British Journal of Radiology (2008) 81, e44-e47
© 2008 British Institute of Radiology
doi: 10.1259/bjr/25797982
Dural arteriovenous fistula causing primary intraventricular haemorrhage
R PADMANABHAN, MD
1
R STACEY, MD
2
S WIMALARATNA, MD
3 and
W KUKER, MD
1
Departments of 1 Neuroradiology, 2 Neurosurgery and 3 Neurology, The Radcliffe Infirmary, Oxford, UK
Correspondence: Dr Wilhelm Kuker, Department of Neuroradiology, The Radcliffe Infirmary, Woodstock Road, Oxford OX2 6HE, UK. E-mail: Wilhelm.Kuker{at}ndm.ox.ac.uk
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Abstract
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Primary intraventricular haemorrhage is a rare presentation of a dural arteriovenous fistula. We describe the case of a 52-year-old woman with a past history of idiopathic intracranial hypertension who presented with sudden-onset severe headache
The CT scan on admission showed primary intraventricular haemorrhage with no associated haemorrhage in the brain parenchyma or the extra-axial compartment
The cerebral angiogram demonstrated a dural arteriovenous fistula involving the left sigmoid and transverse sinuses which was successfully embolised transvenously. Subependymal venous congestion and rupture secondary to retrograde venous drainage has been proposed as the cause for this presentation.
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Introduction
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Dural arteriovenous (AV) fistulas are presumed to be acquired vascular malformations that consist of an AV shunt in the wall of a dural sinus without an intervening nidus [1, 2]. Dural AV fistulas can have a wide range of clinical manifestations including stroke due to intracranial haemorrhage, benign pulsatile tinnitus, visual deficits, proptosis, transient ischaemic attacks, seizures, cranial nerve palsies [3].
The most common cause of a dural fistula in one of the main dural sinuses is a thrombotic occlusion with subsequent recanalization. The dural arteries gain access to the sinus lumen through the ensuing granulation tissue. The sinus thrombosis has often been clinically silent or not correctly diagnosed.
Although subarachnoid, subdural, parenchymal and intraventricular haemorrhages in varying combinations have been previously described as a complication of a dural fistula, primary intraventricular haemorrhage in isolation has hardly ever been noted [4–6].
We report a case of woman with a dural fistula presenting with primary intraventricular haemorrhage and suggest that, although rare, this possibility should be considered in all patients presenting with primary intraventricular haemorrhage and all such patients should have external carotid artery injections as a part of their angiographic work up.
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Case report
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A 52-year-old woman was admitted to our hospital with a sudden onset of severe headache which had come on at rest that evening.
Over the past year, she had been investigated for progressive visual impairment with optometry, neuroimaging (MRI) and cerebrospinal fluid studies, and a diagnosis of idiopathic intracranial hypertension (IIH) had been made for which a lumboperitoneal shunt had been inserted 5 months prior to this admission. The only other past medical history was of hysterectomy for menorrhagia and appendicetomy many years back.
An MRI scan 3 years before the recent admission had been normal (Figure 1
).

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Figure 1. MRI 3 years before admission. (a) The T2 weighted image shows normal flow void in the left sigmoid sinus (arrow). The brain parenchyma appears normal. (b) The proton density image is also consistent with normal flow in the left sigmoid sinus (arrow). Because the MRI was normal, no MR venogram or flow-sensitive sequences were performed.
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On admission, the woman was alert and there were no abnormal neurological signs to find on examination.
A cranial CT scan showed intraventricular haemorrhage in the left lateral ventricle and third ventricle (Figure 2
). No associated haemorrhage was noted in either the brain parenchyma or the extra-axial compartment. There were no parenchymal hypodensities to note.

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Figure 2. CT scan at the time of admission.(a) The CT at the level of the basal ganglia shows blood in the left lateral ventricle. Neither hydrocephalus nor cerebral oedema are encountered. (b) The CT slice at a lower level shows blood in the third ventricle, again without parenchymal reaction. The CT did not indicate the source or aetiology of the intraventricular bleed.
