Spontaneous internal carotid artery rupture during neck dissection in osteogenesis imperfecta patient.

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Spontaneous

internal

carotid

artery

rupture

during

neck

dissection

in

osteogenesis

imperfecta

patient

Giuditta

Mannelli

MD

*

,

Alberto

Deganello

MD,

Maria

Rosa

Lagana` MD,

Oreste

Gallo

MD

FirstClinicofOtolaryngology,DepartmentofSurgeryandTranslationalMedicine,UniversityofFlorence,ViaLargoBrambilla3,50134Florence,Italy

1. Introduction

Manystudiesreportthepresenceof structuralalterationsof

vascular walls in patients suffering from connective tissue

disorderssuchasEhlers–Danlossyndrome (eDS),ﬁbromuscular

dysplasia, osteogenesis imperfecta (OI) and Marfan syndrome;

theseconnective abnormalitiesareknowntoberiskfactorsfor

arterydissectionorrupture[1].Unfortunately,commonimaging

analyses (TC scan, MRI, Doppler sonography) could diagnose

vascular clinicalfeatures only, while themicroscopicabnormal

distributionofcollagenﬁbersformingthemicrostructureofthe

vascularwallunderliesasilentarteriopathyknownas‘‘weakness

ofthevesselwall’’,whichistherealpredisposinggeneticriskfor

arteryrupture and it does not present any clinical features or

symptoms[2].

Ischemic and hemorrhagic vascular events, due to artery

dissectionor rupture, have beendocumented in thesepatients

who usually claim headache, ipsilateral neck pain, incomplete

Horner’ssyndrome(ptosisandmyosis),bruits,transientischemic

attack,cerebrovascularaccident signs suchasamaurosis fugax,

syncope, encephalopathy and focal signs [2,3]. Cervicocephalic

arterialdissectionsandrupturecouldbetraumaticor

spontane-ous, and they could follow mild efforts as head and neck

maneuvering,forcefulcoughandvomiting[4].Fewauthorsreport

a syndrome called ‘‘spontaneous cervical arterial dissection

(SCAD)’’, usedto describea nontraumatictear ordisruption in

thewallofthebrain-supplyingarteries[5].

Herewereporta femalepatientsufferingfromOI[6,7] who

experiencedaspontaneousruptureofinternalcarotidartery(ICA)

duringaneckdissection.

2. Casereport

A 52-year-oldfemale patient referred toour Institution for

severedysphoniaandaleftnecklump.Hermedicalhistorywas

positive for OItype I withseveral bone fractures and bleeding

complications after adeno-tonsillectomy in 1980. The physical

examinationshowedarightvocalfoldparalysis,increasedthyroid

glandvolume,andseveralbulkynecklymphnodesonbothsides.

Onelymphadenopathywas5cm6cminsizeandseemedtobe

adherenttotherightcervicalneurovascularbundleatlevelII.A

preoperative CAT scan of theneck and chest showedmultiple

calciﬁcations in neck lymph nodes (from level II to level VI)

bilaterally and a thyroid glandmass with similarcalciﬁcations

AurisNasusLarynxxxx(2014)xxx–xxx

* Correspondingauthor.Tel.:+390557947112;fax:+390557947939. E-mailaddress:mannelli.giuditta@gmail.com(G.Mannelli). ARTICLE INFO Articlehistory: Received4April2014 Accepted24May2014 Availableonlinexxx Keywords:

Connectivetissuedisorder Osteogenesisimperfecta Majorcervicalvesselrupture SpontaneousruptureofICA Weaknessofthevesselwall

ABSTRACT

Objective:Severalconnectivetissuedisordersareassociatedwithvascularwallabnormalities,including spontaneousdissectionofthecervicalarteries.Osteogenesisimperfecta(OI)isahereditarydisorder, withrareneurovascularcomplications,whichpotentiallyleadtolife-threateningevents.

Methods:Wepresentedacaseofspontaneousinternalcarotidartery(ICA)rupturethatoccurredina 52-year-old-woman, suffering from OItype I, and who underwent a bilateralmodiﬁed radical neck dissection(mRND) plustotalthyroidectomy foraT4aN1b thyroidcancer. DuringmRND, anICA’s spontaneousruptureoccurred.

Results:Histopathologic report suggested a structural defect of the arterial wall without cancer inﬁltration.Thepatientdidnotexperienceanyneurologiccomplications.

Discussion: Headandnecksurgeonshavetobeawareaboutearlyclinicalrecognitionofpossiblecervical vascularabnormalities,inpatientswithconnectivetissuedisorders,potentiallyresponsiblefordramatic vascularruptureduringcervicalsurgicalprocedures.

ß2014ElsevierIrelandLtd.Allrightsreserved.

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Pleasecitethisarticleinpressas:MannelliG,etal.Spontaneousinternalcarotidarteryruptureduringneckdissectioninosteogenesis

imperfectapatient.AurisNasusLarynx(2014),http://dx.doi.org/10.1016/j.anl.2014.05.025

ContentslistsavailableatScienceDirect

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Nasus

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http://dx.doi.org/10.1016/j.anl.2014.05.025

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(Fig.1).Vascularabnormalitieswerenotevidenced.Subsequently,

a ﬁne needle aspiration biopsy was performed and a thyroid

papillarycarcinomawasfound.

Thus,weperformedtotalthyroidectomywithrightrecurrent

nerve sacriﬁce and bilateral modiﬁed radical neck dissection

includinglevelVI.

