Arteriovenous malformations (AVMs) of the orbit are progressively enla
rging communications between arteries and veins that bypass normal cap
illary beds. In contrast to arteriovenous fistulas (AVFs), AVMs are co
ngenital lesions with multiple large feeding arteries, a central nidus
, and numerous dilated draining veins. Management of AVMs of the orbit
may be difficult due to the threat of hemorrhage, vascular occlusion
during treatment, and collateral damage to surrounding organs. We mana
ged AVM of the orbit and periorbital tissues in four patients. Neuroim
aging studies, clinical decision making, operative experience, and lon
gterm postoperative results were retrospectively reviewed. Four cases
of AVM of the orbit and periorbital tissues were successfully treated
with preoperative embolization and subsequent excision of the central
nidus of the AVM. There was no evidence of recurrence in any of the ca
ses over follow up ranging from 2 to 5 years. We conclude that identif
ication of all arterial feeders, from both internal and external carot
id systems, is critical in developing a therapeutic plan. AVMs may be
treated by surgical excision alone, or embolization alone. However, in
the hands of an experienced interventional neuroradiologist and an ap
propriately chosen surgical team, most AVMs can and should be treated
by a combined approach of preoperative embolization followed by surgic
al excision of the vascular mass. The goal of therapy is closure of th
e low-resistance shunt.