BrazJOtorhinolaryngol.2015;81(1):109---112
Brazilian
Journal
of
OTORHINOLARYNGOLOGY
www.bjorl.org
CASE
REPORT
Allergic
fungal
sinusitis
eroding
the
pterygoid
plates:
a
rare
case
series
夽
Sinusite
fúngica
alérgica
como
causa
de
erosão
das
lâminas
pterigóideas:
série
de
casos
raros
Osama
Marglani,
Ahmed
Masood
Shaikh
∗DepartmentofOtolaryngologyandHeadNeckSurgeryKingAbdullahMedicalCity,UmmulQuraUniversity,Makkah,SaudiArabia
Received24January2013;accepted27August2013 Availableonline3July2014
Introduction
Allergic fungal sinusitis (AFS) is a non-invasive form of allergicsinusitis,resultingfromtheIgE-mediated hypersen-sitivity reactionto fungal antigenin atopic individuals. It wasfirstdescribedin1983byKatzenstein.1Itusuallyfollows
aslow,non-aggressivecourse,andawidevarietyoffungal agentshavebeenimplicated,withthevastmajority belong-ingtotheDematiaceaefamily,suchasBipolarisspiciferaor
Curvularia lunata,or totheAspergillusfamily,such asA. fumigatus,A.flavus,orA.niger.2,3AFScanpresentwitha
continuumofsymptoms,rangingfromsimplenasal obstruc-tiontodiplopia,facialdeformity,osteolyticdestruction,and involvement ofthe skullbase.4 CTscanof thesepatients
is always abnormal, andevidencingbone erosion,chronic rhino sinusitis, and central areas of hyper-attenuation
夽 Pleasecitethisarticleas:MarglaniO,ShaikhAM.Allergicfungal
sinusitiserodingthe pterygoidplates:a rare caseseries.Braz J
Otorhinolaryngol.2015;81:109---12.
∗Correspondingauthor.
E-mail:[email protected](A.M.Shaikh).
(doubledensity). Incidenceof bone erosionvaried signifi-cantlyindifferentseries.5
In this article, three cases with erosion of pterygoid platesarepresented.Althoughbone erosionsarecommon andareobserved in20%of thepatientwithAFS,5 erosion
ofthepterygoidplatesisaveryrarephenomenon,andhas notyetbeenreportedintheliterature(accordingtoPubMed search).Thesepatientpresentswithdistinctsymptoms. Uni-lateral disease, in addition to bony erosions and typical presentationofsymptomsarerareinpatientswithAFS.
The pathophysiology, clinical features, and detailed managementofthesethreepatientsarediscussed.
Case
report
Twoofthepatientswerefemale,andonewasamaleinthe agegroupof18---30years.Theypresentedtothisoutpatient clinicwiththehistory ofunilateral nasalobstruction, rhi-norrhoea,repeatedsneezingepisodes,andheadache,with mediandurationofsymptomsfromeighttoninemonths.All thepatientswereresidentsofMakkah,SaudiArabia.
On inquiry,these patients alsoprovidedhistory ofdull aching,unilateral,ill-defined,intermittenthemifacialpain,
http://dx.doi.org/10.1016/j.bjorl.2014.05.028
1808-8694/©2014Associac¸ãoBrasileirade OtorrinolaringologiaeCirurgiaCérvico-Facial.Published byElsevierEditoraLtda.Allrights
110 MarglaniO,ShaikhAM
andthepainwasexacerbatedwithchewingandopeningof mouth, withnorelieving factors. Upon endoscopic exam-ination, multiple nasal polyps were seen, with unilateral involvement.Nolocaltemporomandibularjointtenderness waspresent.
All these patients were investigated with computed tomography(CT)scanoftheparanasalsinusesintheaxial andcoronalplanes,serumIgElevels,differentialleukocyte count,absoluteeosinophiliccount,andfastingsugarlevels. Thesurgicallyremoved(endoscopic)nasalpolypsand inter-sinusdebrisweresentforhistopathologicalexaminationand fungalculture,whichyieldedAspergillusterreusinonecase andA.fumigatusintheothertwocases.
CToftheparanasalsinusesrevealedpresenceofchronic rhinosinusitis, involving multiple unilateral sinuses, with areasofhyper-attenuationinthecenter.
Erosionofthepterygoidplateswaspresentinthesideof hemifacialpain,andnoextensionofdiseasewaspresentin infra-temporalfossa.
All the patients were treated with endoscopic clear-anceofthesinuses,withpost-operativesteroidsandnasal irrigation. Erosion of the pterygoid plates to a variable extentwaspresentinallthethreecases.
Post-surgically,allthepatientswererelievedof unilat-eralhemifacialpain.
Therewerenorecurrencesduringthefollow-up period ofninemonthsfor onepatient,andofsix monthsforthe othertwopatients.
Discussion
Fungalsinusitiswasonceconsideredararedisorder,buthas beenreportedwithincreasingworld-widefrequencyinthe lasttwodecades.Histopathologically,twodistinctformsof fungal sinusitisarerecognized:invasive andnon-invasive. Invasivefungal sinusitisincludes acute fulminant, chronic invasive,andgranulomatousinvasiveformsofsinusitis;the non-invasiveformsincludeAFSandfungalmycetoma.AFSis clinically,pathologically,andprognosticallyadistinctform ofrhinosinusitis,anditoccursinatopicimmunocompetent individuals. AFS is most common among adolescents and youngadults;themeanageofpresentationis21years.The male-to-female(M/F)ratioisequal.
