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 |
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.