43
Eagle’s syndrome: a case report
Chang-Sig Moon, Baek-Soo Lee, Yong-Dae Kwon, Byung-Jun Choi,
Jung-Woo Lee, Hyun-Woo Lee, Sun-Ung Yun, Joo-Young Ohe
Department of Oral and Maxillofacial Surgery, School of Dentistry, Kyung Hee University, Seoul, Korea
Abstract (J Korean Assoc Oral Maxillofac Surg 2014;40:43-47)
Eagle’s syndrome is a disease caused by an elongated styloid process or calcified stylohyoid ligament. Eagle defined the disorder in 1937 by describ-ing clinical findings related to an elongated styloid process, which is one of the numerous causes of pain in the craniofacial and cervical region. The prevalence of individuals with this anatomic abnormality in the adult population is estimated to be 4% with 0.16% of these individuals reported to be symptomatic. Eagle’s syndrome is usually characterized by neck, throat, or ear pain; pharyngeal foreign body sensation; dysphagia; pain upon head movement; and headache. The diagnosis of Eagle’s syndrome must be made in association with data from the clinical history, physical examination, and imaging studies. Patients with increased symptom severity require surgical excision of the styloid process, which can be performed through an intraoral or an extraoral approach. Here, we report a rare case of stylohyoid ligament bilaterally elongated to more than 60 mm in a 51-year-old female. We did a surgery by extraoral approach and patient’s symptom was improved.
Key words: Eagle syndrome, Elongated styloid process[paper submitted 2013. 12. 18 / revised 2014. 2. 3 / accepted 2014. 2. 4]
ThecarotidarterytypeofEagle’ssyndromepresentswith
othersymptoms,suchasmigraines,andneurologicalsymp-
toms,causedbyirritationofthesympatheticnerveplexus.
Iftheinternalcarotidarteryiscompressed,thenipsilateral
headachescanoccur.If theexternalcarotidarteryiscom-
pressed,thentherecanbepaininthetemporalandmaxillary
branchareas4,6.
Here,wereportararecaseofthestylohyoidligamentbilat-
erallyelongatedtomorethan60mmina51-year-oldfemale.
Wedidasurgerybyextraoralapproachandpatient’ssymp-
tomwasimproved.
II. Case Report
A51-year-oldfemalepatientwiththroatpainanddizziness
wasreferredto theDepartmentofOralandMaxillofacial
SurgeryatKyungHeeUniversityDentalHospitalinKorea.
Uponturningherhead,thepatientfeltfacialswelling,dizzi-
ness,aswellasneckandshoulderpain.Shealsohadasense
offullnessinherneck.Herpastmedicalhistoryincludeda
thyroidnodulectomy1yearpriortopresentation.Duringthe
clinicalexam,wecouldnotfeeleitherstylohyoidligament
intheoralcavity.Howeverafterimaging,wecouldseethat
bothligamentswereelongatedinthepanoramicview.(Fig.1)
I. Introduction
In1652,PietroMarchettifirstdescribedanelongatedsty-
loidprocessrelatedtoanossifyingprocessofthestylohyoid
ligament.Eagle,anotolaryngologist, laterdefinedEagle’s
syndromein19371,2.Eagleconsideredanystyloidprocess
longerthan25mminanadulttobeabnormal.Hefoundthat
4%ofthepopulationhadelongatedstyloidprocesses,but
only4%ofthosewiththistraithadsymptoms3-5.Hedivided
thesyndromeintotwoforms:classictypeandcarotidartery
type.
Theclassic typeofEagle’ssyndromecandevelopafter
tonsillectomy,whenscartissueunderthetonsillarfossacom-
pressesandstretchescranialnervesV,VII,IX,andX.This
typeincludessymptomssuchasforeignbodysensation,pain
referredtotheear,anddysphagia.
