J Neurosurg 65:417-420, 1986

Extensive dural arteriovenous malformation
Case report
OSSAMA AL-MEFTY, M.D., JOHN R. JINKINS, M.D., AND JOHN L. FOX, M.D.

Department of Neurosciences, King Faisal Specialist Hospital and Research Centre, Riyadh,
Saudi Arabia
v- A child with an extensive dural arteriovenous malformation (AVM) of the sigmoid sinus and bilateral
occlusion of the transverse sinus is reported. His intracranial venous flow had become rerouted through the
right cavernous sinus and then into the fight ophthalmic veins. The AVM was associated with severe
hydrocephalus and a progressive devastating neurological deficit. The hemodynamic and intracranial pressure
changes as well as the mechanism of sinus thrombosis are discussed. The role of aggressive surgical treatment
is emphasized.
KEY WORDS

9 arteriovenous malformation

hydrocephalus

9 thrombosis

D

URAL arteriovenous malformations (AVM's) in
the region of the transverse sigmoid sinus,
although rare, represent a well-defined entity
from both clinical and angiographic aspects. 1'7-9'13'14
The etiology and pathology of these anomalies remain
controversial, t,7,~2-t4,24'25 The clinical findings are determined by the volume, direction, and route of the
venous drainage. The disease often progresses, leading to increasing neurological deficits, elevation of intracranial pressure, hydrocephalus, seizures, and hemorrhage. 2'7'8'13"24 Unilateral thrombosis of the sigmoid
sinus is recognized to be associated with these lesions. 4A~
In rare cases, the thrombosis is bilateral. 2,4,5,20.24
We report a case that exemplifies the devastating
neurological deficit that results from intracranial hemodynamic and pressure changes associated with this
type of AVM. The patient made a remarkable recovery
after radical surgical treatment.
Case Report

This 13-year-old Syrian boy was admitted to the King
Faisal Specialist Hospital on October 30, 1984. His
medical history started 6 years before, when his right
cheek was struck by a stone. The injury was mild.
Several months later a gradually increasing proptosis of
the right eye and swelling of the forehead and nose was
noticed. One year prior to admission, the patient had

J. Neurosurg. / Volume 65/September, 1986

9 dura mater

9 sigmoid sinus

9

increasingly severe headaches and deteriorating consciousness. He had been seen in several medical centers
prior to his present referral.
Examination. The child was severely cachectic, had
several decubitus ulcers, and was debilitated, with flexion contractures of all four limbs. He had proptosis of
the right eye with marked dilatation of the fight superior
ophthalmic and facial veins. A loud bruit was heard
over the right side of the head. He was awake, but did
not follow commands and spoke only a few words.
Bilateral papilledema was present. The ocular movements were intact.
Pre- and postcontrast computerized tomography
(CT) scans demonstrated hydrocephalus and extensive
dilatation of vascular structures within the cranium and
right orbit. Peripheral rim calcifications of these vascular structures were present, suggesting dystrophic
changes in the walls of arterialized veins (Fig. 1). The
initial arteriograms showed a direct dural arteriovenous
fistula (Fig. 2 left). The major feeding vessel was a
markedly hypertrophied right middle meningeal artery
communicating with the dural venous sac, which
drained into the sigmoid sinus. Additional communications with this dural venous sac were via some unnamed branches originating from the right internal
carotid artery in the petrous bone and also from a large
meningo-orbital branch (also known as "the superior
orbital branch of the middle meningeal artery") ema-

417

O. A1-Mefty, J. R. Jinkins and J. L. Fox
nating from the lacrimal division of the ophthalmic
artery (Fig. 2 right). The venous phase of the arteriogram demonstrated bilateral transverse sinus occlusions, and aneurysmal dilatation of the sigmoid sinus
and torcula Herophili (Fig. 3). Extracranial drainage
was entirely via the right cavernous sinus and then into
the superior and inferior ophthalmic veins.
Operations. At the first operation, the right middle
meningeal artery was occluded by a Fogarty catheter
balloon. A similar balloon was lodged more proximally
in the internal maxillary artery, after which the external
carotid artery was ligated. A few days later a small fight
frontotemporal craniotomy was performed with clipping of the meningo-orbital artery. Angiography then

demonstrated that the fistula was filled from hypertrophied branches of the intrapetrous portion of the internal carotid artery (Fig. 4 left).
At the third operation, temporal craniotomy was
performed with clipping of the internal carotid artery
feeders behind the petrous pyramid and total excision
of the aneurysmal sac contained within the two leaves
of the dura mater. Postoperative angiography confirmed complete obliteration of the AVM (Fig. 4 right).
The route for cerebral venous drainage via the ophthalmic veins remained, but the pressure and size of the
dilated vascular structures were much reduced. The
ophthalmic veins collapsed with the patient in an upright position.
Postoperative Course. The patient underwent grafting of the decubitus ulcers, a ventriculoperitoneal shunt
was placed, and the flexion contractures of his limbs
were treated orthopedically. After 6 months of rehabilitation, he was able to walk and converse with continually improving mental and physical skills. One year
after surgery, he has nearly normal mentation and
neurological function. He is being treated for hip dislocation.
Discussion

FIG. 1. Plain computerized tomography scan at the level
of the lateral ventricles demonstrating hydrocephalus and
calcifications.

