Brazilian
Journal
of
OTORHINOLARYNGOLOGY
www.bjorl.org
REVIEW
ARTICLE
Congenital
laryngeal
anomalies
夽
,
夽夽
Michael
J.
Rutter
DivisionofPediatricOtolaryngology,HeadandNeckSurgery,CincinnatiChildren’sHospitalMedicalCenter,Cincinnati,USA
Received30April2014;accepted24May2014 Availableonline21August2014
KEYWORDS
Congenitallaryngeal anomalies;
Difficultintubation; Laryngomalacia; Subglotticstenosis; Vocalfoldparalysis
Abstract
Introduction:Itisessentialforclinicianstounderstandissuesrelevanttotheairway manage-mentofinfantsandtobecognizantofthefactthatinfantswithcongenitallaryngealanomalies areatparticularriskforanunstableairway.
Objectives: Tofamiliarizeclinicianswithissuesrelevanttotheairwaymanagementofinfants andtopresentasuccinctdescriptionofthediagnosisandmanagementofanarrayofcongenital laryngealanomalies.
Methods:Revisionarticle,inwhichthemainaspectsconcerningairwaymanagementofinfants willbeanalyzed.
Conclusions: Itiscriticalforclinicianstounderstandissuesrelevanttotheairwaymanagement ofinfants.
© 2014Associac¸ãoBrasileira de Otorrinolaringologiae CirurgiaCérvico-Facial. Publishedby ElsevierEditoraLtda.Allrightsreserved.
PALAVRAS-CHAVE
Anomaliascongênitas dalaringe;
Intubac¸ãodifícil; Laringomalacia; Estenosesubglótica; Paralisiadascordas vocais
Anomaliascongênitasdalaringe
Resumo
Introduc¸ão: Asanomaliascongênitasdalaringeestãoemriscodeumaviaaéreainstável,sendo essencialqueoclínicotenhaumaboacompreensãodosproblemasrelevantesparaomanuseio dasviasaéreasdebebês.
Objetivos: Familiarizarosclínicoscomosproblemasrelevantesparaomanuseiodasviasaéreas de bebêseapresentar umadescric¸ão sucintadodiagnósticoe tratamentodeumasériede anomaliascongênitasdalaringe.
Método: Artigoderevisão,noqualserãotratadososprincipaisaspectosrelacionadosao manu-seiodasviasaéreasdebebês.
夽
Pleasecitethisarticleas:RutterMJ.Congenitallaryngealanomalies.BrazJOtorhinolaryngol.2014;80:533---9.
夽夽
Institution:CincinnatiChildren’sHospitalMedicalCenter,Cincinnati,OH,UnitedStates. E-mail:mike.rutter@cchmc.org
http://dx.doi.org/10.1016/j.bjorl.2014.08.001
Conclusões:Éessencialqueoclínicotenhaumbomconhecimentodosproblemasrelevantes aomanuseiodasviasaéreasdebebês.
©2014Associac¸ãoBrasileira deOtorrinolaringologiaeCirurgiaCérvico-Facial.Publicadopor ElsevierEditoraLtda.Todososdireitosreservados.
Overview
Giventhatinfantswithcongenitallaryngealanomalies are atriskforanunstableairway,itiscriticalforcliniciansto understand issues relevant to the airway management of infants.Withthatinmind,thisreviewprovidesanoverview oftheseissuesandsubsequentlypresentsabriefdiscussion ofthediagnosisandmanagementofaspectrumof congen-itallaryngealanomalies.
General
considerations
Preventionofcomplications
Althoughprolongedintubationmaybetoleratedforweeks orevenmonthsinneonates,thistolerance decreaseswith age.Thelongertheinfantisintubated,thehigherthe rela-tiveriskofdevelopingsubglotticstenosis(SGS)orposterior glotticstenosis(PGS).Acombinationoffactorsmayincrease therisk oflaryngealinjurysecondary tointubation.These factorsinclude (1) thecomposition and sizeof the endo-tracheal tube, (2) the duration of intubation, (3) patient agitation while intubated, and (4) problems that predis-pose patients to mucosal damage (e.g., extra-esophageal refluxandairwayburns).Ofthesefactors,themost criti-calis thesize of the endotracheal tube. To minimizethe likelihoodofpost-intubationinjury,endotrachealtubesize shouldbebasedonthe ventilatoryneedsof each individ-ualchildratherthantheageofthechild.Morespecifically, thetube shouldbelargeenough toallowadequate venti-lationbutsmallenoughtopermitaleakofairthroughthe subglottisatasubglotticpressureof<20cmH2O.Although
theaverage termnewbornshouldaccommodatea3.5-mm endotrachealtube,anewbornwithmild,asymptomatic con-genitalSGS may require a 2.5-mm endotracheal tube. In someinfants,thesizeoftheendotrachealtubeidealforthe larynxmaynotallow adequate pulmonary ventilationand toilet.Inthesechildren,alargerendotrachealtubewithout aleakmaybetoleratedforaperiodoftime,astheactual riskofdevelopingSGSisstilllow.
