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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: ojyoung81@hanmail.net

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.

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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

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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.

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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