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.