Asimilarproblemmayoccurwithpoorpulmonary compli-ance,wherebyaleakpressureof<20cmH2Omaynotallow
adequateventilation.Onepossible solutionistheuseofa low-pressurecuffedendotrachealtubethatpermits higher-pressureventilationwhilestillminimizinglaryngealtrauma. Forsome infants, the risks associatedwith tracheal intu-bation may be circumvented by the use of alternatives suchascontinuouspositiveairwaypressure(CPAP),bilevel
positiveairwaypressure(BiPAP),highflownasalcannula,or tracheotomy.
Difficultintubation
Infants and children with anatomic anomalies of the mandibleareparticularlydifficulttointubate.Inaddition, children with microsomia, temporomandibular fixation, macroglossia,andmaxillofacialtraumapresentachallenge totheclinician.Childrenwithretrognathia,especiallythose withPierre---Robinsequence,maybeextremelydifficultto intubateirrespectiveofthedegreeofretrognathiaorairway obstruction.
Standardintubationtechniquesalsomaybechallenging inchildreninwhomneckextensionshouldbeavoided,such asthechildwithanunstablecervicalspine.Theriskmaybe known,asinachildwithDownsyndrome,orunknown,asin anunconsciouschildwithaheadinjuryandpossiblecervical injury.Ifintubationiselective,flexion---extensionplainfilms of thecervical spine ofchildren at riskfor cervical insta-bility are useful. Although children withDown syndrome, mucopolysaccharidestorage disorders,andmajor chromo-somalanomaliesareathigherriskofcomplications,inmost, intubation without neck extensioncan stillbe safely per-formed.
Itis usuallypossible tointubatein a standard fashion, with the largest possible anesthetic laryngoscope blade, a styletted endotracheal tube (with the tip of the stylet angledanteriorly30---45◦ inaretrognathicchild),andwith
laryngeal pressure applied.Ifthis is unsuccessful, intuba-tion maybe performed with flexiblefiberoptic transnasal endoscopy,rigidventilatingbronchoscopy,anendotracheal tubethreadedoveraHopkinsrodtelescope,oralaryngeal maskairway.Eveninchildrenwhoaredifficulttointubate, abagandmaskwithanoralairwaymaybesufficientuntil amoredefinitivesolutioncanbeattained.Forchildren in whomintubationisparticularlydifficult,eitherelectiveor emergent tracheotomyis generally required. When possi-ble,itisdesirabletoplaceatracheotomywiththeairway alreadysecuredwithanendotrachealtube.
Thechildwithatracheotomy
maturing the stoma (the skin is sewn directly tothe tra-chealcartilage)andplacingstaysutures.Theseprecautions aretakensothatifthetracheotomyisdisplaced,traction onthe stay sutures will open the tracheotomyandaid in replacementofthetube.Flexiblebronchoscopyatthetime oftracheotomyplacementpermitsassessmentofthetube position,assuring that it is nottooclose tothecarina or downabronchus.
Respiratorysymptomsmayoccurfromobstructionwithin thetubeordistaltothetube.Regularsuctioningtothetipof thetube,butnotbeyond,usuallypreventstubeobstruction. Suctioningistheinitialinterventionforsuspected obstruc-tion.Ifdifficultiespersist,thetube shouldbereplaced. If symptoms continue, obstruction distal to thetube should besuspected.Ideally, flexiblebronchoscopydownthe tra-cheotomytube willconfirm the siteofobstruction, and a longertubemaybeallthatisrequiredtobypassthe obstruc-tion.Positivepressurealleviatesobstructionstemmingfrom tracheomalaciaorbronchomalacia.Inanemergency situa-tion, a longertracheotomy tube or an endotracheal tube placedthroughthestomawillgenerallybypassthe obstruc-tion.
