Abstract
Abstract: :
Purpose: To investigate the clinical correlation between electroretinograms (ERG) and optical coherence tomography (OCT) and fundus appearance in patients with X–linked recessive retinoschisis (XLRS). Methods: We studied ERGs from 118 eyes (65 patients) and examined 20 eyes (11 patients) using OCT. The fundus appearance was classified based on the involvement of peripheral retinoschisis: group 1, macular abnormality without peripheral schisis; group 2, macular abnormality and typical ballooning peripheral retinoschisis; and group 3 macular abnormality and extensive peripheral retinoschisis with loss of retinal blood vessels and/or diffuse pigmentation. We used age–matched normal control data to compare ERG data among patients. Results: We recorded a– and b–waves in 118 eyes of all patients, photopic b–waves in 71 eyes, scotopic b–waves in 86 eyes, and photopic flicker in 80 eyes. The amplitude of b–wave and b/a ratio recorded with bright flashes under dark adaptation was below normal in all patients (group 1, 52 eyes; group 2, 65 eyes; and group 3, 1 eye). The mean amplitudes in groups 1 and 2, respectively, were: a waves, 90.942±20.597 and 77.215±21.088%; b wave, 51.750±18.166 and 38.388±14.473%; photopic b–waves, 32.452±22.993 and 26.341±17.676%; scotopic b–wave, 33.622±16.927 and 18,062±14.857%; and photopic flicker, 60.306±24.199 and 38.860±24.390%. The mean b/a ratios were 0.696±0.151 and 0.630±0.193, respectively. All recordings in 1 eye in group 3 were nonrecordable. OCT in 20 eyes (11 patients) showed splitting of the neurosensory retina in the macula in 18 eyes (10 patients). Two eyes (1 patient) had no retinoschisis due to retinal atrophy. Six eyes in group 1 had splitting of the retina beyond the macula where no retinoschisis was detectable by ophthalmologically. Conclusions: Patients with XLRS have widespread ocular dysfunction in which only the macula is ophthalmologically involved (group 1). The ERG is more depressed in group 2 as compared with group 1, but the difference is not great. Majorities stayed in the same group in the follow–up for 10 years. There is rarely diffuse pigmentation with complete loss of sight (group 3).
Keywords: electroretinography: clinical • retina • imaging/image analysis: clinical