The Impact of Optical Coherence Tomography in the Early Identification of Children with Sickle Cell Retinopathy
Table 2
Relation of different Hb genotypes and study variables.
Variables
Hb SS n = 8 (%)
Hb SC n = 3 (%)
Hb SB0n = 1 (%)
Hb SB + n = 3 (%)
-value
Age (years)
11.7 ± 1.3
11.3 ± 0.3
11.0
9.5 ± 0.3
0.064
Age at diagnosis (months)
21.0 ± 9.8
24.7 ± 13.0
18.0
26.7 ± 4.2
0.780
Disease duration (years)
10.0 ± 1.2
9.2 ± 0.9
9.5
7.3 ± 0.6
0.027
Gender
Male
5 (62.5)
2 (66.7)
0 (0.0)
2 (66.7)
0.652
Female
3 (37.5)
1 (33.3)
1 (100.0)
1 (33.3)
Hb prior to study
7.5 ± 0.4
8.5 ± 0.1
6.9
9.8 ± 0.3
<0.001
Hb F (%) prior to study
6.4 ± 0.5
8.0 ± 1.0
6.0
15.0 ± 2.6
<0.001
Retics (%) prior to study
10.9 ± 1.5
8.7 ± 0.6
9.5
5.7 ± 1.5
0.001
Simple blood transfusion
Present
8 (100.0)
3 (100.0)
1 (100.0)
0 (0.0)
0.002
Exchange transfusion
One time (suspected stroke)
1 (12.5)
0 (0.0)
0 (0.0)
0 (0.0)
0.208
One time (acute chest syndrome)
1 (12.5)
0 (0.0)
1 (100.0)
0 (0.0)
Neurological symptoms
Seizures (silent stroke)
1 (12.5)
0 (0.0)
0 (0.0)
0 (0.0)
0.569
Frequent headache
3 (37.5)
1 (33.3)
1 (100.0)
0 (0.0)
Ocular symptoms
Decreased visual acuity
2 (25.0)
1 (33.3)
1 (100.0)
0 (0.0)
0.270
Painful crisis
Frequent
4 (50.0)
1 (33.3)
1 (100.0)
0 (0.0)
0.011
Infrequent
4 (50.0)
2 (66.7)
0 (0.0)
0 (0.0)
Hydroxyurea therapy
Not compliant
3 (37.5)
1 (33.3)
1 (100.0)
0 (0.0)
0.010
Compliant
5 (62.5)
2 (66.7)
0 (0.0)
0 (0.0)
Hb SS n = 16 (%)
Hb SC n = 6 (%)
Hb SB0n = 2 (%)
Hb SB + n = 6 (%)
-value
Fundus exam
Abnormal findings1
10 (62.5)
1 (16.7)
1 (50.0)
0 (0.0)
0.032
OCT
Macular thinning
9 (56.3)
1 (16.7)
0 (0.0)
0 (0.0)
0.036
OCTA
Flow void at the deep retinal capillary plexus
9 (56.3)
1 (16.7)
0 (0.0)
0 (0.0)
0.036
Flow void at the superficial retinal capillary plexus
2 (12.5)
1 (16.7)
1 (50.0)
0 (0.0)
0.345
Significant.1Children with Hb SS showed tortuosity, salmon patches, and black sunspots in 3 children (6 eyes), tortuosity and salmon patches in one child (2 eyes), and another one child (2 eyes) showed tortuosity only. A child with Hb SC showed tortuosity, salmon patches, and black sunspots in one eye and the child with Hb SB0 showed tortuosity, salmon patches, and black sunspots in one eye.
