Airway management in the neonate with Moebius syndrome 夽

AnPediatr(Barc).2019;91(4):264---271

www.analesdepediatria.org

SCIENTIFIC LETTER

Airway management in the neonate with Moebius syndrome ^夽

Manejo de la vía aérea en el neonato con síndrome de Moebius

DearEditor:

Moebiussyndrome(MS)ischaracterisedbynon-progressing congenitalfacialpalsyand impairedocular abductiondue to involvement of the VII (facial) and VI (abducens) cra- nialnerves,respectively.Itsprevalenceisestimatedat1in 125000livebirths,withnodifferencebetweenthesexes.¹ TheaetiologyofMSisunknown;thedisorderhasbeenassoci- atedwithgeneticandenvironmentalfactors,suchasfoetal ischaemiaandtheuse ofdrugssuchasmisoprostolduring earlypregnancy.² Althoughitis widelyknownasMS,from theperspectiveofdysmorphologyitwouldbemorecorrectly termedasasequence,asaninitialchangeduringembryonal developmentwouldresultinacascadeofsecondaryevents.

Moebiussyndromecanbediagnosedearlyintheneonatal period.Itscharacteristicfeaturesaredeficientsuckingdue toincompleteclosure of thelips,the absenceof a facial expression,incompleteclosureoftheeyelidsduringsleep, droolingandesotropia.¹

Abnormalitiesinswallowingandbreathingarefrequent innewbornswiththisdisease.Approximately 90%ofthese patients had craniofacial anomalies that predispose the newborntoairwayobstruction.³

Wemadearetrospectivereviewofthecasesof7new- borns (5 male) with MS managed at the department of otorhinolaryngologyinthe2006---2018period,analysingthe clinicalmanifestations, thefindings ofendoscopy andthe videofluoroscopicswallowstudyandthetreatmentreceived (Table1).

Allthepatientshadstrabismusandbilateralfacialpalsy (Fig.1).Three patients(42.9%) hada historyof maternal use of a druguse to induceabortion (misoprostol) in the firsttrimesterofpregnancy.Threepatientspresentedasso- ciatedmalformations:Polandsyndromein1(hypoplasiaof themajorpectoralismuscle)andPierre-Robinsequencein 2(retrognathia,cleftpalateandglossoptosis).

Theexaminationdetectedmicrognathia,palateanoma- liesandglossoptosisin6patients(85.7%)andretrognathia in 3 (42.9%). The 3 patients that experienced severe

夽 Please citethisarticle as: CuestasG, Quiroga V,Zanetta A, GiménezE.Manejodela víaaéreaen elneonato consíndrome deMoebius.AnPediatr(Barc).2019;91:264---267.

respiratory distress underwent intubation witha Holinger anteriorcommissurelaryngoscopebyanear,noseandthroat (ENT) specialist with specific training in complex airway managementin children,followed byperformance oftra- cheotomy.

Thevideofluoroscopicswallowstudyrevealedlaryngeal penetrationinallpatientsandlowerairwayaspirationin3 (42.9%).All7patientsunderwentplacementofanasogastric tubeinthefirstdaysoflife.Allreceivedtreatmentforreflux andearlyswallowingrehabilitation.

Other findings included involvement of other cranial nerves(n=4),hypotonia(n=6),clubfoot(n=7),heartdis- ease(n=2),chestandouterearmalformations(n=4).

Themeandurationoffollow-upwas6years.Twopatients stillneedtracheostomyandgastrostomytubesandhavelan- guage delay, autism and self-harming behaviours (P1 and P3).Anotherpatient(P2)underwentdecannulationatage3 years,andatpresentcanconsumefoodsofanyconsistency bymouthandexhibitsadequatecognitivefunctioning,asdo theremainingpatients.

The clinical picture of MS is heterogeneous. Its dif- ferential diagnosis must includevarious diseases that can cause facial paralysis: neonatal trauma (use of forceps), hereditary congenital facial paresis, myotonic dystrophy, infectious diseases (otitis, herpeszoster), variouscancers orGuillain-Barrésyndrome,amongothers.

