Low-Flow Orbital Venous Malformation Masquerading as Rhabdomyosarcoma

A 15-month-old male was referred for biopsy of presumed rhabdomyosarcoma in the setting of rapidly progressing left-sided proptosis. Examination revealed left periorbital edema and left hypoglobus. MRI revealed a soft-tissue density mass within the left lateral retrobulbar space. Several days later, he developed acute periorbital ecchymosis and increasing edema. With high suspicion for a vascular lesion, a CT scan was performed with dynamic arterial and venous imaging. Central filling was noted in the lateral retrobulbar component with increased enhancement on delayed venous imaging in the middle cranial fossa component favoring the diagnosis of a low-flow orbital venous malformation. In the setting of spontaneous orbital hemorrhage and risk of future vision loss, the decision was made to proceed with a combined neurosurgical approach treating the intracranial component and debulking the orbital component. This case highlights the importance of thorough radiographic evaluation prior to proceeding with a surgical procedure in the appropriate clinical context. Accepted for publication August 7, 2018. The authors have no financial or conflicts of interest to disclose. Presented at the American Society of Ophthalmic Plastic & Reconstructive Surgery Conference, June 24, 2017 Vancouver BC, Canada. Address correspondence and reprint requests to Michael Kazim, M.D., Edward S. Harkness Eye Institute, 635 West 165th Street, New York, NY 10032. E-mail: mk48@cumc.columbia.edu © 2018 by The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc., All rights reserved.