Orbital Drainage from Cerebral Arteriovenous Malformations
Nicholas J. Volpe, M.D., Mithlesh C. Sharma, M.D.,
Steven L. Galetta, M.D., David J. Langer, M.D.,
Grant T. Liu, M.D., Robert W. Hurst, M.D.,
Eugene S. Flamm, M.D.
Departments of Ophthalmology and Neurology (NJV, MCS, SLG, GTL),
Scheie Eye Institute, Philadelphia, Pennsylvania; Departments of Ophthalmology and
Neurology (NJV, MCS, SLG, GTL) and Radiology (RWH), Hospital of the
University of Pennsylvania, University of Pennsylvania School of Medicine,
Philadelphia, Pennsylvania; and Department of Neurosurgery (DJL, ESF),
Beth Israel Medical Center, Albert Einstein College of Medicine,
Yeshiva University, New York, New York

OBJECTIVE: To describe the neuro-ophthalmic findings in patients with orbital drainage from cerebral arteriovenous
malformations (AVMs).
METHODS: We reviewed the records of 100 consecutive adult patients with cerebral AVMs who presented to our
institution during a 4-year period. All patients with orbital drainage were identified, and their neuro-ophthalmic
evaluations were reviewed.
RESULTS: Three patients (3%) were identified with orbital drainage from a cerebral AVM. The first patient presented with
typical chiasmal syndrome (reduced visual acuity, bitemporal hemianopia, and optic atrophy). Magnetic resonance
imaging demonstrated a large left temporal and parietal lobe AVM with compression of the chiasm between a large
pituitary gland and a markedly enlarged carotid artery. The second patient presented with headaches and postural
monocular transient visual obscurations. Examination revealed normal visual function with minimal orbital congestion
and asymmetrical disc edema, which was worse in the left eye. Magnetic resonance imaging revealed a large right
parietal and occipital lobe AVM without mass effect or hemorrhage and an enlarged left superior ophthalmic vein. The
third patient had no visual symptoms and a normal neuro-ophthalmic examination; a right parietal lobe AVM was
discovered during an examination for the cause of headaches.
CONCLUSION: Orbital drainage from cerebral AVMs is rare. Manifestations may include anterior visual pathway
compression, dilated conjunctival veins, orbital congestion, and asymmetrical disc swelling.
(Neurosurgery 46:820-824, 2000)
Key words: Asymmetrical optic disc edema, Cerebral pial arteriovenous malformation, Chiasmal syndrome, Elevated intracranial pressure, Orbital
drainage

I

ntracranial arteriovenous malformations (AVMs) are
broadly categOrized as dural, pial, or mixed dural-pial on
the basis of their arterial supply. Dural AVMs receive their
blood supply from the meningeal branches, and pial AVMs
are supplied by the cerebral or cerebellar arteries (16). Dural
AVMs and direct fistulae, particularly those involving the
cavernous sinus with drainage into the orbit, have a characteristic neuro-ophthalmic presentation resulting from arteriolization of the orbital venous system. Symptoms include increased intraocular pressure, cranial nerve palsy, conjunctival
chemosis, arterialized conjunctival vessels, bruit, and venous

820

stasis retinopathy (14). Patients with pial AVMs commonly
present with headaches, seizures, and intracranial hemorrhage (7, 14, 15, 18). Depending on location, size, and pattern
of venous drainage, the ophthalmic signs and symptoms of
pial AVMs may include visual field defects and positive visual phenomena (4, 5, 14). Venous drainage of pial AVMs
usually occurs into the deep venous system associated with
the ventricles, or it may follow the pattern of the transcerebral
venous system (14). We examined three patients with orbitally draining cerebral AVMs, two of whom exhibited distinct
neuro-ophthalmic signs and symptoms.

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Orbital Drainage from Cerebral Arteriovenous Malformations
PATIENTS AND METHODS
We reviewed the clinical records of 100 consecutive adult
(> 18 yr) patients with pial AVMs who presented to the Hospital of the University of Pennsylvania between January 1991
and December 1994 for neurosurgical consultation and who
subsequently underwent cerebral angiography. We also analyzed the cerebral angiograms of these 100 patients with
AVMs. Three patients with orbital drainage were identified,
two of whom had relevant neuro-ophthalmic signs and symptoms. All three patients with orbitally draining AVMs underwent complete neuro-ophthalmic examinations, including assessment of visual acuity, color vision, intraocular pressure,
and pupillary reactions, orbital and slit lamp examinations,
formal examination of visual fields, and dilated funduscopy.
Angiograms of the three patients included in the study displayed features of classic AVM architecture: an arterial feeding vessel or vessels, a nidus of arterial and venous tissue, and
an early draining vein. No patients with solely venous angiomas, dural AVMs, or dural fistulae were included. Various
angiographic characteristics of these lesions were considered.
Venous drainage was characterized as occurring through the
deep venous system alone, combined superficial and deep, or
superficial alone. The orbitally draining AVMs were detected
by abnormal filling of the superior ophthalmic veins.

