Orbital compression syndrome in sickle cell disease.
OBJECTIVE
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.
Department of Ophthalmology, Albert Einstein College of Medicine (AECOM)/Montefiore Medical Center (MMC), Bronx, New York, USA. jason.sokol@mssm.edu
Jason A
JA
Sokol
Edward
E
Baron
George
G
Lantos
Michael
M
Kazim
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D002437:Cefazolin / Q000627:therapeutic use; D002648:Child; D004359:Drug Therapy, Combination; D005122:Exudates and Transudates; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007238:Infarction / Q000150:complications*; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009408:Nerve Compression Syndromes / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D009901:Optic Nerve Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D019315:Retrobulbar Hemorrhage / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D013100:Sphenoid Bone / Q000098:blood supply*
D005938:Glucocorticoids; D002437:Cefazolin; D008775:Methylprednisolone