The American Academy of Ophthalmology (AAO) announced the presentations of a study of sickle retinopathy, an age-dependent process that can develop in patients with sickle cell disease, of whom older patients are at substantially higher risk than younger patients.

To learn more about how this condition manifests in children, investigators at the University of Tennessee Health Science Center in Memphis, Tennessee, conducted a large, retrospective review at their institution. According to an AAO press release, the study showed that one in three children had retinopathy, of whom 9% required treatment, which suggests that children need to be screened for vision problems as often as adults with sickle cell disease.

The study was presented at AAO 2023, held November 3-6 in San Francisco, California.

“Our data underscores the need for patients—including pediatric patients—with sickle cell disease to get routine ophthalmic screenings along with appropriate systemic and ophthalmic treatment,” commented lead researcher, Mary Ellen Hoehn, MD, professor of Ophthalmology at University of Tennessee Health Science Center.

Dr. Hoehn and colleagues also evaluated the effectiveness of therapies for sickle cell disease. They found that hydroxyurea and chronic transfusions were associated with decreased rates of retinopathy, even when accounting for different genotypes.

As summarized in the AAO press release, the study investigators evaluated records for 652 patients (age, 10 to 25 years; median age, 14 years) who underwent eye exams (2,240 visits) over a 12-year period.

In these patients, the investigators found the following:

  • 33% had nonproliferative retinopathy.
  • 6% had proliferative retinopathy (PR).
  • 33 eyes were treated with panretinal photocoagulation, most commonly for PR stage 3 (43%). Intravitreal anti-VEGF therapy was given to five eyes, all with PR.
  • Other complications included retinal detachment and retinal artery occlusion in two patients each.
  • Vision loss (final best corrected visual acuity 20/60) following complications from sickle cell disease was noted in only one patient with a central retinal artery occlusion.

“We hope that people will use this information to better care for patients with sickle cell disease, and that more timely ophthalmic screen exams will be performed so that vision-threatening complications from this disease are prevented,” concluded Dr. Hoehn in the AAO press release.