Sickle Cell Maculopathy: Prevalence, Associations and Impact on Visual Acuity

Area of Science:

• Ophthalmology
• Hematology
• Retinal Diseases

Background:

• Sickle cell disease is a genetic blood disorder with potential ocular complications.
• Sickle cell maculopathy (SCM) is a known complication affecting the macula.

Purpose of the Study:

• To determine the prevalence of SCM in adults with sickle cell disease.
• To explore associations between SCM and patient demographics, genotype, and disease stage.
• To assess the impact of SCM on visual acuity.

Main Methods:

• A consecutive series of 74 adults with sickle cell disease were evaluated.
• Data collected included age, sex, and visual acuity.
• Spectral domain OCT and ultra-wide-field imaging were used to assess the macula and retina.

Main Results:

• SCM was present in 54.1% of patients and 42.3% of eyes.
• Prevalence varied by genotype: HbSS (54.8%), HbSC (62.5%), HbS/BThal or other (25%).
• SCM was found in 39.4% of eyes with early proliferative sickle cell retinopathy (PSR) and 51.2% with advanced PSR. Mild visual impairment was rare and secondary to other conditions.

Conclusions:

• SCM is a frequent ocular finding in adults with sickle cell disease.
• SCM prevalence is similar in HbSS and HbSC genotypes and not linked to PSR stage.
• High-contrast distance visual acuity is generally maintained in patients with SCM.