QUIZ22ndFeb2017

1. HowdoyoudiagnoseKawasaki’sdisease?

2. WhatisthemanagementofKawasaki’sdisease?

3. Wherecanyouseeextra-axialhaemorrhageonCTbrain?

4. Howdoesafasciailiacablockwork?

5. DescribeandinterpretthefollowingECGfroma37yearoldmanwithsyncope.

QUIZanswers22ndFeb20171.HowdoyoudiagnoseKawasaki’sdisease?Kawasaki’sdiseaseisa(mediumsizedarterial)vasculitis,overwhelminglyofchildhood.Thenaturalcourseisself-limitingfeverandmucocutaneousinflammationlastinganaverageof12daysifuntreated.Therearesignificantcomplicationsthatmayoccurfromcoronaryarteryinvolvementleadingtocoronaryaneurysmsandocclusionscausingarrhythmias,infarctsandheartfailure.Peripheralarterialocclusionscanalsooccur.Treatmentwithin10daysreducestherateofcomplications,placingimportanceonearlydiagnosis.Diagnosisisclinical,basedonevidenceofsystemicinflammation(fever)alongwithmucocutaneousinflammation.

1. Fever≥5days-typically>38.5withpoorresponsetoantipyretics

2. Fouroutoffiveofthefollowing(withoutalternateexplanation)

a. Bilateralnon-purulentbulbarconjunctivitis(Fig.1)

- >90%ofcases- Classicallylimbicsparing- 70%haveassociateduveitis

b. Oralmucousmembranechanges- Crackedredlips- Strawberrytongue(Fig.2)- NOTdiscretelesions,ulcersor

tonsillarexudatec. Peripheralextremitychanges–lastsigns

toappear- Erythemaofpalmsorsoles- Oedemaofhandsorfeet

(early)- Periungualdesquamation

(late)d. Polymorphousrash

- Infirstfewdays- Oftenstartsatperineumbeforespreading

e. Cervicallymphadenopathy–atleast1node>1.5cm- Oftenonelargeanteriorcervicalnodefelt- Notdiffuselymphadenopathyelsewhereinbody

Thesecriteriaweredescribedbeforecardiacinvolvementwasrecognised.Wenowknowthat~10%childrenthatdevelopcoronaryarteryaneurysmsdon’tmeetthefullcriteria.ThisiscalledincompleteKawasaki’sdisease.Othersymptomscanalsooccurbutarenotspecific–vomiting,diarrhoea,cough,coryza,irritabilityandjointpain.

Figure1ConjunctivitisofKD

Figure2Strawberrytongue

Supportinglaboratoryfindings:

• Elevatedacute-phasereactants(CRP≥3mg/dLorESR≥40mm/hour)• WBCcount≥15,000/microL• Normocytic,normochromicanaemia• Plateletcellcount≥450,000/microLaftersevendaysofillness• Sterilepyuria• Serumalanineaminotransferaselevel>50units/L• Serumalbumin≤30g/L

IncompleteKawasaki’sdiseasemaybepresentinchildrenhavefever≥5dayswithlessthan4signsofmucocutaneousinflammation.Theyarestillatriskforcoronaryarterychanges.Itseemsthatthemucocutaneouschangesarethemostcommonlypresentchangesandcervicallymphadenopathymostcommonlyabsent.IncompleteKawasaki’sdiseaseismostcommonunder6monthsofage,andshouldbeconsideredandinvestigatedforinanyunexplainedfever≥7daysinthisagegroup.TheAmericanHeartAssociationandAmericanAcademyofPediatricshasanalgorithmtoassistthediagnosisofincompleteKawasaki’sdiseasethatinvolveslaboratoryvalues,ECHOandclosefollowup;

ΔPatientcharacteristicssuggestingdiseaseotherthanKDincludeexudativeconjunctivitis,exudativepharyngitis,discreteintraorallesions,bullousorvesicularrash,orgeneralizedadenopathy.Consideralternativediagnoses.Pediatrics,Vol.114,Pages1708-33,Copyright©2004bytheAAP.SundelRUpToDateKawasakiDisease;InitialTreatmentandprognosisJune2016