Aswasthecaseinotherpublishedcaseseries,wefounda strongassociationbetweenattemptedterminationofpreg- nancy with misoprostol and the subsequent birth of an individualwithMS.²

Earlyrehabilitationofswallowingisessential.Thecom- bination of motor rehabilitation and dietary modification allow improvement in swallowing. In most cases, eating skillsimproveasthechildgrows.Gastrostomyisindicated in cases where safe and effective oral feeding cannot be achieved.

Retromicrognathiacan causeposterior displacement of the tongue toward thepharynx (glossoptosis),obstructing the airway. Most patients respond to positional ther- apy, although extreme casesmayrequiretracheotomy. At present, jawdistractionis thefirst-linetreatmentfor this condition, as it prevents the need for tracheotomy and other,lesseffectiveproceduressuchasglossopexy.

Many children with MS require multiple surgeries, for instance,facialreconstructivesurgeryand/orsurgicalcor- rectionof strabismus.Thereis ahigh riskassociatedwith theuseofanaesthesia.⁴

Limitedmouthopening,retromicrognathia,glossoptosis andcleftpalate arefrequent featuresinpatientswithMS thathavebeenassociatedwithfailedordifficultintubation.

SCIENTIFICLETTER265

Table1 Clinicalmanifestations,craniofacialanomalies,findingsofendoscopicexaminationandvideofluoroscopyswallowstudy,andtreatmentofpatientswithMS.

P1 P2 P3 P4 P5 P6 P7

Respiratorysymptomsassociatedwithdysphagia^a

Inspiratorystridor Yes Yes Yes Yes Yes No Yes

Sleepapnoea Yes Yes Yes No No No No

Respiratorydistress Severe Severe Severe Mild No Mild No

Impairedsucking Yes Yes Yes Yes Yes Yes Yes

Chokingandcoughingonoralfeeding Yes Yes Yes Yes No No Yes

Drooling Yes Yes Yes Yes Yes Yes Yes

Craniofacialabnormalities

Micrognathia Yes Yes Yes Yes Yes Yes No

Retrognathia Yes Yes Yes No No No No

Palateanomaly Cleftpalate High-archedpalate Cleftpalate Submucouscleftpalate High-archedpalate Cleftpalate No Endoscopicfindings^b

Glossoptosis Severe Severe Severe Moderate Mild Mild No

Larynx GERsigns GERsigns LMGERsigns GERsigns LM Normal LM

Pharynx Pharyngealpooling Pharyngealpooling Pharyngealpooling Smallpharynx Normal Normal Normal

Findingsofvideofluoroscopic swallowstudy^c

Nasalpharyngealreflux Yes Yes Yes Yes Yes Yes Yes

Impairedposteriorcontainment Yes Yes Yes Yes Yes Yes Yes

Residues Yes Yes Yes Yes Yes Yes Yes

Penetration Yes Yes Yes Yes Yes Yes Yes

Aspiration Yes Yes Yes No No No No

Gastro-oesophagealreflux Yes Yes Yes Yes Yes Yes Yes

Treatment

Tracheotomy Yes Yes Yes No No No No

Pureedfood No No No Yes Yes Yes Yes

Gastrostomy Yes Yes Yes No No No No

Nissenfundoplication Yes No Yes No No No No

LM,laryngomalacia;MS,Moebiussyndrome;P,patient.

GERsigns:indirectsignsofgastro-oesophagealreflux(arytenoidswellingandredness,congestionofthemucosaoftheposteriorpharyngealwall).

a InvolvementofIXcranialnerveinP2andP4,IX,XandXIIcranialnervesinP1andIX,X,XIIandVIIIcranialnervesinP3.

b Vocalcordmovementwasnormalinallpatients.

c Impairedposterior containment(bolusinhypopharynxmorethan2safterstartofpharyngealphase),presenceofresidues(in pyriformsinuses,valleculaandpharyngealwalls), penetration(entryofboluscontentstolaryngealvestibule)andaspiration(passageofboluscontentstolowerairway).