CASE REPORT
Joo-Young OheDepartment of Oral and Maxillofacial Surgery, Kyung Hee University Dental Hospital, 26, Kyungheedae-ro, Dongdaemun-gu, Seoul 130-701, KoreaTEL: +82-2-958-9360 FAX: +82-2-966-4572E-mail: [email protected]
This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
CC
Copyright Ⓒ 2014 The Korean Association of Oral and Maxillofacial Surgeons. All rights reserved.
http://dx.doi.org/10.5125/jkaoms.2014.40.1.43pISSN 2234-7550·eISSN 2234-5930
J Korean Assoc Oral Maxillofac Surg 2014;40:43-47
44
tachmentsusingarongeurforcepandsmallosteotome.(Figs.4,
5)Inaddition,weextractedallofthewisdomteeth.Theday
aftersurgery,weconfirmedthatmostoftheelongatedstylo-
hyoidligamentswereremovedusingpanoramicradiography
andCBCT.(Figs.6,7)Afterresection, thepatient’sneck
andshoulderpainanddizzinessuponheadmovementdisap-
peared,butshehaddiscomfortwithswallowing.Shealso
complainedthattheskinaroundthesurgicalsitewasloose.
Atthe3-monthfollow-up,wereevaluatedthesurgicalsite
usingpanoramicradiographyandCBCT.Wedeterminedthat
thepatient'ssymptomssuchaspain,dizziness,swollenfeel-
ing,andodynophagiahadresolved.However,thepatientstill
complainedoflooseskinaroundthesurgicalsite.
III. Discussion
Thestyloidprocessisderivedfromthesecondbranchial
archofReichert’scartilage.Thiscartilageconsistsoffour
components:1) the tympanohyale,whicharisesfromthe
perioticcapsuleof thetemporalboneandisaprocessat-
tachedtotheinferiorsurfaceofthepetrouspartofthetem-
poralbone;2)thestylohyale,whichusuallyformsthegreater
partofthestyloidprocessproper;3)theceratohyale,which
formsthestylohyoidligament;and4)thehypohyale,which
formsthelessercornuofthehyoidbone7.Thestyloidpro-
cessisanelongatedtaperedprojectionthatoriginatesinthe
petrousportionofthetemporalbone,lyingmediallyandan-
teriorlytothestylomastoidforamen,betweentheinternaland
externalcarotidarteries,andlaterallytothetonsillarfossa.
Thestylopharyngeal,stylohyoid,andstyloglossalmusclesare
LateralcephalometryandreverseTowneprojectionfilm
showedthatthestylohyoidligamentsextendeddowntothe
hyoidbone.(Fig.2)Usingcone-beamcomputedtomography
(CBCT),wedeterminedthat their lengthsweremorethan
60mmandtheirdiameterswerealmost5mmatthethickest
site,andtheligamentsdisplayedatwigmorphology.(Fig.3)
WediagnosedthispatientwithEagle’ssyndromebyclini-
calandradiographicexams.Weplannedforcompleteremov-
alofbothelongatedstylohyoidligamentsusinganextraoral
approachundergeneralanesthesia.
Ahorizontalincisionwasplacedinaskincrease5cmin-
feriortothelowerborderofthemandible.Acervicalincision
wasmadefromtheproximalsideofthesternocleidomastoid
muscletothehyoidbone,andtheelongatedstylohyoidliga-
mentsweredetachedandthemuscularattachmentsremoved
viasubperiostealdissection.Weresectedtheligamentsandat-
Fig. 1. Panoramic view at the first visit shows calcification of both stylohyoid ligaments.Chang-Sig Moon et al: Eagle’s syndrome: a case report. J Korean Assoc Oral Maxillo-fac Surg 2014
Fig. 2. Lateral cephalometry & reverse Towne shows that both calcified stylo-hyoid ligaments come down to hyoid bone.Chang-Sig Moon et al: Eagle’s syndrome: a case re-port. J Korean Assoc Oral Maxillofac Surg 2014
Eagle’s syndrome
45
toexplainthedevelopmentofEagle’ssyndrome.Thefirst
theoryiscongenitalelongationofthestyloidprocessdueto
thepersistenceofthecartilaginousprecursor,thesecondis
thecalcificationofthestylohyoidligamentbyamysterious
process,andthethirdisthegrowthofosseoustissueatthe
insertionof thestylohyoid ligament.Steinmannproposed
threemechanismsthatmightcaseossification:1)reactivehy-
attachedtothestyloidprocess.Thisbonyprocesssupports
thestylohyoidandstylomandibularligaments.Thestylohy-
oidligamentconnectstheapexofthestyloidprocessandthe
lesserhornofthehyoidbone,andthestylomandibularliga-
mentextendsfromthestyloidprocesstotheparotideomas-
setericfasciabetweenthemastoidprocessandthemandible8.