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The possibility of an underlying occult AV malformation was considered and catheter angiography was carried out (Figure 3
) which showed a large dural AV fistula located in the left transverse–sigmoid sinus with extensive cortical venous reflux. Feeders originated from the occipital and middle meningeal branches of the left external carotid artery and the tentorial branches of the left internal carotid artery.

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Figure 3. Cerebral angiogram in the digital subtraction angiography technique. Contrast injection into the left external carotid artery is demonstrated. The(a) lateral and (b) frontal views show an arteriovenous shunt with abnormal filling of the left transverse and sigmoid sinus. There is an extensive area of shunting in the sinus wall. Note the dilated occipital artery. There is reflux into numerous cortical veins. The affected part of the sinus was occluded using platinum coils without complication. This resulted in an occlusion of the fistula and restitution of a normal intracranial pressure.
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Transvenous embolisation of the left sigmoid and distal transverse sinus with detachable platinum and fibred coils was carried out until there was complete cessation of the associated cortical venous reflux. The patient remained well after the procedure and was scheduled to have an outpatient follow-up MRI scan after the procedure.
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Discussion
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Primary intraventricular haemorrhage in adults is usually associated with conditions such as hypertension, coagulation disorders, tumours, AV malformations, moyamoya disease, fibromuscular dysplasia, arteritis and choroid plexus cysts [7–9], and accounts for less than 3% of all intracranial haemorrhages in the adult population [10].
Dural AV fistulas account for approximately 10–15% of all intracranial vascular malformations and occur most commonly along the transverse and sigmoid sinuses [3, 11].
Intracranial haemorrhage has been reported in up to 35–42% of dural AV fistulas [7, 10, 12] and is thought to be due to the rupture of congested cortical veins (secondary to retrograde venous drainage) [3]. This haemorrhage may be intraparenchymal or within the subarachnoid/subdural space.
The classification of dural AV fistulas is based upon the pattern of their venous drainage and any associated venous outflow restriction because these are the factors determining the severity of their clinical presentation and the probability of intracranial haemorrhage [12–15].
Intraventricular haemorrhage in the setting of a dural venous fistula is usually due to the extension of an associated intraparenchymal haematoma and is very rarely seen in isolation.
Halbach et al [4] were the first to report dural AV fistulas presenting with primary intraventricular haemorrhage. In their series of 28 patients, 1 patient with a dural AV fistula in the transverse–sigmoid sinuses had two episodes of primary intraventricular haemorrhage. Subsequently, to our knowledge, only three other cases of primary intraventricular haemorrhage due to dural AV fistulas (two in the Japanese and one in the English literature) have been reported [5, 6, 16].
The venous outflow pattern in this case corresponded to grade 2 according to the Lalwani classification [15] and to II a + b according to Cognard's classification [12]; presumably, the venous outflow restriction caused retrograde diversion of the venous outflow to the cortical veins and then to deep medullary veins with resultant subependymal venous congestion [6].
It is possible that the rupture of the congested fragile subependymal venous network caused the intraventricular haemorrhage in our patient.
Prompt investigation of primary intraventricular haemorrhage using MRI or angiography is necessary as these patients may harbour underlying aneurysms or AV malformations, which have a high propensity of rehaemorrhage in the acute period. Our patient went on to have catheter angiography directly after CT without an intervening MRI scan as the suspicion of an underlying AV malformation or aneurysm was paramount because of the sudden onset of her headache and also because of reportedly normal cranial MR imaging in the previous year.
Once the diagnosis of a dural AV fistula with cortical venous reflux was made, we proceeded to treatment immediately as one-third of patients with dural AV fistulas and retrograde cortical venous drainage have been reported to rebleed within 2 weeks after the initial haemorrhage [17].
The lesion was successfully embolised transvenously with detachable platinum coils placed within the distal transverse and proximal sigmoid sinuses until the retrograde cortical venous filling was completely obliterated.
Received for publication July 31, 2006.
Revision received October 25, 2006.
Accepted for publication November 15, 2006.
