Table 1. The clinical features of aniridia patients in two unrelated Tunisian families.
Families |
F1
|
F2
|
|||||
---|---|---|---|---|---|---|---|
Patients | II 8 | III 11 | III 12 | II 8 | III 12 | III 13 | III 14 |
Gender | F | F | M | M | F | F | F |
Age (years) | 56 | 11 | 14 | 39 | 11 | 9 | 9 |
CVA
| |||||||
R | 1/20 | 1/10 | 1/10 | 1/10 | 1/10 | 1/10 | NA |
L | 1/20 | 1.5/10 | 1.5/10 | 1.5/10 | 1/10 | 1/10 | NA |
Nystagmus | Congenital | Congenital | Congenital | Congenital | Congenital | Congenital | Congenital |
Cataract | Congenital bilateral | Congenital bilateral subcapsular type | Congenital bilateral subcapsular type | Congenial unilateral nuclear and cortical progressive type (R) | Congenital bilateral anterior pyramidal type | Congenital bilateral | Congenital bilateral |
Aniridia | Complete | Complete | Complete | Partial (L) | Complete | Complete | Complete |
Muscle tone | N | N | N | N | N | N | NA |
OCT | N | Foveal hypoplasia | Foveal hypoplasia | N | N | N | NA |
Gonioscopy | N | N | N | N | N | N | NA |
Other features | Corneal dystrophy | — | — | Implant of the posterior chamber R: crystalline lens and iris coloboma | Remnants of pupillary membrane | L: decrease in foveal reflection R: sclerocornea | — |
Abbreviations: CVA, corrected visual acuity; F, female; L, left eye; M, male; OCT, optical coherence tomography; N, normal; NA, not available; R, right eye.