- Department of Neurosurgery, S.C.B. Medical college and Hospital, Cuttack, Odisha, India
Department of Neurosurgery, S.C.B. Medical college and Hospital, Cuttack, Odisha, India
DOI:10.4103/2152-7806.116926Copyright: © 2013 Mishra SS This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Mishra SS, Panigrahi S, Satpathy PC, Das D. Intraorbital arteriovenous fistula with thrombosed varix: Diagnosis and treatment without catheter angiography in a developing country. Surg Neurol Int 23-Aug-2013;4:107
How to cite this URL: Mishra SS, Panigrahi S, Satpathy PC, Das D. Intraorbital arteriovenous fistula with thrombosed varix: Diagnosis and treatment without catheter angiography in a developing country. Surg Neurol Int 23-Aug-2013;4:107. Available from: http://sni.wpengine.com/surgicalint_articles/intraorbital-arteriovenous-fistula-with-thrombosed-varix-diagnosis-and-treatment-without-catheter-angiography-in-a-developing-country/
Background:Pure intraorbital arteriovenous fistula (AVF) that do not connect to the cavernous sinus are quite rare. Presence of thrombosed varix in association with a spontaneous onset pure orbital AVF is even rarer.
Case Description:We report an interesting case of a 50-year-old female with pure intraorbital AVF of spontaneous onset with thrombosed varicose superior ophthalmic vein (SOV). She presented with 18 months history of right eye proptosis, upper lid swelling, and conjunctival chemosis with recent onset of retro-orbital pain and decreased vision. Computed tomography (CT) and angiography revealed thrombosed varicose SOV in association with pure intraorbital AVF. Direct surgical exposure of the thrombosed SOV through right fronto-orbital approach followed by excision of the fistulous lesion resulted in complete orbital decompression with disappearance of all symptoms by 2 months.
Conclusion:Spontaneous onset pure intraorbital AVF in association with thrombosed varicose SOV is extremely rare. In such cases, direct surgical exposure of the SOV followed by excision may accomplish complete closure of the fistula without significant risk for iatrogenic injury.
Keywords: Direct surgical exposure, pure intraorbital arteriovenous fistula, spontaneous onset, thrombosed varix
Intraorbital arteriovenous fistulas (AVFs) that lie purely within the orbit and do not connect to the cavernous sinus are quite rare.[
A 50-year-old woman, presented with 18-month history of painless progressive proptosis of right eye in association with upper lid swelling and conjunctival chemosis. Initially the proptosis was more marked during episodes of straining or prone/stooping positioning. Only in the last 1-month, she reported acute onset of retro-orbital pain, fixed proptosis, decreased vision, and mucopurulent discharge. There was no history of orbital trauma or family history of vascular disorders. Ophthalmologic examination of the right eye disclosed marked dilatation of conjunctival vessels, chemosis, and exophthalmos [
Computed tomography (CT) of the head and orbits demonstrated a nonenhancing, serpiginous soft tissue mass within right retrobulbar space, extending from the superior ophthalmic fissure to the anterolateral wall of the orbit [
Head and orbit computed tomography scan demonstrating an isodense, serpiginous soft tissue mass within right retrobulbar space, clearly separate from the optic nerve extending from the superior ophthalmic fissure to the anterolateral orbital wall (a). Contrast enhancement was evident within the neighboring artery (b)
As the lesion was symptomatic due to dilated thrombosed SOV posing significant mass effect, open surgical excision was planned in our center On opening the orbital roof through fronto-orbital craniotomy, a thickened serpiginous thrombosed SOV was found, which was resected [
AVFs in the orbit are quite rare, and most are part of facial arteriovenous malformations (AVMs).[
Having the same hemodynamic characteristics, AVFs of the orbit must be considered in the differential diagnosis of carotid-cavernous sinus fistulas (CCFs) and orbital AVMs. Increased orbital venous pressure and signs of orbital congestion, such as proptosis, dilation of conjunctival and retinal vessels, ocular hypertension, dilation of the SOV, and extraocular muscle enlargement can be found in all the three conditions.[
Orbital varices in association with AVF are easily identifiable on either CT or duplex ultrasound imaging. Being a static modality, CT is less reliable in demonstrating flow across fistulae. Fine cut contrast-enhanced CT scans usually show ill-defined, heterogeneous multiloculated enhancing soft tissue mass(es) with connections to the orbital and extraorbital circulation. When thrombosed, orbital varices may or may not show patchy enhancement. Alternatively, duplex ultrasound using a high frequency (5-7.5 MHz) probe on B-mode with color doppler is a repeatable primary imaging modality for the orbit and reliably demonstrates flow across AVF of varying velocities.[
Treatment of intraorbital AVFs remains controversial. These orbital fistulas are usually managed by a conservative treatment, rather than by active vascular intervention.[
Spontaneous onset pure intraorbital AVF in association with thrombosed varicose SOV, as reported here, seems extremely rare. In such cases, direct surgical exposure of the SOV followed by excision may accomplish complete closure of the fistula without significant risk for iatrogenic injury.
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