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Published on: November 5, 2019
Orbital compression syndrome in sickle cell disease
Jason A Sokol1, Edward Baron, George Lantos
1Department of Ophthalmology, Albert Einstein College of Medicine (AECOM)/Montefiore Medical Center (MMC), Bronx, New York, USA. jason.sokol@mssm.edu
Ophthalmic Plastic and Reconstructive Surgery
|June 4, 2008
Summary
Sickle cell disease patients can develop orbital compression syndrome from bone infarction during crises. Prompt diagnosis and treatment are crucial for preventing vision loss.
Area of Science:
- Ophthalmology
- Hematology
- Radiology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder associated with vaso-occlusive crises.
- Orbital complications in SCD are rare but can lead to significant morbidity.
Observation:
- Three cases of orbital compression syndrome in young SCD patients presenting with proptosis, limited ocular motility, and chemosis.
- Magnetic Resonance Imaging (MRI) revealed infarction of the greater wing of the sphenoid bone with subperiosteal hemorrhage and exudate.
Findings:
- Two patients experienced compressive optic neuropathy, with full recovery after high-dose corticosteroid treatment.
- Orbital edema resolved rapidly with methylprednisolone, supplemented with antibiotics in one case.
- Literature review identified 27 similar cases, with most managed medically.
Implications:
- Orbital bone infarction is a potential cause of orbital compression syndrome in patients with sickle cell disease during vaso-occlusive crises.
- Clinicians should consider this diagnosis in SCD patients presenting with proptosis, decreased ocular motility, and/or optic nerve compromise.
- Early recognition and medical management, including corticosteroids, can lead to favorable visual outcomes.
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