Rev. Bras. Anestesiol. vol.64 número2

RevBrasAnestesiol.2014;64(2):128---130

REVISTA

BRASILEIRA

DE

ANESTESIOLOGIA

CLINICAL

INFORMATION

Anesthesia

for

a

child

with

Walker---Warburg

syndrome

Emine

Arzu

Kose

,

Bulent

Bakar

,

Gokay

Ates

,

Didem

Aliefendioglu

,

Alpaslan

Apan

a_{DepartmentofAnesthesiologyandReanimation,SchoolofMedicine,KirikkaleUniversity,Kirikkale,Turkey} b_{DepartmentofNeurosurgery,SchoolofMedicine,KirikkaleUniversity,Kirikkale,Turkey}

c_{DepartmentofNeonatology,SchoolofMedicine,KirikkaleUniversity,Kirikkale,Turkey}

Received30October2012;accepted19December2012 Availableonline11October2013

KEYWORDS Walker---Warburg Syndrome;

Congenitalmuscular

dystrophy;

Generalanesthesia

Abstract

Backgroundandobjectives: Walker---Warburg Syndrome is a rare, autosomal recessive

con-genital muscular dystrophy manifested by central nervous system, eye malformations and

possiblemultisysteminvolvement.Thediagnosisisestablishedbythepresenceoffour crite-ria:congenitalmusculardystrophy,typeIIlissencephaly,cerebellarmalformation,andretinal

malformation.Mostofthesyndromicchildrendieinthefirstthreeyearsoflifebecauseof

respiratoryfailure,pneumonia,seizures,hyperthermiaandventricularfibrillation.

Casereport: Theanestheticmanagementofatwo-months-oldmalechildlistedforelective ventriculo-peritonealshuntoperationwasdiscussed.

Conclusions:A careful anesthetic management is necessary due to the multisystem

involvement. We reported anesthetic management of a two-months-old male child with

Walker---WarburgSyndromewhowaslistedforelectiveventriculo-peritonealshuntoperation. ©2013SociedadeBrasileiradeAnestesiologia.PublishedbyElsevier EditoraLtda.Allrights reserved.

Introduction

Walker---Warburg Syndrome (WWS) is a rare, autosomal

recessivecongenitalmusculardystrophy(CMD).1_The

diag-nosisisgenerallydependsonclinicalmanifestations,genetic diagnosis has been made by DNA analysis only in 10---20% ofthecases.The diagnosis isestablished bythepresence offourcriteria:CMD,typeIIlissencephaly,cerebellar mal-formation,andretinalmalformation.2_{Theotherfrequently}

∗_{Correspondingauthor.}

E-mail:[email protected](E.A.Kose).

observed abnormalities are ventricular dilatation with or without hydrocephalus, anterior chamber malformation of eye, congenital macrocephaly, Dandy---Walker malfor-mation. Congenital microcephaly, microphthalmia, ocular coloboma,congenitalcataract,urogenitalanomalies,cleft lipandcleftpalatearethelessfrequentlyobserved abnor-malities.DifferentialdiagnosiswithFukuyamaCMD, muscle-eye-brain disease, cerebro-ocular-cerebral syndrome is depend on theseverity of theclinical manifestations and radiologicalfindings.2,3_{Clinicalmanifestationarepresentat}

birthororappearsafterinashortperiod.Mostofthe syn-dromicchildrendiesinthefirstthreeyearsoflifebecauseof respiratoryfailure,pneumonia,seizures,hyperthermiaand

0104-0014/$–seefrontmatter©2013SociedadeBrasileiradeAnestesiologia.PublishedbyElsevierEditoraLtda.Allrightsreserved.

AnesthesiainWalker---Warburgsyndrome 129

Figure1 FLAIRMRscanimagesdemonstratingDandy---Walkerabnormality(AandD);andlissencephalywithhydrocephalus(B). T2WIMRscanimagedemonstratingseptumpellucidumdysgenesis(C).

ventricularfibrillation.Prenataldiagnosisispossibleandthe riskofhavinganotherchildwiththissyndromeis25%inthe familieswithoneaffectedchild.4

Case

report

A two-months-old male child weighing 3.3kg was listed forelectiveventriculo-peritonealshuntoperation.Delivery hadbeen bycesareansection atthe38thweeksof gesta-tion.The mother’sprevious gestation had been endedby medicalabortusduetodeterminedhydrocephalusby ultra-sonographicevaluationsandtheparentswererelative.The patient was admitted to intensive care unit of newborn becauseof hydrocephalusand respiratorydistress. Macro-cephalia,lownasalbridge,frontalbulgingoftheforehead, micrognathia,recedingmandible,moderatehypotonia,and bilateral retinaldetachment weredetermined byphysical

examination. Creatine phosphokinase (CPK) levels were measuredas4226uL−1_{(normal24---195uL}−1_).Magnetic res-onance imaging (MRI) was revealed type II lissencephaly, hypomyelinationofwhitematter,dilatedventricles, bilat-eral cerebellar hypoplasia, agesis of septum pellucidum, Dandy---Walker malformation, bilateral hyperplasia of vit-reusandbuphthalmus(Fig.1).

