Braz. j. . vol.82 número4

BrazJOtorhinolaryngol.2016;82(4):484---486

www.bjorl.org

Brazilian

Journal

of

OTORHINOLARYNGOLOGY

CASE

REPORT

Atypical

Kawasaki

disease

presenting

as

a

retropharyngeal

abscess

夽

Doenc

¸a

de

Kawasaki

atípica

manifestando-se

como

abscesso

retrofaríngeo

Jong

Seung

Kim

_,

_Sam

_Hyun

_Kwon

a_{DepartmentofOtolaryngology-HeadandNeckSurgery,ChonbukNationalUniversity,Jeonju,RepublicofKorea}

b_{ResearchInstituteofClinicalMedicineChonbukNationalUniversityandBiomedicalResearchInstituteofChonbukNational}

UniversityHospital,Jeonju,RepublicofKorea

Received3March2015;accepted21April2015 Availableonline9October2015

Introduction

Kawasaki disease (KD) is an acute systemic vasculitis of

unknown etiology,also called an acutefebrile

mucocuta-neouslymphnodesyndrome,whichisfirstidentifiedbyDr.

Tomisaku Kawasaki in 1967. This disease is characterized

byfever,erythematousrash,cervicallymphadenitis,

straw-berrytongue,nonpurulentconjunctivitisanddesquamation

ofhandsandfeet.Ofthesesymptoms,themostinfrequent

symptomiscervicallymphadenitis(50%---75%)whilethe

oth-ers occur in 90% of the cases.1 _{Cervicallymphadenitis as}

initialpresentingsymptomoccursinonly12%ofthetimes2 andmaybemisdiagnosedasotherdiseaseentity.

KD involves all blood vessels, especiallymedium sized vesselslikethecoronaryartery.Inthisregard,KDmaycause severecomplicationswithdelayedtreatment.

Image ofretropharyngeal low-densityarea,in addition toretropharyngealabscessandedemaisusuallyassociated toafatalconditionifnotappropriatelytreated. Otolaryn-gologistsarefamiliarwiththisdiseaseandmaytreatitwith

夽 _{Pleasecitethisarticleas:KimJS,KwonSH.AtypicalKawasaki}

diseasepresentingasaretropharyngealabscess.BrazJ Otorhino-laryngol.2016;82:484---6.

∗_{Correspondingauthor.}

E-mail:[email protected](S.H.Kwon).

surgicaldrainingoftheabscess,preventingblockageofthe airway.

KDwithretropharyngeal abscessis a veryrare disease andcanbeachallengetomanyotolaryngologists.We expe-rienceda9year-oldgirlwhopresentedasaretropharyngeal abscess,thusresultingindelayeddiagnosis.

Case

report

A9-year-oldgirlwastransferredtoourhospitalbecauseof aretropharyngealabscess.Shehadsufferedfromfeverand sorethroat,andhadseekedforasecondaryhealthcare cen-ter.Computed tomography(CT)showedaretropharyngeal abscess with bilateral cervical lymphadenopathy (Fig. 1A and B). She wasadmitted in the intensive care unit and treated with amoxicillin-clavulanate (120mg/kg/day) and clindamycin(100mg/kg/day)for4days.However,her symp-toms did notimprove withthis conservativetherapy, and the patient was transferred to a tertiary medicalcenter. On admission, cardiac murmur wasaudibleand breathing soundswereclearwithoutcrackles.CTwasrecheckedand showeda retropharyngeallow density areafromC2toC5 with bilateral cervical lymphadenitis. Simple lateral neck X-rayrevealedamildsofttissuethickeningofthe retropha-ryngealarea.Theabscesspockethadbeendecreasedwhen comparing with the previously done CT at the secondary healthcareunit.(4cm×1cm→2cm×0.5cm)Whiteblood

http://dx.doi.org/10.1016/j.bjorl.2015.04.014

AtypicalKawasakidiseasepresentingasaretropharyngealabscess 485

Figure1 (A)AxialCTviewrevealed4cm×1cmhypodenseareaintheretropharyngealspace.(B)CoronalCTshowed1cm×4cm

sizedhypodenseareaintheretropharyngealspace,fromC2leveltoC5level.(C)MildinjectedthroatandgradeIItonsilwasnotedon theendoscopy.Swellingofpostpharyngealwallwasalsoidentified.(D)SimpleneckX-rayrevealedmildswellingofretropharyngeal softtissueandnointervalchangecomparedwithpreviousfilm.(E)Polymorphouserythematousrashappearedontheanteriorand posteriortrunk.(F)Erythematousrashandedemaappearedonbothhands.