Recently,Murtaghetal.9presentedthreeetiologictheories
Fig. 3. Cone-beam computed tomogra-phy shows that both calcified stylohyoid ligaments are more longer than 60 mm. Chang-Sig Moon et al: Eagle’s syndrome: a case re-port. J Korean Assoc Oral Maxillofac Surg 2014
Fig. 4. Clinical photographs were taken during operation. A. Right. B. Left.Chang-Sig Moon et al: Eagle’s syndrome: a case re-port. J Korean Assoc Oral Maxillofac Surg 2014
J Korean Assoc Oral Maxillofac Surg 2014;40:43-47
46
ossificationofthestylohyoidligament;and3)anatomicvari-
ance,whichoccurswithoutanydistinctivetrauma10.Camarda
etal.11proposedafourthmechanismofagingdevelopmental
anomalyintheabsenceofapparentradiographicossification.
Murtaghetal.9explainedthatEagle’ssyndromesymptoms
stemfromitspathophysiology,whichincludes:1)thetheory
thattraumaticfractureofthestyloidprocesswithaprolif-
erationofgranulationtissueisabletoapplypressureonthe
perplasia,whentraumaactivatestheremnantsoftheoriginal
connectiveandfibrocartilaginouscells;2)reactivemetapla-
sia,oranabnormalhealingfollowingatraumathatinitiates
Fig. 7. Cone-beam computed tomogra-phy after surgery shows that both calci-fied stylohyoid ligaments were almost removed.Chang-Sig Moon et al: Eagle’s syndrome: a case re-port. J Korean Assoc Oral Maxillofac Surg 2014
Fig. 5. Resected calcified stylohyoid ligament was more than 60 mm on right side.Chang-Sig Moon et al: Eagle’s syndrome: a case report. J Korean Assoc Oral Maxillo-fac Surg 2014
Fig. 6. Panoramic view after surgery shows that most of both cal-cified stylohyoid ligaments were removed. Chang-Sig Moon et al: Eagle’s syndrome: a case report. J Korean Assoc Oral Maxillo-fac Surg 2014
Eagle’s syndrome
47
thestyloidprocess.Theexternalapproachhastheadvantage
ofgoodanatomicexposureofthestyloidprocess.However,
itrequiresmoreinterventionandresultsinavisiblescar3,12.
Inthiscase,weplannedsurgicalexcisionbecausethepa-
tient’ssymptomsweresevere.Asbothelongatedstylohyoid
ligamentswerequite large,weresectedusinganexternal
approach.Wealsoextracted4wisdomteeth.Theresected
stylohyoidligamentsmeasuredmorethan60mmlong,asin-
dicatedintheCTscan.Aftersurgery,thepatient’ssymptoms
improvedbutascarremained.Atthe3-monthpostoperative
follow-up,thepatient’ssymptomswerefurtherdecreasedal-
thoughsomediscomfortremained.
Conflict of Interest
Nopotentialconflictofinterestrelevanttothisarticlewas
reported.
References
1. EagleWW.Elongatedstyloidprocesses:reportoftwocases.ArchOtolaryngol1937;25:584-7.
2. Dunn-RyznykLR,KellyCW.Eaglesyndrome:ararecauseofdysphagiaandheadandneckpain.JAAPA2010;23:28,31-2,48.
3. YavuzH,CaylakliF,ErkanAN,OzluogluLN.Modifiedintraoralapproachforremovalofanelongatedstyloidprocess.JOtolaryn-golHeadNeckSurg2011;40:86-90.
4. ColbyCC,DelGaudioJM.Stylohyoidcomplexsyndrome:anewdiagnosticclassification.ArchOtolaryngolHeadNeckSurg2011;137:248-52.
