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Anomalous venous drainage of a plexiform (pial) arteriovenous malformation mimicking an indirect caroticocavernous sinus fistula
  1. D Squirrell1,
  2. P Puri1,
  3. P A Rundle1,
  4. C Romanowski2,
  5. I G Rennie3
  1. 1Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield S10 2JF, UK
  2. 2Department of Radiology
  3. 3University Department of Ophthalmology and Orthoptics, Floor O, Royal Hallamshire Hospital, Sheffield S10 2RX, UK
  1. Correspondence to: Mr P Puri

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We report a case of a 35 year old man who presented with proptosis of the left globe and congested episcleral and retinal veins. We present angiographic evidence to show that the venous engorgement of the left orbit was related to anomalous venous drainage of a previously posteriorly draining parietal arteriovenous malformation (AVM).

Case report

A 35 year old man presented complaining of a 3 month history of a red eye and a 1 month history of progressive swelling and protrusion of the left eye. His only significant past medical history was that of a subarachnoid haemorrhage from an intracranial AVM 7 years previously for which he had undergone stereotactic radiosurgery. Ocular examination revealed a visual acuity of 6/5 in either eye. There was a 5 mm axial displacement of the left globe with normal ocular motility. The proptosis was non-pulsatile, non-reducible, and Valsalva manoeuvre was negative. There was no audible bruit. Anterior segment examination was unremarkable aside from congested episcleral vessels. Examination of the fundus revealed dilated and tortuous retinal veins but a normal arterial system. There was no disc swelling.

A computed tomography (CT) scan of the brain and orbits revealed an AVM in the left primary sensory cortex, proptosis of the left globe and a dilated superior ophthalmic vein (Fig 1). A magnetic resonance image (MRI) with angiography confirmed the presence of an AVM in the left perirolandic region which was fed by the left middle cerebral artery and the pericallosal branch of the left anterior cerebral artery (Fig 2). The dominant venous drainage was via a large superficial vein upwards to the superior sagittal sinus. Although the AVM did not appear to have changed significantly in overall size when compared with previous angiograms (Fig 3), it was clear that there had been significant changes in the venous structures distant to the AVM. A relative constriction at the junction of the ipsilateral transverse and sigmoid sinuses had developed (Fig 4) and as a consequence there was a relative hold up to the filling of the sigmoid sinus. The AVM therefore now drained anteriorly via a collateral circulation into the cavernous sinus. The later phases of the angiogram revealed that there was relatively little downward venous outflow from the cavernous sinus and the majority of this venous outflow was therefore shunted via the superior ophthalmic vein which was markedly dilated as a consequence. The patient's symptoms of unilateral proptosis and venous engorgement were therefore a manifestation of increased blood flow through collateral shunts that had developed as a consequence of a stricture in the principal drainage channel of the existing AVM, which itself had not changed significantly in size.

Figure 1

CT scan of the orbits showing dilated superior ophthalmic vein. The majority of the venous outflow from the cavernous sinus is via the superior ophthalmic vein which is markedly dilated as seen in this scan.

Figure 2

Anterior segment photographs showing the dilated episcleral vessels in the left eye.

Figure 3

Cerebral angiogram dated 1994. A moderately sized arteriovenous malformation is shown fed by branches of the left middle cerebral and anterior cerebral arteries. It is drained by a large superficial vein which continues into the superior sagittal sinus.

Figure 4

Cerebral angiogram dated 2000. There has been no significant change in the size of the arteriovenous malformation since the angiogram was performed in 1994. There is constriction at the junction of the transverse and sigmoid sinuses with hold up in the transverse sinus. Multiple cortical collateral vessels are seen draining into the cavernous sinus.


Arteriovenous malformations (AVM) are lesions which consist of racemose networks of arterial and venous channels which communicate directly rather than through a normal capillary bed. These communications are of two types: fistulous and plexiform. In the fistulous type an arterial channel empties directly into a venous channel, while in the plexiform type one or more arterial channels feed a vascular conglomerate that comprises multiple arteriovenous communications from which one or more venous channels emerge as draining veins.1 The fistulous types are usually supplied by meningeal branches of the external carotid artery and therefore they are also known as dural AVMs. The plexiform type, in contrast, are supplied by branches of the cerebral or cerebellar arteries and hence are also known as pial AVMs. The AVM most commonly encountered by the ophthalmologist is the acquired caroticocavernous sinus fistula, a dural AVM whose characteristic neuro-ophthalmic presentation results from the arterialisation of the orbital venous system. Proptosis and orbital venous engorgement secondary to direct arterialisation of the venous system has also been reported in dural AVMs involving the torcular herophili2 and the Galenic system.3 The more common plexiform (pial) AVMs typically present with complications that arise from the massive venous run off that is generated by the associated arteriovenous shunts—namely, intracranial haemorrhage, seizures, and recurrent headache.4 Symptomatic orbital drainage of plexiform (pial) AVMs is rare and when it does occur the AVM is usually in an anterior location.5–8

We describe a patient with a posteriorly located plexiform (pial) AVM who presented with proptosis and vascular engorgement of the left globe as a result of the venous outflow of the AVM being shunted anteriorly through newly opened collateral channels. Unilateral proptosis associated with anterior shunting has previously been described in a child with bilateral sigmoid sinus hypoplasia,9 but has never been described in a patient with a posteriorly located plexiform AVM. Our case is also extremely unusual in that we have angiographic evidence which shows that these collateral channels developed as a result of an acquired stenosis at the junction of the transverse and sigmoid sinus, previously its dominant route of venous drainage. The aetiology of this focal stenosis is unknown. It is unlikely to be a consequence of the stereotactic radiosurgery as the site of the stenosis is remote to the previously treated area. It is more likely that the stenosis represents a response to the chronic endothelial damage that is known to accompany the hyperdynamic circulation of AVMs.1

This case serves to illustrate that not all adults who present with proptosis and venous engorgement of the globe have a caroticocavernous sinus fistula and other anomalies of venous drainage must occasionally be considered.