Neurol Med Chir (Tokyo) 46, 441¿445, 2006

Surgical Treatment of Non-ruptured Giant Occipital
Arteriovenous Malformations With Frequent
Migraine-Like Headache
—Two Case Reports—
Shinji NAGATA, Takato MORIOKA, Yoshihiro NATORI*, and Tomio SASAKI
Department of Neurosurgery, Kyushu University Graduate School of Medical Sciences, Fukuoka,
Fukuoka; *Department of Neurosurgery, Aso Iizuka Hospital, Iizuka, Fukuoka

Abstract
Two patients with giant occipital arteriovenous malformation (AVM) underwent microsurgical
treatment among 294 patients with intracranial AVM treated between 1981 and 2004. The patients were
aged 52 and 65 years and showed common symptoms consisting of long-term frequent migraine-like
headaches with visual aura and recent homonymous hemianopia. Common neuroimaging findings
were abundant dural feeders plus feeders from the posterior, middle, and anterior cerebral arteries, and
deep drainers to the galenic system. Both patients underwent preoperative staged embolization and total
microsurgical removal of the nidus with a neuronavigation system. Both patients returned to ordinary
life without the need for assistance. These findings emphasize that giant occipital AVM, even if
unruptured, is surgically treatable with acceptable morbidity.
Key words:

giant arteriovenous malformation,

occipital lobe,

Introduction

Case 1: A 52-year-old woman had suffered from
severe frequent migraine-like headaches with visual
aura for 30 years. She gradually noticed left visual
field cut 3 years previously without sudden symptoms or other neurological deficits. She visited a
local hospital. Brain computed tomography (CT) and
magnetic resonance (MR) imaging showed a giant
right occipital AVM (Fig. 1). The maximum
diameter of the nidus was more than 6 cm on MR
imaging. The nidus partially extended to the major
forceps of the corpus callosum and anteriorly
compressed the trigone of the lateral ventricle. The
main feeders were the posterior cerebral artery and
dural feeders from the middle meningeal and

Case Reports
We treated 294 patients with intracranial AVM at
September 14, 2005;

Accepted

microsurgery

the Department of Neurosurgery, Kyushu University
Graduate School of Medical Sciences, and the
Department of Neurosurgery, Aso Iizuka Hospital
between 1981 and 2004. These cases included 27
occipital AVMs, of which two were Spetzler and
Martin grades IV and V. These patients presented
with severe frequent migraine-like headaches and
complete homonymous hemianopia, so we selected
microsurgical treatment combined with staged
embolization with n-butyl 2-cyanoacrylate (NBCA).

Surgical treatment is not recommended for most
Spetzler-Martin grade IV and V arteriovenous
malformations (AVMs)4) because of the generally
low rate of hemorrhage, high risk of hemorrhage
after incomplete excision of the nidus, and high
mortality and morbidity after total resection. The
American Heart Association Scientific Statement11)
and Japanese Guidelines14) on the management of
intracranial AVMs also recommend conservative
follow up for grade IV and V AVMs unless accompanied by massive hematoma, associated aneurysm, or
progressive neurological deterioration.4,10,12,14,16)
However, giant occipital AVMs are good candidates
for microsurgery despite their size.22) Here we
describe two cases of giant occipital AVM cured by
microsurgery.

Received
2006

migraine,

April 3,

441

442

Fig. 1

S. Nagata et al.

Case 1. Brain T1- (left) and T2-weighted
(right) magnetic resonance images showing
flow voids in the whole right occipital lobe
and enlarged draining veins in the retrosplenial region. The medial wall of the
trigone of the lateral ventricle was compressed by the extension of the nidus to the
major forceps of the corpus callosum.

vertebral arteries. The anterior two-thirds of the
nidus was supplied by pial feeders and the posterior
one-third by dural feeders. Deep venous drainage
was identified and the perimesencephalic veins
were enlarged (Fig. 2). The diagnosis was Spetzler
and Martin grade V. She was referred to us for
surgery.
On admission, she had complete left homonymous
hemianopia confirmed by formal ophthalmologic
visual field testing and mild left hemiparesis. To
reduce the blood flow and prevent overload of the
surrounding brain, preoperative embolization of
both pial and dural feeders was performed in six
sessions with NBCA. Fifty days after the last
embolization, the nidus of the AVM was completely
removed with the aid of a neuronavigation system.
Postoperative angiography detected neither the
residual nidus nor arteriovenous shunts (Fig. 3).
After surgery, she showed deterioration of the left
hemiparesis. Rehabilitation helped her recover and
return to normal life without the need for assistance
in 3 months. The headaches completely disappeared
after surgery without the need for medication.
Case 2: A 65-year-old man had suffered from almost
daily migraine-like headaches with visual aura for
20 years. He gradually noticed difficulty in driving
his car due to visual field cut 3 years previously,
without sudden symptoms or other neurological
deficits. He visited a local hospital. Brain CT and
MR imaging showed a giant left occipital AVM with
a maximum diameter of more than 6 cm (Fig. 4). He
was referred to us for surgery.

Fig. 2

Case 1. upper row: Preoperative left vertebral angiograms showing enlarged pial
feeders from the right posterior cerebral
artery and dural feeders from the vertebral
artery. middle row: Preoperative right internal carotid angiograms showing participation of the middle cerebral artery in the
anterior part of the nidus. The nidus was
clearly visualized via the well-developed
posterior communicating artery. lower
row: Preoperative right external carotid
angiograms showing enlarged middle
meningeal arteries feeding the torcular
portion of the arteriovenous malformation.

