Eli L Chang1, Peter A D Rubin. 1. Ophthalmic Plastic, Orbital and Cosmetic Surgery Service, Harvard Medical School, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114, USA.
Abstract
PURPOSE: To report the clinical findings and surgical treatment of multifocal, bilateral orbital hemangiomas in a patient with the blue rubber bleb nevus syndrome, a rare vascular disorder characterized by distinctive cutaneous and visceral hemangiomas. DESIGN: Interventional case report. METHODS: Review of clinical findings, radiologic studies, and treatment of the patient. RESULTS: A 36-year-old female with multifocal, bilateral orbital lesions was seen with severe proptosis and decreased visual acuity of the left eye. Surgical debulking of multiple cavernous hemangiomas of the orbit resulted in decreased proptosis and an improvement in visual acuity. Subsequent histopathologic analysis and the results of a systemic workup revealing multiple, distinctive, cutaneous and visceral lesions were found to be consistent with the blue rubber bleb nevus syndrome. CONCLUSIONS: Although hemangiomas of the orbit are relatively common occurrences, multifocal, bilateral lesions may exist in the setting of underlying systemic hemangiomatosis. Bilateral or multifocal hemangiomas associated with cutaneous or visceral lesions should alert the clinician to the possibility of a number of syndromes that may have life-threatening consequences.
PURPOSE: To report the clinical findings and surgical treatment of multifocal, bilateral orbital hemangiomas in a patient with the blue rubber bleb nevus syndrome, a rare vascular disorder characterized by distinctive cutaneous and visceral hemangiomas. DESIGN: Interventional case report. METHODS: Review of clinical findings, radiologic studies, and treatment of the patient. RESULTS: A 36-year-old female with multifocal, bilateral orbital lesions was seen with severe proptosis and decreased visual acuity of the left eye. Surgical debulking of multiple cavernous hemangiomas of the orbit resulted in decreased proptosis and an improvement in visual acuity. Subsequent histopathologic analysis and the results of a systemic workup revealing multiple, distinctive, cutaneous and visceral lesions were found to be consistent with the blue rubber bleb nevus syndrome. CONCLUSIONS: Although hemangiomas of the orbit are relatively common occurrences, multifocal, bilateral lesions may exist in the setting of underlying systemic hemangiomatosis. Bilateral or multifocal hemangiomas associated with cutaneous or visceral lesions should alert the clinician to the possibility of a number of syndromes that may have life-threatening consequences.