Rev. Bras. Anestesiol. vol.65 número4

RevBrasAnestesiol.2015;65(4):298---301

REVISTA

BRASILEIRA

DE

ANESTESIOLOGIA

www.sba.com.br

CLINICAL

INFORMATION

Anesthetic

management

for

surgery

of

esophagus

atresia

in

a

newborn

with

Goldenhar’s

syndrome

Rosana

Guerrero-Domínguez

,

Daniel

López-Herrera-Rodríguez

,

Inmaculada

Benítez-Linero

_,

_Antonio

_Ontanilla

a_{ServiceofAnesthesiologyandReanimation,HospitalesUniversitariosVirgendelRocío,Sevilla,Spain}

b_{ServiceofAnesthesiologyandReanimation,HospitalInfantil,HospitalesUniversitariosVirgendelRocío,Sevilla,Spain}

Received3July2013;accepted25July2013 Availableonline28April2015

KEYWORDS

Goldenhar’s syndrome;

Tracheoesophageal fistula;

Esophagealatresia; Difficultairway; Fibrobronchoscope

Abstract

Backgroundandobjectives: Goldenhar’ssyndromeisapolymalformativeconditionconsisting ofacraniofacialdysostosisthatdeterminesdifficultairwayinupto40%ofcases.Wedescribed acaseofanewbornwithGoldenhar’ssyndromewithesophagealatresiaandtracheoesophageal fistulawhounderwentrepairsurgery.

Casereport: Wereportthecaseofa24-h-oldnewborn withGoldenhar’ssyndrome. Hehad

esophageal atresiawithdistal tracheoesophagealfistula.Itwas decidedthatanemergency

surgerywouldbeperformedforrepairingit.Itwascarriedoutundersedation,intubationwith fibrobronchoscopedistaltothefistula,tolimittheairflowintotheesophagus,andpossible abdominal distension.Following complete repair ofthe esophagealatresia andfistula liga-tion,thepatientwastransferredtotheintensivecareunitandintubatedundersedationand analgesia.

Conclusions:The finding of a patient with Goldenhar’s syndrome and esophageal atresia assumesanexceptional situationandachallengefor anesthesiologists,sincetheanesthetic

managementdependsonthepatientcomorbidity,thetypeoftracheoesophagealfistula,the

usualhospitalpracticeandtheskillsoftheanesthesiologistincharge,withthemain

peculiar-itybeingmaintenanceofadequatepulmonaryventilationinthepresenceofacommunication

betweentheairwayandtheesophagus.Intubationwithfibrobronchoscopedistaltothefistula dealswiththemanagementofaprobablydifficultairwayandlimitsthepassageofairtothe esophagusthroughthefistula.

©2013SociedadeBrasileiradeAnestesiologia.PublishedbyElsevier EditoraLtda.Allrights reserved.

∗_{Correspondingauthor.}

E-mail:rosanabixi7@hotmail.com(R.Guerrero-Domínguez).

SurgeryforesophagealatresiainnewbornwithGoldenhar’ssyndrome 299

PALAVRAS-CHAVE

Síndromede Goldenhar; Fístula

traqueoesofágica; Atresiadeesôfago; Viarespiratória difícil;

Fibrobroncoscópio

Manejoanestésicoparacirurgiadeatresiadeesôfagoemumrecém-nascidocom síndromedeGoldenhar

Resumo

Justificativaeobjetivos: AsíndromedeGoldenharéumquadrodepolimalformac¸ãoque con-sisteemumadisostosecraniofacialquedeterminaumaviarespiratóriadifícilematé40%dos

casos.Nósdescrevemosumcasodeumrecém-nascidocomsíndromedeGoldenharcomatresia

deesôfagoefístulatraqueoesofágicaparaaqualfoirealizadacirurgiadereparo.

Relatodecaso: Apresentamosocasodeumrecém-nascidode24hdevidacomsíndromede Goldenhar.Eleapresentavaatresiadeesôfago,comfístulatraqueoesofágicadistal. Decidiu-seporumacirurgiadeemergênciaparareparo.Elafoirealizadasobsedac¸ão,intubac¸ãocom fibrobroncoscópiodistalàfístula,paralimitarpassagemdoarparaoesôfagoepossíveldistensão abdominal.Apósoreparocompletodaatresiadeesôfagoeligaduradafístula,opacientefoi transferidoparaaunidadedeterapiaintensivaeintubadocomsedoanalgesia.

