Table 1
Summary of general ocular findings
| Age seen | Cycloplegic refraction | VA | Colour vision | Axial length (mm) | Lens scleral ultrasound | VEP | ERG | |
|---|---|---|---|---|---|---|---|---|
| 1 | 7 years | R +16.00DS L +14.5/0.75DC |
R 6/24 L 6/24 |
normal | R 15.9 L 15.3 |
Lens 4.4 mm, Sclera 2 mm R + L | Pattern VEP of normal latency though marginally smaller than average | Normal mixed rod/cone response |
| 2 | 5 years | R +14.5DS L +13.25 DS |
R 6/18 L 6/18 |
normal | R 15.2 L 15.5 |
Lens 4.2 mm, Sclera 1.4 mm R + L |
Pattern VEP normal latency slightly small amplitude | Normal mixed rod/cone response |
| 3 | 3 years | R +16.0DS L +16DS/0.5DC |
R 6/18 L 6/18 |
normal | NA | NA | Pattern VEP of normal latency, marginally smaller than average | Normal mixed rod/cone response. |
| 4 | 8 months | R +17DS L +15.5DS |
CSM CSM | NA | NA | NA | Pattern VEP of normal latency, amplitudes less than lab average | Normal mixed rod/cone response |
| 5 | 5 years | R +16.25DS L +17.0/1.5DC |
R 6/36 L 6/24 |
normal | R 15.2 L 15.4 |
Sclera 2.1 mm R + L |
NA | NA |
| 6 | 8 years | R +14.5DS L +14.5DS |
R 6/60 L 6/36 |
NA | R 12 L 12 |
Sclera 2.2 mm R + L |
NA | NA |
| 7 | 3 years | R +17.0DS L +17.0DS |
R 6/60 L 6/60 |
NA | NA | NA | NA | NA |
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In all cases the intraocular pressure was normal. All cases also exhibited anterior segments with small corneas and shallow anterior chambers but normal appearing lens size giving rise to a very crowded appearance.
