Abstract ID: 26-622

Bilateral Cavernous Venous Malformations of the Orbit: A Case Report and Literature Review

Author: Ryan Zukerman
Base Hospital / Institution: University of Pittsburgh Medical Center

Presentation Type: ePoster Presentation

Purpose

To describe clinical features and management of a patient with bilateral orbital cavernous venous malformations (CVM) and to review the previous literature on bilateral CVMs.


Methods

A 48-year-old man presented for evaluation of several years of uneven eyes. His best corrected visual acuity was 20/20 bilaterally, pupils were equal, round and reactive without a relative afferent pupillary defect, and extraocular movements were full and without pain in all directions. His exam was significant for a right inferolateral palpable lesion, 4mm of left-sided hypoglobus, and 5mm of left-sided proptosis.


Results

Computed tomography angiogram (CTA) demonstrated heterogeneously enhancing bilateral orbital masses without large feeder vessels or dilation of either ophthalmic artery; the mass on the right was in the inferolateral intraconal space, while the mass on the left was in the superomedial intraconal space abutting the superior rectus muscle, medial rectus muscle, and the optic nerve. Delayed CTA also demonstrated a gradual increase in enhancement with Valsalva maneuvers. Magnetic resonance imaging (MRI) demonstrated bilateral contrast-enhancing masses, specifically with delayed filling on T1 fat-suppressed MRI. The patient underwent sequential orbitotomies with excisional biopsy. Histopathology of the specimens demonstrated dilated vascular channels with walls of smooth muscle cells lined by flattened endothelial cells within a fibrous capsule, with a diagnosis of bilateral CVMs. Post-operative exam demonstrated resolution of the patient’s left-sided hypoglobus and left-sided proptosis.


Conclusion

CVMs, formerly cavernous hemangiomas, are the most common benign neoplasms of the orbit in adults, typically presenting as solitary, unilateral intraconal masses with large venous channels and a surrounding fibrous capsule. This case represents a rare presentation of bilateral orbital CVMs. To date, very few cases of bilateral CVMs have been documented without systemic association, though it has been suggested that bilateral orbital CVMs may be more common than currently recognized.


Additional Authors

First name Last name Base Hospital / Institution
Stephen Klapper Klapper Eyelid & Facial Plastic Surgery

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