0148-396X/83/1305-0587$02.00/0.
NEUROSURGERY
Copyright < 1983 by the Congress of Neurological Surgeons

x’ oo € ' 7‘

Vol. 13. No. 5. 1983
Printed in U.S.A.

Bilateral Parietal Arteriovenous Malformations: Report of a
Case

James L. Stone, M.D., Robert M. Crowell, M.D., Blaine M. Lisner, M.D., Mohammad Naseem, M.D., and
John B. Oldershaw, M.D.

Division of Neurosurgery and Neuroradiology, Cook County Hospital; Department of Neurosurgery, University of Illinois at Chicago; and
Tektoen Institute for Medical Research, Chicago, Illinois

A patient with bilateral parietal arteriovenous malformations (AVMs) is reported. The clinical presentation included
seizures, coma, and signs of tentorial herniation. After the evacuation of a large left intraparietal hematoma, cerebral
angiography identified a small left parietal AVM, which was excised. Mass effect and increased intracranial pressure
were present at the time of the initial angiography, and the presence of a right parietal AVM was not appreciated.
Postoperative cerebral angiography to document excision of the left parietal AVM then disclosed the small right parietal
AVM, which was later excised. The patient has recovered very well. The natural history of AVMs and the occurrence
of multiple AVMs are discussed. (Neurosurgery 13:587-592, 1983)

Key words: Arteriovenous malformation, Bilateral symmetry, Cerebral angiography, intracerebral hemorrhage. Multiple

lesions

INTRODUCTION

Cerebral aneurysms and arteriovenous malformations
(AVMs) are frequently thought of as developmental in origin
and may coexist in the same patient. Multiplicity of lesions
and bilateral symmetry is common with aneurysms and rare
with AVMs. We report a case of bilateral parietal AVMs and
discuss the relevant literature.

CASE REPORT

This 24-year-old right-handed man was well until he expe-
rienced the sudden onset of headache with malaise and then
lapsed into unconsciousness. After transfer to Cook County
Hospital, intermittent generalized seizures and bilateral de-
cerebrate posturing were noted. The general physical exami-
nation was normal without cutaneous nevi or angiomas. A
computed tomographic (CT) brain scan revealed a large left

parietotemporal intracerebral hematoma with extension into
the ventricular system and moderate left-to-right shift of
midline structures (Fig. 1). After this study, the patient devel-
oped an enlarging left pupil and was brought directly to the
operating room for decompressive craniotomy. At operation,
a large clot was evacuated from the left parietotemporal area
via a posterior parietal cortisectomy. The hematoma cavity
at its depth communicated with the ventricle. Hemostasis was
not a problem, and no AVM tissue was encountered. A
catheter was left in the ventricle for external drainage. After
closure, a left frontal subarachnoid bolt was placed to monitor
the intracranial pressure (ICP). Postoperatively, the patient
was decerebrate with equal reactive pupils, intact extraocular
motions, and regular respirations. One day after operation,
he began to flex all four extremities, and over the next 5 days
he gradually awoke. For several days postoperatively, the ICP
rose intermittently to range around 20 mm Hg, but it was
easily controlled with intravenous mannitol. The ventriculos-

Fic. 1. Plain CT brain scan shows the large left parietotemporal intracerebral hematoma with intraventricular extension. There is moderate

left-to-right shift of midline structures.

588 STONE et al.

Neurosurgery, Vol. 13, No. 5

Fic. 2
into the i

ilateral vein of Labbé.

tomy drained pinkish fluid for 48 hours and then clear fluid
before removal at 72 hours.

Four-vessel cerebral angiography 3 days after operation
disclosed a left parietal AVM (Fig. 2). The malformation was
fed by angular and posterior temporal branches of the left
middle cerebral artery and drained into the ipsilateral vein of
Labbé. A mild left-to-right midline shift was still present, and
no definite abnormality was initially appreciated in the right
carotid study. A CT scan with enhancement disclosed resolv-
ing intracerebral hematoma. By | week after operation, the
patient was awake with full motor strength. but there was a
fluent aphasia with poor comprehension and paraphasic er-
rors. In addition to diplopia from a left 3rd nerve paresis, he
had a right homonymous hemianopsia to confrontation and
a decreased response to pinprick of the right foot.

One month after admission, excision of the left parietal
AVM was carried out. The left temporoparietal craniotomy
was enlarged. and a 1- by 1.5-cm lesion was totally removed
with microtechnique. Large tortuous vessels, not seen on the
angiogram. were found at the deepest apex of the malforma-
tion. The ventricle was not entered. The resection cavity from
the AVM communicated inferiorly with the parietotemporal
intracerebral hematoma cavity.

