Rev. Bras. Anestesiol. vol.66 número1

RevBrasAnestesiol.2016;66(1):72---74

REVISTA

BRASILEIRA

DE

ANESTESIOLOGIA

CLINICAL

INFORMATION

Role

of

noninvasive

ventilation

in

perioperative

patients

with

neuromuscular

disease:

a

clinical

case

Ana

Marinho,

Mario

José

Guimarães,

Neusa

Cristina

Ribeiro

Lages

_,

_Carlos

_Correia

CentroHospitalardoAltoAve,HospitaldeGuimarães,Fafe,Portugal

Received5March2013;accepted10June2013

Availableonline6April2014

KEYWORDS

Noninvasive ventilation; Locoregional anaesthesia; Inclusionbody myositis

Abstract Theinclusionbodymyositisisaninflammatorymyopathythatleadstochronicmuscle inflammationassociatedwithmuscleweakness.Itischaracterizedbyarestrictiveventilatory syndromerequiringventilatorysupportundernon-invasiveventilation.The authorsdescribe aclinicalcaseandthe anaestheticmanagementofapatientwith inclusionbodymyopathy candidateforvertebroplasty,whichhighlightstheimportanceoflocoregionalanaesthesiaand ofnoninvasiveventilationandincludesassistedcoughtechniques,maintainedthroughoutthe perioperativeperiod.

©2014SociedadeBrasileiradeAnestesiologia.PublishedbyElsevier EditoraLtda.Allrights reserved.

PALAVRAS-CHAVE

Ventilac¸ãonão invasiva; Anestesia locorregional; Miositeporcorposde inclusão

Papeldaventilac¸ãonãoinvasivanoperíodoperioperatóriodedoentescompatologia neuromuscular:casoclínico

Resumo A miosite por corpos de inclusão é uma miopatia inflamatória que cursa com inflamac¸ãocrônicamuscularassociadaàfraquezamuscular.Caracteriza-seporumasíndrome ventilatóriarestritivacomnecessidadedesuporteventilatóriosobventilac¸ãonãoinvasiva.Os autoresdescrevemcasoclínicoerespectivomanuseioanestésicodepacientecommiopatiapor corposdeinclusãopropostaparavertebroplastiaquerealc¸aaimportânciadaanestesia locor-regionaledaventilac¸ãonãoinvasivaeincluiastécnicasdetosseassistida,mantidasdurante todooperíodoperioperatório.

©2014SociedadeBrasileiradeAnestesiologia.PublicadoporElsevierEditoraLtda.Todosos direitosreservados.

∗_{Correspondingauthor.}

E-mail:lages.neusa@gmail.com(N.C.R.Lages).

Introduction

Neuromuscular diseases (NMDs) are a challenge to inva-siveprocedures,thankstotherespiratoryrisktheyentail. Thedecreaseinrespiratorymusclestrength,theineffective

Roleofnoninvasiveventilationinperioperativepatientswithneuromusculardisease 73

coughandaccumulationoforopharyngealsecretionsimply high risk of failure in tracheal extubation after posi-tivepressureventilation inpatients withNMD. Therefore, these patients are considered at high risk for general anaesthesia.1,2 _{Theyarealso usuallypatients with}

associ-atedcomorbidities,whichmakesthempreferredcandidates forregionalanaesthesiatechniques.3

The inclusion body myositis is a rare disease that is part of a group of muscle diseases known as inflamma-torymyopathies,whicharecharacterizedbychronicmuscle inflammationassociatedwithmuscleweakness.Decreased muscle strength is usually progressive (occurs gradually over months or years)and affects both proximaland dis-tal muscles, andcan affect onlyhalf the body.It is more frequentinmalesanditssymptomsusuallybeginafterage 50.Thefamilialformsusuallyoccurinchildhoodandshow no inflammatory changes. Both forms have intracytoplas-micandintranuclearinclusions inmuscletissue.4_Thereis

neitheracureforthisdisease,noraclearroutineofcare. The involvementoftherespiratorymuscles, character-ized by a restrictive ventilatory syndrome of progressive evolution,leads totheneed forventilatorysupportunder non-invasiveventilation(NIV).Giventheacknowledged effi-cacyofNIVassociatedwiththetechniquesofassistedcough in NMD, these should be continued in the perioperative periodinpatientswhowerepreviouslyunderNIVandthus simplifybothsurgeryandtheanaestheticact.5_The

mechan-ical ventilation ensures better stability of the breathing physiology,withfulfilmentofgasexchange,thereby reduc-ingtheeffectsofsurgicalstress.4

Clinical

case

Femalepatient,71yearsold,accompaniedinlung consulta-tionduetoinclusionbodymyositis,withanaestheticriskASA III(concomitantcomorbidities:hypertension,atrial fibrilla-tion);ventilationabout fiveyearsagowithBiPAPat home (18h/day), with indication for percutaneous kyphoplasty in reason of osteoporotic (or post-traumatic) fracture of T11---T12.