PatientswithAFSnormallypresentwithsignsand symp-toms of allergicrhinitis, or chronic sinusitis that includes nasalcongestion,purulent rhinorrhea,postnasaldrainage, or headache. The presentation of AFS is often subtle. Patientstypicallycomplainofgradualnasalairway obstruc-tionlastingfrommonthstoyears,andsymptomsareusually unilateral.
Painisanuncommonsymptom,butinthepresentstudy, all the patients had a history of typical pain. It was a dull-aching,non-localised,hemifacialpain,whichwas exac-erbated by chewing andexcessive opening of the mouth; all themovements of mastication caused increase in this pain. This associated symptom has never been described withsinonasaldiseasesintheliterature.
ThepathophysiologyofAFShasnotyetbeenfully under-stood; however, it is postulated to be similar to that of allergic bronchopulmonary fungal disease (a term replac-ing bronchopulmonary aspergillosis). Manning et al. have
suggestedthatseveralinterrelatedfactorsandeventslead tothe developmentand perpetuation of AFS.6 Theyhave
providedevidenceofroleofanIgEmediatedreaction.First, anatopichostisexposedtofungivianormalnasal respira-tion,whichprovidestheinitialantigenicstimulus.Aninitial inflammatory response ensues as the result of both type I (IgE-mediated)and typeIII (immune complex-mediated) reactions, causingsubsequenttissueedema. Theresulting obstruction of sinus ostia, which may be accentuated by anatomicfactorssuchasseptaldeviationorturbinate hyper-trophy, results in stasis within the sinuses. This creates an ideal environmentfor further proliferation of the fun-gus, thus increasing the antigenic exposure to which the host is allergic. This process leads to further inflamma-tion and obstruction, thus leading to a self-perpetuating cycle;italsoleadstocollectionofallergicmucin,i.e.the secretions which fills the sinuses, which contains fungal hyphae.
The production of this allergic mucin and its even-tualclinical,histologic,andradiographiccharacteristicsare uniquetoAFSandserveasahallmarkofthedisease.Grossly, allergic fungal mucin is thick, tenacious, and highly vis-cous.Oftendescribedashavingapeanutbutterappearance, accumulation of allergicfungal mucin eventuallyleads to theincreasinglywell-recognizedradiographicfindings char-acteristicofAFS,i.e.heterogeneousareasofsignalintensity within the paranasal sinuses on CT scans, although these findingsarenotspecificforAFS.
Theareasofhighattenuationareseenduetothe collec-tionofheavymetals(iron,manganese),alongwithcalcium crystalsintheinspissatedmucin.6
Expansion,remodeling,orthinningoftheinvolvedsinus walls is common in AFS, and is thought to be caused by the expansile nature of the accumulating mucin, which alsoleadstothebonyerosionsseenonCTscan.Thebone resorptionispresumablycausedbycytokinespresentinthe allergicmucin.
Different criteria have been proposed for the diagno-sisofAFS, outofwhichBentandKuhncriteriaarewidely accepted.5In1994,BentandKuhnpublishedspecific
diag-nosticcriteriabasedaoncaseseriesof15patients,which included five criteria: (1) typeI IgE-mediated hypersensi-tivity;(2)nasalpolyposis;(3)characteristicCTfindings;(4) eosinophilicmucin;and(5)positivefungalsmearorculture. In1997,DeShazoproposedsimilardiagnosticcriteriafor patientswithoutatopy6;BentandKuhn’scriteriawereused
Allergicfungalsinusitis 111
Figure1 Navigationimageshowingsiteoferosion.
presentbetweenthemediaandlateralpterygoidmuscles, especiallythebuccaldivision.Thesechronicinflammatory changesofmuscles,alongwiththebranchesofthe mandibu-lardivisionofthetrigeminalnervecanexplainthetypical painexperiencedbythesepatients.
The authorshavetermedthisasMarglaniSyndrome,as thecharacteristicsofthisentityweredescribedbythemain author(Dr.OsamaMarglani),whichincludeunilateral hemi-facialdullachingpain,whichincreasesuponchewing and jaw opening in patients diagnosed withAFS with involve-mentofthepterygoidplates.
Managementofthesepatientsinvolvessurgeryfollowed byrigorousmedicaltherapy.Allthepresent patientswere treated with functional endoscopic sinus surgery under imageguidance.Thisinvolvesremovalofpolypsand evac-uationofsinuses,withwideningofsinusostiatofacilitate sinusdrainage,preservingthenasalsinusmucosaasmuch aspossible. It is always difficult tovisualize theareas of pterygoid erosion.Medical therapy is required toprevent recurrences.Allthepatients weretreatedwithintranasal steroidspray.Thesepatientsalsoreceivelocalbudesonide irrigation,which delivers alargerdose of topicalsteroids comparedtonasalsprays.Ashortcourseoforalsteroidsis prescribedinpost-operativeperiods.
Inpostoperativefollow-up,allthepatientswererelieved ofunilateral pain.This retrospectivelyconfirmsthecause ofpainaspterygoidplateerosionwithinflammationofthe pterygoidmuscles.
Closefollow-upofthesepatientsiswarranted,because recurrent disease may silently progress until the patient developssignificantnasalobstruction(Fig.1).
Conclusion
InanerawhendiagnosisofbonyerosionsinAFSpatientsis alwaysconfirmedbyradiologicalinvestigations,itcanstill beclinicallysuspectedbasedonsignsandsymptomsthatare associatedwiththesiteoferosions.Asthiscaseserieswas described,theerosionsofthepterygoidplatespresentwith auniquesetofsymptoms,andwarrantearlysurgical inter-vention,astheymayprogresstoinvolvetheinfratemporal fossa.
Conflicts
of
interest
112 MarglaniO,ShaikhAM
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