Postoperatively the patient showed a worsening and then
subsequent improvement in his Wernicke’s aphasia. Cerebral
angiography several days after operation showed no trace of

Left carotid angiogram demonstrates the left parietal AVM fed by branches of the angular and posterior temporal arteries and draining

the parietal AVM (Fig. 3). The right carotid angiogram now
clearly disclosed a right parietal cone-shaped AVM fed by the
right angular branch of the middle cerebral artery and drain-
ing into the ipsilateral veins of Labbé and Trolard (Fig. 4).
Speech and language comprehension improved gradually with
a speech therapy program.

Six weeks after initial admission. the right parietal AVM
was excised. A right parietotemporal craniotomy disclosed a
2 by |-cm malformation occupying the supramarginal and
angular gyri. The AVM was carefully removed with micro-
technique. Postoperatively the patient was somewhat con-
fused and showed worsening of his aphasia. Postoperative
angiography confirmed complete excision of the right AVM
(Fig. 5). The deficit gradually improved, and he was dis-
charged to a rehabilitation center. Six weeks after the last
operation, there was a mild Wernicke’s aphasia, a right infe-
rior quadrantanopsia. and subjectively decreased pinprick
sensation on the right foot. Although the pupils were equal
and reactive to light and extraocular motions were full. the
patient complained of occasional diplopia and there was an
intermittent left exotropia. His motor strength was full with-
out Babinski’s sign. but the deep tendon reflexes were slightly
increased on the right side. His station and gait were normal.
On examination | year after the last operation. the diplopia
had resolved and his language functions were almost normal.
The patient was living at home with family support

Fic. 4. Right carotid angiogram demonstrates the right parietal AVM fed by the angular artery and draining into the ipsilateral veins of
Labbé and Trolard

590 STONE et al.

Neurosurgery, Vol. 13, No. 5

Fic. 5. Right carotid angiogram after excision of the right parietal AVM. The malformation is gone. and the angular artery is preserved.

DISCUSSION

Our patient deteriorated so rapidly that there was no time
for preoperative cerebral angiography to delineate a possible
cause for the massive intracerebral and intraventricular hem-
orrhage. Helpful surgical adjuncts included rapid generous
craniotomy, corticotomy avoiding dominant speech centers,
and external drainage of bloody ventricular fluid. The ICP
monitor provided an objective guide to further therapy. After
excision of the symptomatic left parietal AVM, complete
cerebral angiographic evaluation identified the right parietal
AVM. Retrospective examination showed that this lesion was
present on the initial right carotid angiogram, but was ob-
scured by mass effect and increased ICP. Complete four-vessel
cerebral angiography is recommended for adequate evalua-
tion of AVMs and associated vascular anomalies (1, 30, 32,
38, 47).

Palliative procedures have proved unsatisfactory in the
treatment of AVMs (30, 36), and complete excision is the

only method of cure. Without surgical excision, 17 to 33% of

AVM patients will die, frequently from hemorrhage (12, 28,
30, 37, 45). Significant neurological impairment or death is
expected in 40 to 60% of patients with unoperated AVMs
(37, 45, 49). Mortality with radical surgical excision in recent
years has been about 10% (28) and with the advent of micro-
surgical technique has been 5% or less (23. 26, 32). Conse-
quently, total excision of all accessible A V Ms is recommended
even in eloquent cortical areas (10, 22, 23. 28, 32). Small

AVMs (<2 to 3 cm) have an increased tendency to enlarge
(9, 20, 28, 51), to hemorrhage (3, 10, 12. 23, 27, 28, 32. 34,
50), and to produce larger intracerebral hematomas (28).

AVMs of the brain are congenital lesions arising at an early
embryonic stage (about 3 weeks) before the differentiation of
arteries, capillaries, and veins (8, 24, 30, 42, 43). Develop-
mental aberration results in the persistence of direct arterial
to venous communications without an intervening capillary
bed (13, 19, 31). The shunting vessels enlarge further during
later development in childhood and adult life (16, 19, 32. 51).
AVMs are also believed to enlarge by repeated. small, silent
hemorrhages (9, 34). The more common occurrence of symp-
toms during the 2nd to 4th decades rather than during child-
hood suggests evolution of the lesions (16, 32, 35, 38. 42).

