Unusual association of diseases/symptoms

CASE REPORT

A rare association of cerebral dural arteriovenous
fistula with venous aneurysm and contralateral
flow-related middle cerebral artery aneurysm
David O Onu,1 Andrew W Hunn,1 Robin A Harle2
1

Department of Neurosurgery,
Royal Hobart Hospital, Hobart,
Tasmania, Australia
2
Department of Radiology,
Royal Hobart Hospital, Hobart,
Tasmania, Australia
Correspondence to
Dr David O Onu,
dr_donu@yahoo.com

SUMMARY
The association of cerebral dural arteriovenous fistula
(DAVF) and ipsilateral flow related aneurysm has
infrequently been reported. We describe a male patient
who presented with an acute haemorrhagic stroke and
was found to have a large right fronto-parietal intraparenchymal haemorrhage from the ruptured Borden
type II DAVF in addition to a large venous aneurysm and
a flow related intraosseous aneurysm of the contralateral
middle meningeal artery (MMA) all clearly delineated by
CT and DSA. He underwent emergency stereotactic
evacuation of the intraparenchymal haemorrhage and
successful surgical treatment of all the vascular lesions at
the same time with residual neurological deficit. To our
knowledge, this is the first such reported case. We
discuss the challenging surgical treatment, emphasising
the role of CT/DSA in management, and provide a
literature review.

BACKGROUND
The association of cerebral dural arteriovenous
fistula (DAVF) with cerebral aneurysms is rare; the
first case was reported by Kaech et al1 in 1987 and
subsequently by a few other case reports and
series.2–9 A previous series described a 60-year-old
man who presented with subarachnoid/intraparenchymal haemorrhage and had right DAVF fed by
the right middle meningeal artery (MMA) and associated with aneurysms of the right anterior cerebral
and left internal carotid-posterior communicating
arteries considered to be remote.3 Hashiguchi
et al10 also demonstrated that the development of
venous aneurysm over time may be a part of the
natural history of DAVF and significantly contributed to the haemorrhagic complications.
In this report, we discuss the rare association of
ruptured right DAVF with a venous aneurysm and
contralateral flow related MMA aneurysm in a
patient presenting with a right haemorrhagic
stroke. He underwent emergent stereotactic evacuation of the intraparenchymal haemorrhage and
bilateral craniotomies for the obliteration of all the
vascular lesions at the same time with residual
neurological deficit. We discuss the challenging surgical treatment, emphasising the role of CT/DSA in
management, and provide a literature review.
To cite: Onu DO, Hunn AW,
Harle RA. BMJ Case Rep
Published online: [please
include Day Month Year]
doi:10.1136/bcr-2013200764

CASE PRESENTATION
The ambulance service was called to attend to our
patient who is a man in his 60s, who developed
sudden onset of right temporal headaches

Onu DO, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-200764

associated with an ‘electric shock’ sensation radiating down his left trunk quickly followed by leftsided hemiplegia. He vomited and also had left
facial droop and slurring of speech. He was not a
known hypertensive and there was no antecedent
trauma or use of anticoagulant.
On arrival to the emergency department, he was
found to be confused, with altered sensation and
florid paralysis of the left upper and lower limbs.
There was right and upward deviation of gaze. He
was immediately intubated and sedated for time critical transfer to the intensive care unit (ICU) of our
hospital, which is the Regional Neurosurgical Centre.

INVESTIGATIONS
On admission, a CT brain showed a large right
fronto-parietal intraparenchymal haemorrhage with
a midline shift and extravasation of contrast suggesting an underlying vascular anomaly. Cerebral DSA
showed Borden type 2 right frontal dural AV fistula
supplied by branches of the bilateral middle meningeal and superficial temporal arteries, and also from
the left anterior falx artery. A flow related saccular
aneurysm of the left MMA was also demonstrated.
Venous drainage was into cortical veins and a large
venous aneurysm was seen (figure 1).

