Tracheomalacia

PaulMoore,M.D.
EddiePenn,M.D.
DavidBichell,M.D.
OtisRickman,M.D.

NormalTrachea

Definitions
Tracheomalacia:dynamiccollapse
ofthetracheaduringbreathing,
resultinginairwayobstruction
Tracheobronchomalacia isthe
moregeneraltermfordynamic
collapseoflargeorsmallairways

Etiology
Reductionand/oratrophyofthe
longitudinalelasticfibersofthe
parsmembranacea
Impairmentofcartilageintegrity
Faultydivisionoftheforegutinto
thetracheaandesophagusduring
embryonicdevelopmentresultsin
thetracheareceivingtoomuch
tissueinTEF/EAandpossibly
idiopathicprimaryTM

Histology
Reducedcartilageto
muscleratio,resulting
ineasercollapsibility
ofthetrachea
(normalratio4.5:1)
Variabilityinthe
longitudinalmuscle
fibersdeeptothe
transversefibersin
thelowertrachea

Physiology
Byvirtueofitscompliance,thenormalintrathoracic trachea
dilatessomewhatwithinspirationandnarrowswithexpiration,
asareflectionofthedifferencebetweenintrathoracic and
intraluminalpressures.
ThemajorityofcasesofTMareintrathoracic innaturesuch
thatexcessivenarrowingismostprominentwhenintrathoracic
pressureissubstantiallygreaterthanintraluminalpressure,as
itisduringforcedexpiration,cough,ortheValsalva maneuver.
Withextrathoracic TM,negativeintrapleural pressuresare
transmittedtotheextrathoracic tracheaduetopleural
reflectionssuchthattheupperairwaycollapsesduring
inspiration.

Classification:Congenitalor
PrimaryForms
Isolatedfindinginhealthyinfants
Prematurity:inadequatematurityoftracheobronchial
cartilage
Pulsatilecollapsewithnormalinominate artery
AssociationwithTEF(tracheosophageal fistula)
Disorderswithabnormalcartilaginousmatrixofthe
trachea(polychondritis,chondromalacia,
mucopolysaccharidoses)
Othergeneticsyndromesthatmayaltercollagen
maturationorairwaytone

Classification:Acquiredor
SecondaryForms
Protractedendotrachealintubation(withmore
significanteffectsinprematureinfants)
Tracheotomy
Severetracheobronchitis
Externaltrachealcompression

Vascular
Cardiac
Skeletal
Tumorsandcysts

Associatedconditions

Cardiovascularabnormalities(2058%)
Bronchopulmonary dysplasia(upto52%)
Gastroesophageal refluxdisease(~50%)
Subglotticstenosisandlayrngomalacia
Neurologicimpairment(848%)
Severedevelopmentaldelay(26%)

MAC(MajorAirwayCollapse)
ClassificationSystem
TypeI:intrinsicdefectinthecartilaginousportionofthe
trachealeadingtoanincreasedproportionof
membranoustrachea
TypeII:extrinsictrachealcompressionbycardiovascular
structures,tumors,lymphnodes,orothermasses
TypeIII:prolongedpositivepressureventilationoran
infectious/inflammatoryprocessthatunderminesthe
intrinsiccartilaginoussupportofthetrachea

Incidence
Congenitalform:est.1per1,445infants
Incidenceoftracheomalacia incongenital
diseasesparallelstheincidenceofthose
diseases
Incidenceonincreaseduetogreatersurvival
fromNICUandincrease#ofbronchoscopies
MortalityofsevereTMcanapproach80%

Clinicalfeatures
Expiratorystridorandcough,oftendescribed
asbarkingorbrassy
Coughduetothejuxtapositionoftheanterior
andposteriorwallsofthetrachea,resultingin
recurrentvibrationsandirritationoftheairway
whethersymptomspresentatbirthorafter
afewweeks
Inspiratorystridorsuggestiveofextrathoracic
compression

Additionalclinicalfeatures
Noisy,mediumpitchedtohighpitchedbreathing
Recurrentrespiratorydistress
Wheezing
Cyanosis
Spontaneoushyperextensionoftheneck
Breathholdingspells
Bagpipesign,anexpiratorysibilantnotethatpersistsafterthe
endofvisibleexpiration
Feedingdifficulties
Reflexapnea

ClinicalSeverityRating
Mild:Respiratorydifficultiesassociatedwith

infectiousprocessessuchascrouporbronchiolitis
Moderate:classicfindings,includingstridor,
wheezing,recurrentrespiratoryinfections,andeven
cyanosisassociatedwithexacerbations
Severe:stridorduringtidalbreathing,marked
sputumretention,upperairwayobstruction,reflex
apnea,andevencardiacarrest

Diagnosis:Imaging
Chestradiograph:62%sensitivity
StandardCTandMRI:notwellsuitedbecause
imagesareobtainedendexpiration
Multidetector CT:allowsendexpiratoryandend
inspiratoryimaging
FreebreathingcineCT:doesnotrequirepatient
cooperationwithexpiratorymaneuvers
CTAngiogramorAngioMRIcanbeusedtodiagnose
vascularanomalies

Tracheomalacia:Computedtomographicscanshowsanarrowsagittal
diameterandwidecoronaldiameterofthetrachea.
UpToDate,2013

4yearoldgirlwithrecurrentcough.PairedinspiratoryexpiratoryvolumetricMDCT
wasperformedforevaluationforpossibleunderlyingtracheomalacia.
A,AxialendinspiratoryCTimageobtainedwithlungwindowshowsnormalappearance
oftracheaandlungparenchyma.
B,AxialendexpiratoryCTimageobtainedwithlungwindowatsamelevelasAshows
excessivecollapseoftrachea(curvedarrow)consistentwithtracheomalacia.Areasof
geographicallymarginated radiolucency(straightarrows)inbothlungsareconsistent
withairtrapping.
Lee,AJR,2009

5yearoldgirlwithrecurrentcough.PairedinspiratoryexpiratoryvolumetricMDCTwasperformedfor
evaluationofpossibleunderlyingtracheomalacia.
A,AxialendinspiratoryCTimageobtainedwithlungwindowshowsnormalappearanceoftrachea.
B,AxialendexpiratoryCTimageobtainedwithlungwindowatsamelevelasAshowsflattening(arrow)
ofposteriormembranouswalloftrachea,whichisnormalfindingatendexpiration.Mildexpiratory
decreaseincaliberoftracheallumendoesnotmeetCTcriteriafortracheomalacia.Generalized
increasedattenuationoflungparenchymaispresentwithoutevidenceofairtrapping.Decreasedlung
volumeassociatedwithdecreasedanteroposterior dimensionofchestalsoisevident.Thesefindingsare
normalatendexpiration.

Lee,AJR,2009

Trachealshapesonfreebreathing cineCTaccordingtotheratioofthe
transversediametertotheanteroposterior diameterofthetrachea.
a Roundshapeshowsaratioofbetween0.8and1.2.
b Lunateshapeshowsaratioof1.2.
c Elongatedshapeshowsaratioof<0.8.
dCrescentic shapeshowsaratio2.0withposteriorconcavity GooPed Rad.2013

Management
Mostinfantsimproveby6to12monthsofageas
airwaycaliberincreasesandcartilagedevelops.
Indicationsforintervention:lifethreateningepisodes
ofairwayobstruction,recurrentinfection,respiratory
failure,orfailuretothrive.

PossibleInterventions

Splinting
Tracheotomy
Positivepressureventilatory support
Aortopexy
Stentplacement
Posteriortrachealwallsurgery