Intraorbital arteriovenous fistula with thrombosed varix: Diagnosis and treatment without catheter angiography in a developing country
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 [] with gross reduction of visual acuity (Perception of light). Ocular movements were almost normal. Intraocular pressure was 22 mmHg in the right eye and 10 mmHg in the left eye.\nComputed 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 [] with no change in the size of the lesion on valsalva maneuvre. Contrast enhancement was evident within the neighboring artery. It was clearly separated from the optic nerve and extraocular muscles. CT angiogram with 3D reconstruction revealed intraconal serpiginous vascular lesion arising from right ICA and ending at the level of SOV, which is grossly dilated and tortuous (2 cm) and filled with nonenhancing thrombous []. The SOV drained anteriorly into several small tributaries with slow outflow. No nidus and no communication into the cavernous sinus were observed []. These findings were consistent with the diagnosis of thrombosed varicose SOV in association with pure intraorbital AVF, which was further confirmed by high frequency (5-7.5 MHz) B-scan ultrasound with color doppler.\nAs 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 [] completely as far back toward the orbital apex as possible after ligation of the proximal feeding artery. Postoperative course was uneventful with good clinical outcome. CT scan obtained at the time of discharge demonstrated complete decompression of the orbit []. At 6 month follow, she was doing well with no recurrence.