Congenital
anomalies
of
the
larynx
Laryngomalacia
Laryngomalaciaisthemostfrequentcauseofstridorinthe neonate,1 andmost children withthis anomaly are
symp-tomaticatbirthorwithinthefirstfewdaysoflife.Stridor isgenerallymild,butisexacerbatedbyfeeding,crying,and lyingin a supine position.In 50% of the cases, symptoms worsenduringthefirst6monthsoflife,and,invirtuallyall childrenwithlaryngomalacia,symptomsresolveby1yearof age.1Inchildrenwithseverelaryngomalacia(approximately
10%),surgicalinterventionisrequired1---3;symptomsinthese
children may includeapnea, cyanosis, severe retractions, and failureto thrive.In extremely severe cases, cor pul-monaleisobserved.
Diagnosis is confirmed by flexible transnasal fiberop-tic laryngoscopy. Characteristic findings include short aryepiglottic folds, with prolapse of the cuneiform carti-lages.Insomecases,atightlycurled(-shaped)epiglottis is alsoobserved. Becauseof the Bernoullieffect, charac-teristic collapse of the supraglottic structures is seen on inspiration. Inflammation suggestive of reflux laryngitis is also frequently present. The need for intervention is not determinedbytheendoscopicappearanceofthelarynx,but ratherbythesymptomsoftheinfant.
Childrenwithlaryngomalaciararely presentwithacute airwaycompromise.Inthe10%ofchildrenwhorequire oper-ativemanagement,thismaybearrangedinasemi-elective fashion within one to two weeks of presentation. Preop-erative management of gastroesophageal reflux (GER) is prudent.Supraglottoplasty(alsotermedepiglottoplasty)has replacedtracheotomyasthepreferredintervention.Thisis arapidandeffectiveendoscopicprocedure,directedatthe infant’sspecificlaryngealpathology.Botharyepiglotticfolds usuallyaredivided.Inaddition,oneorbothcuneiform car-tilagesmayberemoved.Ifthearyepiglotticfoldsaloneare divided,postoperativeintubationisgenerallynotrequired;
however,overnightintubationshouldbeconsideredifmore extensivesurgeryhasbeenperformed.
Followingsupraglottoplasty,overnightobservationinthe intensive care unit is desirable, as laryngeal edema may compromise the airway, necessitating reintubation. Extu-bation is usually possible within 24h of the surgery. In some children, obstruction persists postoperatively.4
Bed-side fiberoptic laryngoscopy can differentiate between laryngeal edema and persistent laryngomalacia. Reflux managementhelpsmitigatelaryngealedema.Occasionally, thepostoperativeappearanceofthelarynxisadequate,but theinfantisstillstruggling.Insuchcases,thereissometimes an underlying neurologic component to the laryngomala-cia. Although the neurologic problems may be extremely subtleinitially,theymaybecomemuchmoreevidentwith time.1,5Thissubsetofchildrenisfarmorelikelytorequire
tracheotomyplacement.
Bilateralvocalfoldparalysis
Incontrasttounilateralvocalfoldparalysis,bilateral paral-ysisisusuallycongenital.Althoughthisconditionisgenerally idiopathic, it may be seen with central nervous system pathology,includinghydrocephalusandChiarimalformation ofthebrainstem.6Foraninfantwithstridorandretractions
duetobilateralparalysis,tracheotomyisindicated.Aswith laryngomalacia,thediagnosisisestablishedwithawake flex-ibletransnasalfiberopticlaryngoscopy.Stabilizationmaybe achievedwithintubation,CPAP,orhigh-flownasalcannula asanalternativetemporizingmeasure.Inupto50%of chil-dren,theparalysisspontaneouslyresolvesby1yearofage. Surgicalinterventiontoachievedecannulationistherefore usuallypostponeduntilafterthisperiod.
Theaimof surgeryistoachieveanadequate decannu-latedairwaywhilemaintainingvoiceandnotexacerbating aspiration.Surgicaloptionsincludelasercordotomy,partial orcomplete arytenoidectomy (endoscopicor open), vocal processlateralization(endoscopicallyguidedoropen),and posteriorcricoidcartilagegrafting.1,7Nosingleoptionoffers
aperfectresult.Inachild withatracheotomy,it isoften desirable tomaintain the tracheotomy toensure an ade-quateairwaybeforedecannulation.
