Embolization of a Suspected Cavernous Venous Malformation of the Orbit
Authors: Georges Nassrallah, Patrick Nicholson, Dan DeAngelis.
Author Disclosure Block: G. Nassrallah: None. P. Nicholson: None. D. DeAngelis: None.
Purpose: We report the presentation and management of an orbital tumor, masquerading as an orbital cavernous venous malformation (CVM). We also review the relevant literature and discuss optimal management using a multidisciplinary team in this clinical context.
Study Design: Case report and review of the literature.
Methods: The patient’s medical chart was reviewed, and a comprehensive literature review was conducted.
Results: A 27-year-old male presented with a seven-year history of progressive right proptosis and right upper eyelid fullness. He was referred to our service following a Computed Tomography (CT) scan of his orbits done 3 months prior revealing a vascular lesion suspicious for CVM of his superonasal right orbit. He had no pain or diplopia. His visual acuity was 20/20 and his intraocular pressure was 19 in the right eye. He had 3 mm of proptosis by Hertel exophthalmometer. A careful review of his imaging revealed that the lesion had a feeder vessel. Magnetic Resonance Imaging (MRI) with intravenous gadolinium showed relatively homogenous enhancement of the mildly lobulated lesion that was involving the intraconal and extraconal space, and CVM was thought to be the most likely diagnosis. A follow-up MRI 5 months later revealed no progression. Given the significance of the mass effect, the patient opted for resection of the mass. The patient was referred to interventional radiology to consider pre-operative embolization of the lesion via the prominent feeder vessel. Ultraselective transarterial embolization of the lesion was performed using Onyx via the feeder vessel off the ophthalmic artery two days prior to surgical resection. The latter was performed using a medial lid splitting approach and cryoprobe after careful dissection and strict hemostasis. There was negligible blood loss and the lesion was resected in full. Pathological examination revealed a 3 cm mass with spindle cells, branching “staghorn” vasculature, and a partially myxoid stroma consistent with solitary fibrous tumor (SFT). The lesion had low mitotic index and no necrosis.
Conclusions: There have only been six previous cases in the literature of SFT primarily excised following
transarterial embolization. This approach greatly aided with intraoperative hemostasis and potentially the success of the resection. This case highlights the importance of identifying a feeder vessel on imaging studies for both its utility in embolization and its implication of a potentially more malignant process.