Aneurysm of the Cervical Internal Carotid Artery Associated with Marfan's Syndrome —

Takashiro

OHYAMA,

Department

Case Report—

Susumu

of Neurosurgery,

OHARA

and

Yao Tokushukai

Fumiyuki Hospital,

MOMMA Yao, Osaka

Abstract An aneurysm

of the internal

carotid

old female with a 2-year history size. Left carotid angiography carotid

artery.

The

aneurysm

artery

associated

of a pulsating demonstrated was excised

with

Marfan's

syndrome

occurred

in a 23-year

lesion in the left neck, which progressively increased in a giant saccular aneurysm at the origin of the internal

and end-to-end

anastomosis

performed

without

postopera

tive morbidity. Key words:

Marfan's

syndrome,

cervical

internal

Introduction

Marfan's syndrome is associated with arterial aneurysms secondary to cystic medionecrosis of the vessel wall, including the intracranial major arteries. The most common site is the intracranial internal carotid artery,2,a,b,1o,13,1a> and sometimes the ex tracranial carotid artery.',') We report a case of aneurysm of the cervical inter nal carotid artery associated with Marfan's syn drome, treated by excision of the aneurysm and end to-end anastomosis of the internal carotid artery. Case On August female with

21, 1989, Marfan's

complaining of a lump area, persisting for about

carotid

artery,

aneurysm

digits. The thorax was very thin and the chest mildly funnel-shaped. Her pulse rate and blood pressure were normal. There was a 3 x 4 cm pulsating lesion with a bruit in the left submandibular area or below the mastoid process (Fig. 1). She had no neurological deficit. The plain skull x-ray films appeared normal. Her cervical spine x-ray films showed no distinct ab normalities except for a deformed atlas. The brain computed tomographic (CT) scans appeared normal. Left carotid angiograms showed a 3.3 x 4.2 cm aneurysm at the proximal portion of the left internal carotid artery. No intracranial aneurysms were detected (Fig. 2). The cervical CT scans also demon

Report a 23-year-old right-handed syndrome visited our clinic in her left submandibular 2 years. She underwent im

plantation of two Harrington's distraction rods at age 15 years for severe scoliosis. She also had heart murmur and had been treated by a cardiologist for the last few years. About 2 years previously, the car diologist had noticed the small lump and recommend ed consulting a neurosurgeon. The lump gradually enlarged before she came to our clinic. She was very tall and thin with quite long slender Received 1992

November

8,

1991;

Accepted

June

16,

Fig.

1

Left

neck

mandibular

view,

showing

or submastoid

the lump process

in the area.

sub

Fig.

2

Left

carotid

3.3

x 4.2 cm

tion

of the

cm distal

angiograms, aneurysm

left internal

from

showing

the

at the

proximal

carotid

artery

giant por

about

1

the bifurcation.

strated this aneurysm. Echocardiographic examina tion detected mild mitral regurgitation. No tolerance test, such as the balloon Matas test with somatosensory evoked potential (SEP) monitor ing, was performed preoperatively because the col lateral blood supply from the contralateral internal carotid artery via the anterior communicating artery seemed to be adequate, and an occlusion test at the internal carotid artery distal to the aneurysm might have caused complications. The aneurysm was excised and end-to-end anastomosis of the internal carotid artery performed (Fig. 3). No graft vessel was prepared because the preoperative angiograms showed that the artery after aneurysmectomy would be long enough for end-to-end anastomosis. Intraoperative SEP moni toring with right median nerve stimulation during internal carotid artery occlusion with vessel tape for 7 minutes showed no significant change in the central conduction time (CCT). In addition, the stump pressure of the internal carotid artery mea sured was 85 mmHg. The internal carotid artery was extensively dissected around the aneurysm to expose an adequate length for end-to-end anasto mosis. Aneurysmectomy and anastomosis required internal carotid artery occlusion for 31 minutes, during which the CCT did not change significantly. Histological examination of the aneurysmal wall revealed extensive medionecrosis with deposition of a mucoid-like substance, compatible with arterial changes due to Marfan's syndrome 13)(Fig. 4). The postoperative course was uneventful returned to work after 2 months postoperative

Fig. 3

Operative findings, showing the aneurysm (up per) and excision of the aneurysm and end-to end anastomosis of the internal carotid artery (lower).

Fig. 4

morbidity.

