Andersons pediatric cardiology 1391
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FIG.53.1 Epicardialcoronaryartery(right)andepicardialvein(left)from
a19-month-oldchildwhodied10monthsaftertheonsetofKawasaki
disease.Theepicardialveincontainsbloodandshowsmildthickeningof
thewall,whereasthecoronaryarteryshowsalmostcompleteocclusionby
aluminalmyofibroblasticproliferationwithafineslit-likelumen.(From
McCrindleBW,RowleyAH,NewburgerJW,etal.Diagnosis,treatmentand
long-termmanagementofKawasakidisease:ascientificstatementfor
healthprofessionalsfromtheAmericanHeartAssociation.Circulation.
2017;135[17]:e927–e999.)
MyocarditisisfoundinalmostallpatientswithKawasakidiseaseandisthe
earliestcauseofdeath.47Smallhistopathologicserieshavedemonstrated
predominantmyocardialedemaandavariablecellularinflammatoryinfiltrate
withneutrophils,monocytes,macrophages,and/oreosinophils.21,46Afterthe
first10days,deathwasmostoftencausedbymyocardialinfarctiondueto
thrombosis.Mortalitypeaksbetween15and45daysaftertheonsetoffever,
whenpatients,whoareinahypercoagulablestate,havethrombocytosisand
disruptedvascularendothelium.48Beyondthefirstyearafteronsetoftheillness,
mortalitydeclinessignificantly,butmyocardialinfarctionmayoccurmanyyears
laterbecauseofprogressivecoronaryarterystenosis.
ClinicalDiagnosis
GeneralAspectsofInitialDiagnosis
DiagnosticcriteriaaresummarizedinBox53.1.49Theepidemiologicdefinition
fordiagnosisincludesfeverfor4dayswithatleastfourprincipalclinicalcriteria
(Fig.53.2)orfeverandfewerthanfourprincipalcriteriainthepresenceof
coronaryarteryabnormalities.Byconvention,thefirstdayofthediseaseis
consideredtobethefirstdayonwhichfeveroccurs.Allclinicalfeaturesare
rarelypresentatthesametime,sothediagnosisrequiressequentialevaluation.
Intheabsenceoftreatment,themeandurationoffeveris11days,andfever
rarelypersistsbeyond4weeks.
Box53.1
DiagnosisofClassicKawasakiDisease
ClassicKDisdiagnosedinthepresenceoffeverforatleast5days(thedayof
feveronsetistakentobethefirstdayoffever)togetherwithatleastfourofthe
fivefollowingprincipalclinicalfeatures.Inthepresenceoffourormore
principalclinicalfeatures,particularlywhenrednessandswellingofthehands
andfeetarepresent,thediagnosisofKDcanbemadewith4daysoffever,
althoughexperiencedclinicianswhohavetreatedmanypatientswithKDmay
establishthediagnosiswith3daysoffeverinrarecases(seeFig.53.2):
1.Erythemaandcrackingoflips,strawberrytongue,and/orerythemaoforal
andpharyngealmucosa.
2.Bilateralbulbarconjunctivalinjectionwithoutexudate.
3.Rash:maculopapulardiffuseerythroderma,orlikeerythemamultiforme.
4.Erythemaandedemaofthehandsandfeetinacutephaseand/or
periungualdesquamationinsubacutephase.
5.Cervicallymphadenopathy(≥1.5cmdiameter),usuallyunilateral.
Acarefulhistorymayrevealthatoneormoreprincipalclinicalfeatureswere
presentduringtheillnessbutresolvedbythetimeofpresentation.
PatientswholackfullclinicalfeaturesofclassicKDareoftenevaluatedfor
incompleteKD(Fig.53.3).Ifcoronaryarteryabnormalitiesaredetected,the
diagnosisofKDisconsideredconfirmedinmostcases.
Laboratoryteststypicallyrevealanormalorelevatedwhitebloodcellcount
withneutrophilpredominanceandelevatedacute-phasereactantssuchasCreactiveproteinanderythrocytesedimentationrateduringtheacutephase.Low
serumsodiumandalbuminlevels,elevatedserumliverenzymes,andsterile
pyuriacanbepresent.Inthesecondweekafterfeveronset,thrombocytosisis
common.
Otherclinicalfindingsmayincludethefollowing:
▪Cardiovascular
▪Myocarditis,pericarditis,valvarregurgitation,shock
▪Coronaryarteryabnormalities
▪Aneurysmsofmedium-sizednoncoronaryarteries
▪Peripheralgangrene
▪Aorticrootenlargement
▪Respiratory
▪PeribronchialandinterstitialinfiltratesonCXR
▪Pulmonarynodules
▪Musculoskeletal
▪Arthritis,arthralgia(pleocytosisofsynovialfluid)
▪Gastrointestinal
▪Diarrhea,vomiting,abdominalpain
▪Hepatitis,jaundice
▪Gallbladderhydrops
▪Pancreatitis
▪Nervoussystem
▪Extremeirritability
▪Asepticmeningitis(pleocytosisofcerebrospinalfluid)
▪Facialnervepalsy
▪Sensorineuralhearingloss
▪Genitourinary
▪Urethritis/meatitis,hydrocele
▪Other
▪Desquamatingrashingroin
