An Arteriovenous Malformation Masquerading as a Carotid-Cavernous Sinus Fistula

doi:10.1016/j.ajo.2005.03.028

American Journal of Ophthalmology

Volume 140, Issue 3, September 2005, Pages 548-550

https://doi.org/10.1016/j.ajo.2005.03.028 Get rights and content

Purpose

To describe a case of an occipital arteriovenous malformation (AVM) presenting with unilateral signs mimicking a carotid-cavernous sinus fistula (CCF).

Design

Interventional case report.

Methods

A 67-year-old normotensive male presented with loss of vision, pain, and proptosis of the right eye. Best-corrected visual acuity was right eye 6/36 and left eye 6/5. Examination of the right eye revealed orbital congestion with arteriolization of the episcleral vessels and an intra-ocular pressure of 44 mm Hg which was refractory to medical treatment.

Results

Computed tomography (CT) angiogram and cerebral angiography found an occipital AVM draining into the right sphenoparietal sinus and thereafter the right ophthalmic vein, bypassing the cavernous sinus. Embolization followed by excision of the AVM resulted in recovery of vision, reversal of proptosis, and normalization of intraocular pressure.

Conclusions

Rapid diagnosis and immediate intervention resulted in a rewarding visual recovery despite a persisting left homonymous hemianopia.

Section snippets

Case report

A 67-year-old normotensive male presented with sudden loss of vision in the right eye associated with severe pain and proptosis. He had no history of recent trauma but gave a 2-year history of progressive redness and prominence of the right eye.

His best-corrected visual acuities were right eye 6/36 and left eye 6/5. Examination of the right eye revealed eyelid edema, associated with a non-pulsatile proptosis of 7 mm. There was an afferent pupil defect, reduced color vision discrimination, and

References (5)

A.R. Forman et al.
Ocular findings in patients with arteriovenous malformations of the head and neck
Am J Ophthalmol
(1975)
T.A.S. Buchanan et al.
Bilateral proptosis, dilatation of conjunctival veins, and papilloedemaa neuro-ophthalmological syndrome caused by arteriovenous malformation of the torcular Herophili
Br J Ophthalmol
(1982)

There are more references available in the full text version of this article.

Cited by (10)

