Laryng Slides 090331

8/8/2019 Laryng Slides 090331

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Current Concepts inCurrent Concepts inDiagnosis andDiagnosis and anagementanagement ofofLaryngomalaciaLaryngomalacia

ShraddhaShraddha Mukerji, MDMukerji, MDHarold Pine, MDHarold Pine, MDDepartment of OtolaryngologyDepartment of Otolaryngology

University of Texas Medical Branch, GalvestonUniversity of Texas Medical Branch, Galveston

March 31, 2009March 31, 2009


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What isWhat is laryngomalacialaryngomalacia??

LaryngomalaciaLaryngomalacia

(LM) is the commonest(LM) is the commonest

congenital laryngeal anomaly of thecongenital laryngeal anomaly of thenewborn characterized bynewborn characterized by flaccidflaccid

laryngeal tissue and inward collapse of thelaryngeal tissue and inward collapse of the

supraglotticsupraglottic structures leading tostructures leading to upperupper

airway obstructionairway obstruction

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


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EtiopathogenesisEtiopathogenesis

Cartilage immaturityCartilage immaturity

Anatomic abnormalityAnatomic abnormality

Neuromuscular immaturityNeuromuscular immaturity


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Cartilage immaturityCartilage immaturity

First proposed by Sutherland and Lack inFirst proposed by Sutherland and Lack in

the late 19the late 19thth centurycenturyDelayed development of theDelayed development of the

cartilageneouscartilageneous support of the larynxsupport of the larynxTheory has been disprovedTheory has been disproved No histological evidence ofNo histological evidence ofchondropathychondropathy

Incidence not different in premature infantsIncidence not different in premature infants


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Anatomic abnormalityAnatomic abnormality

LM is a result of the exaggeration of an infantileLM is a result of the exaggeration of an infantile

larynx (Iglauer1922)larynx (Iglauer1922)

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

stridorstridor is not seen in all infants withis not seen in all infants with omegaomegaepiglottisepiglottisCongenital laryngeal stridor (laryngomalacia): etiologic factors and associated disorders. Belmont JR, GrundfastK. Ann Otol Rhinol Laryngol. 1984 Sep-Oct;93(5 Pt 1):430-7.


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Neuromuscular immaturityNeuromuscular immaturity

There is a highThere is a high prevalanceprevalance

of neurologicof neurologic

disorders with LMdisorders with LM

Some believe that neuromuscularSome believe that neuromuscularimmaturity leads to laryngealimmaturity leads to laryngeal hypotoniahypotonia and LMand LMMay be one of the several components ofMay be one of the several components ofLMLM


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LaryngomalaciaLaryngomalacia and GERDand GERD8080--100% of infants with LM have GERD100% of infants with LM have GERD

It is not clear whether GERD is a cause orIt is not clear whether GERD is a cause oran effect of LMan effect of LM

EMPIRIC REFLUX THERAPYEMPIRIC REFLUX THERAPY choking,choking,

frequent emesis,frequent emesis,

regurgitationregurgitation

or feeding difficultyor feeding difficulty


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Proposed pathogenesis of GERD in LMProposed pathogenesis of GERD in LM

Respiration against afixed obstruction

Large ve

intrathoracic

pressure

Reflux into esophagus and

LPR

Laryngeal edema

Increased

prolapse

Increased

obstruction


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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


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Conditions that worsen LMConditions that worsen LM

PrematurityPrematurity

Neuromuscular disorders: higherNeuromuscular disorders: higherincidence, increased severityincidence, increased severity

Synchronous airway lesionSynchronous airway lesion 20% incidence20% incidence

TracheomalaciaTracheomalacia,, sublglotticsublglottic stenosis,stenosis,

bronchomalaciabronchomalacia,, pharyngomalaciapharyngomalacia,, vallecularvallecularcystcyst Potentiates GERDPotentiates GERD

Surgical failuresSurgical failures

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


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Clinical presentationClinical presentation

StridorStridor 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, feedingor in the supine positionor in the supine position

Feeding symptomsFeeding symptoms Choking, coughing, prolonged feeding time, recurrent emesis,Choking, coughing, prolonged feeding time, recurrent emesis,

dysphagiadysphagia, weight loss, weight loss

GERD symptomsGERD symptoms

ComplicationsComplications


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Complications of LMComplications of LM

1010--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 delayPulmonary hypertension, developmental delayand cardiac failureand cardiac failure


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Classification schemesClassification schemes

Based onBased on symptomatologysymptomatology/flexible/flexible

laryngoscopylaryngoscopy MildMild

ModerateModerate

SevereSevere

Based on mechanism of collapseBased on mechanism of collapse

Anterior: epiglottisAnterior: epiglottis Posterior: large arytenoidsPosterior: large arytenoids

