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.