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 Table of Contents  
Year : 2021  |  Volume : 9  |  Issue : 1  |  Page : 43-45

Cranio-vertebral junction arteriovenous fistula presenting with subarachnoid haemorrhage: A case report

Department of Neurosurgery, GB Pant Institute of Postgraduate Medical Education and Research, New Delhi, India

Date of Submission08-Feb-2021
Date of Decision14-Jun-2021
Date of Acceptance18-Jul-2021
Date of Web Publication27-Aug-2021

Correspondence Address:
Dr. Anita Jagetia
Department of Neurosurgery, GB Pant Institute of Postgraduate Medical Education and Research, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcvs.jcvs_6_21

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Perimedullary arteriovenous fistulas are uncommon vascular malformations particularly if they involve the craniovertebral junction. The complexity of the angioarchitecture of these lesions poses a further diagnostic challenge. Moreover, the therapeutic management is controversial and can include observation alone, endovascular occlusion, or surgical exclusion, depending on both patient and the angiographic characteristics of the lesion.

Keywords: Angiography, arteriovenous fistula, craniovertebral junction, perimedullary, subarachnoid haemorrhage

How to cite this article:
Katyal A, Anil Kumar B C, Bodeliwala S, Jagetia A, Srivastava AK. Cranio-vertebral junction arteriovenous fistula presenting with subarachnoid haemorrhage: A case report. J Cerebrovasc Sci 2021;9:43-5

How to cite this URL:
Katyal A, Anil Kumar B C, Bodeliwala S, Jagetia A, Srivastava AK. Cranio-vertebral junction arteriovenous fistula presenting with subarachnoid haemorrhage: A case report. J Cerebrovasc Sci [serial online] 2021 [cited 2021 Dec 1];9:43-5. Available from: http://www.jcvs.com/text.asp?2021/9/1/43/324819

  Introduction Top

Craniovertebral junction (CVJ) arteriovenous fistulas (AVFs) are rare disorders which present with different clinical symptoms and pose difficulty in the management due to its specific location and treatment. There is still controversy regarding the choice between surgery and endovascular management. Few patients present with symptoms of headache and vomiting suggesting subarachnoid haemorrhage (SAH), may also present with weakness of limbs and neck pain due to myelopathy or symptoms of ischaemia. We present a rare case of ventral perimedullary AVF (PMAVF) of the CVJ, giving an insight into its diagnosis and management.

  Case Report Top

A 55-year-old man patient presented with a history of sudden onset of severe headache which was associated with multiple episodes of vomiting. He had no neurological deficit. Non-contrast computed tomography (CT) of the head showed SAH predominantly in basal cisterns [Figure 1].
Figure 1: Non-contrast CT scan of the head showing SAH predominantly in basal cisterns.

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CT angiography showed dilated nidus of vessels in the posterior circulation likely AVF arising from right vertebral artery. There were two blister aneurysms at M1 segment of the right middle cerebral artery (MCA) and MCA bifurcation on the left side [Figure 2].
Figure 2: CT Angiography showing two blister aneurysms at the M1 segment of the Right MCA and at the Left MCA bifurcation, and AVF arising from the Right Vertebral Artery

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The patient underwent diagnostic digital subtraction angiography (DSA) which showed an AVF at the CVJ proximal to the origin of the right PICA [Figure 3] with dilated and prominent draining veins [Figure 4]. We decided to obliterate the communication. Right suboccipital craniotomy, removal of C1 posterior arch (far lateral suboccipital approach), was performed. Intraoperatively, a fistulous arteriovenous malformation was identified intradurally at C1 level with feeders from the right vertebral artery and venous drainage into medullary veins with partially thrombosed nidus. The PMAVFs was excised along with the thrombosed nidus. Post-operative CT angiography showed a complete excision of the lesion with no nidus and AVF could be identified [Figure 5]. Post-operative period was uneventful. The patient was subsequently discharged and is under follow-up for the definitive treatment of MCA aneurysms.
Figure 3: DSA showing an AVF at the CVJ proximal to the origin of the Right PICA