Consideringthatourswasapatientathigh anaesthetic risk,givenherlungfunction(forcedvitalcapacity=1.05L, withlossof22%withdorsaldecubitumandpeakcoughflow of120L/mincough---severerestrictivesyndromewithfrank reductionofpeakcough),weoptedinitiallyforamedical treatmentwithanalgesiaandimmobilizationinhospitalfor 12days.

Despite this treatment, the patient persevered with intensepaincomplaints;thus,afteramultidisciplinary dis-cussion of her clinical case, we decidedto proceed with percutaneouskyphoplastyunderintraoperativeNIV,withthe supportofmechanicalin-exsufflatortoensuretheremoval ofsecretionsthatcouldeventuallyariseduringsurgery.

To improvetheconditionsin thepreoperative,assisted cough was initiated and taught to the nursing staff and to patient’s relatives in the week before surgery, with improvement in ventilatory parameters according to noc-turnaloximetryandarterialbloodgas.

Then,thesurgerywasperformed underthoracic epidu-ral anaesthesia, at the level T12-L1, with administration of 60mg of 0.75% ropivacaine. A higher level of superior

sensory block in T7 was achieved, and the surgery pro-ceededwiththepatient positionedin ventraldecubitum. Intheoperatingtheatrenotonlywerepresentthemedical teamsoforthopaedicsandanaesthesiology,butalsoof pul-monology,tosafeguardtheeventualpossibilityofarequired intubationbyfibreopticbronchoscopy(inreasonofher posi-tioning)andconsequentinvasiveventilation.

ThroughoutthesurgerythepatientremainedunderNIV by bi-level positive airway pressure (BiPAP) with a nasal mask,atpressuresof5cmH2Oforexpiratorypositive air-waypressure(EPAP)and18cmH2Oforinspiratorypositive airwaypressure(IPAP)inspontaneous/timed(ST) module, withnoneedforadditionalassistedcoughtechniques intra-operatively. The patient always remained without pain, hemodynamicallystable, andwith noepisodesof desatu-ration, shortness of breath or accumulation of secretions alongabout120minofdurationoftheprocedure.Itis note-worthythat there wasnoneed for supplemental oxygen. The peripheral oxygen saturation always remained above 97% under room air ventilation. In the Post-Anaesthetic CareUnit,due todesaturation andthe patientcomplaint ofa feeling of presence of bronchial secretions,we used theassistedcough.Theepisodereversedimmediatelyafter removing the plug of mucus secretions. In the absence ofadditionalcomplications,thepatientwasdischargedto inpatientorthopaedicswardafterafewhoursofrecovery, andduringthistimesheremaineduneventfuluntilthedate ofherhospitaldischarge.

Preventivemeasuresontheambulationofthepatientat homeweretaken.Theuseofawheelchairwasencouraged andthepatientmaintainedclinicalcriteriaforthe contin-uoususeofassistedcough techniquesathome.Abouttwo weeksafterthesurgery,thepatientwasreassessedinalung consultation.

Discussion

Thepercutaneouskyphoplasty/vertebroplasty,aminimally invasive technique usedin the treatment of osteoporotic fracturesandosteolytic tumoursofvertebralbodies, con-sists of a percutaneous introduction of a balloon into theaffected vertebra;its inflation andsubsequent defla-tion/removalcreatesavertebralspacethatissubsequently filledwithacrylic or biologicalcement.5 _{This isa surgical}

procedure that involves the placement of the patient in aprone position,and usually theprocedure is performed undergeneral anaesthesia.5 _{Over time, withthe}

increas-ingexperienceoforthopaedistswiththistechnique,ithas beenpossiblein manycasestoreduce thetimenecessary forperformingthistypeofsurgery. Italsohasbeen possi-bletoadjustthe anaesthetictechnique associated.Thus, if initially nearly all patients were operated under gen-eralanaesthesia,recentlythesedationhasemergedasan increasinglyusedoption,althoughinvariablythistechnique needs to be supplemented by a good local anaesthesia, particularlyfortheperiosteum.5_{Nonetheless,thereare}

spe-cifictimes whenpatientsendupfeelingsome discomfort, especiallywithintroductionoftrocars,ballooninflationand injectionofcement.5