Histological

Postoperative

findings

of the

showing

extensive

tion

a mucoid-like

with drome.

of

arterial

change

HE stain,

substance, due x 40.

angiograms

aneurysmal

medionecrosis to

and she without

with

wall, deposi

compatible Marfan's

syn

Table

1

Cerebral Marfan's

Fig.

5

Postoperative rysm

totally

angiograms,

showing

the

aneurysms

associated

with

syndrome

aneu

removed.

showed complete disappearance of the aneurysm (Fig. 5). Clinical follow-up examination 13 months later showed no abnormality. Discussion

Marfan's syndrome is a congenital connective tissue disease, transmitted as an autosomal dominant trait.") The syndrome is characterized by a disorder of collagen formation.") The joints are commonly affected resulting in overextension. "> The skeletal system is also involved, resulting in abnormal height, long extremities, arachnodactyly, and deformity of the spine and the thorax." Ocular involvement in cludes blue sclera, turbid cornea, and lens disloca tion.") Cardiovascular lesions occur in 95% of pa tients, such as dissecting aortic aneurysm, annulo aortic ectasia, cardiac septal defect, and mitral valve prolapse syndrome.") The prognosis depends on the cardiovascular lesions, and 93-100% of patients with Marfan's syndrome die due to such dis orders.', 11) Aneurysms of the cerebrovascular system are less common. Thirteen intracranial aneurysms and two extracranial internal carotid artery aneurysms were previously reported'-6,10,13.14) (Table 1). The incidence of stroke in patients with extracranial internal carotid artery aneurysms approaches 50%, resulting from trauma, degenerative arteriosclerosis, and previous carotid surgery.',') All three extracranial carotid artery aneurysms associated with Marfan's syndrome started to present as asymptomatic masses.

The methods of management are aneurysmec tomy, aneurysmorrhaphy, and ligation. 1,7,12) The brain must be protected from intraoperative ischemia. The collateral blood supply via the anterior communicating artery, posterior com municating artery, etc., should be examined by cerebral angiography. The preoperative balloon Matas test with SEP monitoring and intraoperative SEP monitoring are useful to predict cerebral ischemia.8 If these are not possible, the angiographic appearance of the circle of Willis and carotid artery stump pressure mea surements should be evaluated. If the pressure is low, an intraoperative shunt should be considered. In our case, the balloon Matas test on the internal carotid artery may have caused complications. We therefore carried out intraoperative SEP monitor ing during occlusion of the internal carotid artery

with vessel tapes. Previously reported intracranial aneurysms associ ated with Marfan's syndrome occurred in five males and eight females. The mean age is 36.5 years. Seven aneurysms were located in the internal carotid artery, three in the posterior communicating artery, four in the anterior cerebral artery, one in the ante rior communicating artery, five in the middle cere bral artery, and one in the posterior cerebral artery. Five cases (38.5%) had multiple aneurysms. These aneurysms tend to develop multiply, and in young females. The location is more commonly in the internal carotid portion than the vertebrobasilar system. Symptoms included headache in five cases, cranial nerve pareses in two, and altered con sciousness and hemiparesis in one. Autopsy after subarachnoid hemorrhage revealed cerebral aneurysms in three cases. Conventional cerebral angiography revealed cerebral aneurysms in three asymptomatic cases. Although the incidence of intracranial aneurysms associated with Marfan's syndrome is not high, rup ture causes severe morbidity. Therefore, cerebral angiography should be considered for patients with Marfan's syndrome. Intracranial or extracranial carotid artery aneurysms associated with Marfan's syndrome should be treated surgically as with any other aneurysm. References 1)

2)

3)

Busuttil RW, Davidson RK, Foley KT, Livesay JT, Barker WF: Selective management of ex tracranial carotid arterial aneurysms. Am J Surg 140: 85-91, 1980 Finney HL, Roberts TS, Anderson RE: Giant in tracranial aneurysm associated with Marfan's syn drome. Case report. J Neurosurg 45: 342-347, 1976 Hardin CA: Successful resection of carotid and ab dominal aneurysm in two related patients with Marfan's syndrome. New Eng J Med 260: 141-142, 1959

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12)

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14)

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Address reprint requests to: T. Ohyama, M.D., Depart ment of Neurosurgery, Yao Tokushukai Hospital, 3 15-38 Kyuhoji, Yao, Osaka 581, Japan.