2.WhatisthemanagementofKawasaki’sdisease?Thefrequencyofcoronaryarteryaneurysmshasbeendramaticallyreducedasaresultofimmuneglobulintherapy.Thebenefitforpatientswhoarealreadydevelopedaneurymsismoreequivocal.AllchildrendiagnosedwithKawasaki’sorincompleteKawasaki’sdiseasearetreatedatthetimeofdiagnosis,aswecannotclearlystratifytheriskofdevelopingcoronaryarterychanges.Initialtreatment

• IVimmuneglobulin(2g/kg)infusionover8-12hours• Aspirin3-5mg/kg/day

Someadministerhighdoseaspirin(30–50mg/kg)forfirstfewdaysbutunclearbenefitAppropriatepaediatricfollowupwithatleastanotherECHOat6-8weeks.SundelRUpToDateKawasakiDisease;InitialTreatmentandprognosisJan2017

3.Wherecanyouseeextra-axialhaemorrhageonCTbrain?Extra-axialisadescriptivetermtodenotelesionsthatareexternaltothebrainparenchyma,incontrasttointra-axial,whichdescribeslesionswithinthebrainsubstance.Extra-axialhaemorrhageisthereforeextradural,subduralorsubarachnoid.Bloodintheacutephaseishyperdense.Extraduralhaemorrhageisseenasalensshapedsubperiostealopacification,commonlyovertheareaofthemiddlemeningealartery.Itislimitedbycranialsutures.Subduralhaemorrhageisacrescentshapedopacificationacutely,betweentheskullandthebrain,notlimitedbysuturelines.Itdoesn’tenterthesulcibutthesulcimaybeeffacedonthesideofthehaemorrhage.SubarachnoidbloodcanpassintoanyoftheCSFspaces,andsomaybeseeninthesulci,fissures,ventriclesandbasalcisterns.www.radiopaedia.org

4.Howdoesafasciailiacablockwork?Fasciailiacablockisalow-techalternativetoafemoralnerveoralumbarplexusblock.Themechanismbehindthisblockisthatthefemoralandlateralfemoralcutaneousnerveslieundertheiliacusfascia.Therefore,asufficientvolumeoflocalanestheticdepositedbeneaththefasciailiaca,evenifplacedsomedistancefromthenerves,hasthepotentialtospreadunderneaththefasciaandreachthesenerves.Traditionally,itwasbelievedthatthelocalanestheticcouldalsospreadunderneathfasciailiacaproximallytowardthelumbosacralplexus;however,thishasnotbeendemonstratedconsistently.Thenon-ultrasoundtechniqueinvolvedplacementoftheneedleatthelateralthirdofthedistancefromtheanteriorsuperioriliacspineandthepubictubercle,usinga"double-pop"techniqueastheneedlepassesthroughfascialataandfasciailiaca.However,blocksuccesswiththis"feel"techniqueissporadicbecausefalse"pops"canoccur.Theultrasound-guidedtechniqueisessentiallythesame;however,monitoringoftheneedleplacementandlocalanestheticdeliveryassuresdepositionofthelocalanestheticintothecorrectplane.NewYorkSchoolofRegionalAnaesthesiawww.nysora.com

5.DescribeandinterpretthefollowingECGfroma37yearoldmanwithsyncope.Sinusrhythm70/minPwaves

• UprightinII,thereforelikelysinusinorigin• BiphasicinV1–maybeleftatrialenlargement

PRintervalisnormalQRS

• Narrow• Normalaxis• RBBBconfiguration

STsegments• SaddleshapedSTelevationinV2~1-2mm

TwavesnormalImpressionSaddleshapedSTelevationinV2suspicousforBrugadaType2(>2mm)orType3(<2mm)Noteitsays“correctposition”ontheECG.ThiscouldbebecauseanotherECGwasperformedwiththeleadsV1-3placedinaBrugadaposition(2ndand3rdintercostalspaces)toincreasethechanceofseeingBrugadapatterns.

Type1CoveshapedSTelevation>2mm

Type2SaddleshapedSTelevation>2mmwithbottomofsaddle>1mm

Type3Coveorsaddleshapedbut<2mm

http://www.csanz.edu.au/documents/guidelines/clinical_practice/Brugada_Syndrome.pdf

ThisECGwasfroma37yearoldmanwhopresentedwithsyncopeandwithahistoryofuninvestigatedsyncope.HisECGabnormalitywaspickedupbyFarzadandthepatientwasreferredtoourelectrophysiologists.Aflecainidechallengewasperformedandwasnormal,buthewillbehavingfurthermonitoring.