Orbital Compression Syndrome in Sickle Cell Disease

Purpose: To present 3 cases of orbital compression syndrome caused by infarction of the greater wing of the sphenoid in patients with sickle cell disease. Methods: Case report and review of the literature. Results: Three patients with sickle cell disease (2 males aged 22 and 16 years, and a 10-year-old girl) who presented with proptosis, limited ocular motility, and chemosis were found to have an infarction of the marrow space of the greater wing of the sphenoid that produced an orbital subperiosteal hemorrhage and exudate demonstrated on MRI. Two patients suffered compressive optic neuropathy; both patients recovered normal optic nerve function. Orbital edema resolved within 48 hours of receiving 1 g methylprednosolone daily. The third patient had normal optic nerve function and his orbital edema improved with methylprednisolone 250 mg/day and intravenous Kefzol over 3 days. In the literature, there are 27 similar cases; 5 were treated surgically and the remainder were managed medically. Conclusions: Patients with sickle cell disease are at risk for orbital compression syndrome secondary to orbital bone infarction, in the setting of vaso-occlusive crises. This diagnosis should be considered when a patient with sickle cell disease presents with headache, proptosis, decreased motility, and/or optic nerve compromise.