On admission, he had no neurological deficits
except complete right homonymous hemianopia.
Cerebral angiography showed pial feeders mostly
from the posterior and middle cerebral arteries
supplying the anterior two-thirds of the nidus. Dural
feeders from the middle meningeal and vertebral
arteries supplied the posterior one-third (Fig. 5).
The diagnosis was Spetzler and Martin grade IV

Neurol Med Chir (Tokyo) 46, September, 2006

Surgical Treatment of Non-ruptured Giant Occipital AVM

Fig. 5
Fig. 3

Case 1. Postoperative right internal
carotid (upper row) and left vertebral angiograms (lower row) showing no residual
nidus or arteriovenous shunt confirming
total removal of the arteriovenous malformation.

443

Case 2. Left internal carotid (upper row),
external carotid (lower left), and vertebral
angiograms (lower right) showing the
anterior two-thirds of the nidus was
supplied mainly by the posterior cerebral
artery together with the middle and anterior
cerebral arteries, and the posterior one-third
was supplied by the middle meningeal
artery and dural branches of the vertebral
artery.

the AVM was completely removed with the aid of
the neuronavigation system.
The postoperative course was uneventful and
postoperative angiography showed no residual
nidus or arteriovenous shunt (Fig. 6). He was
discharged without headaches or neurological
deficits except the pre-existing right homonymous
hemianopia.
Fig. 4

Case 2. Brain T1- (left) and T2-weighted
(right) magnetic resonance images showing
flow voids in the left occipital lobe and
enlarged draining veins in the retrosplenial
region. The trigone of the lateral ventricle
was anteriorly shifted due to compression of
the major forceps of the corpus callosum by
the arteriovenous malformation.

because of the complete homonymous hemianopia.
Two-stage embolization of the pial and dural feeders
with NBCA was performed before surgery. Twentyseven days after the last embolization, the nidus of

Neurol Med Chir (Tokyo) 46, September, 2006

Discussion
A large series of 500 intracranial AVMs included 34
occipital AVMs (6.8%), three of which were giant
(more than 6 cm in diameter).22) Surgery in 30 cases
including three giant AVMs resulted in favorable
outcomes without morbidity or mortality, suggesting that the outcome was independent of size, probably due to the characteristic features of occipital
AVMs. The occipital lobe is an eloquent part of the
brain, particularly for the optic system. However,
optic function has often already been lost in patients
with giant occipital AVMs, as in our patients.

444

Fig. 6

S. Nagata et al.

Case 2. Postoperative left vertebral (upper
row) and common carotid angiograms
(lower row) showing no residual nidus or
arteriovenous shunt, confirming total
removal of the arteriovenous malformation.

Moreover, the risk of additional functional deficits
is low.
Endovascular embolization is important in
multimodality treatment of AVMs, particularly
for patients with a nidus volume of more than
10 ml.6,13,15,21) Pre-surgical embolization is particularly effective for giant occipital AVMs, because the
nidus is usually attached to both the falx and tentorium and receives blood supply from the dural feeders. Both pial and dural feeders are candidates for
embolization, but embolization of the dural feeders
is reliable and safe, allowing precise obliteration of
the compartment of the nidus being fed. Therefore,
in contrast to other grade IV and V AVMs, giant
occipital AVMs can be cured by surgery with acceptable morbidity.
Headache is a well-known symptom of occipital
AVM. Migraine-like headaches occurred in 0–31%
of reported cases of intracranial AVM.1) However,
AVM is found in less than 1% of patients with
migraine. This symptom is now classified as secondary headache attributed to unruptured AVM.5)
The pathogenesis of the headache is unclear, but
larger nidus volume, tortuous change of the feeding
artery, and cortical drainage with reflux in the
superior sagittal sinus are associated with higher
incidence of headache.9) Five of 34 patients with

occipital AVMs had ophthalmic migraine (14.7%).22)
Frequent migraine-like headaches prevent a normal
life, so resolution by removal of the occipital AVM is
desirable.3,8,17,20) Frequent migraines or migraine-like
headaches should be a factor when considering
surgical treatment for occipital AVM, even if the
AVM is non-ruptured and/or giant. Middle age is not
an indication for more conservative treatment. In
fact, patients with AVM identified at higher age
show a higher incidence of AVM hemorrhage.18)
The present cases of giant occipital AVM were
characterized by recent development of visual field
defects and abundant participation of dural feeders.
Moreover, both patients noticed gradual visual field
cut. Because neither showed signs of AVM rupture,
such as sudden headache or other neurological
symptoms, the recent visual field cut might be
attributable to developmental growth of the AVM.
An immunohistochemical study suggested that
transient regional hypoxia within the AVM nidus
might mediate neoangiogenesis related to growth of
the nidus.19) Previous cases of AVM recurrence after
surgical excision and postoperative angiographic
disappearance suggest that regrowth of the remnant
arteriovenous shunt will result in development of de
novo AVM nidus.2,7) We speculate that development
of the dural feeders might further enhance gradual
growth of the nidus, resulting in homonymous
hemianopia. Giant occipital AVMs might enlarge
beyond the extent of the occipital lobe, becoming
inoperable and incurable, and as a result, the severe
migraine-like headaches and AVM will persist.
Therefore, microsurgical treatment of giant
occipital AVMs is indicated in patients with complete homonymous hemianopia, even if the AVM is
not ruptured.

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Address reprint requests to: S. Nagata, M.D., Department
of Neurosurgery, Kyushu University Graduate
School of Medical Sciences, 3–1–1 Maidashi,
Higashi–ku, Fukuoka 812–8582, Japan.
e-mail: snagata＠ns.med.kyushu-u.ac.jp