Neurol

Med

Chir

(Tokyo)

20, 67•`72,

1980

A Complete Form of Unilateral Retinocephalic
Vascular Malformations: A Case Report
Akinori

KONDO, Junichiro

ISHIKAWA, Junya

and Tsunemaro

HANAKITA

KOYAMA

Department of Neurosurgery, Fukui Red Cross Hospital,
Fukui, Japan
Summary
A complete

form

of

vascular

malformation"

15 years

is presented.

lies

such

between

rare

aneurysm

arteriovenous

in terms

of the

incidence

briefly

commented

upon.

Key words:

malformation

named

woman

with

This case was also associated

as a saccular
usual

vascular

in a 25-year-old

Extensive

and

an

malformation
of intracranial

vascular

malformation,

Introduction

An
extensive
arteriovenous
malformation
involving
the face, retina and brain all on the
ipsilateral
side was named
"unilateral
retinocephalic
vascular malformations"
by Theron
et al. in 19744) after reviewing
and analyzing
22 cases in the foregoing literature
and of their
own three cases. We present a typical case of
this vascular
malformation
which
combines
other cerebral vascular anomalies.

"unilateral

a clinical

with other

azygos

retinocephalic

history

cerebral

of more

vascular

than

anoma-

pericallosal

artery.

The

and this extensive

vascular

malformations

hemorrhage

aneurysm,

and

also

epileptic

difference
seizures

is

azygos artery

temple with small cutaneous
telangiectasia
over the same area, but they did not extend
beyond the midline. She exhibited a mild
exophthalmos
and also moderately
dilated
bulbar conjunctival vessels of the right eye.
Bruit was loudly heard over all the parts of
her right frontal and temporal areas. Her right
fundus
photograph
demonstrated
typical
arteriovenous malformation of the retina which
completely obscured the optic disc (Fig. 1) and
vision of her right eye was almost lost. She

Case Report
A 25-year-old
woman
was referred
to us
because
of disturbed
consciousness
and left
hemiparesis.
She suddenly started complaining
of severe
throbbing
headache
in the right
frontal region after taking a bath 2 days prior
to her admission
and became
semicomatose
thereafter
for several hours.
E xaminations
She was slightly
lethargic
but responsive
to
verbal
commands
upon
admission.
She was
short and slender with slight mental retardation.
She displayed
remarkably
dilated
cutaneous
venous
channels
on her right forehead
and

Fig. 1

Fundus

malformations
the optic

disc.

photograph

shows

arteriovenous

of the right

retina

which

obscure

A. KoNDo ET AL.

68

showed gaze-fixed nystagmus to the right and
upward. Dysarthria was evident and deglutition was moderately disturbed. She showed
spastic left hemiparesis with positive Babinski
sign on the left. All sensations were reduced on
the left side of the body except for the face
where they were impaired on the right side.
Lumbar spinal tap revealed watery clear CSF
with no pleocytosis and normal Queckenstedt's
sign. Approximately
5 days after admission,
she regained clear consciousness and dysarthria
slightly improved, but deglutition
was still
moderately disturbed. About 2 weeks after
admission, she was able to sit on the bed for a
while and dysarthria improved greatly. Almost
I month after admission, she was able to walk
with an aid. When she was discharged from the
hospital after 5 weeks stay, she was able to
walk by herself although her gait was still
clumsy and her left spastic hemiparesis,
especially of her left thumb and fingers, was still
present.

Past History
She first noticed right exophthalmos with
bulbar conjunctival hyperemia and cutaneous
venous engorgement in the area of her right
forehead at approximately 9 years of age. At
age 10 years, she underwent right common
carotid ligation in the neck in another hospital
and the surgery was effective to reduce her
symptoms for a while. Since about 14 years of
age, however, left spastic hemiparesis developed
gradually, right exophthalmos with venous
engorgement on the right forehead recurred
and the right visual acuity and her intelligence
deteriorated. She neither experienced epileptic
seizures nor cerebral ischemic attacks. Her
familial history was non-contributory.
Laboratory Findings
Hematocrit was 37 %, hemoglobulin 12.2
gm % and red blood cell count was 3.8 million
per cu mm. Her hypothalamo-hypophyseal
hormonal function and other routine laboratory
tests were all in the normal range. Electroencephalogram displayed slight but diffuse
cerebral dysfunction. Electrocardiogram was
normal and she did not show cardiomegaly
on plain chest film.