Laterally: AE foldsLaterally: AE folds


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Classification scheme based onClassification scheme based on

symptoms, flexiblesymptoms, flexible laryngoscopylaryngoscopy

MILD SEVERE


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Classification scheme based onClassification scheme based on

mechanism of LMmechanism of LM

ANTERIOR

POSTERIOR

LATERAL


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DiagnosisDiagnosis

HistoryHistory

Physical examinationPhysical examination

FlexibleFlexible laryngoscopylaryngoscopy


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DirectDirect laryngoscopylaryngoscopy videovideo

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


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Complementary studiesComplementary studies

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

aspirationaspiration

EsophagramEsophagram Extent and degree of refluxExtent and degree of reflux

r/or/o concomitant GI disorderconcomitant GI disorderpH study ifpH study ifNissenNissenss surgery is necessarysurgery is necessarySleep Study to document severity ofSleep Study to document severity ofapnea in severe LM and in surgicalapnea in severe LM and in surgical

failuresfailures


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ManagementManagement

MedicalMedical

Empiric reflux acid suppressionEmpiric reflux acid suppressionFeeding modificationsFeeding modifications

Posture repositioningPosture repositioningSurgicalSurgical

SupraglottoplastySupraglottoplasty

EpiglottopexyEpiglottopexy

TracheostomyTracheostomy

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 JPediatr Otorhinolaryngol 1998;43:1120.


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Empiric reflux acid suppressionEmpiric reflux acid suppression

8080--100% of patients with LM have GERD100% of patients with LM have GERD

H2 receptor antagonist (RA) or ProtonH2 receptor antagonist (RA) or Protonpump inhibitor (PPI)pump inhibitor (PPI)

H2RA: ranitidine 3mg/kg three times dailyH2RA: ranitidine 3mg/kg three times dailyPPI: 1mg/kg dailyPPI: 1mg/kg daily

If symptoms worsenIf symptoms worsen6mg/kg of ranitidine6mg/kg of ranitidineat 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 newtheory of etiology. Laryngoscope 2007;117:133.


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Feeding modificationsFeeding modifications

PacingPacing

Thickening formula feedsThickening formula feeds

Upright feeding positionUpright feeding position

Small, frequent feedsSmall, frequent feeds


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Evolving concepts in surgicalEvolving 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


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Indications for surgeryIndications for surgeryAbsolute indicationsAbsolute indications Relative indicationsRelative indications

CorCor

pulmonalepulmonale AspirationAspiration

Pulmonary hypertensionPulmonary hypertension DifficultDifficult--toto--feed child who hasfeed child who has

failed medical interventionfailed medical interventionHypoxiaHypoxia Weight loss with feeding difficultyWeight loss with feeding difficulty

ApneaApnea

Recurrent cyanosisRecurrent cyanosis

Failure to thriveFailure to thrive

PectusPectus

excavatumexcavatum

StridorStridor

with respiratorywith respiratory

compromisecompromise

StridorStridor

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.


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ContraindicationsContraindications

Relatively uncommonRelatively uncommon

Proceed with cautionProceed with caution Patients with comorbiditiesPatients with comorbidities

Patients with multiple levels of airwayPatients with multiple levels of airwayobstructionobstruction

Postpone surgery till resolution of URIPostpone surgery till resolution of URI

WEIGHT AND AGE ARE NOT CI TOWEIGHT AND AGE ARE NOT CI TO

SURGERYSURGERY


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PrePre--operative counselingoperative counseling

Overnight hospitalization in the ICUOvernight hospitalization in the ICU

TheThe stridorstridor will improve, butwill improve, but NOT DISAPPEARNOT DISAPPEARExpect feeding improvementExpect feeding improvement

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

Risk of revision surgeryRisk of revision surgery


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Anesthetic considerationsAnesthetic considerations

SpontaneousSpontaneousbreathing analgesiabreathing analgesia

ETT in the nasopharynx, mouthETT in the nasopharynx, mouth

Spray (1%Spray (1% lidocainelidocaine


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Surgical SetSurgical Set--upup

RigidRigid bronchoscopybronchoscopy

VisualizeVisualize subglottissubglottis,,trachea and bronchitrachea and bronchi

R/O synchronousR/O synchronous

airway lesionairway lesionAssess VC mobility ifAssess VC mobility if

not assessednot assessed

previouslypreviously


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Surgical Steps ofSurgical Steps ofSupraglottoplastySupraglottoplasty

AE folds

Pharyngoepiglottic

fold

Arytenoids

Extent of AE fold dissection

1

2

AE fold trimming with forceps and scissors


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AE fold trimming with forceps and scissors


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Surgical steps,Surgical steps, contdcontd

Pre-op Post-op


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Removal of redundantRemoval of redundant arytenoidarytenoid mucosamucosa

AchieveAchieve hemostasishemostasis

using Afrinusing Afrin pledgetspledgets

Laser precautionsLaser precautions

CO2 laser to remove

redundant soft tissue over

both arytenoids

Preserve inter-arytenoid

mucosa


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How much supraHow much supra--arytenoidarytenoid mucosamucosais 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 severelaryngomalacia. Ann Otol Rhinol Laryngol 1990;99:6257.

