Ocular Manifestations of Sickle Cell Disease: Signs, Symptoms and Complications

ABSTRACT

Background

Sickle cell disease is an inherited hematological disorder that can affect any organ in the body including the eyes (1–6). Previous studies on ocular manifestations of sickle cell disease generally included samples of less than 100 patients. In this study, we aim to assess the frequency of different ocular signs, symptoms and complications among sickle cell disease patients.

Methods

This study was conducted using data from the Cooperative Study of Sickle Cell Disease (CSSCD). Patients with major sickle cell hemoglobinopathies (SS, SC, S β-thal) were eligible for enrollment. Patients from all age groups were included. Patients underwent detailed ophthalmological examination under standardized conditions.

Results

A total of 1904 patients were included in this study, with a mean age of 27.67 (±11.72) years. 1,802 (96.4%) patients had BCVA of more than 20/40 in the better-seeing eye. On slit lamp examination, the presence of vascular loops and segment, representing a positive conjunctival sign, was the most common reported abnormal finding (54.1%). The most common complication was peripheral retinal artery occlusion detected in225 patients (20.3%) bilaterally and 77 patients (6.9%) unilaterally.

Conclusion

In this study that included one of the largest samples ever studied to assess ocular complications of sickle cell disease, we identified the frequency and percentages of different ocular signs, symptoms and complications in different age groups.

KEYWORDS:

• Sickle Cell
• ocular
• conjunctival sign
• sunburst
• retinopathy

Acknowledgments

We would like to thank the Biologic Specimen and Data Repository Information Coordinating Center (BioLINCC) for providing the data for the Cooperative Study of Sickle Cell Disease (CSSCD) https://biolincc.nhlbi.nih.gov/studies/csscd/.

Data availability statement

The data described in this article are openly available in the Open Science Framework at DOI:10.17605/OSF.IO/TPA6U.

Declaration of interest

All authors have no conflicts of interest to disclose.