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Published on: November 5, 2019
Orbital infarction in sickle cell disease.
Anuradha Ganesh1, Sana Al-Zuhaibi, Anil Pathare
1Department of Ophthalmology, Sultan Qaboos University Hospital, Muscat, Oman. gananu@gmail.com
American Journal of Ophthalmology
|July 30, 2008
Summary
Orbital infarction is a serious vision threat in sickle cell disease (SCD). The Benin haplotype increases risk, and severity is unrelated to SCD systemic disease severity.
Area of Science:
- Ophthalmology
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder.
- Vaso-occlusive crises in SCD can lead to various complications.
- Orbital infarction is a rare but severe complication affecting vision.
Purpose of the Study:
- To investigate hematological and genetic factors contributing to orbital infarction in SCD.
- To identify risk factors for orbital infarction in sickle cell disease patients.
Main Methods:
- Retrospective case series of 14 SCD patients with orbital infarction.
- Comparison with matched SCD controls without orbital infarction.
- Analysis of clinical, radiological, and sickle haplotype data.
Main Results:
- Orbital infarction presented with periorbital pain, swelling, and potential vision loss during crises.
- Radiological findings included orbital swelling, hematoma, and abnormal bone marrow.
- Orbital involvement severity did not correlate with systemic SCD severity.
- The Benin haplotype was significantly more common in patients with orbital infarction.
Conclusions:
- Orbital infarction poses a significant risk to vision in SCD patients.
- Magnetic resonance imaging (MRI) is superior for evaluating orbital changes.
- Orbital infarction severity is independent of systemic SCD severity.
- The Benin haplotype is a key genetic factor associated with increased risk of orbital infarction during vaso-occlusive crises.
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