TREATMENT
The vascular lesions described above were considered to be better suited for surgical treatment.
After gaining informed consent from the patient’s
partner, we then proceeded with a lengthy emergency stereotactic right frontoparietal craniotomy
for the evacuation of the large right frontoparietal
intraparenchymal clots, resection and clipping of
the right frontal DAVF and venous aneurysm utilising the BrainLab neuronavigational system. He also
had a left pterional craniotomy for clipping of the
left intraosseous MMA aneurysm and subsequent
insertion of a Codman microsensor intracranial
pressure monitor. The inner skull table was seen to
be unduly grooved by the rather tortuous middle
meningeal vessels. The dura was reinforced with
Duraform (Codman & Shurtleff Inc), Duraseal
(Confluent Surgical Inc), Tisseel glue (Baxter
International Inc), and bone flap was secured with
miniplates and screws.
He received blood transfusion for acute perioperative blood loss, anti-seizure, antibiotic and
DVT prophylaxis. His postoperative course in the
ICU was, however, complicated by ventilator associated Staphylococcus aureus pneumonia and vocal
1

Unusual association of diseases/symptoms
Figure 1 CT scan and cerebral digital
subtraction angiography (DSA) upon
admission. (A) CT scan showing right
fronto-parietal intraparenchymal
haemorrhage with abnormal vessels
and midline shift. (B) Arterial-phase
DSA of the left internal carotid artery
showing right frontal dural
arteriovenous fistula (AVF) (green and
blue arrows) with filling from the left
middle meningeal artery which also
has a flow-related saccular aneurysm
(red arrow). (C), Arterial-phase DSA of
the right external carotid artery
showing the dural AVF with supply
from the middle meningeal and
superficial temporal arteries. There is
retrograde filling of the cortical veins
(blue arrow), consistent with Borden
type II d-AVF. (D), Arterial-phase DSA
of the left external carotid artery
showing the dural AVF (green arrow)
being supplied from the middle
meningeal artery, superficial temporal
artery as well as the left middle
meningeal aneurysm (red arrow). Note
the retrograde filling of the cortical
veins (blue arrow), consistent with
Borden type II d-AVF.

cord oedema requiring prolonged ventilatory support. Steroid
therapy used to reduce vocal cord oedema, further compounded
his stress-induced hyperglycaemia. Additional complications
included transient acute renal insult and left lower limb deep
vein thrombosis, resulting in a 3-week stay in the ICU. He was
subsequently transferred to the high dependency unit and later
to the general ward where upon satisfactory clinical recovery, he
resumed very vigorous physical, cognitive and speech rehabilitation for his residual neurological deficits which included dense
left hemiplegia, left-sided neglect, visual and speech defects.

OUTCOME AND FOLLOW-UP
On discharge home, he continued to receive community-based
allied health support and remains well motivated. He is currently
continent, has a normal speech, intact memory and is able to
mobilise with assistance on a quad stick through left hip swing.
A follow-up CT showed desorption of the right frontoparietal haemorrhage with resultant encephalomalacia. Cerebral
DSA showed significant occlusion of the right DAVF and venous
aneurysm with no retrograde filling of the cortical veins, and
the left MMA aneurysm was completely occluded (figure 2).

DISCUSSION
The terms DAVF and dural arteriovenous malformation (DAVM)
are often used interchangeably. Intracranial DAVF are acquired
arteriovenous shunts confined to the dura, supplied mainly by
branches of the external carotid artery and usually drained
either anterogradely via the dural venous sinuses or retrogradely
into other dural or leptomeningeal venous channels.11 They are
frequently idiopathic but can be associated with venous
2