RESULTS
Orbitally draining cerebral pial AVMs were identified in
only 3 of the 100 patients observed during this period. The first
patient had a 7.5-cm AVM in the left temporal and parietal lobes.
The second had a 3-cm AVM in the right parietal and occipital
lobes. The third patient had a 3.5-cm lesion in the posterior
aspect of the right side of the splenium of the corpus callosum.

Case reports
Patient 1
A 40-year-old right-handed woman presented with blurred vision in
both eyes. Approximately 10 years before, she had developed seizures
and been diagnosed with an AVM. Her adherence to her anticonvulsant
regimen was poor, and she had experienced approximately one seizure
per year for many years. She intermittently used cocaine.
Her visual acuity was 20/20 in the right eye and 20/25 in the left
eye. When given the Ishihara test, the patient correctly identified 10 of
15 plates on the right side and 3 of 15 plates on the left side. A left
afferent pupillary defect and a mild bilateral proptosis were observed.
The globes were nonpulsating. Mild soft tissue swelling of the eyelids
along with dilated veins in the conjunctival fornices was observed.
Bilateral temporal optic disc pallor was observed (Fig. 1), and a bruit
was heard over the left orbit. A computerized visual field test revealed complete right homonymous hemianopia. In addition, a superotemporal visual field defect was evident in the left eye. The rest
of the neurological examination results were normal. Magnetic resonance imaging revealed a large left hemispheric AVM involving the
temporal and parietal lobes as well as the left optic tract. The superior

FIGURE 1. Patient
1. Fundus
photograph of the
left eye
demonstrating
temporal pallor of
the optic disc and
corresponding
loss of nerve fiber
striations
(arrows).
ophthalmic vein was dilated on both sides (Fig. 2). The intracavemous
carotid artery was enlarged, and veins around the chiasm were dilated. The chiasm was observed to be compressed between the carotid
artery and an enlarged pituitary gland (Fig. 2). A cerebral angiogram
confirmed the presence of large veins around the chiasm, which were
draining into the orbit. The AVM was noted to be supplied by the
middle cerebral and lenticulostriate arteries. We decided to follow
the patient unless evidence of progressive visual field loss emerged.
Her automated fields have remained stable for 3 years.

Patient 2
A 38-year-old right-handed woman with a long history of seizures
developed an intermittent "pulling" pain around her left eye. She also
experienced episodic visual graying in the same eye. The graying was
diffuse in the left eye, was not hemianopic, and was not present in the
right eye. Each episode, which was precipitated by standing, lasted
for only a few seconds. Other symptoms included colored spots in her
left visual fields, dejii vu phenomenon, and unusual smells.
As revealed by examination, visual acuity was 20/15 in each eye
and color vision was normal. There was no proptosis or evidence of
orbital congestion. Intraocular pressure was 14 mm Hg in each eye,
and no afferent pupillary defect was detected. No bruit was detected,
and Goldmann visual fields were full. The right optic nerve appeared
normal, with possible fullness to the nerve fiber layer at the 6 and 12
o'clock positions. The left optic disc was markedly swollen without
hemorrhages or exudates (Fig. 3). There were no visible spontaneous

FIGURE 2. Patient 1. A, coronal Tl-weighted magnetic resonance image demonstrating a large left hemispheric AVM
(short arrow) and optic chiasm (long arrow) compressed
between the enlarged pituitary gland (arrowhead) and
enlarged carotid artery. H, axial T1-weighted magnetic resonance image demonstrating the enlarged left superior ophthalmic vein (arrow).

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Volpe et al.

FIGURE 3.
Patient 2. Color
fundus
photograph of the
left eye
demonstrating
disc edema at
presentation.

venous pulsations in either eye. The rest of the neurological examination results were normal. Magnetic resonance imaging revealed a
3-cm right posterior parietal and occipital lobe AVM without hemorrhage, mass effect, or edema. The left superior ophthalmic vein was
dilated. These findings were confirmed by cerebral angiography, and
the AVM was noted to have a dual blood supply from both the
middle and posterior cerebral arteries. The AVM drained initially into
the superior sagittal sinus. As a result of congestion within the sinus,
abnormal flow into the left orbit was noted in the venous phase of the
angiogram (Fig. 4). This patient underwent embolization and complete surgical excision of the AVM. Postoperatively, her disc edema
resolved completely in 3 months. She has a residual inferior left-sided
homonymous visual field defect, and she occasionally experiences
formed visual hallucinations in this area.