cell count (WBC) was 14,040mm3 _{(neutrophil 11,170),} erythrocytesedimentationrate (ESR)58mm/h,Creactive protein(CRP)waselevated,165mg/dL.Endoscopicfindings consistedofenlargedtonsils,injectedthroat,andmild nar-rowed airway. She had no dyspnea. (Fig. 1C) We treated thepatientwithhighdoseceftriaxone(80mg/kg/day)and metronidazole(22.5mg/kg/day).On day 2,fever had dis-appearedandWBC,CRPhaddecreased.However,thefever reappeared on the third day of hospitalization, twice a day.Urinecultureandbloodculturewereallnegative.Her inflammatory lab results had ameliorated, and neck lat-eralX-rayshoweddecreasingoftheretropharyngealedema, butspikingfever hadnotdisappearedinasimilarfashion. Her lip wasfissured and the color of the tongue became redon3rd admissionday; wereferred hertothe depart-mentofpediatrics,suggestingadiagnosisofscarletfeveror Kawasakidisease.However,thepediatricianbelieved that thefocusofthefeverwastheretropharyngealabscess,not fromaninflammatorydisease.Althoughthenecklateral X-rayrevealednoincreaseofthicknessintheretropharyngeal space, the patient was inevitably treated with high dose of antibiotics(Fig.1D). WBCandCRP decreased withthis therapybutherfeverwasnotcontrolled.

Onthe6thday,erythematousrashturnuponthetrunk, inbothhandsandfeet(Fig.1EandF).Therashwas poly-morphouswithnovesiclesinassociation.WBCandCRPhad

been normalized,7130mm3 _{and 16.8mg/dL, respectively.} Virallabtestswereallnegative.

Desquamation of hands and feet appeared on the 8th day of hospitalization and her echocardiogram proved to benormal. The pediatrician made a diagnosis of atypical Kawasaki disease and administered high dose of intra-venous immunoglobulin (IVIG) (2.5g/kg/day) and aspirin (100mg/kg/day).Feverandrash didnot disappearwithin 48h. On day 10,booster steroid injection(30mg/kg/day) wastriedfortwodaysandthefeverfinallydisappearedon the11thday.Onthe13thday,desquamationofhandsand feetdisappearedandthepatientwasdischarged.

After 6 weeks follow up, the patient was uneventful, echocardiogram revealed no abnormality of the coronary artery,withnormalventricularmotion.

Discussion

486 KimJS,KwonSH

includingerythemaofthepalmsandsoles,nonpittingedema ofhandsandfeet,ordesquamationand(5)cervical lympha-denopathyover 1.5cm indiameter. Otherdisease causing thesymptomsmustbeexcluded.3

Delayeddiagnosismaybedisastrous.Itinvolvesmiddle sizedarteries,andseverecomplicationslikecoronaryartery aneurysmor myocardial infarctioncan happen.4 _Coronary arteryaneurysmappearsin7%ofchildrenofKDand myocar-diacinfarctiondevelopin0.2%---0.5%ofthesechildren.

Despite of these seriouscomplications, the problem is thattheonlydiagnostictoolsarethesymptomsandsigns.No specificdiagnostictesthasbeendevelopedforKD.Atypical KDisadiseasethatsymptomsdonotoccursimultaneously ornotmettheabovedescribedcriteria.Itinvolves periton-sillarabscess,retropharyngealabscess,renal impairment, acuteotitismedia,pulmonary infiltrates,abdominalpain, arthritisand lymphadenitis.5 _{Even in atypical KD likethis} patient,earlydiagnosis isveryimportant.Itismeaningful toinvestigatetheclinicalcharacteristicsofatypicalKDwith retropharyngealabscessoredema.

Our case had an afebrile period of 2 days. There are twoothercase reportsofafebrileperiodinKDbeforethe IVIGtreatment.5,6_{Itcanbeanimportantcluefromseveral} case reports of atypicalKD withretropharyngealabscess. Propermanagementincludingwidespectrumantibioticsand surgerycanproceedbeforethediagnosisofKD,howeverit iscrucialtoadministerearlyIVIGtreatmentafterthefinal diagnosis,inordertopreventfromseriouscomplications.

Conclusion

Any children who present with fever and lymphadenitis, colorchange ofskin andmucosaincludingoral cavityand conjunctivashouldbeobservedcarefully.Eventhoughthese

symptoms donotappear simultaneously,otolaryngologists shouldruleouttheatypicalKD.Inaddition,afebrileperiod andretropharyngealabscess arealsotobeconsiderate in atypicalKD.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

Acknowledgement

This paper was supported by the Fund of Biomedical ResearchInstitute,ChonbukNationalUniversityHospital.

References

1.BurgnerD, Festa M, Isaacs D. Delayeddiagnosis of Kawasaki diseasepresenting withmassive lymphadenopathyand airway obstruction.BMJ.1996;312:1471---2.

2.YoskovitchA,TewfikTL,DuffyCM,MorozB.Headandneck man-ifestationsofKawasakidisease.IntJPediatrOtorhinolaryngol. 2000;52:123---9.

3.HungMC,WuKG,HwangB,LeePC,MengCC.Kawasakidisease resemblingaretropharyngealabscess---casereportand litera-turereview.IntJCardiol.2007;115:94---6.

4.Pontell J, Rosenfeld RM, Kohn B. Kawasaki disease mim-icking retropharyngeal abscess. Otolaryngol Head Neck Surg. 1994;110:428---30.

5.Ganesh R, Srividhya VS, Vasanthi T, Shivbalan S. Kawasaki disease mimicking retropharyngeal abscess. Yonsei Med J. 2010;51:784---6.