RevBrasAnestesiol.2016;66(5):551---553
REVISTA
BRASILEIRA
DE
ANESTESIOLOGIA
PublicaçãoOficialdaSociedadeBrasileiradeAnestesiologiawww.sba.com.br
CLINICAL
INFORMATION
Airway
management
in
newborn
with
Klippel---Feil
syndrome
Nuray
Altay
a,∗,
Hasan
H.
Yüce
a,
Harun
Aydo˘
gan
a,
Mustafa
E.
Dörterler
baHarranUniversitySchoolofMedicine,DepartmentofAnesthesiologyandReanimation,Sanliurfa,Turkey bHarranUniversitySchoolofMedicine,DepartmentofPediatricSurgery,Sanliurfa,Turkey
Received16January2014;accepted13March2014 Availableonline29April2014
KEYWORDS
Neonatal; Difficultairway; Congenital anomalies; Congenitalheart disease;
Surgery; Criticalcare
Abstract Klippel---Feilsyndrome(KFS)hasaclassicaltriadthatincludesshortneck,lowhair lineandrestrictioninneckmotionandisamongoneofthecongenitalcausesofdifficultairway. Herein,wepresenta26-day,3300gnewbornwithKFSwhowasplannedtobeoperatedfor cor-rectionofanintestinalobstruction.ShehadfeaturesofsevereKFS.Anesthesiawasinducedby inhalationofsevoflurane2---3%inpercentage100oxygen.Sevofluraneinhalationwasstopped after2min.HerCornmackLehanescorewas2andoralintubationwasperformedwith3.5mmID non-cuffedendotrachealtubeinfirstattempt.Operationlastedfor45min.Following unevent-fulsurgery,shewasnotextubatedandwastransferredtothenewbornreanimationunit.On thepostoperativethirdday,thepatientdiedduetohyperdynamicheartfailure.Thiscaseis theyoungestchildwithKlippel---Feilsyndromeinliteratureandonwhomoralintubationwas performed.Wealsothinkthatpositioningofthisyoungeragegroupmightbeeasierthanolder agegroupsduetoincompleteossificationprocess.
©2014SociedadeBrasileiradeAnestesiologia.Publishedby ElsevierEditoraLtda.Thisisan openaccessarticleundertheCCBY-NC-NDlicense( http://creativecommons.org/licenses/by-nc-nd/4.0/).
PALAVRAS-CHAVE
Neonatal;
Viasaéreasdifícil; Anormalidadeas congênitas; Doenc¸acardíaca congênita; Cirurgiageral; Cuidadosintensivos
Manejodeviasaéreasemrecém-nascidocomsíndromedeKlippel-Feil
Resumo AsíndromedeKlippel-Feil(SKF)envolveumatríadeclássicaqueincluipescoc¸ocurto, linhadeimplantac¸ãodoscabelosbaixaerestric¸ãodomovimentodopescoc¸oeéumadascausas congênitasdeviaaéreadifícil.Apresentamosocasodeumarecém-nascida,de26dias,3.300g depeso,comSKF,quefoiagendadaparacirurgiadecorrec¸ãodeumaobstruc¸ãointestinal.A pacienteapresentavacaracterísticasacentuadasdaSKF.Aanestesiafoiinduzidacominalac¸ão desevofluranoa2-3%em100%deoxigênio.Ainalac¸ãodesevofluranofoiinterrompidaapós doisminutos.OescoredeCormack-Lehanedapacienteera2eaintubac¸ãoorotraquealfoifeita naprimeiratentativa,comtuboendotraquealde3,5mmIDsembalão.Otempodecirurgiafoi
∗Correspondingauthor.
E-mail:nurayaltay@ymail.com(N.Altay). http://dx.doi.org/10.1016/j.bjane.2014.03.006
552 N.Altayetal.
de45minutos.Apósacirurgiasemintercorrências,apacientefoitransferidaparaaunidadede reanimac¸ãoneonatalaindaintubada.Noterceirodiadepós-operatório,foiaóbitoporcausa deumainsuficiênciacardíacahiperdinâmica.Essecasoapresentaacrianc¸amaisjovemcom síndromedeKlippel-Feilnaliteraturaenaqualaintubac¸ãoorotraquealfoifeita.Acreditamos tambémqueoposicionamentodepacientesdessafaixaetáriamaisjovempodesermaisfácildo queodepacientesdefaixasetáriasmaisaltas,porcausadoprocessodeossificac¸ãoincompleto. ©2014SociedadeBrasileiradeAnestesiologia.PublicadoporElsevierEditoraLtda.Este ´eum artigoOpen Accesssobumalicenc¸aCCBY-NC-ND( http://creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction
Klippel---Feil syndrome(KFS) has an incidence of 1:42,000 birthsandisamongoneofthecongenitalcausesofdifficult airway.Theclassicaltriadincludesshortneck,lowhairline, restrictioninneckmotion,andfusionofatleasttwocervical segments.Shortneckwithalimitedrangeofmovementand cervicalinstabilitycanleadtoneurologicaldamageduring laryngoscopy,intubationandpositioningforsurgery.1
Case
description
Herein,wepresenta26-day,3300gnewbornwithKFSwho
wasplannedtobeoperatedforcorrectionofanintestinal
obstruction.On physical examination,she hadfeaturesof
severeKFS:includingshortneck,restrictioninneckmotion,
high palate, class II modified Mallampati score, low hair
lineand atypicalfacialappearance (Fig.1).The
echocar-diogramrevealedASD,VSD,aorticpseudocoarctation,PDA,
widecoronarysinus, persistentpulmonary HT.Blood
pres-sure, oxygen saturation, and ECG were monitored in the
operatingroom.VariouslargebladesofMillerandMacintosh
type,laryngealmaskairways,trachealtubeswithstilettes,
fiberopticbronchoscope,andatracheostomysetwerekept
ready.Anesthesiawasinducedbyinhalationofsevoflurane
2---3%inpercentage100oxygen.Sevofluraneinhalationwas
stoppedafter2min.HerCornmackLehanescorewas2and
oral intubationwas performed with3.5mm ID non-cuffed
Figure1 Lowhairlineandatypicalfacialappearanceofthe patient.