Patient 3
A 57-year-old right-handed woman presented with chronic daily
headaches. Neuro-ophthalmic examination results were normal, including the absence of a bruit. Magnetic resonance imaging revealed
a right parietal AVM. A cerebral angiogram confirmed the presence
of the AVM and revealed orbital drainage (Fig. 5).

DISCUSSION
We identified three patients with orbital drainage from
cerebral pial AVMs. The retrospective nature of this study and
the referral patterns of our tertiary care facility have the
potential to bias any conclusions regarding the prevalence of
this type of drainage pattern. These three patients, however,
were part of a series of 100 consecutive patients with pial
AVMs. Their presentations were similar to those of other
patients with pial AVMs (headaches and seizures), but two of
the patients had unique neuro-ophthalmic manifestations
of orbital drainage.
The most common neuro-ophthalmic symptoms and signs
found in patients with hemispheric pial AVMs are headaches,
visual field defects, and visual hallucinations. Involvement of
the chiasm and the optic nerve with pial AVMs is rare (11, 14,
21). Two of our three patients with orbitally draining AVMs
developed symptoms and signs that were attributable to the
abnormal drainage pattern and its effect on the anterior visual
pathways.
Patient 1 had dilated (nontortuous) conjunctival vessels and
eyelid soft tissue swelling, and she developed a typical chiasmal syndrome with reduced visual acuity, bitemporal hemianopia, and optic atrophy. Visual loss resulted from compression of the chiasm between an enlarged carotid artery, dilated
veins, and an enlarged pituitary gland. The venous drainage

FIGURE 4. Patient 2. A, lateral view of the arterial phase of
an angiogram showing an occipital AVM. The supply is via
the middle cerebral artery (arrow). H, lateral view of the
venous phase of the angiogram demonstrating flow to the superior
ophthalmic vein (short arro~, and missing the superior sagittal
sinus anteriorly (long arro~.

into the orbit caused the dilation of conjunctival vessels and
eyelid soft tissue swelling. Despite the markedly enlarged
superior ophthalmic vein, it did not result in an elevation of
intraocular pressure, arterialization of conjunctival vessels,
conjunctival chemosis, or venous stasis retinopathy. This patient's visual loss was probably the result of optic tract and

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Orbital Drainage from Cerebral Arteriovenous Malformations

FIGURE 5. Patient 3. Lateral view of the venous phase of an
angiogram demonstrating flow to the orbital venous system
(black arrows), and location of the AVM in the posterior portion of the splenium of the corpus callosum (white arrow).
chiasmal involvement by the AVM and compression of the
chiasm between the vascular elements of the AVM and an
enlarged pituitary gland. Sibony et a1. (21) reported two patients and identified four others in the literature with chiasmal
syndrome secondary to AVMs (3, 5, 6, 22). This series represents a heterogeneous group composed of patients with
Wyburn-Mason syndrome, venous angiomas of chiasm, and
AVMs intrinsic to the chiasm. Only one of these six patients
was similar to ours, with chiasmal compression caused directly by vascular elements of a large pial AVM.
In a series of 202 patients with pial AVMs, Kupersmith (14)
found only two with significantly reduced vision. One patient
had bilateral optic atrophy and severely reduced vision as a
result of venous drainage and damage to the optic chiasm and
intracranial optic nerve. Although the degree of visual loss was
different, our Patient 1 also presented with bilateral optic atrophy.
Patient 2 had transient visual obscurations and asymmetrical disc edema, which was worse on the side with orbital
drainage. Elevated intracranial pressure (ICP) is commonly
associated with dural AVMs because they frequently shunt
arterial blood into the transverse or other major venous sinuses. The associated venous sinus thrombosis also causes
elevated ICP (8, 10, 11, 13, 14, 17). Elevated ICP associated
with unruptured pial AVMs is rare (1, 2, 11, 18, 19, 23, 24).
One mechanism of raised ICP in this type of AVM is a massive
runoff from the large shunting vessels, which increases blood
volume and decreases cerebrospinal fluid absorption (1, 2, 23).
Large pial AVMs per se, or as a result of an aneurysmal
dilation of the venous system draining the AVM, may also
exert a mass effect. Although patients with markedly asym-