The cause-and-effect relationship between the presence of the dural AVM and the thrombosis of the sinus
is controversial. Sundt and Piepgras 24 have suggested
that the dural AVM is an acquired abnormality evolving from revascularization of the previously thrombosed sinus - - the sinus thrombosis being the primary
event. Contrary to that, the sinus thrombosis may develop as a result of the hemodynamic and morphological changes caused by the turbulence of the AVM.
Over a century ago, Virchow postulated that the path-

FIG. 2. Left: Right external carotid arteriogram, lateral view, showing the middle meningeal artery
markedly hypertrophied and connecting directly to the dural venous fistula (arrow). Right: Right internal
carotid arteriogram, lateral view, depicting the enlarged fight meningo-orbital artery (closed arrow) arising
from the lacrimal division of the ophthalmic artery (straight open arrow). The curved open arrow shows the
point of arteriovenous connection, and the arrowhead the meningeal feeding vessel arising from the internal
carotid artery in the petrous bone.
418

J. Neurosurg. / Volume 65/September, 1986

Dural arteriovenous malformation
ogenic events that led to thrombus formation included:
1) stasis; 2) changes in blood coagulability; and 3)
alterations in the vessel wall. 3 Such phenomena certainly may occur in dural AVM's. Seeger, et aL, 17
demonstrated radiographic signs of thrombus formation in the cavernous sinuses in eight consecutive patients with carotid-cavernous fistulas. In five of these
patients, progression of thrombosis was revealed on
follow-up angiography. They hypothesized that the arterial shunt into the venous sinus elevates the venous
pressure to a critical point at which the flow in the
tributaries is decreased, resulting in blood stasis within
the sinus.
Many patients with sigmoid sinus AVM's present

with elevated intracranial pressure, 1~ which, if it is of
sufficient magnitude, may compress and collapse the
dural sinus wall.15.19 Osterholml5 demonstrated that this
collapse of the dural sinus occurs in the transverse sinus
area. Stasis within the sinus would then follow.
When these dynamic and morphological changes in
the sinus occur, endothelial and subendothelial changes
can ensue, leading to thrombosis. Stewart, et al., 22
presented experimental data showing leukocytic invasion into a venous wall following stasis in the vein
associated with surgical trauma to adjacent tissue. The
leukocytic invasion caused venous endothelial damage
and desquamation. Endothelial desquamation exposes
subendothelial collagen, initiating platelet adhesion and

FIG. 3. Left: Right carotid angiogram, venous phase lateral view, demonstrating the markedly dilated
cortical veins, bilateral occlusion of the sigmoid sinuses, and venous drainage exiting entirely through the fight
superior and inferior ophthalmic veins. Right: Anteroposterior view showing the dilated dural venous sinus
draining superiorly. There is bilateral occlusion of the sigmoid sinuses. Asterisks mark the location of the
nonvisualized jugular bulbs.

FIG. 4. Left." Right carotid arteriogram, frontal view, after clipping of the meningo-orbital artery. The
arrow indicates the petrous branches arising from the internal carotid artery and communicating with the
dural sinus. Right: Right common carotid arteriogram, lateral view, after clipping of the internal carotid
artery feeders, demonstrating complete obliteration of the dural arteriovenous malformation.
J. Neurosurg. / Volume 65/September, 1986

419

O. A1-Mefly, J. R. Jinkins and J. L. Fox
aggregation as well as activation of the intrinsic clotting
system leading to thrombosis. 2~
The injection of contrast medium may further contribute to the process of thrombosis. Ritchie, et al., ~6
showed experimentally that leukocyte accumulation in
the venous wall may be exaggerated by contrast medium. Clagett and colleagues 3 stated that a direct effect
of the contrast medium on the vascular endothelium
and on red blood cells will contribute to thrombus
formation. This finding is supported by the occurrence
of thrombosis at angiography in dural AVM's, as reported by Kfihner, et al. (their Case 2), 9 and Storrs and
King (their Case 4), 23 and as reported in several cases
of carotid-cavernous fistula thrombosis at or soon after
angiography. ~7 Finally, the turbulence caused by the
shunt may have further contributed to the thrombotic
process. The occurrence of thrombosis of the sinus
would explain the abrupt neurological deterioration in
our own and other reported cases.
Although spontaneous closure of a dural AVM has
been reported, 9'~~.z3complete obliteration of the fistula
is recommended. 24 Simple ligation of the arterial feeder
is rarely successful. Recent techniques of arterial occlusion by embolization have merit, 6'26 and surgical intervention may be curative if the embolization fails to
completely obliterate the fistula. Serial stages of management were recommended by Storrs and King. z3 Embolization and/or ligation of the major feeding vessels,
followed by repeat angiography and then total surgical
excision, minimizes blood loss, decreases the shunt
pressure, may allow total excision, and permits gradual
correction of the disturbed hemodynamics of the cerebral circulation.
Acknowledgment
We thank Miss Virginia D. Lacson for her invaluable
assistance in the preparation of this manuscript.
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Manuscript received September 25, 1985.
Accepted in final form February 19, 1986.
Address reprint requests to: Ossama AI-Mefty, M.D., Department of Neurosurgery, The University of Mississippi
Medical Center, 2500 North State Street, Jackson, Mississippi
39216.
J. Neurosurg. / Volume 65/September, 1986