Radiology
Roentgenogram of the skull showed dilated
deep vascular grooves on the right fronto.
parietal area. Right optic canal was not
enlarged.
A selective internal carotid arteriogram was
performed on the right by direct percutaneous
puncture distal to the previous carotid ligation,
It showed a remarkably dilated right internal
carotid artery and a trunk of the middle cerebral
artery. Extensive arteriovenous malformation
involving the retina, optic pathway, sellar
region, hypothalamus, entire basal ganglia and
thalamic region were visualized and this was
mainly fed by dilated lenticulostriate arteries
and enormously developed and dilated branches
from the insular portion of the right middle
cerebral artery. This arteriovenous malforma.
tion was drained mostly by the internal cerebral
vein, right thalamostriate vein, basal vein,
middle cerebral vein, cavernous sinus, vein in
the frontal base, ascending frontal and parietal
veins which were all dilated extraordinarily
(Fig. 2a, b) (Fig. 3a, b).
A saccular aneurysm-like shadow was seen
on the right carotid siphon (Fig. 4).
The anterior cerebral artery was not filled
from this side. Left common carotid arteriogram revealed moderately dilated internal
carotid artery and also an azygos pericallosal
artery. The arteriovenous malformation was
also supplied from the left carotid artery
(Fig. 5).
Vertebral arteriogram demonstrated megadolicho-basilar artery.
The dilated right
posterior cerebral artery and thalamoperforators sent off many feeders to the conglomerated
arteriovenous malformation occupying the
posterior portion of the thalamus and the upper
brain stem (Fig. 6).
Brachial arteriogram revealed a dilated
innominate artery and the previously occluded
common carotid artery . The right inferior
thyroid artery, the ascending cervical arteries
and the cervical profund artery were all
dilated and tortuous and they made as
abnormal vascular network in the mandibular
and maxillar regions (Fig . 7). The left inferior
thyroid artery and possibly the right superior
thyroid artery, both dilated , were making a

A CASE

OF UNILATERAL

a

RETINOCEPHALIC

VASCULAR

MALFORMATIONS

b

Fig. 2 Frontal projection of the selective right internal carotid arteriogram. a: Late
arterial phase. A huge cerebral arteriovenous malformation mainly fed by
dilated lenticulostriate arteries and branches from the insular portion of the
middle cerebral artery, occupying basal ganglia and thalamic region. b: Venous
phase. Dilated thalamostriate veins and numerous deep draining veins.

b

Fig. 3 Lateral projection of the selective right internal carotid arteriogram. a:
Arterial phase. An extensive vascular malformation involves the retinal region
(arrow heads), optic pathway (arrows), sellar region and thalamus. b: Venous
phase. Extraordinarily dilated numerous draining veins: internal cerebral vein,
basal vein, middle cerebral vein, ascending frontal and parietal veins and so on.

69

70

A.KONDO

Fig.4 Frontal projection of the selective right
internal carotid arteriogram shows a saccular
aneurysm like shadow (arrow) on the carotid
siphon.

ET AL.