endotracheal tube in the first attempt. Correction of ET
tubeplacementwasdonewithauscultationand
capnogra-phy.Anesthesiawasmaintainedwith60%airinoxygenwith
1---1.5%sevofluraneandDuodenoduodenostomywasdonefor
thepartial obstruction induodenum.Operationlastedfor
45min.Followinguneventfulsurgery,shewasnotextubated
and wastransferredtothenewborn reanimationunit.On
thepostoperativethirdday,thepatientdieddueto
hyper-dynamicheartfailure.
Discussion
Klippel---Feilsyndromeisararedisease,initiallyreportedin
1912byMauriceKlippelandAndréFeilfromFrance,
charac-terizedbythecongenitalfusion ofany2ofthe7cervical
vertebrae.Theclassicaltriadincludesshortneck,lowhair
line,restrictioninneckmotion,andfusionofatleasttwo
cervicalsegments.PatientswithKlippel---Feilsyndrome
usu-allypresentwiththediseaseduringchildhoodbutthismay
alsopresent later inlife.2,3 Ourcase wasdetected in the
neonatal period and has classical triad signs of the
syn-drome. Difficult airwayis the main problemof this group
ofpatientinanesthesiologyandintensivecaremedicine.In
literature,most oftheKFS casesinvolveadultagegroups
andisassociatedwithdifficultairwayproblems.1,2Stallmer
etal.haverecentlypublishedthemanagementof10
pedi-atricpatientswithKFSconcludingthattheairwayofthese
patientscanbesettledwithoutdifficultyinahigh
percent-ageofcasessimilartoourcase.4Theyoungestcaseinthe
literatureis two-month-oldgirl.To thebestof our
knowl-edge,thiscaseistheyoungestoneintheliterature.
Inthatcase,inordertoavoidtraumatization,LMA(size
1) was inserted with the reverse technique. This case is
theyoungestchildwithKlippel---Feilsyndromeandonwhom
oralintubationwasperformed.Wealsothinkthat
position-ing of this younger age group might beeasier than older
age groups due to incomplete ossification process. More
extensivefusionstendtobeassociatedwithotherdefects
involvingthecardiovascular,respiratory,andgenitourinary
systems.Cardiovascular anomalies occurin 15---30%of the
cases.Themostcommonproblemistheventricularseptal
defect.3Heartfailureisimportantinpostoperativecare
pro-cess.OurcasehasASD,VSD,aorticpseudocoarctation,wide
coronarysinusandpersistentpulmonaryHT.Thesyndrome
alsomaybeassociatedwithabnormalitiesoftheheadand
face,skeleton,sexorgans,muscles,brainandspinalcord,
AirwaymanagementinKlippel---Feilsyndrome 553
KFScasewithGIsystemanomalies wascolonduplication.5
Upper GI system abnormalities might cause nutritional
disorders and respiratory problems similar to our case.
Detailed preanesthetic evaluation of organ systems
espe-ciallyincludingairwayandcardiovascularsystemiscrucialin
KFS.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
Acknowledgements
Informed consent was obtained from the patient’s
fam-ily. The authors did not receive any outside funding or
grantsinsupportoftheirresearchorinpreparationofthis
work.
References
1.Fernandes ML, Faria NC, Gonc¸alves TF, et al. Anesthesia for tonsillectomy in a child with Klippel---Feil Syndrome associ-ated with Down syndrome. Case report. Rev BrasAnestesiol. 2010;60:315---20.
2.KlippelM,FeilA.Uncasd’absencedesvertebrescervicalesAvec cagethoraciqueremontantjusqu’alabaseducrane(cage tho-raciquecervicale).NouvIconogSalpetriere.1912;25:223---50. 3.TracyMR,DormansJP,KusumiK.Klippel---Feilsyndrome:clinical
featuresandcurrentunderstandingofetiology.ClinOrthopRelat Res.2004;424:183---90.
4.StallmerML,VanaharamV,MashourGA.Congenitalcervicalspine fusionandairwaymanagement:acaseseriesofKlippel---Feil syn-drome.JClinAnesth.2008;20:447---51.
