Fibromuscular Dysplasia of theExtracranial Internal Carotid ArteriesAssociated With an Ulcerative PlaqueBY H. HOOSHMAND, M.D.,* M. E. BOYKIN, M.D.,f F. S. VINES, M.D.,*AND H. M. LEE, M.D.§

Abstract:FibromuscularDysplasiaof theExtracranialInternalCarotidA rteriesAssociatedWith anUlcerativePlaque

• Fibromuscular dysplasia (FMD) of the extracranial internal carotid arteriesis a relatively rare angiographical finding which has been reported as anincidental finding during angiography, as well as in association with a variety ofdifferent clinical conditions. A case is reported of transient attacks of amaurosisfugax and hemiparesis. Angiography in this patient revealed bilateral FMD ofthe internal carotids as well as a small ulcerative plaque involving the rightinternal carotid artery at the region of carotid bifurcation. Surgical treatmentdirected toward the ulcerative plaque and not the FMD of the carotid arterieshas rendered the patient asymptomatic. Lack of symptoms, despite thepersistence of FMD, suggests that FMD is of doubtful pathogenicsignificance.

A small, surgically correctable ulcerative plaque should not be overlookedin the presence of FMD of the internal carotid arteries. The significance of therare finding of FMD of the internal carotid arteries is not clear.

ADDITIONAL KEY WORDS amaurosis fugax transient cerebral ischemiaanticoagulation carotid endarterectomy

Introduction• Fibromuscular dysplasia (FMD) of theextracranial internal carotid arteries is arelatively rare angiographical finding which hasbeen recognized since 1964.]~n It has beenreported in association with a variety ofclinical conditions, including intracranialaneurysms,7' 8>lu intracranial neoplasms,8' n

transient cerebral ischemic attacks,4'5'7-8'1U~1-post-traumatic intracranial disease,8 asympto-matic"'7 and "symptomatic"6 carotid bruits,headache/migraine,6'8 demyelinating disease,8

•Associate Professor of Neurology, fResident inNeurology, ^Assistant Professor of Radiology, §Profes-sor of Surgery, from the Division of Neurology andthe Departments of Radiology and Surgery, MedicalCollege of Virginia, Virginia Commonwealth Universi-ty, Richmond, Virginia, 23219.

This study was supported in part by Grant#5T1NB-NSRA.

Reprint requests to Division of Neurology,Medical College of Virginia, Richmond, Virginia (Dr.Hooshmand).

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subdural hematoma,11 epilepsy,6 arteriovenousmalformation,9 positional cerebral ischemia,13

cerebral infarction,8'8 intracerebral hemor-rhage,8 vertigo,3012 syncope and dizziness,"'10

and personality changes.6 In addition, it hasbeen reported as an incidental finding duringradiological evaluation for renal FMD-' *•7 orother conditions.0'8 This long list of differentclinical conditions has given rise to thequestion whether there is any direct causalrelationship between the FMD and the clinicalsymptomatology with which it has beenreported.8-14

The purpose of this paper is to report acase of FMD of the internal carotid arteriesoccurring in association with a symptomatic,angiographically demonstrable, and surgicallycorrectable arteriosclerotic ulcerative plaque ofthe right carotid artery at its bifurcation.Surgical intervention, directed toward thearteriosclerotic lesion and not the FMD,resulted in complete recovery from the fre-quent attacks of transient cerebral ischemia.

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FIGURE 1

Lateral view of common carotid injections with typical appearance of bilateral fibromusculardysplasia. (A) Right. (B) Left. The changes spare the proximal 2.5 cm of the internal carotid.Note the ulcerative plaque (arrow) at the common carotid bifurcation on the right.

Case ReportA 58-year-old woman was referred to the MedicalCollege of Virginia Hospital with a one-monthhistory of repeated attacks of transient left-sidedhemiparesis and hemisensory loss as well as right-sided amaurosis fugax. Physical examination wasunremarkable. Blood pressure was 130/80. Neu-rological examination was normal.

Anticoagulation with heparin was begun atthe time of admission. Skull films, EEG, brainscan, and lumbar puncture were all normal.

Bilateral carotid angiography via the femoralapproach revealed bilateral FMD of the internalcarotid arteries, involvement being much morepronounced on the right (fig. 1). A 2 mm by 2mm ulcerative plaque of the right carotid artery atits bifurcation was also demonstrated (fig. 1,arrow).

The patient was subsequently anticoagulatedwith dicumarol in the therapeutic range anddismissed from the hospital. During the next fivemonths there were rare attacks of amaurosisfugax and no episodes of transient hemiparesis.

