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Table of Contents    
Year : 2013  |  Volume : 61  |  Issue : 2  |  Page : 200-201

Rare presentation of pial arteriovenous malformations as proptosis: Case report and review of literature

1 Department of Neurosurgery, National Institute of Mental Health and Neurological Sciences (NIMHANS), Bangalore, India
2 Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health and Neurological Sciences (NIMHANS), Bangalore, India

Date of Submission04-Feb-2013
Date of Decision11-Feb-2013
Date of Acceptance09-Mar-2013
Date of Web Publication29-Apr-2013

Correspondence Address:
Paritosh Pandey
Department of Neurosurgery, National Institute of Mental Health and Neurological Sciences (NIMHANS), Bangalore
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.111160

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How to cite this article:
Saligoudar P, Seshadri R, Pandey P. Rare presentation of pial arteriovenous malformations as proptosis: Case report and review of literature. Neurol India 2013;61:200-1

How to cite this URL:
Saligoudar P, Seshadri R, Pandey P. Rare presentation of pial arteriovenous malformations as proptosis: Case report and review of literature. Neurol India [serial online] 2013 [cited 2020 Dec 3];61:200-1. Available from:


Arteriovenous malformations (AVMs) present with hemorrhage, seizures, headache or neurological deficits. Ocular symptoms are very rare in AVMs, unlike dural arteriovenous fistulae (DAVFs) and carotico-cavernous fistula (CCFs). There are many reports of DAVFs with thrombosis of the venous sinus, and re-routing of intracranial venous drainage leading to dilatation of superior ophthalmic vein (SOV) causing ocular symptoms. [1],[2] However, this is very rare in pial AVM, and only 2 similar cases have been previously reported in the literature. [3],[4] We present a case of pial AVM causing sinus thrombosis, leading to rerouting of venous drainage causing dilatation of SOV with resultant proptosis.

A 35-year-old gentleman presented with gradually progressive protrusion and redness of left eye of 8 months duration. Examination of left eye revealed proptosis, dilated episcleral vessels and conjunctival chemosis and congestion [Figure 1]a. Visual acuity was 6/12 in left, 6/6 in the right eye, and fundus examination revealed congested disc margins. Rest of the neurological examination was normal. Magnetic resonance imaging brain showed left parieto-occipital AVM, with draining vein into the superior sagittal sinus (SSS) [Figure 1]b, with complete thrombosis of the SSS distal to the insertion of the draining vein, and thrombosis of transverse sinuses [Figure 1]c. There was retrograde venous drainage, and dilatation of the left SOV causing proptosis [Figure 1]d. Digital subtraction angiogram (DSA) showed left parietooccipital AVM with the nidus measuring 1.9 cm × 1.8 cm × 2.0 cm with early superficial draining vein, with complete occlusion of the SSS just distal to the insertion of the draining vein into SSS, and thrombosis of bilateral transverse sinus. There was no antegrade flow, and the venous drainage was retrograde, with re-routing of venous blood through basal veins, middle cerebral vein to the sphenoparietal sinus bypassing the cavernous sinus causing dilatation of left SOV [Figure 2].
Figure 1: (a) Left orbital proptosis, conjunctival hyperaemia, and chemosis. (b) FLAIR axial images showing the arteriovenous malformations nidus as tangled mass of flow voids in the left parieto-occipital lobe (arrow). (c) Loss of flow voids in both the transverse sinuses and posterior superior sagittal sinus (arrows) on a coronal T2WI, suggestive of thrombosis. (d) Dilated and tortuous left superior ophthalmic vein (arrow) with multiple dilated vessels in the subarachnoid spaces

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Figure 2: Pre-operative digital subtraction angiogram, Left ICA injection arterial phase lateral view (a) and Anteroposterior view (b) showing the arteriovenous malformations nidus with an early draining vein. Venous phase show non-filling of the posterior aspect of the superior sagittal sinus, bilateral transverse-sigmoid sinuses and straight sinus with retrograde flow of blood into dilated superior ophthalmic vein (c and d)

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Patient underwent partial embolization of the AVM with Onyx, followed by surgical excision of AVM. Post-operative DSA showed no residual nidus and complete obliteration of early draining vein noted. There was complete resolution of the proptosis and chemosis [Figure 3].
Figure 3: Post-operative Digital subtraction angiogram. Left ICA injection lateral view, showing complete obliteration of Arteriovenous malformations nidus (a and b), with persistence of retrograde flow with dilated SOV, which is reduced in size compared to the pre-treatment stage (c). Post-operative image of patient showing complete resolution of proptosis, conjuctival congestion and chemosis (d)

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Orbital drainage from the cerebral AVMs is rare. [4] Even in patients with very high-flow AVMs, hemispheric AVMs, and arteriovenous fistulas, the venous drainage is antegrade. Neuro-ophthalmological manifestations of AVMs has rarely been described, and include, anterior visual pathway compression, visual field defects, oculomotor nerve palsies and raised intracranial pressure. [3],[4]

Only 4 cases of pial AVMs presenting with orbital drainage and neuro-ophthalmic symptoms, with 2 of them having proptosis. [3],[4] Gregory et al. [3] describe a case of an occipital AVM presenting with the unilateral signs mimicking a CCF. Volpe et al. [4] described 3 cases of pial AVMs with orbital drainage, but only one of those patients had proptosis secondary to the AVM.

Re-routing of venous drainage through the cavernous sinuses or sphenoparietal sinus causing orbital symptoms secondary to venous sinus thrombosis is typically seen in patients with intracranial DAVFs. Controversy persists whether the sinus thrombosis is the primary or secondary event in formation of DAVFs. Most of the authors back the role of primary sinus thrombosis in the development of DAVF. However, authors have shown that high flow secondary to fistulae may result in endothelial hyperplasia, inflammatory reaction and sinus thrombosis due to turbulent flow, and the sinus thrombosis can be secondary to DAVF. This case clearly shows that high flow in the sinuses may lead to thrombosis of the sinus, leading to re-routing of venous drainage. This provides some support to the theory of secondary sinus thrombosis in patients with DAVF as well.

  Conclusion Top

Pial AVMs can rarely cause secondary sinus thrombosis due to high turbulent flow causing endothelial damage leading to venous hypertension resulting in significant rerouting of venous drainage. Embolization followed by surgery leads to complete recovery in these patients.

  References Top

1.Buchanan TA, Harper DG, Hoyt WF. Bilateral proptosis, dilatation of conjunctival veins, and papilloedema: A neuro-ophthalmological syndrome caused by arteriovenous malformation of the torcular herophili. Br J Ophthalmol 1982;66:186-9.  Back to cited text no. 1
2.Liu HM, Shih HC, Huang YC, Wang YH. Posterior cranial fossa arteriovenous fistula with presenting as caroticocavernous fistula. Neuroradiology 2001;43:405-8.  Back to cited text no. 2
3.Gregory ME, Berry-Brincat A, Ghosh YK, Syed RN, Diaz PL, Jordan TL. An arteriovenous malformation masquerading as a carotid-cavernous sinus fistula. Am J Ophthalmol 2005;140:548-50.  Back to cited text no. 3
4.Volpe NJ, Sharma MC, Galetta SL, Langer DJ, Liu GT, Hurst RW, et al. Orbital drainage from cerebral arteriovenous malformations. Neurosurgery 2000;46:820-4.  Back to cited text no. 4


  [Figure 1], [Figure 2], [Figure 3]


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