5. JohnsonGM,RosdyNM,HortonSJ.ManualtherapyassessmentfindingsinpatientsdiagnosedwithEagle'sSyndrome:acaseseries.ManTher2011;16:199-202.
6. CostantinidesF,VidoniG,BodinC,DiLenardaR.Eagle'ssyn-drome:signsandsymptoms.Cranio2013;31:56-60.
7. GhoshLM,DubeySP.Thesyndromeofelongatedstyloidprocess.AurisNasusLarynx1999;26:169-75.
8. ValerioCS,PeyneauPD,deSousaAC,CardosoFO,deOliveiraDR,TaitsonPF,etal.Stylohyoidsyndrome:surgicalapproach.JCraniofacSurg2012;23:e138-40.
9. MurtaghRD,CaraccioloJT,FernandezG.CTfindingsassociatedwithEaglesyndrome.AJNRAmJNeuroradiol2001;22:1401-2.
10. SteinmannEP.Styloidsyndromeinabsenceofanelongatedpro-cess.ActaOtolaryngol1968;66:347-56.
11. CamardaAJ,DeschampsC,ForestD.I.Stylohyoidchainossifi-cation:adiscussionofetiology.OralSurgOralMedOralPathol1989;67:508-14.
12. LeclercJE.Eaglesyndrome: teachingtheintraoralsurgicalap-proachwitha30degreesendoscope.JOtolaryngolHeadNeckSurg2008;37:727-9.
surroundingarea;2)compressionof theglossopharyngeal
nerve,themandibularbranchofthetrigeminal,orthechorda
tympani;3)thetheoryofinsertiontendonitisduetoinflam-
mationofthetendinouspartofthestylohyoidinsertion;4)
irritationof thepharyngealmucosabydirectcompression
orpost-tonsillectomyscarring(withinvolvementofcranial
nervesV,VII,IX,andX);and5)thetheoryofirritationof
thesympatheticnervessurroundingthecarotidvessels9.
Diagnosisisguidedbytheclinicalhistoryandphysicaland
radiographicexaminations.Thephysicalexaminationcon-
sistsofpalpationofthetonsillarfossaandlocalinfiltration
anesthesia.Transpharyngealpalpationdemonstratesabony
projectionandreproducesthecharacteristicpain.Symptom
reliefshouldfollowanestheticinjectioninthetonsillarfossa.
Radiologicevaluation,suchaspanoramicradiography,lateral
cephalometry,Towneprojectionfilm,orCT,maybeused.In
thepanoramicview,thestyloidprocessisvisualizedposte-
riorlytotheexternalacousticmeatuswithadescendantand
anteriortrajectory.Whenelongated,itattainsoveronethird
ofthelengthofthemandibularramus.CTallowsforthepre-
cisemeasurementofthestyloidprocesslength,direction,and
anatomicvariance, inadditiontoevaluationofstylohyoid
ligamentossification8.
Eagle’ssyndromecanbetreatednonsurgicallyandsurgi-
cally.Apharmacologicalapproachincludestranspharyngeal
infiltrationofsteroidsoranestheticsintothetonsillarfossa.In
addition,therearetwosurgicalapproaches,intraoralandex-
traoral.Intheintraoralapproach,thestyloidprocessisfound
withpalpationofthetonsillarfossa.Theoverlyingmucosa
andsuperiorconstrictormuscleareincisedvertically,andthe
styloidprocessaredissectedoutandresectedusingarongeur
forcep.Ifnecessaryforexposure,astandardtonsillectomy
shouldbeperformedfirst.Theintraoralapproachisgoodfor
aestheticconsiderationasexternalscarringisavoidedandfor
shorteroperativetimes.Nevertheless,exposureoftheretro-
pharyngealspacestointraoralcontentsincreasestheinfection
risk.Theintraoralapproachalsohasthedisadvantagesof
pooraccess,asincasesoftrismus,andriskofintraoperative
injury.Anextraoralapproachinvolvesmakingacervicalin-
cisionfromtheproximalportionofthesternocleidomastoid
muscletothehyoidbone,andthendissectingandremoving