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The patient then suddenly had daily attacks—twoto three per day—of amaurosis fugax, frequentlyassociated with transient hemiparesis and hemi-sensory loss, despite adequate anticoagulation.

Six months after the first hospitalization thepatient was readmitted to the hospital. During thefirst five days of hospitalization dicumarol wasdiscontinued and intravenous heparin, 30,000units per day, was begun. The patient had nofurther episodes of amaurosis fugax or transienthemiparesis.

Four-vessel angiography via the femoralapproach was performed on day ten of hospitali-zation, demonstrating again the small ulcerativeplaque and the bilateral FMD. No angiographicalprogression of the FMD was noted whencompared with the study done six monthsbefore.

The patient underwent right common carotidartery exploration (1/8/71) with the finding of a2 mm by 2 mm ulcerative plaque at thebifurcation (fig. 2). This was excised and theartery was repaired by patch graft angioplasty. No

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FIBROMUSCULAR DYSPLASIA

mmnnnmFIGURE 2

Surgical endarterectomy specimen. Irregular athero-sclerotic plaque with a 2 mm ulcer filled with freshfibrin thrombus. (Scale in mm.)

attempt was made to surgically approach theFMD.

The patient tolerated the procedure with noevidence of any neurological deficit. She wasdismissed from the hospital on no anticoagulants.She has had no further symptoms since surgicaltreatment.

Four-vessel angiography performed threemonths after surgery revealed no progression ofthe FMD and no evidence of the pre-existingulcerative plaque (fig. 3).

DiscussionFMD is a nonarteriosclerotic intrinsic arterialdisease with a characteristic angiographicalappearance.4' 8>8 It is to be differentiated fromstationary arterial waves, circular spasticcontractions, and other abnormalities of theextracranial carotid arteries.6 The angiographi-cal appearance correlates with the pathologicalfindings of irregular focal areas of hypertrophyof the fibrous and muscular tissue alternatingwith focal areas of medial thinning anddisruption of both internal and external elasticmembranes.2'B> 9> 1B

The incidence of FMD of the internal

carotid arteries in the general population isunknown, but it is uncommon.8'12

The natural history of FMD of the carotidarteries is not clear. In one case report, therewas no progression of the FMD over sixmonths' follow-up.14 In another case report,the disease developed bilaterally in the patientafter an initial study three months earlier haddemonstrated unilateral involvement.11 In ourcase, we noted no progression of FMD of theinternal carotid arteries six months and ninemonths following the initial angiographicalstudy.

It has been emphasized that the proximal2.5 cm of the internal carotid artery is usuallyspared from FMD.0 '8 In our case, theulcerative plaque was proximal to the areainvolved with FMD, but isolated to the carotidartery bifurcation. The ulcer was in the base ofan irregular plaque which differentiated it from

' -"-T!

FIGURE 3

Lateral view of right common carotid injection threemonths postoperatively. Intimal defect anteriorly inthe internal carotid is the site of the temporary surgi-cal shunt.

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a single mural aneurysm. On the first admis-sion of the patient to the hospital, theulcerative plaque was considered clinicallyinsignificant when compared to the degree andseverity of FMD. Eventually, however, theremoval of the ulcerative plaque rendered thepatient asymptomatic despite no treatment forthe bilateral FMD of the internal carotidarteries. Morris et al.,10 Ehrenfeld et al.,4 andPolin12 have reported cases of FMD of theinternal carotid arteries in association witharteriosclerotic disease at the carotid bifurca-tion. This combination was discovered eitherangiographically or at the time of surgery,necessitating endarterectomy and patch graftangioplasty. However, the surgical treatment inthese case reports had been originally aimed atFMD. In our case, no attempt was made tocorrect the FMD, and the patient becameasymptomatic after removal of the ulcerativeplaque.

Surgical treatment has not been acceptedas a reasonable approach to FMD of thecarotid arteries.8'14 The indications for surgeryare vague, and no one surgical procedure hasbeen consistently advocated."•10'n-10 On theother hand stereotyped transient cerebralischemia has been frequently reported in casereports of FMD in which carotid arterioscle-rotic plaques or stenoses were simultaneouslypresent.3'4> 10' "•1 7 Since FMD may coexistwith the more commonly occurring arterio-sclerotic carotid artery disease, it would seemadvisable to pursue the patients with FMD whohave transient cerebral ischemia for evidenceof a surgically amenable arteriosclerotic lesionif they can tolerate the surgical procedureotherwise. A known indication for carotidendarterectomy is the clinical picture ofamaurosis fugax, transient neurological signs,and angiographically proved ulcerative plaque.17

This should not be overlooked or disregardedbecause of the coexisting FMD which may notbe responsible for the patient's symptoms de-spite its striking appearance on angiography.

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