Modern OptometryCollaborative Case #006: Red Tied

What could this fascinating case of spontaneous regression mean for future approaches to sickle cell retinopathy?

In a recent exploration of ocular impacts tied to sickle cell disease (SCD), an 11-year-old patient presents a fascinating case study. Highlighting key diagnostic tools and management strategies, the case underscores the importance of vigilant monitoring and the potential for spontaneous regression.

Key Points:

• The patient, an 11-year-old Black female with SCD, presented with traction of the macula in her left eye, initially measuring BCVA at 20/20 OD, 20/30+ OS.
• Dilated fundus examination of the left eye revealed fibrotic tissue in the macula and neovascularization associated with a preretinal hemorrhage and tortuous arteriovenous anastomosis.
• Spectral domain OCT of the right eye showed mild temporal retinal thinning, whereas the left eye exhibited increased retinal thickness and associated traction.
• Ultra-widefield fluorescein angiography (UWFA) revealed areas of nonperfusion in both eyes and superior temporal peripheral leakage in the left eye.
• After 2 months of monitoring, both the neovascularization and the macular fibrotic tissue in the left eye had regressed significantly, and visual acuity improved to 20/20.

Additional Points:

• Sickle cell retinopathy (SCR) presents in nonproliferative and proliferative stages, much like diabetes.
• Diagnostic modalities such as fundus photography, SD-OCT, and UWFA play vital roles in assessing and monitoring SCR.
• SCR management includes close monitoring, laser photocoagulation, anti-VEGF injections, and vitreoretinal surgery, depending on the stage and severity of the disease.

Conclusion:

• Regular dilated fundus examination and ultra-wide imaging are highly recommended for children with SCD, as they may present symptoms of SCR. Close follow-up and monitoring can aid in managing the disease effectively and possibly prevent vision loss.

Optometry Reading

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• Xdemvy Approved for Treatment of Demodex BlepharitisAugust 28, 2023
• With AI Tool, Johns Hopkins Clinician Boosts Diabetic Retinopathy Screening to 95% Among Pediatric PatientsFebruary 26, 2024

Did You Know?
It’s estimated that up to 40% of patients with sickle cell disease will develop proliferative sickle cell retinopathy, the most severe form of the disease which can lead to vision loss.