Acquired bilateral vocal cordparalysis is usually more recalcitrant to treatment than idiopathic cord paralysis, andmore thanoneproceduremayberequiredtoachieve decannulation.Forpatients whohave undergoneanysuch procedures,post-extubationstridormayrespondtoCPAPor high-flownasalcannula.Achild’spostoperativeriskof aspi-rationshouldbeassessedbyavideoswallowstudypriorto resuminganormaldiet.Duringtheinitialweeksfollowing surgery,thereis sometimesanincreasedriskofaspiration withcertaintextures,particularlythinfluids.
Subglotticstenosis
witha smallerthan averagediameter,usuallyelliptical in shape.MildSGSmaymanifestinrecurrentupperrespiratory infectionsinwhichminimalsubglotticswellingprecipitates airwayobstruction.Inayoungchild,thegreatest obstruc-tion is usually 2---3mm below the true vocal folds. More severecasesmaypresentwithacuteairwaycompromiseat delivery.Ifendotrachealintubationissuccessful,thepatient mayrequireinterventionbeforeextubation.When intuba-tion cannot be achieved, tracheotomy placement at the timeof deliverymaybelifesaving.Itisimportanttonote thatinfantstypicallyhavesurprisinglyfewsymptoms.Even thosewithgrade3SGS (71---99% obstruction)8 maynotbe
symptomaticforweeksormonths.
CongenitalSGSisoftenassociatedwithothercongenital headandnecklesionsandsyndromes(e.g.,asmalllarynx in a patient with Down syndrome). After initial manage-mentofSGS,thelarynxwillgrowwiththepatientandmay notrequirefurthersurgicalintervention.However,ifinitial managementrequirestrachealintubation,thereisa consid-erableriskofdevelopinganacquiredSGSinadditiontothe underlyingcongenitalSGS.
Radiologicevaluationofanairwaythatisnotintubated maygivethecliniciancluesaboutthesiteandlengthofthe stenosis. Usefulimagingmodalities include(1) inspiratory andexpiratorylateralsoft-tissueneckfilms,(2)fluoroscopy to demonstrate the dynamics of the trachea and larynx, and (3)chest X-ray.The single most important investiga-tion,however,is high-kilovoltage airway film.These films canidentifytheclassicsteeplingobservedinpatientswith SGSaswellaspossibletrachealstenosis.Thelatter condi-tionisgenerallycausedbycomplete trachealrings,which maypredispose thepatienttoa life-threatening situation duringrigidendoscopy.
EvaluationofSGSrequiresendoscopicassessment. Flex-iblefiberopticendoscopyprovidesinformationondynamic vocalfoldfunction.RigidendoscopywithHopkinstelescopes providesthebestpossible examination.Preciseevaluation oftheendolarynxshouldbecarriedout,includinggrading oftheSGS.8
For children with mild symptoms and a minor degree of SGS, endoscopic intervention may be effective. Endo-scopicoptionsincluderadialincisionsthroughthestenosis with steroid injections and laryngeal dilatation.9 More
severeformsofSGSarebettermanagedwithopenairway reconstruction.Laryngotrachealreconstructionusingcostal cartilagegraftsplacedthroughthesplitlaminaofthecricoid cartilageisreliableandhaswithstoodthetestoftime.10,11
Costalcartilagegraftsmaybeplacedthroughtheanterior laminaofthecricoidcartilage,theposteriorlaminaofthe cricoid cartilage, or both. These procedures maybe per-formed astwo stages, maintaining the tracheal tube and temporarilyplacingasuprastomallaryngealstentabovethe trachealtube.Alternatively,inselectcases,asingle-stage proceduremaybeperformed,withremovalofthetracheal tube on the day of surgery and with the child requiring intubationfor1---14days.12 Forthemanagementofsevere
SGS,betterresultsareachievedwithcricotrachealresection thanwithlaryngotrachealreconstruction.13---15However,this
isatechnicallydemandingprocedurethatcarriesa signifi-cantriskofcomplications.Reconstructionofthesubglottic airwayis a challenging procedure and the patientshould be optimized before undergoing surgery. Preoperative
