June 2020
Volume 61, Issue 7
Free
ARVO Annual Meeting Abstract  |   June 2020
SD-OCT in Monitoring Children with Sickle Cell Disease, More Than Meets the Eye
Author Affiliations & Notes
  • Jing Jin
    Ophthalmology, Nemours A. I. duPont Hospital for Children, Wilmington, Delaware, United States
  • Robin Miller
    Center for Cancer and Blood Disorders, Nemours A. I. duPont Hospital for Children, Wilmington, Delaware, United States
  • Footnotes
    Commercial Relationships   Jing Jin, None; Robin Miller, None
  • Footnotes
    Support  U54-GM104941 and P20GM109021
Investigative Ophthalmology & Visual Science June 2020, Vol.61, 2351. doi:
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      Jing Jin, Robin Miller; SD-OCT in Monitoring Children with Sickle Cell Disease, More Than Meets the Eye. Invest. Ophthalmol. Vis. Sci. 2020;61(7):2351.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : The association between the systemic manifestations of sickle cell disease (SCD) and sickle cell retinopathy (SCR) is not well understood. Data from our earlier study on the detection of retinal changes in SCD by optic coherence tomography (OCT) challenged the notion that subjects with SC genotype develop SCR more frequently than those with SS. This prospective, study confirmed the correlation between SCD genotype and retinal damage identified by OCT using an expanded dataset and examined a potential link between hypoxic ischemic injury in the retina and brain.

Methods : 97 patients (48 males) aged 5-20 years (mean 12.19 ± 4.35) with SCD (34 SC, 53 SS, 5 Sβ+ thalassemia, 5 Sβ0 thalassemia) underwent ophthalmologic examination including funduscopy and Spectral-Domain OCT (SD-OCT) imaging of both eyes. The posterior pole volume scan involves a 30° × 25° cuboid centered at approximately 3 mm temporal to the fovea by adjusting the fixation target. Areas of retinal thinning were determined using the circle grid function on the thickness map. T-test and Chi-square test were performed to compare findings between SCD genotypes and correlate ocular findings with cerebral vascular disease (CVD) in SS/Sβ0 genotype.

Results : Visual acuity ranged from 20/20 to 20/40. On funduscopic exam, 14 of 97 (14.43%) showed signs of retinopathy whereas 59 of 97 (60.82%) showed inner retina thinning on SD-OCT. By SD-OCT, patients with SS/Sβ0 showed a significantly higher frequency of SCR change (70.7% vs 47.1%), bilateral SCR (75.6% vs 43.8%) and foveal involvement (22.0% vs 0%) than those with SC genotype.
Remarkably, while funduscopic findings in our cohort with SS/Sβ0 genotype showed no correlation with CVD, all patients with CVD had evidence of SCR by SD-OCT (Table).

Conclusions : SD-OCT showed greater capabilities than fundoscopy in: 1) a greater detection rate for retinal changes, offering earlier diagnosis of SCR; 2) demonstrating a higher frequency and more extensive retinal changes in the more severe SCD genotypes SS and Sβ0 as compared to SC; 3) demonstrating a correlation between SCR and the presence of any form of CVD, strongly suggesting that retinal exam using SD-OCT may aid in detection and monitoring SCD related CVD. These important findings require further study in a larger patient population undergoing serial exams over time.

This is a 2020 ARVO Annual Meeting abstract.

 

1 vessel stenosis on MRA, 2 abnormal by STOP criteria, * p < 0.05 based on χ2 test for each group

1 vessel stenosis on MRA, 2 abnormal by STOP criteria, * p < 0.05 based on χ2 test for each group

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