Intraorbital arteriovenous fistula with thrombosed varix: Diagnosis and treatment without catheter angiography in a developing country

Abstract

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.

Thrombosis of intraorbital arterio-venous fistula

“…Mishra et al 7 also reported a case of pure intra orbital AVF with thrombosed varicose SOV, which was similar to ours. Their patient, on the other hand, had a lengthy history of proptosis, chemosis, and a recent onset of retro-orbital pain and reduced vision, spanning 18 months.…”

“…AVF, unlike AVMs, begins with a single contact between an artery and a vein but no nidus. 1 , 6 , 7 Intra-orbital AVFs are most usually caused by a physical lesion to the ethmoid artery, such as a fracture of the ethmoid bone and a rupture in the ocular venous system. 6 , 7 Fistulas can develop rapidly in non-traumatic situations due to artery degeneration caused by atherosclerosis, hypertension, or other vascular disorders.…”

“… 6 , 7 Fistulas can develop rapidly in non-traumatic situations due to artery degeneration caused by atherosclerosis, hypertension, or other vascular disorders. 7 …”

Spontaneous Intra-orbital Arteriovenous Fistula: A Rare Cause of Proptosis

“…Most cases are secondary to traumatic events, such as a fracture of the ethmoid bone, or spontaneous. The vast majority of cases have been described in cases after trauma; however, a few cases have been documented spontaneously in patients with a prior history of hypertension, atherosclerosis, and other vascular diseases [1]. Purely intra-orbital AVF are very rare, in fact, only about 26 cases have been reported in the literature [2].…”

“…Intra-orbital AVFs may be easily miss-diagnosed due to its rarity and common presentation. Symptoms usually involve proptosis, diplopia, ocular hypertension, and engorged episcleral vessels [1]. Vision loss and abnormal ocular movement are usually not present.…”