thrombosis, head trauma, tumours, previous neurological
surgery, meningitis or a sinus infection.11 12
In our case, the DAVF was supplied mainly by branches of the
bilateral external carotid arteries and drained into the superior
sagittal sinus via cortical veins/venous aneurysm with venous
reflux quite in keeping with the Borden type II category.
An incidence of 13–21% has been cited in cohort studies for
the association of pial or dural AVF with cerebral aneurysms.
One-third of the aneurysms associated with DAVF are flow
related while the rest are remote aneurysms of the circle of
Willis.2 3 A prior report has found a stronger proclivity for such
association in the anterior cranial fossa and cerebral convexity,
in men and in middle-aged patients.3 Many postulates have
been adduced for the association of DAVF and aneurysms
including genetic predisposition, dynamics of blood flow,
common inciting event like head injury, and chance phenomenon.2 The association of DAVF and contralateral aneurysm in
our case is certainly due to flow dynamics as the parent aneurysmal artery was a prominent blood supply of the DAVF.
The commonest presenting feature in these cases is haemorrhagic complication which can involve the parenchymal, subarachnoid or rarely the subdural compartment. Haemorrhage
results from rupture of the fragile parenchymal veins which had
become arterialised as a result of the cortical venous reflux and
hypertension. This is commoner in the presence of venous
aneurysm and drainage of DAVF into the deep venous sinuses as
in our case.10 13 Our patient presented acutely with intraparenchymal haemorrhage and with features of raised intracranial
pressure such as sudden headache, visual abnormality, disturbance of consciousness and gross hemiplegia.
Onu DO, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-200764

Unusual association of diseases/symptoms
Figure 2 CT scan and Cerebral
digital subtraction angiography (DSA)
after surgery. (A), Follow up CT scan
after 6 months showing desorption of
the haemorrhage with some
encephalomalacia at the site of the
right fronto-parietal haemorrhage. (B),
Arterial-phase DSA of the left internal
carotid artery showing significant
occlusion of the right dural
arteriovenous fistula (AVF) and
complete occlusion of the left
meningeal artery aneurysm. (C),
Arterial-phase DSA of the right
external carotid artery showing
significant occlusion of the right dural
AVF with no retrograde filling of the
cortical veins. (D), Arterial-phase DSA
of the left external carotid artery
showing a significant occlusion of the
right dural AVF and complete occlusion
of the left middle meningeal artery
aneurysm.

Six-vessel DSA as was performed in our case, has been considered the gold standard in patients with DAVF to search for both
flow related and remote aneurysms and to plan treatment
options.2
DAVF can be treated by endovascular embolisation or open
surgical obliteration. The open surgical technique becomes even
more imperative in cases like ours, in which urgent evacuation
of a large intraparenchymal haemorrhage is required. Complete
disruption of the venous cortical drainage should be conducted
for all affected sides of the dural venous sinus, if need be,
bilaterally.14

Learning points
▸ The association of cerebral dural arteriovenous fistula (DAVF)
with contralateral flow-related aneurysm increases the risk of
haemorrhagic complications with attendant higher morbidity
and/or mortality.
▸ Six-vessel digital subtraction angiography should be
performed in all cases of DAVF to search for the associated
aneurysms and for the treatment planning.
▸ Concurrent surgical treatment should be attempted where
feasible to prevent the risk of haemorrhagic complication at
a later date.
▸ The DAVF should be obliterated first before clipping of the
aneurysm so as to prevent the rupture of the DAVF if
retraction is required to gain access to the associated
aneurysm.
Onu DO, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-200764

Where a DAVF and flow-related aneurysm coexist, concurrent
surgical treatment has been strongly advocated by some authors
to prevent catastrophic consequences of rupturing of any of the
vascular anomalies at a later time.7 During surgery, the DAVF
should be secured first before clipping of the aneurysm so as to
prevent the rupture of the DAVF if retraction is required to gain
access to the associated aneurysm.7
Acknowledgements We are grateful to Mrs Favour David-Onu for her able
editorial assistance.
Contributors DOO admitted the patient into the hospital and assisted in the
surgical procedure. He is also the major contributor in analysing the patient’s data
and preparing the manuscript. AWH is the senior author who performed the
procedure and offered useful comments. RAH is the radiologist who performed and
reported the DSA, and provided the relevant images. All the authors read, corrected
and approved the final manuscript.
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.

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2
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Onu DO, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-200764