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References
|
|---|
- Chaudhary MY, Sachdev VP, Cho SH, Weitzner I Jr, Puljic S, Huang YP. Dural arteriovenous malformations of major venous sinuses: an acquired lesion. AJNR Am J Neuroradiol 1982;3:13–19.[Abstract]
- Houser OW, Campbell JK, Campbell RJ, Sundt TM Jr. Arteriovenous malformation affecting the transverse dural venous sinus: an acquired lesion. Mayo Clin Proc 1979;54:651–61.[Medline]
- Lasjaunias P, Chiu M, Ter Brugge K, Tolia A, Hurth M, Bernstein M. Neurological manifestations of intracranial dural arteriovenous malformations. J Neurosurg 1986;64:724–39.[Medline]
- Halbach VV, Higashida RT, Hieshima GB, Goto K, Norman D, Newton TH. Dural fistulas involving the transverse and sigmoid sinuses: results of treatment in 28 patients. Radiology 1987;163:443–7.[Abstract/Free Full Text]
- Kawaguchi T, Kawano T, Kaneko Y, Tsutsuki M, Ooigawa H, Kazzekawa K. Dural arteriovenous fistula of the transverse–sigmoid sinus with intraventricular haemorrhage: a case report. No Shikei Geka 1999;27:1113–18.
- Irie F, Fujimoto S, Uda K, Toyoda K, Hagiwara N, Inoue T, et al. Primary intraventricular haemorrhage from dural arteriovenous fistula. J Neurol Sci 2003;215:115–18.[CrossRef][Medline]
- Angelopoulos M, Gupta SR, Azar Kia B. Primary intraventricular haemorrhage in adults: clinical features, risk factors, and outcome. Surg Neurol 1995;44:433–7.[CrossRef][Medline]
- Gates PC, Barnette HJM, Vinters HV, Simonsen RL, Siu K. Primary intraventricular haemorrhage in adults. Stroke 1986;17:872–7.[Abstract/Free Full Text]
- Marti-Fabregas J, Piles S, Guardia E, Marti-Vilalta JL. Spontaneous primary intraventricular haemorrhage: clinical data, etiology and outcome. J Neurol 1999;246:287–91.[CrossRef][Medline]
- Draby DG, Donnan GA, Saling MA, Walsh KW, Bladin PF. Primary intraventricular haemorrhage: clinical and neuropsychological findings in a prospective stroke series. Neurology 1988;38:68–75.[Abstract/Free Full Text]
- Newton TH, Cronqvist S. Involvement of dural arteries in intracranial arteriovenous malformations. Radiology 1969;93:1071–8.[Medline]
- Cognard C, Gobin YP, Pieroy L, Bailly AL, Houdart E, Casasco A, et al. Cerebral dural arteriovenous fistulas: clinical and angiographic correlation with a revised classification of venous drainage. Radiology 1995;194:671–80.[Abstract/Free Full Text]
- Djindian R, Merland JJ, Theron J. Superselective angiography of the external carotid artery. New York, NY: Springer Verlag, 1977: 606–28.
- Awad I, Little J, Akarawi W, Ahl J. Intracranial dural arteriovenous malformations: factors predisposing to an aggressive neurological course. J Neurosurg 1990;72:839–50.[Medline]
- Lalwani AK, Dowd CF, Halbach VV. Grading venous restrictive disease in patients with dural arteriovenous fistulas of the transverse/sigmoid sinus. J Neurosurg 1993;79:11–15.[Medline]
- Aihara N, Hashimoto N, Watanabe K, Fukushima T, Karasawa K, Takagi T. Dural AvF of the transverse/sigmoid sinus associated with sinus occlusion/stenosis with an unfavourable outcome. Jpn J Neurosurg 1997;6:833–7.
- Duffau H, Lopes M, Janosevic V, Sichez JP, Faillot T, Capelle L, et al. Early rebleeding from intracranial dural arteriovenous fistulas: report of 20 cases and review of literature. J Neurosurg 1999;90:78–84.[Medline]