Visual Acuity in Aniridia and WAGR Syndrome

Figures & data

Table 1 Characteristics of Aniridia and WAGR Spectrum Subjects

	Aniridia	WAGR Spectrum	P value
Number of patients	25	25
Number of eyes	50	50
Gender, male/female, N (%)	13/12 (52/48)	10/15 (40/60)	0.571
Mean age ± SD, years	15.4 ± 11.6	16.8 ± 13.3	0.693
Median age (range), years	13 (2–41)	13 (2–50)
Nonsense mutation, N (%)	25 (100)	0 (0)	0.000
Large* deletion mutation, N (%)	0 (0)	25 (100)

Note: *Multi-gene.

Table 2 Visual Acuity in Aniridia and WAGR Spectrum Subjects

	Aniridia	WAGR Spectrum	P value
Number of patients	25	25
Number of eyes	50	50
LogMAR OD*	0.87±0.25	1.42±0.87	0.004
LogMAR OS, mean ± SD	1.03±0.70	1.60±1.11	0.035
LogMAR eyes, mean ± SD	0.95±0.53	1.51±0.99	<0.001
LogMAR eyes, median (range)	0.9 (0.5–3.7)	1.2 (0.9–4.7)
LogMAR better eye†	0.78±0.15	1.40±0.88	0.001
LogMAR worse eye, mean ± SD	1.12±0.70	1.62±1.10	0.061
Median better eye (range)	0.8 (0.5–1.0)	1.1 (0.9–4.7)

Notes: *Intragroup comparisons of OD with OS were not statistically significantly different in aniridia (P = 0.287) and WAGR syndrome (P = 0.526) subjects. ^†Intragroup comparisons of better-seeing eye with worse-seeing eye were statistically significantly different for aniridia subjects (P = 0.022) but not for WAGR spectrum subjects (P = 0.439).

Figure 1 Scatter plots for LogMAR values in aniridia and WAGR spectrum subjects. (A) Values for both eyes in aniridia (N=50) and WAGR spectrum (N=50) subjects. (B) Values for the better-seeing eye in aniridia (N=25) and WAGR spectrum (N=25) subjects. (C) Values for the worse-seeing eye in aniridia (N=25) and WAGR spectrum (N=25) subjects. Heavy horizontal lines indicate the mean and lighter horizontal lines indicate the standard deviation for each group. The analyses of all eyes and better-seeing eyes comparing aniridia and WAGR spectrum subjects were statistically significantly different in both analyses.

Abbreviations: BCVA, best-corrected visual acuity; WAGR, WAGR spectrum (syndrome) subjects.

Table 3 Average Visual Function Thresholds in Aniridia and WAGR Spectrum Subjects

	Aniridia	WAGR Spectrum
Mean LogMAR, better eye, mean ± SD	0.78 ± 0.15	1.40 ± 0.88
Mean Snellen vision, better eye	20/125	20/500
Visual impairment*	Moderate	Profound
Legal blindness†	No	Yes
Read wall chart in office**	Yes	No
Visual efficiency, both eyes‡	34%	2%

Notes: *WHO criteria for visual impairment: moderate is 20/70-20/160, severe is 20/200 or worse, profound is 20/500-20/1000.^{14 †}American Academy of Ophthalmology (AAO) criteria for legal blindness: 20/200 or worse (and/or 20 degrees or less of VF).¹⁵ **Assumption that the wall chart is a Snellen Chart with range of 20/10 to 20/400. ^‡Lower visual efficiency corresponds to lower acuity of both eyes. Thresholds for disability vary from state to state in the US, generally ranging from 20 to 60%. Visual efficiency less than 20% would likely be considered a severe vision disability.¹⁶

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