The association of single or multiple cerebral aneurysms in
about 5 to 8% of patients with an AVM (5, 6, 35, 44, 50)
suggests a tendency for cerebrovascular maldevelopment (4).
Associated aneurysms are usually on vessels that supply the
malformation, but other arteries may be involved (4. 7. 35).
The association of persistent primitive carotid-basilar anas-
tomoses and AVMs also suggests congenital disturbance dur-
ing the early phase of cerebrovascular development (2. 11,
26).

Such a congenital disturbance might be expected to produce
multiple cerebrovascular lesions. Indeed, multiple intracranial
aneurysms are found in about 20% of patients with cerebral
aneurysms (29. 40), and 37% of these multiple aneurysms are
bilaterally symmetrical (29). However, multiple or bilaterally

November 1983

symmetrical AVMs are distinctly uncommon. The Coopera-
tive Study reported multiple. bilateral intracranial angioma-

tous malformations in 3 of 453 cases (35). but forms of

angioma other than the typical arteriovenous (AVM) type
may have been included in this group. Several series of
cerebral AVMs noted multiple cerebral malformations in 1%
(3 of 301 cases) (11. 26. 27). but many more series make no
specific mention of multiple lesions (3. 12. 21. 28. 30, 32. 38).
Several cases with multiple AVMs have been reported. A
patient with asymmetrical bilateral, large AVMs in both hem-
ispheres with separate arterial supply but sharing one common
abnormal vein has been reported (48). This patient also had

multiple cerebral aneurysms and arterial ectasia. One case of

multifocal unilateral intracerebral AVMs was recently de-
scribed, as were bilateral mirror image parietooccipital AVMs
41). The AVMs in this case each filled from the ipsilateral
anterior cerebral artery and appeared contiguous at the mid-
line on the contrast-enhanced CT scan. We do not know
whether they had a separate or a common venous drainage
pattern. Complete angiography had not been done before the
first operation. and the possibility exists that initially a single.

large. bilateral AVM was present. Four additional cases of

apparently separate but asymmetrical intracranial AVMs have
been reported (16, 18. 34. 46). A case of a large, bilateral.
cerebral arteriovenous communication reported in 1946
would more properly be considered a vein of Galen malfor-
mation today (17). Intracranial AVM has also been associated
with spinal cord AVM (14, 15, 33).

To the best of our knowledge. the present case is the first
report of bilateral, clearly separate, balanced (mirror image)
AVMs each with a separate arterial supply and venous drain-

age. On a statistical basis, because approximately 0.14% of

the population harbors an AVM (25). we would expect about
2 people of | million to have two completely separate mal-
formations. Because the middle cerebral distribution and the
parietal lobe are more common loci for AVMs (18, 30, 35,
38. 39). this occurrence of two separate parietal malformations
may be coincidental.

ACKNOWLEDGMENTS

We thank Dr. Leslie Schaffer for technical assistance and
Mr. A. J. Tydell for photographic work.

Received for publication, April 26, 1983: accepted, July 9, 1983,

Reprint requests: James | one. M.D.. Division of Neurosurgery,
Cook County Hospital. 1835 West Harrison Street, Chicago. Illinois
60612.

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COMMENT

The authors have described another rare case of multiple
arteriovenous malformations. Their case is even more unique
because these were the mirror image type of malformations.
In my experience with approximately 200 arteriovenous mal-
formations, | have not operated on any that have been mul-
tiple. Even though rare, the specter of multiplicity in arterio-
venous malformations underscores the need for comprehen-
sive angiography before or after the treatment of these treach-
erous lesions. The well-known incidence of aneurysms occur-
ring with arteriovenous malformations is another reason for
comprehensive angiography. The authors also emphasize the
treacherous nature of these lesions; one of the lesions pre-
sented primarily as a mass of intracerebral hemorrhage, which
has left modest neurological residua. They were wise in pro-
phylactically removing the malformation in the opposite hem-
isphere. The question of potential bilateral speech centers in
a young person when the primary speech area is injured might
be addressed in this patient. One would wonder whether
resection of the nondominant arteriovenous malformation
from the parietotemporal region might interfere with full
recovery of the individual's speech and comprehension defi-
cits that occurred after the hemorrhage into the dominant
parietotemporal region. This obviously cannot be answered:
however, if the patient should recover from this bilateral insult
to homologous areas of the parietotemporal cortex and sub-
cortical white matter, the contention that cerebral function is
displaced to the margins of an AVM because these are con-
genital lesions would be supported.

Bennett M. Stein, M.D.
New York, New York