74 A.Marinhoetal.

stilluncommon in thelocalhospital (andthereforehighly likelytotakemoretime),generalanaesthesiaandsedation werediscardedasoptions.Itwasthenproposeda neurax-ialblockadethat,inthiscontext,isparticularlyinteresting: thistechniqueavoidsthemanipulationoftheairwayandthe useof invasiveventilation. Therefore,offersclear advan-tages,bothinintra-andpostoperativephases,forpatients withNMD and with involvement of underlying respiratory muscles.Recently,Souvatzisetal.reportedacaseseriesof patientsundergoingkyphoplasty undersubarachnoidblock (SAB).By beinglimitedin time,SAB is ineffectivein pro-longedsurgicalprocedures.5_{Buttheepiduralblock,thanks}

tothepresenceofacatheter,allows,ontheonehand,to overcomethissetbackofalongersurgerythananticipated and,ontheotherhand,theintraoperativeneedfor kypho-plastyonadjacentvertebraeandallowsanadjustmentfor theadequateanaestheticlevel.Additionally, althoughnot describedintheirpaper,thedoseoflocalanaestheticused bySouvatzisetal.usuallylimitstheoccurrenceofmore sig-nificanthaemodynamicalterations,comparedtothecaseof epiduralanaesthesia---andthisisaninstabilitytobeavoided inpatientsathighanaestheticrisk.

Thepropositionofthisanaestheticprocedure,alongwith ventilationsecuredbyNIV,enabledourpatientwithNMDto chooseatherapeuticoptionthatallowedtheimmediate res-olutionofherclinicalcondition.Thepatientwasdischarged fromhospitalonthethirdpostoperativeday,walkingwith supportandwithoutpain.Otherwise,theoptionswould con-sist of conservative treatment (immobilization and all its associated risks),or kyphoplasty under general anaesthe-siaunderinvasiveventilation,whichwouldinvolveintensive careinthepostoperativeperiodandeventualneedfor tra-cheostomyanddecannulation, entailingalongerperiodof hospitalizationandconsequenthigherriskofnosocomial dis-ease.

Themultidisciplinaryevaluationandmonitoring through-outthe perioperativeperiod,includingduringthesurgical procedure,werecriticaltothetherapeuticsuccess,because situations of eventual need for invasive ventilation or of haemodynamicinstabilitythatcouldarisewereguaranteed andsafeguarded.

One should also emphasize the importance of the preoperative improvement of the underlying respiratory dysfunction in this type of patient. Throughout the

perioperative period, the assisted-cough techniques con-tributetominimizetheriskofrespiratorycomplicationsto whichthesepatientsaresubjectto.Inthecasepresented, therewasnoneedfortheuseofassistedcoughtechniques duringsurgery,whichwasattributedtotheextensiveuseof thistechniqueinthepreoperativeperiod.

Withthereassessmentofthepatientonthesecond post-operativeweek,itwaspossibletoconcludethatherquality of life was superior than that presented before the frac-ture/surgicaltreatment,andthisclearlydemonstratesthe benefitsoftheclinicaloptionschosen.

Conclusion

Byavoidingtheairwaymanipulation,theepidural anaesthe-siaoffersmanyadvantagesforpatientswithneuromuscular pathologyinneedofsurgery.

Themultidisciplinaryapproachandtheuseoftechniques ofventilatorysupportensureabetterqualityoflifeforthese patients;therefore, theseshouldbepromotedthroughout theperioperativeperiod.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

References

1.VianelloA,ArcaroG,BraccioniF,etal.Preventionofextubation failureinhigh-riskpatientswithneuromusculardisease.JCrit Care.2011;26:517---24.

2.WhitneyJ,HardenB,KeiltyS.Assistedcough:anewtechnique. Physiotherapy.2002;88:201---7.

3.RobinsonP,DouglasJ,FootC.Respiratorymanagementofadult patientswithprogressive neuromusculardisease: non-invasive ventilationandtheroleoftheintensivist.CurrAnaesthCritCare. 2007;18:237---51.

4.AdamD.Theinfectionsandinflammatorymyopathies.In:Ropper AH,SamuelsMA,editors.Principlesofneurology.6thed.New York:McGraw-Hill;1997.p.1409---10.