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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
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OverviewOverview
Discuss PathogenesisDiscuss PathogenesisClinical presentationClinical presentationLaryngomalaciaLaryngomalacia
and GERDand GERD
DiagnosisDiagnosisMedical and surgical managementMedical and surgical managementParental counselingParental counseling
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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
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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 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
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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.
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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
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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
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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
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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
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.
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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
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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
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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
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Classification scheme based on Classification scheme based on symptoms, flexible symptoms, flexible laryngoscopylaryngoscopy
MILD SEVERE
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Classification scheme based on Classification scheme based on mechanism of LMmechanism of LM
ANTERIOR
POSTERIOR
LATERAL
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DiagnosisDiagnosis
HistoryHistory
Physical examinationPhysical examination
Flexible Flexible laryngoscopylaryngoscopy
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Direct Direct 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 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
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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.
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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.
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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 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
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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 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.
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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
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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
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Anesthetic considerationsAnesthetic considerations
SpontaneousSpontaneous breathing analgesiabreathing analgesia
ETT in the nasopharynx, mouthETT in the nasopharynx, mouth
Spray (1% Spray (1% lidocainelidocaine
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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
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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
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Surgical Steps of Surgical Steps of SupraglottoplastySupraglottoplasty
AE folds
Pharyngoepiglottic
fold
Arytenoids
Extent of AE fold dissection
1
2
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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 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
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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.
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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 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
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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
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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
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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.
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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
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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
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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
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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
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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
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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