Conclusões: OachadodeumpacientecomsíndromedeGoldenhareatresiadeesôfagosupõe

uma situac¸ão excepcionaleum desafiopara os anestesiologistas,pois omanejoanestésico

dependedacomorbidadedopaciente,dotipodefístulatraqueoesofágica,dapráticahospitalar habitualedashabilidadesdoanestesiologistaresponsável,sendoqueapeculiaridadeprincipal

émanter umaventilac¸ãopulmonaradequadanapresenc¸ade umacomunicac¸ãoentreavia

respiratóriaeoesôfago.Aintubac¸ãocomfibrobroncoscópiodistalàfístularesolveomanejo daviarespiratóriaprovavelmentedifícilelimitaapassagemdearparaoesôfagoatravésda fístula.

©2013SociedadeBrasileira deAnestesiologia.PublicadoporElsevierEditoraLtda.Todosos direitosreservados.

Introduction

Goldenhar’ssyndrome(GS)oroculo-auriculo-vertebral dys-plasia was first described by von Arlt in 1845.1 _{This is a}

polymalformative condition resulting from defects of the firstandsecond branchialarchs1 _{consistingof craniofacial}

dysostosis,whereweobserveabnormalitiesoftheeyesand cervical vertebrae, malformations of the ears and unilat-eralmicrothia,facialasymmetry,micrognathia,2_epibulbar

dermoidcysts1_{andotherchangesoftheoralcavity,suchas}

cleftpalateandlingualchange.3,4_{Althoughtheincidenceof}

GSisestimatedat1in5000livenewborns,2,4_{theassociation}

ofesophagealatresia(EA)andthisconditiononlyoccursin 5%ofthesepatients,1_{andisanexceptionalcombination.}

Clinical

case

Wepresentacaseofa24-h-oldnewbornof3.2kgofweight withleftunilateralmicrothia(Fig.1),bilateralureterocele and esophageal atresia with distal tracheoesophageal fis-tula(TEF).Anemergencysurgerywaschosentorepairthe EA andassociatedfistula. On examinationof theairways, mildmicrognathiaanddifficultopeningofthemouthwere found.As supplementarypreoperativetests,achestX-ray withnasogastrictubewasperformed,whichconfirmedthe diagnosis(Fig.2),alongwithbloodcount,biochemistryand coagulationstudy,transthoracicechocardiographyand elec-trocardiography,whichshowednochanges.

In the operating room monitoring was performed with non-invasive blood pressure, pulse oximetry (SpO2),

and electrocardiogram. Under sedation with 2% sevoflu-rane, 0.1mg IV atropine through a venous access with

epicutaneouscatheterwasgiventoreduce oropharyngeal secretions, as well as 10␮g fentanyl. With the patient underspontaneous ventilation the fibrobronchoscope was introducedbymouthuptotheopeningof theglottis,and a rigid tube of 3.5mm in diameter was inserted, being positioned distal to the TEF and near the main carina. The proper placement of the endotracheal tube (ETT) wasconfirmedbycapnographyandpulmonaryauscultation, startingvolume-controlledventilationwithatidalvolumeof 8mlkg−1_{andrespiratoryratebetween17and20.To} main-taintheanesthetic, the concentrationof sevoflurane was increasedto3%and1mgcisatracuriumindosesof6---9␮gof fentanylweregiven,accordingtotheneedforpainkillers.

300 R.Guerrero-Domínguezetal.

Figure2 Radiologicalimage showing aturn of nasogastric tubeduetoimpossibilityofadvancingitinesophageallevel.

During surgery the patient remained hemodynamically stable with a heart rate of 140---150 beats per minute andsystolicbloodpressurebetween70and75mmHg.The respiratorysystemkeptSpO2 at90---95%which surprisingly

reached values close to 100% after complete repair of esophagealatresia andligationof thefistula.The surgery lasted170min withadministrationof40mlof crystalloids withnoneedfortransfusionsofbloodproducts.Upon com-pletion of the surgical procedure, he was transferred to theintensive care unitwithsedoanalgesia, intubatedand mechanicallyventilated,withnocomplications.