evaluation includes assessment and management of GER, EE,andlow-gradetrachealinfection,particularly oxacillin-resistantStaphylococcusaureus(ORSA)andpseudomonas.16
Saccularcysts
The laryngeal saccule is a small diverticulum containing numerous mucousglandsat theanteriorendofthe laryn-gealventricle,extendingsuperiorlybetweenthevestibular foldandtheinner surfaceofthethyroid cartilage.17
Con-genitalsaccularcystsaremucusfilled, andusuallyextend posterosuperiorly intothefalse vocal foldand aryepiglot-tic fold(thelateral saccular cyst).18 These cyststypically
present withrespiratory obstruction in the first few days of life,a muffledcry,and dysphagia.Becauselow muscle tone predisposesto obstruction, oneof the greatestrisks forchildrenwithsaccularcystsis theinduction ofgeneral anesthesia,withsubsequentlossoftheairwayandcomplete obstruction. It is therefore mandatory to perform preop-erativeawaketransnasalflexiblelaryngoscopytoevaluate theairway,asthiswillforewarnofpotentialproblemswith subsequentanesthesia. Duringanesthesia, theairwaymay besecuredwithanappropriateventilatingbronchoscope,a stylettedendotrachealtube,oranendotrachealtubeplaced overasmallHopkinsrodtelescope.Giventherisks associ-atedwithanesthesia,thisshouldbeattemptedonlyinthe presence ofequipmentand personnelcapableof securing theairway.Sendinganinfantforaradiologicevaluationthat mayrequiresedationorananestheticcouldbedisastrous. Itisthusadvisabletoevaluateandsecuretheairwayinthe operatingroom,andconsiderradiologicevaluationafterthe airwayissecured.
Althoughsaccularcystshave historicallybeen managed endoscopically using needle aspiration, marsupialization, or endoscopic excision, the resultshave been poor, often requiringmultipleproceduresandtracheotomyplacement. The author’s preference is to use an anterior cervical approachwhereby the superiorborder ofthe thyroidalar cartilage is identified and the thyrohyoid membrane is incised along its superior border. This approach provides access to the cyst, which may gently be dissected free and removed intact. Part of the thyroid cartilagemay be removed toimproveaccessandreplacedafter thecyst is removed.
Posteriorlaryngealclefts
Posteriorlaryngealcleftsresultfromafailureofthe laryn-gotracheal groove tofuse during embryogenesis. Patients commonlyhaveassociatedanomaliesthataffecttheairway orotherorgansystems.Associatedairwayanomaliesinclude tracheomalacia(>80%)andtracheoesophagealfistula(TEF) formation(20%).Non-airwayassociationsincludeanogenital anomaliesandGER.Themostcommonsyndromeassociated with posterior laryngeal clefting is Opitz---Frias syndrome, characterizedbyhypertelorism,anogenitalanomalies,and posteriorlaryngealclefting.
A useful anatomic classification proposed by Benjamin and Inglis19 dividesposterior laryngeal clefts intothe
Type1: supraglotticinterarytenoidcleftpresent to, but notbelow,thelevelofthetruevocalfolds.Thiscleftcould alsobeconsideredadeepinterarytenoidnotch.
Type 2: partial cricoid cleft that extends into, but not through,theposteriorcricoidcartilage.
Type3:totalcricoidcleftwithorwithoutextensionofthe cleftintothecervicaltrachea.
Type4:cleftextendingtothethoracictrachea.
Ararevariantofthetype4cleft(referredtoastype4 long)isacleftthatextendsintothecarinaorbeyond.
It is important for clinicians to note that there is a great deal of symptom overlap among the various cleft typesandthatsymptomsarenotindicativeofcleftseverity. Aspirationisthehallmarkclinicalfeatureofthisanomaly. Althoughgrossaspirationmayoccurwithassociatedapnea, cyanosis,andevenpneumonia,thesymptomsofteninclude microaspiration,withchokingepisodes,transientcyanosis, andrecurrentchestinfections.Severetracheomalaciamay significantlycompromisetheairway,especiallyinchildren withanassociatedtracheoesophagealfistula.
