Figure 1
Fig 1
Intracranial Dural Arterio-Venous Fistula fed from a Persistent Right Trigeminal Artery.
SectionNeuroradiology
Case TypeClinical Cases
Authors
Aruna S. Pallewatte,
Thushara Muthunayake
Connected authors
29 years, male
Clinical History
A previously healthy 29-year-old male presented with an insidious onset of headache, left-sided neck pain and blurring of left eye vision for 2 months. Clinical examination revealed bilateral papilloedema and dilated left pupil reacting to light. All cranial nerves were normal and no cerebellar signs were detected.
Imaging Findings
Contrast enhanced CT of the brain showed prominent right transverse, straight sinuses and dilated vein of Galen (Fig. 1). There were few prominent abnormal tortuous vessels subjacent to dilated venous sinuses within the right occipital white matter region. No intra cranial hemorrhage was noted.
Cerebral DSA (Figs. 2-4) showed early filling of dilated right sided transverse and sigmoid sinuses, prominent tortuous superficial cortical veins with retrograde flow pattern. A prominent persistent right-sided trigeminal artery was identified and it communicates with the right transverse sinus via multiple vascular channels and also with right anterior inferior cerebellar and posterior inferior cerebellar arteries. Selective catheterisation of the right occipital and posterior auricular artery (Fig. 5) demonstrated a connection between the meningial arterial branches and the right transverse sinus. No filling defect in the deep venous sinuses was noted.
Cerebral DSA (Figs. 2-4) showed early filling of dilated right sided transverse and sigmoid sinuses, prominent tortuous superficial cortical veins with retrograde flow pattern. A prominent persistent right-sided trigeminal artery was identified and it communicates with the right transverse sinus via multiple vascular channels and also with right anterior inferior cerebellar and posterior inferior cerebellar arteries. Selective catheterisation of the right occipital and posterior auricular artery (Fig. 5) demonstrated a connection between the meningial arterial branches and the right transverse sinus. No filling defect in the deep venous sinuses was noted.
Discussion
Intracranial AVF (arterio-venous fistula) are uncommon lesions. Selected cases suggest that they occur only one tenth as frequently as parenchymal AVMs [2].
Posterior fossa only represents 5-7% of the intracranial vascular malformations and associated anomalies involving the ipsilateral persisting trigeminal artery is an extremely rare combination [1].
Persistent trigeminal artery may be associated with vascular anomalies such as cerebral aneurysms, AVMs, AVF, absence of carotid artery [3, 4, 5], and PTA (persistent trigeminal artery) it self may be presented with, cranial nerve palsies, optic douloureux or SAH of unknown etiology. At least 23 cases of intracranial AVMs associated with PTA have been reported in the literature [4, 5].
The dural AVF represents an abnormal connection between dural arteries or parenchymal branches of cerebral arteries [2]. Although dural AVF can occur anywhere in the dura mater covering the brain, they most frequently occur in cavernous and transverse sigmoid sinuses [1]. Most of the lesions are acquired and venous sinus thrombosis, previous surgeries, ear infections and head trauma are potential predisposing factors [7].
Patients present with a spectrum of clinical features, asymptomatic or benign tinnitus to fatal ICH. Several studies have shown that these clinical features are directly related to the venous drainage pattern of AVF. The blurring of vision in our patient is probably due to the pressure effects over the occipital lobe from the dilated transverse sinus, and the left-sided neck pain can be explained by the engorged left-sided sigmoid sinus and internal jugular vein [1].
Although the various classifications of dural AVF have been introduced, extensive venous reflux and the presence or absence of cortical venous drainage is considered to be the most important determinant for the management planning. Cortical veins having retrograde flow patterns are associated with detrimental clinical features such as ICH, seizures, increased intracranial pressure and neuropsychiatric symptoms. Dural AVF involving single venous sinus is extremely aggressive due to cortical venous reflux and it requires urgent definitive management [7].
Possible management strategies are conservative, radiation therapy, endovascular intervention and surgery.
Currently endovascular treatment plays the primary role in dural AVF management including coil embolisation, stent placement and use of the liquid embolic agent like onix [7].
Posterior fossa only represents 5-7% of the intracranial vascular malformations and associated anomalies involving the ipsilateral persisting trigeminal artery is an extremely rare combination [1].
Persistent trigeminal artery may be associated with vascular anomalies such as cerebral aneurysms, AVMs, AVF, absence of carotid artery [3, 4, 5], and PTA (persistent trigeminal artery) it self may be presented with, cranial nerve palsies, optic douloureux or SAH of unknown etiology. At least 23 cases of intracranial AVMs associated with PTA have been reported in the literature [4, 5].