130 E.A.Koseetal.

4.Therefore,thepatient’sheadwasslightlyturnedtoleft andMillersizezerostraightbladewasintroducedextreme rightsideofthemouth.Thetipoftheepiglottisvisualized bytheaid of theexternal manipulationof thelarynxand blindintubationwasperformedbyusinga3.5mmuncuffed endotrachealtube. The endotrachealtube placementwas confirmedwithcapnographyandauscultationoflungs.The endotracheal tube wasfixed and an oropharyngeal throat packwasinserted.Anesthesiawasmaintainedwith sevoflu-rane2%withoxygenandnitrousoxidemixtureintheratio of 50:50. Neither neuromuscular blocker agents nor opi-oidswereusedduringthesurgery. Fluidmanagementwas made intravenous administration of balanced electrolyte solutionregardingthecalculationoftheperioperativefluid deficitsandbasalrequirementofthechild.Attheendofthe surgery,thethroatpackwasremoved,andthetracheawas extubatedwhenthepatientwasfull-awake.Postoperative analgesiawasprovidedwithparacetamol25mgkg---1_.Aftera uneventfulrecoveryperiod,thepatientwastakenintothe intensivecareunit.

Discussion

Acarefulanesthetic managementis necessary due tothe multisystem involvement. Difficult airway should always keepinmindbecauseofshortneck,micrognathia, reced-ingmandibleandsometimesaccompaniedcleft lipand/or cleft palate. After the difficult intubation preparation, we preferred inhalation induction with sevorane without suppressionofspontanventilation.Duetotheintrusion pos-sibilityofacurvedbladeintotheviewline,Millersizezero straight blade was used but visualization of larynx could notbeprovidedbythetraditionalmidlineapproach. There-fore,paraglossal straight blade laryngoscopy described in difficult tracheal intubation was used and blind intuba-tion was performed after visualization of the tip of the epiglottis.5 _{Lateral approach was provided to bypass the}

tongueandmaxillarystructuresandimprovedviewofthe glottis.Incaseofunsuccessfulintubation,ourback-upplan wastoperformfiber-opticlaryngoscopy.The useof laryn-geal mask airway was discussed but was not preferred becausetoprovide asafeairwaycouldbe impossibledue to the movements of head during the operation and the probabilityof theaspirationof theoropharyngeal secreti-ons.Tousethesuccinylcholinewasavoidedbecauseitcan causeexaggeratedpotassiumrelease,fatalcardiac dysrhyt-miasand malign hyperthermia. Because of the increased

hyperthermiariskinWWS,bodytemperaturewasmonitored duringthesurgeryandpostoperativeperiod.2,3

Nondepolar-izingmusclerelaxantsandopioidswerenotusedduetothe possibilityofdelayedrecoveryandpostoperativerespiratory depressionresultingfromthechronicweaknessof respira-torymuscles.2,3_{Postoperativeanalgesiawasprovidedwith}

paracetamol, heavy sedation was avoided. Postoperative pulmonarydysfunctionandconsequentaspiration pneumo-niawaskeptinthemindandoropharyngealsecretionswere suctionedregularly.

Although,ourpatienthadnotanycardiacor genitouri-nary abnormality, renal dysfunction and involvement of cardiacmusclescanobserveinWWSanditisimportantto recognizetheincreasedperioperativecardiacandrenal fail-urerisk.2_{Maintenanceofadequatefluidmanagementshould}

beprovidetoavoidtherenaldysfunctionandcardiovascular depression.

ChildrenwithWWSmayhaveincreasedintracranial pres-sure (ICP), so anesthetic management should include the precautionstoprovideadequatecerebralcirculationsuchas toavoidtheexcessivedecreaseorincreaseinmeanarterial pressure.3_{Itisalsoimportanttorecognizethatthepatients}

withWWSarepronetodevelopseizures,episodesofcentral andobstructiveapnea, difficultyinswallowingand conse-quent cardiorespiratoryfailure,aspirationpneumonia and sepsis.2,3 _{It should be avoid the use of the epileptogenic}

drugsandhypoxicepisodeswhichcanleadtoseizures. In summary, for an unevenful anesthetic management andpostoperativecareitshouldbekeepinmindthatWWS isasevereCMDwithmultisystemicinvolvement.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

References

1.Walker AE. Lissencephaly. Arch Neurol Psychiatry. 1942;48:13---29.

2.DobynsWB,PagonRA,ArmstrongD,etal.Diagnosticcriteriafor Walker---Warburgsyndrome.AmJMedGenet.1989;32:195---210.

3.Sahajanda H, Meneges J. Anaesthesia for a child with Walker---Warburgsyndrome.PediatrAnesth.2003;13:624---8.

4.CroweC,JassaniM,DickermanL.Theperinataldiagnosisofthe Walker---Warburgsyndrome.Diagnosis.1986;6:177---85.