Although diagnostic tools include flexible nasopharyn-goscopy, fiberoptic endoscopic evaluation of swallowing, video fluoroscopy-barium swallow, and flexible bron-choscopy, the gold standard for diagnosis is rigid bron-choscopyandesophagoscopy.Given thatthecleftisoften hiddenby redundant mucosain theposterior glottis, itis notuncommonforittobeundiagnosedormisdiagnosedon initialevaluationwithflexibleorrigidbronchoscopy.
Managementinvolvesmaintaininganappropriateairway while minimizing the risk of aspiration. Initial manage-mentdecisionsshouldconsiderwhethertheinfantrequires tracheotomy placement, gastrostomy tube placement, or Nissenfundoplication.Althoughnoneoftheseis essential, eachincreasesthelikelihoodofsuccessfulcleftrepair. Pro-tection against aspiration is also crucial, and nasojejunal feedingmaybeausefulwayofstabilizinganinfant. Surgi-calrepairmaybeperformedendoscopicallyformosttype1 andsometype2clefts;however,longercleftsthatextend into the cervical or thoracic trachea require open repair. Thetranstrachealapproachprovidesunparalleledexposure ofthecleftwhileprotectingtherecurrentlaryngealnerves. A two-layer closure is recommended, with the option of performing an interposition graft if warranted; a useful interpositiongraft isa freetransferof thesternal perios-teum.
The most challenging cleft torepair isthe raretype 4 long cleft. These clefts are prone to anastomotic break-down and the infant often has multiple anomalies, both airwayandnon-airway.20 Thelattermayinclude
microgas-tria, polysplenia, and annular pancreas. Microgastria may resultinuncontrollablegastroesophageal refluxandis not amenable to fundoplications. Before attempting surgical repairofthecleft,thesurgeonmust decideifthechildis salvageable.
Success rates for posterior laryngeal cleft range from 50% to90%, depending ona numberof factors.The most influentialfactorcompromisingsuccessfulcleftrepairisthe presenceofacoexistentcongenitaltracheoesophageal fis-tula. Other factors include the severity of the cleft, the type of operation chosen by the surgeon,and whether a
double-layerorsingle-layerclosureis performed.Revision surgeryislesssuccessfulthanprimarysurgery.20
Laryngealwebs
Laryngealwebsrepresentafailureofrecanalizationofthe glotticairwayintheearlyweeksofembryogenesis.Virtually all(95%)glotticwebsareanterior;however,thesewebsare rare,comprisingonly5%ofcongenitallaryngealanomalies. Althoughthereiscurrentlynoknowngenethatcauses con-genitalglottic webbing, a significant association between thisanomaly andchromosome 22q11.2deletionsyndrome (velocardiofacial syndrome) has been reported.21,22 Given
thatmorethan 50%of the patientswithan anterior glot-ticwebhavechromosome22q11.2deletionsyndrome,itis prudenttoreferallpatientswithcongenitalanteriorglottic websforgeneticevaluation.
Somewebs aregossamerthin;however,mostarethick andusuallyassociatedwithasubglottic‘‘sail’’ compromis-ingthesubglotticlumen.1Theymaythereforebeconsidered
as a form of partial laryngeal atresia. Neonates usually presentwithanabnormalcryorrespiratorydistressatbirth. Ifaneonatewithananteriorglotticwebpresentswith sig-nificant airwaycompromise in thefirst few hoursor days oflife,thewebissevereandemergentairwayintervention isrequired.Nonetheless,theclinicianshouldbecognizant ofthefact thatinfants areremarkablytolerantofairway compromiseandeventhosewithamoderatetosevere glot-tic web may initially show only subtle airway symptoms. These symptoms typically exacerbate over the first few monthsof life. In infants withmoderate to severe webs, biphasic stridor and retractions become increasingly evi-dent,particularlywhentheseinfantsareupsetorfeeding.
A definitive evaluation requires rigid or flexible bron-choscopy.Both the severityof the web andits subglottic extensionareevaluated.Flexiblebronchoscopyprovidesan excellentview of the anterior commissure,whereas rigid bronchoscopyismoreadvantageousinevaluatingthedegree ofassociatedSGS.Inchildrenwithasevereweb,caremust betakennottofurthercompromiseanalreadycompromised airway,andspontaneousventilation withthe infant main-taininghisorherownairwayispreferabletointubationor emergenttracheotomy.