The dural AVF represents an abnormal connection between dural arteries or parenchymal branches of cerebral arteries [2]. Although dural AVF can occur anywhere in the dura mater covering the brain, they most frequently occur in cavernous and transverse sigmoid sinuses [1]. Most of the lesions are acquired and venous sinus thrombosis, previous surgeries, ear infections and head trauma are potential predisposing factors [7].
Patients present with a spectrum of clinical features, asymptomatic or benign tinnitus to fatal ICH. Several studies have shown that these clinical features are directly related to the venous drainage pattern of AVF. The blurring of vision in our patient is probably due to the pressure effects over the occipital lobe from the dilated transverse sinus, and the left-sided neck pain can be explained by the engorged left-sided sigmoid sinus and internal jugular vein [1].
Although the various classifications of dural AVF have been introduced, extensive venous reflux and the presence or absence of cortical venous drainage is considered to be the most important determinant for the management planning. Cortical veins having retrograde flow patterns are associated with detrimental clinical features such as ICH, seizures, increased intracranial pressure and neuropsychiatric symptoms. Dural AVF involving single venous sinus is extremely aggressive due to cortical venous reflux and it requires urgent definitive management [7].
Possible management strategies are conservative, radiation therapy, endovascular intervention and surgery.
Currently endovascular treatment plays the primary role in dural AVF management including coil embolisation, stent placement and use of the liquid embolic agent like onix [7].
Differential Diagnosis List
Dural arterio-venous fistula associated with right-sided persistent trigeminal artery.
Dural arterio-venous fistula
Dural arterio-venous malformation
Final Diagnosis
Dural arterio-venous fistula associated with right-sided persistent trigeminal artery.
References
[1] Newton TH, Cronqvist S. (1969) Involvement of dural arteries in intracranial arteriovenous malformations. Radiology 93:1071-8
[2] Will Adams, Peter Whitfield. (2007) Intracranial Dural Arteriovenous Fistulae. ACNR 7: 10-12
[3] Cognard C, Gobin YP, Pierot L, et al. (1995) Cerebral dural arteriovenous fistulas: clinical and angiographic correlation with a revised classification of venous drainage. Radiology 194:671-680
[4] Agnoli AL. (1982) Vascular anomalies and SAH associated with persisting embryonic vessels. Acta Neurachair (Wien) 60; 183-199
[5] Abe T, Matsumotok. (1994) Primitive trigeminal artery associated with intracranial vascular malformations, Case report. Neurol Med Chir 34:104-107
[6] Tomsick TA, ChamberS AA. (1979) Perisstent Trigeminal artery, Unusual associated abnormalities. Neuroradiology 17:253-257
[7] Guptha AK, Periakaruppan AL. (2009) Intrcranial dural AVF: A review. Indian J Radiol Imaging 19:43-48
Case information
| URL: | https://www.eurorad.org/case/9604 |
| DOI: | 10.1594/EURORAD/CASE.9604 |
| ISSN: | 1563-4086 |
Figure 2
Fig 2
Cerebral digitally subtracted angiography (DSA) demonstrating right sided prominent persisting trigeminal artery and its communication with the right transverse sinus via multiple vascular channels.
Figure 3
Fig 3
Cerebral DSA showing early filling of dilated right sided transverse and sigmoid sinuses , prominent and tortuous superficial cortical veins with retrograde flow pattern.
Figure 4
fig 4
Cerebral DSA reveals right sided prominent right anterior inferior cerebellar artery and posterior inferior cerebellar artery communicating with transverse sinus via abnormal meningial branches.
Figure 5
Fig 5
Selective catheterisation of right occipital and posterior auricular artery demonstrates connection between the meningial arterial branches and the right transverse sinus.
Fig 2
Cerebral digitally subtracted angiography (DSA) demonstrating right sided prominent persisting trigeminal artery and its communication with the right transverse sinus via multiple vascular channels.
Aruna
Aruna
Fig 3
Cerebral DSA showing early filling of dilated right sided transverse and sigmoid sinuses , prominent and tortuous superficial cortical veins with retrograde flow pattern.
Aruna
Aruna
fig 4
Cerebral DSA reveals right sided prominent right anterior inferior cerebellar artery and posterior inferior cerebellar artery communicating with transverse sinus via abnormal meningial branches.
Aruna
Aruna
Fig 5
Selective catheterisation of right occipital and posterior auricular artery demonstrates connection between the meningial arterial branches and the right transverse sinus.
Aruna
Aruna