Discussion

Craniofacial changes in GS can condition a difficult air-way(DAW) in up to40% of thecases,5 _{mainly due tothe}

combination of micrognathia, limitation of cervical joint movement, and tracheal shift.5,6 _{This is a challenge for}

anesthesiologists and may require the use of alternative methodsfor thedirect laryngoscopytoensure proper air-waypatency, such asintubation withfibrobronchoscope,6

theuseoflaryngealmaskLMA4_{andalsotheperformanceof}

tracheostomywith retrograde intubation.4,5 _{A mandibular}

andcraniocervicalradiologicalevaluationbeforescheduled surgicalproceduresis recommended.7 _Othermedical

con-ditions associated with this syndrome, such as cardiac, tracheoesophageal, genitourinary anomalies, and mental retardment1,2_{shouldberuledout.}

TheEA,withorwithoutTEF,isthemostcommon congen-italesophagealanomaly,withanincidencerangingfrom1in 2500---4500livenewborns.2_{TheclassificationofEAis}

deter-minedbythelocationofatresiaandthepresenceorabsence ofassociatedTEF.Fivedifferenttypes weredescribed, so thatEAwithdistal fistulaassumes 85%of thecases.8 _The

diagnosisofEAisconfirmedbytheinabilitytomakea naso-gastrictubemovetothestomach,abundantoralsecretions andcoughingorepisodesofcyanosisafterfoodingestion.8_A

chestX-raywithanasogastricororogastrictubecanprovide confirmationofthediagnosis.8_{AlthoughThomasGibsonhad}

describedEAassociatedwithTEFin1697,itwasonlyin1941

thatCameronHaightperformedthefirstsurgicalcorrection ofthisproblem.9_{SurgerytorepairEAandTEFhasimportant}

anestheticimplications10_{andisachallengefor}

anesthesiol-ogists.Often,newborns mayhaverespiratorydiseasesand associatedcardiacmalformationsthat,alongwithaweight oflessthan2kg,areriskfactorsforpostoperativemortality andshouldbeassessedpriortosurgery.10,11

Themainfeatureoftheanestheticmanagementinthis surgeryis themaintenance ofadequate ventilationin the presenceofacommunicationbetweentheairwayandthe esophagus, and desaturation episodes may occur during inductionandduringanestheticmaintenance.Ontheother hand,theperformanceofathoracotomymaydeterminethe presentationofhemodynamicandrespiratorychanges,and difficultmaintenanceofanappropriatelevelofanalgesia.10

Theanestheticmanagementdependsonpatient comorbidi-ties,thetypeofEAwithorwithoutTEF,theusualhospital practice,andtheskillsoftheanesthesiologistincharge.

NewbornswithTEF areatincreasedrisk ofgastric dis-tension with potential risk of pneumoperitoneum11 _which

canincreasewiththeintroductionofmechanicalventilation withpositivepressure.DuringrepairofTEFthereareseveral alternativesforthemaintenanceofmechanicalventilation, suchasleftbronchialselectiveintubation,11 _{assuming the}

riskofatelectasis,andintubationwith fibrobronchoscope-guidedplacementofETTdistaltothefistula,whichwasthe techniqueusedinthiscase.11 _{BothformsofETTplacement}

requireclosemonitoringbytheanesthesiologistinchargeto detectabadpositioningofthetubeduringtheprocedure. Anothertechniquethatcouldbeusedistheocclusionofthe fistulausingaFogartycatheteruntilitscompleteligation.12

Itisrecommendedthatspontaneousventilationis main-tainedthroughinhalationinductionwithoutneuromuscular blockade nor positive pressure ventilation until the cor-rectplacementoftheETTisachieveddistaltothefistula8

toavoid gastric hyperinflation. Other authors recommend maintaining spontaneous ventilation tothe complete clo-sureofthefistulatominimizegastricdistensionassociated withpositivepressureventilation,withsome anesthesiolo-gistsevenrecommendingtheperformanceofagastrostomy beforeanestheticinductiontodecompressthestomachand improvemechanicalventilation.11,12

Inthecase ofGS, thefindingofaDAWassociatedwith EA with TEF could seriously impair oxygenation in these patients duringanesthetic induction, significantly increas-ing the risk of respiratory complications that are already highduetothesurgicalprocedureitself.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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