Introduction/Purpose Intraorbital Arteriovenous Fistulas (IOAVFs) are a rare entity often misdiagnosed initially as carotid cavernous fistulas (CCFs). Although patients present in similar fashion and they appear angiographically similar, the diagnosis is differentiated by the location of the fistulous point within the orbit rather than posteriorly in the cavernous sinus. Like other dAVFs, treatments include observation, transarterial embolization (TAE), transvenous embolization (TVE), surgical disconnection, or combination of multiple approaches. There have only been a few previous reports utilizing direct puncture (DP) to treat these lesions. We present a series of 3 unique cases of DP with TVE and a review of the literature from 1978.

Methods The presenting symptoms, treatment and outcome are all described. A literature review is performed back to 1978.

Results All 3 patients presented with classic symptoms (proptosis, chemosis, and extra-ocular movement restriction). All 3 patients were ultimately cured using a direct puncture (DP) and venous embolization. Patient 1 underwent two procedures: an incomplete transfemoral/TAE through the ECA followed by a successful DP/TVE with coils/Onyx utilizing an enlarged vein in the eyelid. The patient suffered an intra-orbital hematoma during the DP requiring a lateral canthotomy (LC). Patient 2 also underwent two procedures: an unsuccessful transfemoral TVE followed by a DP/TVE with Onyx utilizing a sub-ciliary incision and puncture along the inferior orbital rim. Patient 2 also suffered an orbital hematoma requiring an endoscopic/endonasal evacuation from the medial orbit. Patient 3 underwent one procedure: a failed DP attempt and then a successful DP after three-dimensional angiographic guidance in the same setting. The patient was treated curatively after the second successful direct puncture. All patients were angiographically cured and have had resolution of orbital symptoms with normal vision.

Our survey of the literature revealed 49 total cases of IOAVFs of which 17 were managed conservatively, 8 underwent transarterial embolization, 18 underwent transvenous embolization, and 5 underwent percutaneous punctures. Conservative management and transarterial treatments resulted in the worst outcomes with 23.5% and 37.5% of patients with worsened visual function respectively.

Conclusions IOAVFs present similarly to CCFs but have slightly different angiographic anatomy. Like other dAVFs, IOAVFs frequently will require multiple treatments, but DP appears to be a reasonable option for obtaining a cure. Orbital hematoma is not infrequent, and awareness/preparation for a secondary procedure (e.g., LC) is important to preserve vision.

Disclosures S. Pathuri: None. W. Johnson: None. K. Russell: None. A. Robledo: None. F. Albuquerque: None. P. Kan: None. J. Mascitelli: None.