Table 1

Summary of general ocular findings

Age seenCycloplegic refractionVAColour visionAxial length (mm)Lens scleral ultrasound VEPERG
17 yearsR +16.00DS
L +14.5/0.75DC
R 6/24
L 6/24
normalR 15.9
L 15.3
Lens 4.4 mm, Sclera 2 mm R + LPattern VEP of normal  latency though  marginally smaller  than averageNormal mixed  rod/cone response
25 years R +14.5DS
L +13.25 DS
R 6/18
L 6/18
normalR 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
33 years R +16.0DS
L +16DS/0.5DC
R 6/18
L 6/18
normalNANAPattern VEP of normal  latency, marginally  smaller than average Normal mixed  rod/cone response.
48 months R +17DS
L +15.5DS
CSM CSMNANANAPattern VEP of normal  latency, amplitudes  less than lab average Normal mixed  rod/cone response
55 years R +16.25DS
L +17.0/1.5DC
R 6/36
L 6/24
normalR 15.2
L 15.4
Sclera 2.1 mm
R + L
68 yearsR +14.5DS
L +14.5DS
R 6/60
L 6/36
NAR 12
L 12
Sclera 2.2 mm
R + L
73 years R +17.0DS
L +17.0DS
R 6/60
L 6/60
  • 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.