Zalzal GH, Collins WO. Microdebrider-assisted supraglottoplastyInt

J Pediatr

Otorhinolaryngol.

2005

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

2004 Dec 8.


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Pre and Post op resultsPre and Post op results

Pre-op

Post-op


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InstrumentationInstrumentation

Microdebrider

CO2 laserCold instruments


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PostPost--operative careoperative care

Intubation versus immediateIntubation versus immediate extubationextubation

Feeding 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 steroids

Aggressive empiric reflux therapyAggressive empiric reflux therapy

FollowFollow--up in 2up in 2--4 weeks4 weeks

Monitor airway symptoms,Monitor airway symptoms, apneicapneic

spells andspells and

feeding adequacyfeeding adequacy


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Complications after surgeryComplications after surgery

8%, relatively uncommon8%, relatively uncommon

Increases with multiple comorbiditesIncreases with multiple comorbiditesSiteSite--specific complications includespecific complications include

bleeding, infection, web formation,bleeding, infection, web formation,granulation tissuegranulation tissue

Technical complications includeTechnical complications include

supraglotticsupraglottic stenosisstenosis difficult to treat, sodifficult to treat, sobest is preventionbest is prevention


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EpiglottopexyEpiglottopexy

Indicated if the primary level of obstructionIndicated if the primary level of obstruction

is a retroflexed epiglottisis a retroflexed epiglottisCommonly seen in infants with globalCommonly seen in infants with global

delay,delay, hypotoniahypotonia & neurological disorders& neurological disordersTell parents thatTell parents that tracheostomytracheostomy may bemay benecessarynecessaryMain risks are aspiration,Main risks are aspiration, supraglotticsupraglottic stenosisstenosis


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EpiglottopexyEpiglottopexy: Surgical technique: Surgical technique

Suspension of the patientSuspension of the patient

Mucosa of the epiglottis is denuded withMucosa of the epiglottis is denuded withCO2 laser (1CO2 laser (1--10W) under microscopic10W) under microscopic

guidanceguidanceAdditionally the epiglottis can be securedAdditionally the epiglottis can be secured

to the tongue base with 4.0to 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 Surg2006;132:97882.


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Indications for tracheotomyIndications for tracheotomy

Presence of > 3 comorbiditiesPresence of > 3 comorbidities

Severe sleep apneaSevere sleep apnea

Worsening symptoms after revisionWorsening symptoms after revision

supraglottoplastysupraglottoplasty


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Proposed algorithm for the treatment of mildProposed algorithm for the treatment of mild

and moderateand moderate laryngomalacialaryngomalacia

Mild LM Moderate LM

Acid suppression

FU @3m till resolution

3m FU + FL

2m FU + FL

1m FU + FL

+

Symp worsen, persist

Complications

SURGERY

Feeding modification


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Proposed algorithm for treatment ofProposed algorithm for treatment of

severe LMsevere LMSevere LM

Maximum acidsuppression and SGP

FU 2-4 weeks post op

FU as recommended formild/moderate LM

Symptoms

worsen

Revision SGPSymptoms

worsen

pH study andNissensfundocplication

ConsiderPSG

Considertracheotomy


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So what did we learn?So what did we learn?

LM is the commonest congenital anomaly of theLM is the commonest congenital anomaly of thenewborn larynx.newborn larynx.

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

High pitchedHigh pitched inspiratoryinspiratory stridorstridor is the hallmark clinicalis the hallmark clinicalpresentationpresentationFeeding difficulties and GERD are seen in 80Feeding difficulties and GERD are seen in 80--100% of100% of

patients with LMpatients with LM

History, PE and FlexibleHistory, PE and Flexible laryngoscopylaryngoscopy

aid diagnosisaid diagnosis


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Learning pearlsLearning pearls contdcontd

Identifying patients who will benefit mostIdentifying patients who will benefit most

from surgery is of paramount importancefrom surgery is of paramount importance

Less is MoreLess is More when performing surgerywhen performing surgeryon the infant larynxon the infant larynx

Strict FU and reflux therapyStrict FU and reflux therapy


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Laryng Slides 090331