Orbital Lesions in the Blue Rubber Bleb Nevus Syndrome

Purpose: To identify ophthalmologic manifestations of the blue rubber bleb nevus syndrome, a rare cutaneovisceral hemangiomatosis. Methods: The authors report two patients with a diagnosis of blue rubber bleb nevus syndrome with orbital hemangiomas. Results: In one patient, the orbital lesion presented with signs and symptoms similar to an orbital varix and in the other with lid ecchymosis from an eyelid lesion. Conclusion: Patients with the blue rubber bleb nevus syndrome may have vascular orbital lesions associated with intermittent proptosis. Ophthalmologists should be familiar with the syndrome and its life-threatening complication of gastrointestinal hemorrhage.