During therightneckdissection,thecervicallymph nodeat

sublevelIIAappearedtoinﬁltratetheinternaljugularvein(IJV)

thatwassacriﬁced(Fig.2).DuringIJVligation,manipulationofthe

vascularbundledetermineda bleedingcoming froma minimal

leakageofICA’swallwhichrequireditsclamping.Duringthese

procedureswedocumentedanevidentretrogradebloodﬂowfrom

theWilli’sCircle(Fig.3).Withthehelpofvascular surgeon,we

removed the damaged tract of the ICA and he performed a

transpositionofthecranialstumpoftheICAontheexternalcarotid

artery(ECA).Therestorationofnormalbloodﬂowwas

intraopera-tivelyconﬁrmedbyultrasoundechography.Finally,no

cerebro-vascular complications were found at the emergence from

anesthesia.

Thehistopathologicevaluationconﬁrmedapapillary

carcino-maofthethyroidglandandbilateralneckmetastases,pT4aN1b

accordingtoAmericanJointCommitteeonCancer[8].

ThehistopathologicanalysisofthedamagedICAtractdidnot

showanycancerinﬁltrationsoftheartery’swalls,whilethesigns

ofarterialwalldissectionweredocumented(Fig.4).

The patient was discharged 10 days later and she had not

experiencedanydiseaserelapsesafter12months.

3. Discussion

Connectivetissuedisordersalterthestructureofvascularwalls,

causing asilentarteriopathy knownas‘‘weakness ofthevessel

wall’’[2],whichdoesnotpresentanyclinicalfeaturesanditisnot

diagnosedbycommonimagingtechniques;dissectionofcarotid

andvertebralarteriesisreportedascommonfeatureinpatients

sufferingfromconnectivetissuedisorders,butusuallytheyclaim

alert symptoms.Themostcommonlocationsof cervicocephalic

arteryanomaliesareregionswherethesevesselsaremobile,not

ﬁrmlyanchored tootherarteries orbonystructures.TheICAis

relativelyﬁxedproximally,attheoriginfromthecommoncarotid

artery, and distally, at the point of penetration in the petrous

portionofthetemporalbone.Thearterialsegmentbetweenthese

two points of anchorage is mobileand thereforevulnerable to

stretching, especially during neck hyperextension with head

rotation, which can stretch the ICA against an upper cervical

vertebraoraprominentstyloidprocess.Thestrainoftenoccurs

around3cm abovethecarotidbifurcation[5].Usually,patients

whoexperiencecervicalarterydissectionorrupturemightreport

eitherprecipitatingeventsorpriorminornecktrauma[9],evenifa

clearcause–effectrelationshipisstilldebatable[10].

OurpatientwasaffectedbyOItypeI.Vascularcomplications

are rare in OI when compared with other diseases involving

connectivetissue[6,7].Themostcommonvasculardisordersare

dysplasia of aortic and mitral valves, and congenital cardiac

Fig.1.TCscanimagingshowingmultiplecervicallymphnodemetastasis:(a)sagittalsection,(b)coronalsectionand(c)axialsection.

Fig.2.Ligationoftheright internaljugularvein(IJV)duringneck dissection procedure:multipleandbulkylymphnodemetastasesarevisibleinthesurgical ﬁeld.TwosilkystitcheshavebeenplacedaroundthecranialportionoftherightIJV.

Fig.3.Retrogradebloodﬂowcomingfromthecranialresectedstumpoftheinternal carotidartery(ICA):arrowspointthearterialbloodjetcomingfromthecranial portionoftheresectedICA.Redloopshavebeenplacedaroundthecaudalstumpof theICAinordertoperformthefollowinganastomosis.(Forinterpretationofthe referencestocolorinthisﬁgurelegend,thereaderisreferredtothewebversionof thearticle.)

G.Mannellietal./AurisNasusLarynxxxx(2014)xxx–xxx 2

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malformations.Theascendingpartoftheaortacouldbeinvolved

[11].Spontaneoushemorrhagesinvolvingretinaandconjunctivae,

epistaxisandmelenamayoccurduetocapillaryfragility.Onthe

other hand, vascular cerebral complications are very rare

[11]. Thus, therelationship between cervical arteryevents and

OIisdifﬁculttodemonstrate[3].

Herewereported,tothebestofourknowledge,theﬁrstcaseof

spontaneous ICA’s rupture that occurred during surgical neck

dissectioninafemalepatientsufferingfromOIwithnoevidenceof

vascularanomaliesattheCATscan.Theruptureoccurredduringa

gentlemanipulationofthismajorvesselduringastandardsurgical

procedure,withoutanydirecttraumaonit;thus,inouropinion,

and in accordance with literature [9,10,12], the intrinsic wall

weaknesswasthepredisposingcauseofitsrupture.Moreover,the

factthat itwasnot acatastrophic arteryrupturesteerstoward

consideringtheconnectivetissuedisorderasthemainriskfactor

for this kind of potential life-threatening event, excluding

improper surgical approaches and the absence of reported

neoplasticvesselinﬁltration.

In accordance with our experience, we assert that surgical

manipulationofthecarotidbundle,whichcanoccurduringneck

dissectionprocedures,shouldbeperformedcarefullyinpatients

withcollagendisorders,becauseitcouldleadtowardmajorvessel

rupture and then to its sacrify, with potential severe clinical

consequences. Additional imaging techniques suchas

contrast-enhanced CAT scan, including angio-MRI, 3D-CTA, and carotid

arteryultrasonography[1]mighthelpindiagnosingthe

arterio-pathywhenevident,butthehigherriskforspontaneouscervical

arteryruptureshouldbetakenintoconsiderationanyway.

Conﬂictofinterest

Allauthorsdisclose noﬁnancial supportor relationshipthat

mayposeaconﬂictofinterest.

Sourcesofsupportforthework

Nogrants;nodrugs;nospecialequipments.

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Fig.4. ICA’swallalterationsatopticmicroscopy:thickenedintimaandinitial dissectionattheadventitialevel.

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