Background
Cavernous hemangiomas are the most common intraorbital tumors found in adults. These benign, vascular lesions are slow growing and can manifest as a painless, progressively proptotic eye. Most of these tumefactions are exceedingly unilateral. Bilateral cases have been reported but are rare.
Pathophysiology
Orbital cavernous angiomas can increase intraorbital volume with a resultant mass effect. Although cavernous hemangiomas are histologically benign, they can encroach on intraorbital or adjacent structures and can be considered anatomically or positionally malignant. Visual acuity or field compromise, diplopia, and extraocular muscle or pupillary dysfunction can result from compression of intraorbital contents by the angioma. Lagophthalmos can result in exposure keratopathy, keratitis, and corneal perforation.
Epidemiology
Frequency
International
Henderson reported an incidence of 4.3% among orbital neoplasms. [1]
Mortality/Morbidity
The morbidity associated with cavernous hemangiomas is the threat of compressive optic neuropathy, extraocular muscle dysfunction, and cosmetic disfigurement.
Mortality can result from intraoperative complications, such as bleeding and the risk of general anesthesia.
Race
No predilection exists for race or ethnicity.
Sex
Harris and Jakobiec found a 7:3 occurrence ratio of women to men, while Henderson reported an almost equal ratio, 8:7 in women and men. [2, 1]
Age
Patients usually manifest symptomatically during the third to fifth decades of life.
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Extirpation of an orbital cavernous hemangioma. Note en bloc removal and preservation of capsule. Courtesy of Robert Alan Goldberg, MD.
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MRI demonstrates enhancing mass in apex of left orbit. White arrow points to the superior portion of the optic nerve, showing its deviation. Mass was pressing on superotemporal optic nerve and displacing it inferomedially at apex. Patient had 6 months of progressive decreased vision and visual field loss. Courtesy of M. Duffy, MD, PhD.
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In A, final preoperative visual field of same patient as in Media file 2, demonstrating significant inferior altitudinal field loss; in B, postoperative visual field at approximately 3 weeks after orbital apex decompression and removal of mass; and in C, postoperative visual field at approximately 6 months.
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Intraoperative photo of same patient as in Media file 2. Neurosurgical service performed craniotomy and decompression of the superior orbital fissure and optic canal (yellow arrows) at request of ophthalmology service. Orbital surgery service then opened the periorbita over a bulge (double black arrows) between optic nerve and cranial nerves (single black arrow) and bluntly dissected out mass. Pathology confirmed mass as a cavernous hemangioma. Cranial nerves V and IV were adhered, and careful blunt separation was performed. Postoperatively, a small left hypertropia resolved over 6 weeks. Courtesy of M. Duffy, MD, PhD.