266 SCIENTIFICLETTER

Figure1 Newbornwithbilateralfacialpalsyatrest(A)andduringcrying(B):(A)incompleteclosureofeyelids(lagophthalmos).

(B)Evidentfacialasymmetryduringcrying.‘‘Maskface’’withpartialconservationoffunctionofrightinferiorfacialmusculature (deviationofhomolateralcornerofthemouth).(C)Samepatient featuredinA andB atage3monthswithnasogastricanda tracheostomytubes.Evidenceofesotropiaandlowpositionoftheears.(D)Bilateralclubfoot.

Inthispatients,spontaneousventilation shouldbeconsid- eredwithavoidanceofmusclerelaxants.Facemaskswith Guedelairwaysmaybeuseful.Laryngealmaskairwaysand flexiblebronchoscopy arealternative methodsthatshould beavailabletopatients.^4---6

ThemanagementofchildrenwithMSrequiresamultidis- ciplinaryteam.Earlyrehabilitationisessentialtooptimise therecoveryofthedifferentimpairedfunctionsandimprove outcomesandqualityoflifeinthesepatients.

References

1.PiccioliniO,PorroM,CattaneoE,CastellettiS,MaseraG,Mosca F,etal.Moebiussyndrome:clinicalfeatures,diagnosis,manage- mentandearlyintervention.ItalJPediatr.2016;42:56.

2.JotzGP,SchwarzK.TheetiologyofMöbiussyndrome:asocial problem?IntArchOtorhinolaryngol.2014;18:227---8.

3.HobaikaAB,NevesBS,FernandesML,GuedesVC.Anesthesiaina patientwithmoebiussequence:casereport.RevBrasAnestesiol.

2009;59:341---3.

SCIENTIFICLETTER 267

4.ArpaciH,KadiogluM,Tuzuner-OnculA.Anestheticmanagement ofacasewithMoebiussyndrome.IntJExpDentSci.2012;1:37---9.

5.Budi´cI, ˇSurdilovi´cD,Slavkovi´cA,Marjanovi´cV,Stevi´cM,Simi´c D.Moebiussyndrome:challengesofairwaymanagement.Acta ClinCroat.2016;55Suppl.1:S94---7.

6.Poveda R. Secuencia de Moebius en anestesiología. Rev Chil Anest.2012;41:140---3.

GiselleCuestas^a,∗,VíctorQuiroga^b,AdriánZanetta^b, ElianaGiménez^b

aSeccióndeEndoscopiaRespiratoria,Divisiónde

Otorrinolaringología,HospitalGeneraldeNi˜nos«Dr.Pedro deElizalde»,BuenosAires,Argentina

bServiciodeOtorrinolaringología,HospitalInterzonal EspecializadoMaternoInfantil«DonVictorioTetamanti», MardelPlata,BuenosAires,Argentina

∗Correspondingauthor.

E-mailaddress:[email protected](G.Cuestas).

2341-2879/

©2019PublishedbyElsevierEspa˜na,S.L.U.onbehalfof Asociaci´onEspa˜noladePediatr´ıa.Thisisanopenaccessarticle undertheCCBY-NC-NDlicense(http://creativecommons.org/

licenses/by-nc-nd/4.0/).

Surveillance of multiresistance:

Vancomycin-resistant Enterococcus spp. ^夽

Vigilancia de multirresistentes: Enterococcus spp. resistente a vancomicina

DearEditor:

Multidrug-resistantbacteriaarea significantpublichealth problem due tothe limited treatment alternatives, their capacity for epidemic spread from colonized individuals andthepossibilityofhorizontaltransmission,includingthe emergence of outbreaks. In 2007, the Sociedad Espa˜nola deMicrobiologíaClínicayEnfermedadesInfecciosas(Span- ishSocietyofClinicalMicrobiologyandInfectiousDiseases) publishedadocumentonsurveillancecultureofmultidrug- resistant organisms relevant in nosocomial infections, which was updated in 2015.^1,2 Each hospital establishes strategies for the active detection of colonization by multidrug-resistantbacteriaadaptedtolocalepidemiolog- ical characteristics, although a general protocol for this purposehasalsobeenpublished.³Giventhelowprevalence ofvancomycin-resistantenterococcus(VRE)speciesinSpain andthefactthatnostrainswiththisphenotypehadbeen isolatedinourhospital,VREwasexcludedfromourinitial surveillance protocol. Data fromthe European Antimicro- bialResistanceSurveillanceNetwork(EARS-Net)from2014 showedaproportionof7.9%(95%CI,6---11%)ofvancomycin resistanceininvasivestrainsofEnterococcusfaecium,with a significant increase between 2011 and 2014. In Spain, theproportionwasof2.4%(95%CI,1---4%),without signifi- cantchangesinthe2011---2014period.⁴Thesedatareferto Enterococcusfaeciumbecauseinthisspeciesresistanceto glycopeptidesiscompoundedbya highlevelof resistance to ␤-lactam agents (infrequent in Enterococcus faecalis), whichrestrictsthetreatmentalternativesevenfurther.The

夽 Pleasecitethisarticle as:González-AbadMJ,AlonsoSanz M.

Vigilanciademultirresistentes:Enterococcusspp.resistenteavan- comicina.AnPediatr(Barc).2019;91:267---268.

aimofourstudywastodescribethecorrectivemodification ofourprotocolfollowingtheisolationofthefirststrainsof VRE.These strains were detected by rectal swab culture in a chromogenic medium (Brilliance^TM VRE Agar, Oxoid) withconfirmation of their identity and antimicrobial sus- ceptibilitytestingbybrothmicrodilution(Vitek^®2Compact, bioMeriéux)andtheE-test(E-test^®,Oxoid).Wesubmitted theVREisolatesinvolvedininfectionorsuspectedcontagion totheInstitutodeSaludCarlosIII(ISCIII)forgenotypingand investigationofmolecularepidemiology.Between2007and June2018,therewere109casesofbacteraemiaduetoEnte- rococcusspp. in 102patients atthe HospitalInfantilNi˜no JesúsofMadrid.TwoE.faeciumisolates(2017)wereresis- tanttovancomycin(vanAphenotype).Theseisolateswere obtained 9 days apart from immunosuppressed patients hospitalisedinthesameroom.Theminimuminhibitorycon- centrations (MICs) of teicoplanin in these patients were 32 and64mg/L,and the MICs of daptomycin were 2 and 4mg/L, while the MIC for vancomycin was greater than 256mg/Lforbothpatients.Thegeneticprofilesoftheiso- lateswereidentical.TheactivesearchofVREthroughJune 2018resulted indetectionof8 casesofcolonization (in7 cancer patients and1 patient withhydrocephalus staying inthePICU).Threeof thecasesof VREcolonizationwere detected8,16 and17daysafter theidentification ofthe first case of bacteraemia due to VRE in cancer patients hospitalised in the same room as the patients with bac- teraemiaduetoVRE.Oneisolatecorrespondedtoastrain thatwasgeneticallyrelatedtotheisolatesinpatientswith bacteraemia, andthe geneticprofileof another coloniza- tionisolatewasprobably alsorelatedtothe bacteraemia isolates.After the inclusion of VRE in our active surveil- lanceprotocol, wefound arateof colonizationby VREof 1%(April2017---June2018).Therehave beennoadditional outbreaksorcasesofcolonizationsinceFebruary2018.The magnitudeofthemultipledrugresistancephenomenonhas compelledmajorhealthcarefacilitiestoinvestinthecon- tainmentoftheseorganismsthroughtheearlydetectionof carriagestatus.⁵Inlinewiththis,in2014themicrobiology laboratoryofourhospitalintroducedroutinesurveillanceof themicroorganismswiththehighestclinicalandepidemio- logicalimpact(methicillin-resistantStaphylococcusaureus and extended-spectrum ␤-lactamase- or carbapenemase- producing Enterobacteriaceae)³ in patients likely to have