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Published on: November 5, 2019
Orbital involvement in sickle cell disease: A systematic review
Jai Paris1, Abdullah I Almater2, Charlotte Mackenzie1
1Discipline of Ophthalmology and Visual Sciences, The University of Adelaide, Adelaide, South Australia 5000, Australia; Department of Ophthalmology, The Royal Adelaide Hospital, Adelaide, South Australia 5000, Australia.
Survey of Ophthalmology
|June 3, 2026
Summary
Orbital complications in sickle cell disease (SCD) are rare but can cause vision loss. Early recognition and diagnosis, often requiring MRI, are key for successful treatment and recovery in patients with sickle cell orbitopathy.
Area of Science:
- Ophthalmology
- Hematology
- Radiology
Background:
- Orbital complications in sickle cell disease (SCD) are uncommon but can mimic infectious conditions.
- Misdiagnosis of sickle cell orbitopathy can lead to delayed treatment and potential vision loss.
Purpose of the Study:
- To systematically review and characterize orbital complications in patients with confirmed sickle cell disease.
- To identify common presentations, diagnostic findings, and management strategies for sickle cell orbitopathy.
Main Methods:
- Systematic review of case reports and series following PRISMA and MOOSE guidelines.
- Inclusion of 76 cases from 53 studies detailing orbital complications in SCD patients.
- Analysis of clinical presentations, laboratory findings, radiological data, and treatment outcomes.
Main Results:
- Orbital subperiosteal hematoma (70%) and bone infarction (19.7%) were common radiological findings.
- Periorbital edema, proptosis, and restricted ocular motility were frequent clinical signs.
- Leukocytosis (73%) and elevated inflammatory markers (86.7%) were prevalent laboratory findings.
- Magnetic resonance imaging (MRI) is crucial for accurate diagnosis.
Conclusions:
- Sickle cell orbitopathy presents with diverse orbital manifestations, often involving hematoma and bone infarction.
- While complete recovery is common (93.1%) with conservative management, prompt diagnosis is vital.
- Early recognition of sickle cell orbitopathy is critical to prevent severe vision-threatening complications.
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