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. Author manuscript; available in PMC: 2018 Nov 1.
Published in final edited form as: J Pediatr Hematol Oncol. 2017 Nov;39(8):618–625. doi: 10.1097/MPH.0000000000000919

Table 1. Literature on the epidemiology of pediatric sickle cell retinopathy.

Study Number of patients Age Range Retina Exams Done Prevalence Study Type Additional Findings
Abiose et al. (1978) [24] 91 (91 HbSS, 0 HbSC) 5 to 14

  1. Direct Ophthalmoscopy

  2. Indirect Ophthalmoscopy

  3. Three-mirror contact lens where indicated

 Any retinopathy - 58%
PSR – 1% with neovascularization and 5.5% with peripheral arteriolar occlusion		 Cross-sectional Vascular tortuosity - 27%; Black sunbursts - 11%; Salmon patch hemorrhage - 4%; Abnormal conjunctival vasculature – 81%
Condon et al. (1974) [79] 54 (0 HbSS, 54 HbSC) 2 to 15

  1. Direct Ophthalmoscopy

  2. Indirect Ophthalmoscopy

  3. Three-mirror contact lens where indicated

  4. No fluorescein unless indicated on exam (5%)

 Any retinopathy (peripheral retinal vessel disease) - 94%
PSR - 11%		 Cross-sectional Vascular tortuosity - 32%; Black sunbursts - 4%; Arterio-venous fistulae - 17%; Angioid streaks - 0%; Retinoschisis – 2%
Downes et al. (2005) [1] 474 (307 HbSS, 166 HbSC) 5 to 26

  1. Fundus examination through dilated pupils (exact methodology not specified)

  2. Fluorescein for all

 PSR - 43% of HbSC and 14% of HbSS by ages 24-26
CHANGE TO 18-20 AGE RANGE		 Longitudinal observed over 20 yrs Spontaneous regression in 32% of PSR-affected eyes
El-Ghamrawy et al. (2014) [16] 40 (26 HbSS, 14 HbS/Beta-thal) 2 to 28

  1. Biomicroscopy

  2. Indirect Ophthalmoscopy

  3. Fluorescein for age 12+ or when indicated on exam

 Any retinopathy - 47.5% (46.2% of HbSS and 50% of HbS/Beta-thal)
PSR - 32.5%
NPR - 27.5%		 Cross-sectional Vascular tortuosity - 25%; Black sunbursts - 5%
Eruchalu et al. (2006) [77] 37 (37 HbSS, 0 HbSC) 3 to 14

  1. Direct Ophthalmoscopy

  2. Indirect Ophthalmoscopy

 Any retinopathy - 32.4%
PSR - 5.4%
NPR - 27%		 Cross-sectional Retinal infarcts - 14.9%; Vascular tortuosity - 54%; Black sunbursts - 6.8%; Salmon patch hemorrhage - 6.8%; Comma vessels – 70.3%
Estepp et al. (2013) [13] 123 (123 HbSS, 0 HbSC) 11 to 16

  1. Dilated fundoscopic examination (exact methodology not specified)

  2. No fluorescein unless indicated on exam

 Any retinopathy - 10.6%
PSR- 1.6%
NPR - 8.9%		 Retrospective review between 2003-2010
Gill et al. (2008) [11] 236 (163 HbSS, 73 HbSC, 27 HbS/Beta-thal) 1 to 18

  1. Dilated fundoscopic examinations (exact methodology not specified)

  2. No fluorescein unless indicated on exam

 Any retinopathy - 18.7%
PSR- 2.7% (8.2% of HbSC, 0.6% of HbSS, and 0% of HbS/Beta-thal)
NPR - 18.6% (24.7% of HbSC, 14.1% of HbSS, and 11.1 % of HbS/Beta-thal		 Retrospective longitudinal survival analysis between 1987-2005
Kimmel et al. (1987) [15] 135 (Breakdown not available) 0 to 20

  1. Dilated fundoscopic examination (exact methodology not specified)

  2. No fluorescein unless indicated on exam

 PSR - 5.2% Retrospective review between 1976 to 1985
Oliveira et al. (2014) [12] 51 (36 HbSS, 15 HbSC) 4 to 18

  1. Biomicroscopy

  2. Indirect Ophthalmoscopy

  3. Fluorescein for age 10+

 Any retinopathy (ages 10 to 18) - 17.5% (80.0% of HbSS and 22.2% of HbSC ages 10-18)
PSR (ages 4 to 18) - 39.2% (30.6% of HbSS and 60.0% of HbSC)
NPR (ages 4 to 18) - 39.3% (38.9% of HbSS and 40.0% of HbSC)		 Cross-sectional Neovascularization - 0%; Vascular tortuosity - 19.6%; Black sunbursts - 9.8%; Salmon patch hemorrhages - 2.0%; Angioid streaks - 0%; Iridescent spots - 7.8%
Rosenberg et al. (2011) [17] 258 (186 HbSS, 55 HbSC, 12 HbS/Beta-thal 10 to 18

  1. Dilated fundoscopic examination (exact methodology not specified)

 Any retinopathy - 20.9% (17.2% of HbSS, 32.7% of HbSC, 33.3% of HbS/Beta-thal)
PSR - 4.3% (0.54% of HbSS, 16.4% of HbSC, 8.3% of HbS/Beta-thal)
NPR - 16.3%		 Retrospective review over 10 year period
Talbot et al. (1982) [25] 96 (59 HbSS, 37 HbSC) 5 to 7.5

  1. Direct Ophthalmoscopy

  2. Indirect Ophthalmoscopy

  3. Fluorescein for age 6.5+ and when indicated on exam for younger

 PSR - 0.0% Cohort Peripheral arteriolar closure- 24% HbSS and 16% HbSC; Arteriolar sheathing- 51% HbSS and 30% HbSC; Arteriovenous anastomoses - 3.1%; Retinal patches - 37% of HbSS and 24% of HbSC
Talbot et al. (1983) [19] 85 (54 HbSS, 31 HbSC) 6.5 to 8.5

  1. Direct Ophthalmoscopy

  2. Indirect Ophthalmoscopy

  3. Fluorescein for all

 Cohort Peripheral arterial closure - 66.6% HbSS and 51.6% HbSC
Talbot et al. (1988) [14] 389 (219 HbSS, 135 HbSC, 24 HbS/Beta-thal, 11 HbS/Beta-thal0) 5 to 13

  1. Direct Ophthalmoscopy

  2. Indirect Ophthalmoscopy

  3. Fluorescein for age 6+

 PSR - 0.3% Cohort Peripheral arteriolar closure – 90% of HbSS and HbSC by age 12
Tantawy et al. (2013) [78] 60 (28 HbSS, 32 HbS/Beta-thal) 3 to 18

  1. Indirect Ophthalmoscopy

  2. Fluorescein for all

 Any retinopathy – 17% (25% of HbSS and 9.4% of HbS/Beta-thal)
PSR - 0%
NPR- 17% (25% of HbSS and 9.4% of HbS/Beta-thal)		 Cohort

NPR- non-proliferative retinopathy; PSR - proliferative sickle retinopathy