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.