Author(s): Pahl DA, Green NS, Bhatia M, Lee MT, Chang JS, Licursi M, Briamonte C, Smilow E, Chen RWS. Optical coherence tomography angiography and ultra-widefield fluorescein angiography for early detection of adolescent sickle retinopathy. Am J Ophthalmol. 2017 Nov;183:91-98. doi: 10.1016/j.ajo.2017.08.010. Epub 2017 Aug 30. PMID 28860042
Journal: American Journal Of Ophthalmology, Volume 183, Nov 2017
PURPOSE Based on standard screening techniques, sickle retinopathy reportedly occurs in 10% of adolescents with sickle cell disease (SCD). We performed a prospective, observational clinical study to determine if ultra-widefield fluorescein angiography (UWFA), spectral-domain optical coherence tomography (SD-OCT), and optical coherence tomography angiography (OCT-A) detect more-frequent retinopathy in adolescents with SCD.
DESIGN Cross-sectional study.
METHODS Setting: Institutional.
SUBJECTS Sixteen adolescents with SCD, aged 10-19 years (mean age 14.9 years), and 5 age-equivalent controls (mean age 17.4 years).
OBSERVATION PROCEDURES Examinations including acuity, standard slit-lamp biomicroscopy, UWFA, SD-OCT, and OCT-A were performed.
MAIN OUTCOME MEASURES Sickle retinopathy defined by biomicroscopic changes, Goldberg stages I-V, Penman scale, flow void on OCT-A, or macular thinning on SD-OCT.
RESULTS While 22 of 32 SCD eyes (68.8%) had retinopathy on biomicroscopy, by UWFA 4 of 24 (16.7%) SCD eyes had peripheral arterial occlusion (Goldberg I), and 20 of 24 eyes (83.3%) had peripheral arteriovenous anastomoses (Goldberg II) in addition. No patients had Goldberg stages III-V. By SD-OCT and OCT-A, thinning of the macula and flow voids in both the superficial and deep retinal capillary plexus were found in 6 of 30 (20%) eyes.
CONCLUSIONS All 24 eyes with adequate UWFA studies demonstrated sickle retinopathy. SD-OCT and OCT-A, which have not been previously reported in the adolescent population, detected abnormal macular thinning and flow abnormalities undetected by biomicroscopy. These findings suggest that pediatric sickle retinopathy may be more prevalent than previously suspected. If these findings are confirmed with larger cross-sectional and prospective analyses, these approaches may enhance early screening for sickle retinopathy.