Table 1 of D’Souza, Mol Vis 2013; 19:2209-2216.


Table 1. Clinical presentation of XLRS patient #645 and patient #22 with retinal schisis.

Clinical criteria Patient #645 Patient #22
Age (yrs) when data recorded 7 21
Age (yrs) when symptoms noticed 7 5
BCVA-OD, OS 20/800, 20/50 20/50+1, 20/40–1
Central visual field defect present (both OD and OS) Yes. OD Yes. Both OD and OS
Foveal/macular cystic changes (both OD and OS) Yes. Both OD and OS Yes. Both OD and OS
Macular atrophy (both OD and OS) No No
Peripheral visual field defect present (both OD and OS) Yes. Inferonasally OD and OS No
Peripheral schisis (both OD and OS) Yes, peripheral flat schisis superiorly and inferotemporally OS>OD. No
Retinal detachment present or treated (both OD and OS) No No
Inheritance Pattern based on family history Only the proband is affected Only the proband is affected
ERG Dark-adapted combined/mixed response wave values- OD Not performed a wave=237,
b wave=312(μV)*
ERG Dark-adapted combined/mixed response wave values- OS Not performed a wave=240,
b wave=318(μV)*