Managementoftheinfantwithagossamer-thinanterior glotticwebis differentfrommanagementofthefarmore frequent thick web with subglottic extension. The infant witha thin glottic web may never be diagnosed, as it is thoughtthatinachildwithneonatalairwaycompromisedue tosuchaweb,intubationforairwaystabilizationmay actu-allylysethewebandcompletelyresolvetheproblem.Inan infantpresentingwithathinanteriorwebonbronchoscopy, divisionofthe webwitha sickleknife while theinfant is suspendedonasmallLindholmlaryngoscopeiscurative.
witha more severe degree of glottic compromise, repair maybeperformedearly.Alternatively,atracheotomymay beplacedandlaterepairmaybeplanned.Thisapproachis requiredinapproximately40%ofpatientswiththickwebs. Optionsfor repair of a congenitalanterior glotticweb includeopenkeelplacementandopenreconstructionofthe anteriorcommissure.Endoscopicproceduressuchaslaser divisionoftheweb orendoscopic keelplacementarenot advocated. Laser divisionis likelyto resultin web refor-mationanddoes notadequatelyaddress theinherentSGS thatisgenerallypresent.Similarly,endoscopickeel place-mentdoesnotaddress SGS.Furthermore,althoughitmay beanappropriateoptioninanolderchild,itistechnically demandinginaninfant.
Subglottichemangioma
Althoughhemangiomasgenerallypresentcutaneously,they can occur in any anatomic site. The larynx, particularly the subglottis, is the most common site of presentation within the airway. More than 50% of the patients with a subglottichemangiomaalsohavecutaneoushemangiomas, whichprovideanindicationfor thepossiblepresenceofa synchronoussubglotticlesion.Patientswithahemangioma occurringinabearddistribution(i.e.,thechin,jawline,and preauricularareas)areatthe highestrisk for asubglottic hemangioma.23 The natural historyof these hemangiomas
mirrorsthat ofcutaneous lesions,withaninitial phaseof proliferation followed by a phase of involution; however, subglottichemangiomasexpandandinvolutemorerapidly. Asthehemangiomaincreasesinsize,progressive deteriora-tionoftheairwayusuallyoccurs.Symptomsincludebiphasic stridorwithretractions,especiallywhenthechildisupsetor feeding.Abarkingcoughsimilartothatseenwiththecroup may also be present. When airway obstruction is severe, apnea,cyanosis,and‘‘dyingspells’’mayoccur.
Optimally, initial evaluation is performed with awake transnasal flexible laryngoscopy. This may allow for visualization of the compromised subglottis, and more importantly, should also rule out other causes of neona-talstridorsuchaslaryngomalaciaandvocal foldparalysis. A child with progressive stridor and a normal glottic and supraglotticexaminationrequiresalaryngoscopyand bron-choscopyperformed in theoperating room undergeneral anesthesia. As mentioned earlier in this article, because infantsaresoremarkablytolerantofprogressivesubglottic airwaycompromise,theremaybean80---90%compromiseof thesubglotticlumenbythetimethechildpresentsforrigid bronchoscopy. In infants in whom extralaryngeal involve-mentissuspected(e.g.,thosewithcutaneoushemangiomas occurring in a beard distribution), magnetic resonance imaging(MRI)withT2-weightedgadoliniumcontrastis per-formed. Becauseof the risk of hemorrhage,biopsy is not advised.
Mostpatientsrequiretreatment,andtreatment modal-itiesareoftencombined.At CincinnatiChildren’sHospital MedicalCenter,symptomaticpatients withsignificant stri-dor are managed with systemic steroids combined with propranolol, a nonselective beta-blocker used to treat infants withcardiovascularconditions.Over the past sev-eralyears,aplethoraofreportshavedocumenteddramatic
results achieved with the use of this agent,24---29 thereby
changingthetraditionalparadigmofpharmacotherapyand surgicalmanagement.Forthemostpart,propranolol ther-apy hasreplacedbothopen andendoscopicresectionand has obviated the need for tracheotomyplacement. More-over, a recent (2013) review of 41 propranolol studies revealed a low rate of serious adverse advents.30
Treat-mentistypicallymaintainedforaminimumofsixmonths; patientsarethenweanedoffthepropranolol.Because pre-maturecessationofthistherapymayresultinhemangioma regrowth,29carefulmonitoringduringtheweaningprocess
isessential.
Conflicts
of
interest
Theauthordeclaresnoconflictsofinterest.
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