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metrical and even unilateral papilledema have been reported
to have elevated ICP (9, 12, 20), the unusual venous drainage
pattern of this AVM may have contributed to this finding.
Angiographically, there was evidence of missing superior
sagittal sinus anteriorly (because of maldevelopment or acquired thrombosis), which, by compromising the normal venous outflow of the brain, probably worsened the effect of
venous hypertension caused by the high-flow arteriovenous
shunting. Postulated mechanisms of the asymmetrical disc
swelling include direct compression of the optic nerve by a
dilated and enlarged superior ophthalmic vein, or disc edema
resulting from increased venous pressure. Finally, reduced
pliability of the optic nerve sheath, caused by engorged pial
vessels as a result of venous back pressure, may be an additional factor in causing asymmetrical papilledema on the side
of orbital drainage.
Two patients with intracranial hypertension secondary to
AVMs, the first with a mixed dural-pial AVM and the second
with a pure pial AVM, were reported by Kashii et a1. (11). The
first patient had asymmetrical papilledema with markedly
asymmetrical vision loss. Although a steal phenomenon from
the ophthalmic artery to the AVM was postulated as a cause
for the asymmetrical vision loss, no specific explanation was
offered for asymmetrical disc swelling. The AVM drained
through the straight sinus into the Sigmoid sinus in their first
patient, whereas, in the second patient, the AVM drained into
the superior sagittal sinus. Orbital drainage of the AVMs was
not described in either of these patients.
Patients with pial A VMs that are draining orbitally may
present with a more heterogeneous constellation of ophthalmic
signs and symptoms compared with the arteriovenous connections that are dura-based in the cavernous sinus. For example,
despite flow from the AVM into the superior ophthalmic vein in
all three of our patients, none had the classic findings of episcleral arteriolization, glaucoma, retinal venous congestion, and
proptosis. However, two of our three patients manifested signs
and symptoms related to the orbital drainage.

CONCLUSION
Three percent of patients with cerebral pial AVMs were
found to have orbital drainage. Patient 1 exhibited orbital
signs and symptoms related to the ophthalmic venous system
drainage and congestion. Two of the three patients had visual
symptoms caused by involvement of the anterior visual pathway; Patient 1 exhibited direct compression of the chiasm,
and Patient 2 had asymmetrical papilledema. Our patients
demonstrate that orbital drainage from cerebral pial AVMs
may be associated with visual manifestations by heterogeneous mechanisms.
Received, June 7, 1999.
Accepted, November 12, 1999.
Reprint requests: Nicholas J. Volpe, M.D., Scheie Eye Institute, 51 N.
39th Street, Philadelphia, PA 19104.

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retrospective study of 100 patients with AVMs who presented
to their institution. Only three of these patients were identified as having cerebral AVMs that drained the orbit, and only
two experienced visual loss.
First, we must deal with the statistics of small numbers. It
would be inappropriate to conclude that visual loss is a more
common manifestation than ophthalmoplesia for cerebral
AVMs that drain into the orbit. Furthermore, no conclusions
as to incidence can be established, given the retrospective
nature of this study. Issues such as the type of hospital and the
types of referring physicians would significantly slant any
interpretation. For example, had the authors' hospital been an
eye and ear infirmary, most of the remaining patients might
have experienced hearing loss or other symptoms associated
with involvement of the petrous bone.
In summary, the authors add three more case reports of
cerebral AVMs that produced visual symptoms. They also
add the information that orbital drainage from cerebral AVMs
may produce symptoms via a variety of mechanisms.
Alfredo A. Sadun
Neuro-ophthalmologist
Los Angeles, California
Although dural AVMs and dural arteriovenous fistulae,
especially those located anteriorly or in the cavernous sinus
region, commonly demonstrate venous drainage into the orbit, pial AVMs rarely demonstrate orbital drainage. Volpe et al.
present three patients with moderate to large pial AVMs that
demonstrate this unusual pattern of retrograde orbital venous
drainage. Two of the patients presented with ophthalmic symptoms and signs. It has been suggested that AVMs develop in the
setting of maldeveloped cerebral venous drainage (1), and this
might provide one explanation for retrograde orbital venous
drainage. A more probable explanation is simply that elevated
venous pressure in association with an AVM forces venous
blood out through available venous channels, including the orbital veins via the cavernous sinus. Once this occurs, visual
symptoms result from elevated intraorbital pressure, pressure
on the visual pathway by engorged veins, and papilledema. The
second patient presented here did not seem to have a definite
venous pathway from the AVM to the orbital veins; elevated
cerebral venous pressure or maldeveloped veins (such as absence of the anterosuperior sagittal sinus) might be the explanation for the abnormal drainage in this patient. This study is a
reminder that pial AVMs occasionally drain into the orbit and
cause symptoms and signs related to anterior visual pathway
compression or orbital congestion. This report should prompt
careful review of cerebral angiograms and thorough ophthalmological assessment of patients with cerebral AVMs.
Marcus A. Stoodley
Gary K. Steinberg
Stanford, California

COMMENTS
Volpe et al. point out that although orbital drainage from a
cerebral arteriovenous malformation (AVM) is rare, it can
produce neuro-ophthalmic problems. This is useful and interesting. However, the authors fully recognize that this is a

1. Mullan S, Mojtahedi S, Johnson DL, Macdonald RL: Embryological basis of some aspects of cerebral vascular fistulas and malformations. J Neurosurg 85:1-8, 1996.

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