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Figure 4: AVF with prominent draining veins

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Figure 5: Post-operative CT Angiography showing complete excision of the lesion

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  Discussion Top

PMAVFs of the CVJ are rare entities representing <1% of all cranial or spinal AVFs[1] which pose a great diagnostic and management challenge in view of complex vascular anatomy. PMAVFs present direct shunts between the vessels at the level of the pia mater, superficially to the spinal cord, and they can be located anteriorly or anterolaterally to the spinal cord. These can present as subarachnoid haemorrhage, as in the present case, myelopathy presenting with limb weakness due to venous hypertension or arterial steal phenomenon due to high-flow shunt presenting with signs of cerebral ischaemia. Lawton et al. suggested that angiogenesis could be induced by venous hypertension, responsible for the development of AVFs in experimental models.[2] In literature, haemorrhagic presentation is reported as the most common onset (SAH in 63% and intramedullary haemorrhage in 10%), frequently caused either by venous dilation, due to an ascending drainage pathway into an intracranial vein, or by the higher venous flow rates that can be associated with intracranial drainage.[1] On the other hand, spinal dural AVFs are formed by abnormal and low-resistance fistulous connections between branches of the external carotid artery, ICA and/or VA to a venous sinus and/or cortical veins. Hiramatsu et al.[3] and Sato et al.[4] described that arterial feeders from the anterior spinal artery and aneurysmal dilations are associated with haemorrhagic presentation. PMAVFs are characterised by small, tortuous feeding arteries and arteries originating from the VA which may present an acute angle, as observed in this case, can be technically challenging for endovascular treatment. Similar findings were observed by Guiseppe et al. in their study. Mendes et al.[5] also reported an AVF at the level of foramen magnum which was managed via transvenous route. In the present case, in view of improper visualisation of draining venous channels in CT angiography, the patient was subjected to DSA and venous drainage into perimedullary veins was identified. In cases of CVJ AVFs, adequate shoots of external carotid vessels with venous phase are essential to delineate the exact feeding arteries and draining venous channels. Due to high angulation of the feeding vessel in this case, adequate cannulation of feeder was not possible; hence, the patient was subjected to open microsurgery. Moreover the insufficiency to delineate all the feeders, technical difficulties encountered in tortuous vasculature and risks of recanalization do not make endovascular treatment superior to microsurgery.

  Conclusion Top

These AVFs are commonly associated with haemorrhage and are frequently fed by both radiculomeningeal and spinal pial arteries that drain into intradural or epidural veins. AVFs of the CVJ are rare vascular malformations which can present with controversies both for radiological diagnosis and treatment indications. Therefore, a combined endovascular and neurosurgical approach is suggested to treat this entity, for obtaining the best post-operative outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Umana GE, Scalia G, Chaurasia B, Fricia M, Passanisi M, Graziano F, et al. Perimedullary arteriovenous fistulas of the craniovertebral junction: A systematic review. J Craniovertebr Junction Spine 2020;11:157-62.  Back to cited text no. 1
Lawton MT, Jacobowitz R, Spetzler RF. Redefined role of angiogenesis in the pathogenesis of dural arteriovenous malformations. J Neurosurg 1997;87:267-74.  Back to cited text no. 2
Hiramatsu M, Sugiu K, Ishiguro T, Kiyosue H, Sato K, Takai K, et al. Angioarchitecture of arteriovenous fistulas at the craniocervical junction: A multicenter cohort study of 54 patients. J Neurosurg 2018;128:1839-49.  Back to cited text no. 3
Sato K, Endo T, Niizuma K, Fujimura M, Inoue T, Shimizu H, et al. Concurrent dural and perimedullary arteriovenous fistulas at the craniocervical junction: Case series with special reference to angioarchitecture. J Neurosurg 2013;118:451-9.  Back to cited text no. 4
Mendes GA, Caire F, Saleme S, Ponomarjova S, Mounayer C. Retrograde leptomeningeal venous approach for dural arteriovenous fistulas at foramen magnum. Interv Neuroradiol 2015;21:244-8.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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