Intermittent visual loss and exophthalmos due to the Blue rubber bleb nevus syndrome.

PURPOSE To describe the occurrence of intermittent visual loss in a patient with orbital hemangioma due to the blue rubber bleb nevus syndrome. METHODS Case report. RESULTS A 70-year-old woman complained of intermittent blurred vision combined with pressure behind her left eye occurring after head bending or Valsalva maneuvers. Examination revealed 2 mm of enophthalmos on the left. After downward bending of the head the eye became exophthalmic by 12 mm and visual acuity decreased to finger counting. Neuro-ophthalmologic examination was otherwise normal. Magnetic resonance imaging revealed an intraconal hemangioma. Bluish, soft, rubber-like, compressible and slowly refilling hemangiomas on the inferior lip, sublingually, on the hand, and vagina led to the diagnosis of BRBNS. CONCLUSIONS Intermittent visual loss may be the presenting symptom of an orbital hemangioma in BRBNS. Recognition of this rare condition is important because of its potentially life-threatening complications.