Fig.6 Lateral projection of vertebral arteriogram.
Late arterial phase. Posterior part of an extensive
arteriovenous malformation involves posterior
thalamic region and brain stem, but not the
posterior fossa. Megadolichobasilar artery is
also noted. (arrows)
crossing anastomotic channel in the anterior
portion of the neck through which the right
carotid artery was visualized distal to the
occlusion (Fig. 8).
Pyelogram on both sides taken during carotid
arteriography showed no abnormalities, such
as polycystic kidney.
Comments

Fig.5 Frontal projection of the selective left
internal carotid arteriogram. Arterial phase.
A part of arteriovenous malformation of the right
hemisphere is supplied from the left side. No
abnormal vascular malformation on the left
hemisphere. An unpaired pericallosal artery is
visualized. (arrows)

Multiple arteriovenous malformations involving both the brain and the retina reported
by Yates and Paine5) are a rare clinical and
anatomical entity and this vascular disorder is
peculiar since it is always mainly lying on one
side of the face and brain although the malformation is wide and extensive. The case
presented here shows a complete form of this
extensive vascular abnormality which involved
the retina, optic pathway, thalamus, brain
stem and also the face and mandibular region
all on the right side and also had mental
dullness.1,6) She suddenly lost consciousness for

A CASE

OF UNILATERAL

RETINOCEPHALIC

Fig. 7 Frontal projection. Right brachial arteriogram shows dilated vertebral artery and also
dilated and tortuous inferior thyroid artery
(arrow heads) and ascending cervical arteries
(arrows) which make abnormal vascular network
in the mandibular and maxillar regions.

several hours and revealed signs of increased
intracranial pressure thereafter. Her spinal tap
did not show bloody CSF throughout this
episode, but her clinical history suggested the
presence of a right intracerebral hemorrhage.
Among the reviews of previous cases and their
own cases reported by Theron et al.,4) 2 cases
developed subarachnoid hemorrhage and 3
cases developed intracerebral hemorrhage. The
incidences of intracranial hemorrhage from
extensive arteriovenous malformations are
much less frequent than that of usual cerebral
arteriovenous malformation according to Pool
and Potts who described that intracranial
hemorrhage occurred in 40-60% of the total
cases. 3) The patient reported here did not
experience epileptic seizures, and only one case
of petit mal attack was reported in the summarizing review of 25 cases by Theron et al.4)

VASCULAR

MALFORMATIONS

71

Fig. 8 Frontal projection
of the left subclavian
arteriogram.
Left inferior thyroid artery (arrow
heads) and possibly the right superior thyroid
artery (lower arrow) make a crossing anastomotic
channel which supplies distal portions of the
right internal carotid artery (upper arrows).

This incidence of epilepsy is also extraordinarily
rare when compared to the results of 30-50
incidence of epileptic seizures in usual arteriovenous malformation.3)
Theron et al.4) did not mention other vascular
anomalies concomitant
with "unilateral retinocephalic vascular malformations"
in their
own series or in their summarizing review of
other cases. Since these extensive arteriovenous
malformations
arise from multiple developmental anomalies, they likely combined other
types of vascular anomalies, such as a saccular
aneurysm and azygos artery, which were seen
in our case.2)
References
1) Brown, D. G., Hilal, S. K. and Tenner, M. S.:
Wyburn-Mason Syndrome. Report of two
cases without retinal ineolvement. Arch Neurol

72

A. Koi mo ET AL.

28: 67-68, 1973.
2) LeMay, M. and Gooding, C. A.: The clinical
significance of the azygos anterior cerebral
artery (A.C.A.). Am J Roentgenol Radium
Ther Nucl Med 98: 602-610, 1966.
3) Pool, J. L. and Potts, D. G.:"Aneurysm and
Arteriovenous Anomalies of the Brain."
Harper & Row Publishers, New York, 1965,
330 p.

4)

Theron, J., Newton, T. H. and Hoyt, W. F.:
Unilateral retinocephalic vascular malforma.
tions. Neuroradiology 7: 185-196, 1974.
5) Yates, A. G. and Paine, M. B.: A case of
arteriovenous aneurysm within the brain.
Brain 53: 38-46, 1930.
6) Wyburn-Mason, R.: Arteriovenous aneurysm
of mid-brain and retina, facial naevi and
mental changes. Brain 66 (3) : 163-203, 1943,