Choroidal occlusive disease in sickle cell hemoglobinopathies

Area of Science:

• Ophthalmology
• Hematology
• Vascular Biology

Background:

• Sickle cell retinopathy is a known complication of hemoglobin SS disease.
• Posterior ciliary artery occlusions are typically observed post-photocoagulation, but spontaneous occurrences are less documented.

Observation:

• A 32-year-old man with hemoglobin SS disease experienced two episodes of posterior ciliary artery occlusion and amaurosis fugax.
• Histopathologic examination of three additional eyes from patients with sickle hemoglobinopathies revealed potential evidence of prior small vessel occlusions.

Findings:

• The study details the clinical manifestations of spontaneous posterior ciliary artery occlusion in sickle cell disease.
• Histopathology suggests that small vessel occlusive disease, possibly from prior occlusions, is related to sickling hemoglobinopathies.

Implications:

• This research highlights a less-recognized complication of sickle cell disease affecting ocular circulation.
• Understanding spontaneous posterior ciliary artery occlusion is crucial for managing visual impairment in patients with hemoglobinopathies.