Congenital isolated idiopathic episcleral arteriovenous malformation
2020, American Journal of Ophthalmology Case Reports
Citation Excerpt :
Mozaffarpor M. described a patient with dilated episcleral congestion that was reported to have intracranial AVM.3 Gregory et al. reported a casse where in an occipital AVM presented with unilateral signs mimicking a caroticocavernous fistula (CCF).4 Isolated dilated episcleral vessels need to differentiated from orbital varices which show unilateral intermittent non-pulsatile proptosis with manoeuvres that increase venous pressure.
To report the occurrence of an episcleral AVM and to discuss the gravity as well as management of dilated tortuous episcleral vasculature.
A 16-year-old Asian male presented with redness in the left eye since birth and a history of gradual painless progressive increase in size since the past two years, causing a cosmetic concern for the patient. There was no history of any diminution of vision, diplopia, headache, trauma or any similar lesions anywhere in the body.
On examination, unaided visual acuity (UCVA) was 6/6 Snellen in both eyes. Hirschberg's reflex was central and extraocular movements were full and free in both the eyes.
On slit-lamp examination, dilated tortuous episcleral vessels were found in the temporal aspect of the palpebral aperture, shaped like a single continuous vascular frond, vertically measuring 10–11mm in extent and horizontally commencing 6mm from the temporal limbus but the posterior limit could not be discernible. The episcleral plane was freely mobile from the overlying conjunctiva and underlying sclera. The dilated episcleral vasculature remained unchanged on Valsalava maneuver and on bending forward.
Intraocular pressure was 14 mmHg by Goldmann's appalanation tonometer in both eyes. Iris was normal in configuration and gonioscopy revealed open angles without any evidence of any neovascularisation in both eyes. Fundus examination was unremarkable.
USG B-scan was grossly normal. MR Angiography of head and orbit with using thin high-resolution cuts (1mm using 3T machine) was done and was later followed up with a Contrast Enhanced MRI. Both imaging studies revealed an isolated episcleral AVM with a possible feeder vessel from the anterior ciliary artery to the lateral rectus and without any intraorbital or intracranial lesion.
A diagnosis of isolated episcleral AVM was made. The episcleral plane was dissected and dilated tortuous vessels were excised. After cautery, conjunctival autograft was obtained from the fellow-eye was used to cover the defect. Histopathological examination revealed both thick and thin walled vessels of varying calibre corresponding to arteries and veins confirming the diagnosis of isolated episcleral AVM. Ref. Fig. 2. No recurrence has been observed over a follow-up period of 1 year.
AND IMPORTANCE: Isolated idiopathic episcleral AVM should be a diagnosis of exclusion. A thorough systemic and ophthalmological evaluation including a glaucoma workup is necessary for both diagnosis and management. UBM and MRI/MRA may be required in select cases to rule out any associations or complications. Management includes observation, embolization and excision.
Diagnosing and managing functional visual complications after brain injury
2008, Optometry
Citation Excerpt :
In most cases, when an AVM is bleeding or causing a neurologic deficit, treatment is performed immediately. Open craniotomy with excision of the AVM is performed in the case of orbital drainage from an intracranial AVM and can lead to improved visual symptoms.25 Endovascular embolization is an option for treatment in which the vessel is surgically obstructed.12
Brain injury caused by an arteriovenous malformation (AVM) hemorrhage is an uncommon occurrence in a teenager. An AVM is a congenital anomaly of unknown etiology, often described as a tangle of arteries and veins that may vary in length and width leading to a loss of capillary bed. The vessels can break down with time and cause hemorrhage or aneurysm. Hemorrhage occurs in a significant number of patients with AVM. Intracranial hemorrhage causes brain injury, which can lead to systemic and ocular complications, neurologic deficits, and death.
A 15-year-old girl presented to our clinic with a chief complaint of horizontal diplopia while reading, lasting from seconds to 5 minutes. Nine months before the examination she had a ruptured cerebral AVM, then surgery to control the bleeding. She spent 6 weeks in a coma after surgery and woke up a hemiplegic. There were no visual problems before the bleeding. Our evaluation showed accommodative insufficiency, oculomotor dysfunction, dry eye syndrome, and a right beating latent nystagmus in both eyes. Perceptual testing showed a severe visual sequential memory deficit. The patient was given a reading prescription to aid with accommodation and was to use artificial tears and lacrilube ointment. At the follow-up examination, she reported increased efficiency and fewer episodes of diplopia while reading with bifocals and fewer dry eye symptoms since using the artificial tears.
After a brain injury, a young adult can present with perceptual and visual efficiency problems as well as nystagmus. It is important to test all areas that may be affected by this type of event and to investigate all complaints thoroughly with continued follow-up care. A latent nystagmus was discovered during thorough near point testing. The visual perceptual testing helped to confirm the visual sequential memory deficit, which may otherwise have been overlooked. It is believed that the diplopic complaint and poor oculomotor skills were directly related to the newly acquired nystagmus.
Frontal Arteriovenous Malformation Presenting as Painful Unilateral Conjunctiva Injection
2022, Clinical Practice and Cases in Emergency Medicine
Ocular signs caused by dural arteriovenous fistula without involvement of the cavernous sinus: A case series with review of the literature
2016, American Journal of Neuroradiology
Rare presentation of pial arteriovenous malformations as proptosis: Case report and review of literature
2013, Neurology India
Arteriovenous malformations and carotid-cavernous fistulae
2009, International Ophthalmology Clinics

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Brief reportAn Arteriovenous Malformation Masquerading as a Carotid-Cavernous Sinus Fistula

Purpose

Design

Methods

Results

Conclusions

Section snippets

Case report

Am J Ophthalmol

Bilateral proptosis, dilatation of conjunctival veins, and papilloedemaa neuro-ophthalmological syndrome caused by arteriovenous malformation of the torcular Herophili

Br J Ophthalmol

Brief report
An Arteriovenous Malformation Masquerading as a Carotid-Cavernous Sinus Fistula