Optical Coherence Tomography Angiography and Ultra-widefield Fluorescein Angiography for Early Detection of Adolescent Sickle Retinopathy

Abstract

Purpose Using 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, ages 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 22/32 SCD eyes (68.8%) had retinopathy on biomicroscopy, by UWFA 4/24 (16.7%) SCD eyes had peripheral arterial occlusion (Goldberg I), and 20/24 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 6/30 (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.

Sickle cell retinopathy. A focused review

“…Spectral-domain optical coherence tomography and optical coherence tomography angiography (SD-OCT and OCT-A) identified abnormal macular thinning and flow voids in the superficial and deep retinal capillary plexus in 20% of eyes that had been unobserved by biomicroscopy. These findings suggest that pediatric sickle retinopathy may be more prevalent than previously suspected [49,52]. Temporal macular thinning caused by paracentral acute middle maculopathy may be the result of occlusions at the level of the deep retinal capillary plexus (Fig.…”

“…Pahl et al conducted a crosssectional study in pediatric patients (age 10-19 years old) with SCD, demonstrating that all 24 eyes examined by UWFA had sickle cell retinopathy, even if this had not been detected on biomicroscopy. Findings included peripheral arterial occlusion in four of the 24 eyes and peripheral arteriovenous anastomoses in the remaining 20 eyes [49].…”

High Healthcare Utilization in Adolescents with Sickle Cell Disease Prior to Transition to Adult Care: A Retrospective Study

“…10,11,[13][14][15]21,22 Published data are limited on acute and chronic complications, specifically associated with SCD in children prior to the time of transition to adult care. 13,20,[23][24][25][26][27] More information is needed about healthcare resource utilization (HCRU) and treatment patterns in young patients with SCD before they transition to adult care. 15,21,[28][29][30][31][32] Improved understanding of the HCRU in adolescents may help predict individuals at higher risk post-transition.…”

Correlation between Basal Macular Circulation and Following Glaucomatous Damage in Progressed High-Tension and Normal-Tension Glaucoma

“…While comparing OCTA examination to traditional methods, previous studies have demonstrated that OCTA is a reliable tool for the analysis of retinal vasculature, similar to fluorescein angiography [21, 22], and that the retinal vasculature layers can be imaged more precisely compared to fluorescein angiography and indocyanine green angiography [23, 24]. With regard to glaucoma, HTG, after evaluation by OCTA, showed decreased peripapillary vessels that correlate with the RNFL and VF defects and impaired macular VD [9, 14, 17, 18, 25-28], while the OCTA evaluation in NTG subjects showed similar results and precision in the peripapillary and macular vasculature [10, 15, 17, 18, 29].…”