative counselingoperative counselingOvernight hospitalization in the ICUOvernight hospitalization in the ICU

The The stridorstridor

will improve, but will improve, but NOT DISAPPEARNOT DISAPPEAR

Expect feeding improvementExpect feeding improvement

Reflux precautions and medications to be continuedReflux precautions and medications to be continued

Risk of revision surgeryRisk of revision surgery

Anesthetic considerationsAnesthetic considerations

SpontaneousSpontaneous breathing analgesiabreathing analgesia

ETT in the nasopharynx, mouthETT in the nasopharynx, mouth

Spray (1% Spray (1% lidocainelidocaine

Surgical SetSurgical Set--upup

Rigid Rigid bronchoscopybronchoscopyVisualize Visualize subglottissubglottis, , trachea and bronchitrachea and bronchiR/O synchronous R/O synchronous airway lesionairway lesion

Assess VC mobility if Assess VC mobility if not assessed not assessed previouslypreviously

What is What is SupraglottoplastySupraglottoplasty

It is a surgery designed to treat LM that It is a surgery designed to treat LM that aims to aims to trim the trim the aryepiglotticaryepiglottic foldsfolds and and remove soft tissueremove soft tissue, overriding the , overriding the arytenoidsarytenoids

Surgical Steps of Surgical Steps of SupraglottoplastySupraglottoplasty

AE folds

Pharyngoepiglottic

fold

Arytenoids

Extent of AE fold dissection

1

2

AE fold trimming with forceps and scissors

Surgical steps, Surgical steps, contdcontd

Pre-op Post-op

Removal of redundant Removal of redundant arytenoidarytenoid mucosamucosa

Achieve Achieve hemostasishemostasis

using Afrin using Afrin pledgetspledgetsLaser precautionsLaser precautions

CO2 laser to remove redundant soft tissue over both arytenoids

Preserve inter-arytenoid

mucosa

How much supraHow much supra--arytenoidarytenoid

mucosa mucosa is to be removed?is to be removed?

Suction testSuction test

Polonovski JM, Contencin P, Francois M, et al. Aryepiglottic fold excision for the treatment of severe laryngomalacia. Ann Otol Rhinol Laryngol 1990;99:6257.

Zalzal GH, Collins WO. Microdebrider-assisted supraglottoplasty Int

J Pediatr

Otorhinolaryngol.

2005 Mar;69(3):305-9. Epub

2004 Dec 8.

Pre and Post op resultsPre and Post op results

Pre-op

Post-op

InstrumentationInstrumentation

Microdebrider

CO2 laserCold instruments

PostPost--operative careoperative care

Intubation versus immediate Intubation versus immediate extubationextubationFeeding may be started when infant is awakeFeeding may be started when infant is awakeOne or two doses of postOne or two doses of post--operative steroidsoperative steroidsAggressive empiric reflux therapyAggressive empiric reflux therapyFollowFollow--up in 2up in 2--4 weeks4 weeksMonitor airway symptoms, Monitor airway symptoms, apneicapneic

spells and spells and

feeding adequacyfeeding adequacy

Complications after surgeryComplications after surgery

8%, relatively uncommon8%, relatively uncommonIncreases with multiple comorbiditesIncreases with multiple comorbiditesSiteSite--specific complications include specific complications include bleeding, infection, web formation, bleeding, infection, web formation, granulation tissuegranulation tissueTechnical complications include Technical complications include supraglotticsupraglottic

stenosis stenosis

difficult to treat, so difficult to treat, so

best is preventionbest is prevention

EpiglottopexyEpiglottopexy

Indicated if the primary level of obstruction Indicated if the primary level of obstruction is a retroflexed epiglottisis a retroflexed epiglottisCommonly seen in infants with global Commonly seen in infants with global delay, delay, hypotoniahypotonia

& neurological disorders& neurological disorders

Tell parents that Tell parents that tracheostomytracheostomy

may be may be necessarynecessaryMain risks are aspiration, Main risks are aspiration, supraglotticsupraglottic

stenosisstenosis

EpiglottopexyEpiglottopexy: Surgical technique: Surgical technique

Suspension of the patientSuspension of the patientMucosa of the epiglottis is denuded with Mucosa of the epiglottis is denuded with CO2 laser (1CO2 laser (1--10W) under microscopic 10W) under microscopic guidanceguidanceAdditionally the epiglottis can be secured Additionally the epiglottis can be secured to the tongue base with 4.0 to the tongue base with 4.0 vicrylvicryl

Whymark 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;132:97882.

Indications for tracheotomyIndications for tracheotomy

Presence of > 3 comorbiditiesPresence of > 3 comorbidities

Severe sleep apneaSevere sleep apnea

Worsening symptoms after revision Worsening symptoms after revision supraglottoplastysupraglottoplasty

Proposed algorithm for the treatment of mild Proposed algorithm for the treatment of mild and moderate and moderate laryngomalacialaryngomalacia

Mild LM Moderate LM

Acid suppression

FU @3m till resolution

3m FU + FL

2m FU + FL

1m FU + FL

+

Symp worsen, persist

ComplicationsSURGERY

Feeding modification

Proposed algorithm for treatment of Proposed algorithm for treatment of severe LMsevere LM

Severe LM

Maximum acid suppression and SGP

FU 2-4 weeks post op

FU as recommended for mild/moderate LM

Symptoms worsen

Revision SGP Symptoms worsen

pH study and Nissens fundocplication

Consider PSG

Consider tracheotomy

So what did we learn?So what did we learn?LM is the commonest congenital anomaly of the LM is the commonest congenital anomaly of the newborn larynx.newborn larynx.

8080--90% of patients have a benign course90% of patients have a benign course

High pitched High pitched inspiratoryinspiratory

stridorstridor

is the hallmark clinical is the hallmark clinical presentationpresentation

Feeding difficulties and GERD are seen in 80Feeding difficulties and GERD are seen in 80--100% of 100% of patients with LMpatients with LM

History, PE and Flexible History, PE and Flexible laryngoscopylaryngoscopy

aid diagnosisaid diagnosis

Learning pearls Learning pearls contdcontd

Identifying patients who will benefit most Identifying patients who will benefit most from surgery is of paramount importancefrom surgery is of paramount importance

Less is MoreLess is More when performing surgery when performing surgery on the infant larynxon the infant larynx

Strict FU and reflux therapyStrict FU and reflux therapy

Current Concepts in Diagnosis and anagement of LaryngomalaciaOverviewWhat is laryngomalacia?EtiopathogenesisCartilage immaturityAnatomic abnormalityNeuromuscular immaturityLaryngomalacia and GERDProposed pathogenesis of GERD in LMProposed pathogenesis of GERD in LMConditions that worsen LMClinical presentationComplications of LMClassification schemesClassification scheme based on symptoms, flexible laryngoscopyClassification scheme based on mechanism of LMDiagnosisDirect laryngoscopy videoComplementary studiesManagementEmpiric reflux acid suppressionFeeding modificationsEvolving concepts in surgical management of LMIndications for surgeryContraindicationsPre-operative counselingAnesthetic considerationsSurgical Set-upWhat is SupraglottoplastySurgical Steps of SupraglottoplastySlide Number 31Surgical steps, contdRemoval of redundant arytenoid mucosaHow much supra-arytenoid mucosa is to be removed?Pre and Post op resultsInstrumentationPost-operative careComplications after surgeryEpiglottopexyEpiglottopexy: Surgical techniqueIndications for tracheotomyProposed algorithm for the treatment of mild and moderate laryngomalaciaProposed algorithm for treatment of severe LMSo what did we learn?Learning pearls contdSlide Number 46