June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
Case Series of Pediatric Sickle Retinopathy by Ultra-Widefield Fluorescein Angiography and OCT-Angiography Suggests Frequent Early Pathology
Author Affiliations & Notes
  • Daniel A. Pahl
    Columbia College of Physicians and Surgeons, New York, New York, United States
  • Nancy S. Green
    Department of Pediatrics, Columbia University Medical Center, New York, New York, United States
  • Monica Bhatia
    Department of Pediatrics, Columbia University Medical Center, New York, New York, United States
  • Maureen Licursi
    Department of Pediatrics, Columbia University Medical Center, New York, New York, United States
  • Courtney Briamonte
    Department of Pediatrics, Columbia University Medical Center, New York, New York, United States
  • Elana Smilow
    Department of Pediatrics, Columbia University Medical Center, New York, New York, United States
  • Jonathan S Chang
    Department of Ophthalmology, Columbia University Medical Center, New York, New York, United States
  • Margaret T. Lee
    Department of Pediatrics, Columbia University Medical Center, New York, New York, United States
  • Royce W.S. Chen
    Department of Ophthalmology, Columbia University Medical Center, New York, New York, United States
  • Footnotes
    Commercial Relationships   Daniel Pahl, None; Nancy Green, None; Monica Bhatia, None; Maureen Licursi, None; Courtney Briamonte, None; Elana Smilow, None; Jonathan Chang, None; Margaret Lee, None; Royce Chen, None
  • Footnotes
    Support  This work was supported by a research award from the Irving Institute for Clinical and Translational Research (1UL1TR001873, PI H. Ginsberg) to RWC, a training award (2T35HL007616, PI R. Leibel) to DAP, and an unrestricted grant from Research to Prevent Blindness.
Investigative Ophthalmology & Visual Science June 2017, Vol.58, 1547. doi:
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      Daniel A. Pahl, Nancy S. Green, Monica Bhatia, Maureen Licursi, Courtney Briamonte, Elana Smilow, Jonathan S Chang, Margaret T. Lee, Royce W.S. Chen; Case Series of Pediatric Sickle Retinopathy by Ultra-Widefield Fluorescein Angiography and OCT-Angiography Suggests Frequent Early Pathology. Invest. Ophthalmol. Vis. Sci. 2017;58(8):1547.

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

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Abstract

Purpose : Sickle cell retinopathy (SCR) has been reported to occur in 10% of children using standard screening techniques. We performed a prospective, observational clinical study to determine if novel retinal imaging devices, including ultra-widefield fluorescein angiography (UWFA), spectral-domain optical coherence tomography (SD-OCT), and optical coherence tomography angiography (OCT-A), would detect a higher frequency of disease in children with sickle cell disease (SCD).

Methods : A consecutive series of children with SCD of any phenotype, ages 10-19 years, and age-equivalent controls are described. Examinations including acuity, standard slit-lamp biomicroscopy, UWFA, SD-OCT and OCT-A were performed. 2 children were not able/refused to undergo UWFA testing and two UWFA tests (4 eyes) were considered inadequate. Only one control to date has undergone UWFA testing.

Results : Sixteen children with SCD (mean age 14.9 years) and 3 controls (mean age 16.3 years) were evaluated. No patients had visual complaints at time of examination. While 22/32 SCD eyes (68.8%) had retinopathy on biomicroscopy, UWFA was more sensitive: all 24/24 SCD eyes (100%) had peripheral arterial occlusion (Goldberg I), and 21/24 eyes (87.5%) had peripheral arteriovenous anastomoses (Goldberg II) that were undetectable by biomicroscopy. No patients had neovascular seafans (Goldberg III), vitreous hemorrhage (Goldberg IV), or retinal detachment (Goldberg V). SD-OCT showed thinning of the temporal macula in 6/32 eyes (18.8%), and OCT-A of the same 6/32 eyes (18.8%) revealed flow voids in both the superficial and deep retinal capillary plexus in the area of thinned retina. Both the SD-OCT and OCT-A abnormalities were undetectable with biomicroscopy. Control patients demonstrated no abnormalities by biomicroscopy, SD-OCT, OCT-A, or UWFA.

Conclusions : All 16 children with SCD in this case series demonstrated evidence of SCR using the combination of biomicroscopy, UWFA, SD-OCT, and OCT-A imaging. Findings from these sensitive imaging modalities suggest that pediatric SCR is more prevalent than previously suspected. Larger cross-sectional and prospective analyses are needed to confirm these findings and identify their significance for detecting pathology. If confirmed, these approaches may enhance early screening for patients at risk of the vision-threatening consequences of SCR.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.

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