a 21-year - old male presented with a progressive decrease in vision that had begun at the age of 8 years . the patient 's best corrected visual acuity , with + 4.00 ds / -0.50 dc  180 , was counting fingers at 50 cm in the right eye , and , with + 1.00 ds / -1.50 dc  20 , was 6 / 24 in the left eye . indirect ophthalmoscopy revealed multiple vitreous membranes , peripheral vitreous detachment , peripheral retinal epithelial alterations , pigment clumping , equatorial chorioretinal atrophy inferior to the macula , lamellar macular holes , and foveal microcystic spaces in the patient 's right eye . time domain ( stratus ) optical coherence tomography demonstrated confluent macular cystoid changes , as well as foveal retinoschisis in both eyes , with macular holes in the right eye . during electroretinogram evaluation , isolated rod responses were unrecordable for the right eye , and showed delayed implicit time with grossly reduced amplitude in the left eye . there was a gross delay in implicit time in both eyes with 30 hz flicker . fundus fluorescein angiography ( ffa ) revealed window defects corresponding to areas of atrophy of the retinal pigment epithelium ( rpe ) . cataract formation was noted in his right eye . he had developed a bicycle wheel pattern of foveal schisis in his left eye . imaging with spectral domain optical coherence tomography ( sd - oct ; copernicus , optopol technologies , zawierci , poland ) of the right eye revealed lamellar macular holes with macular schisis , microcystic spaces , and vitreo - macular traction . sd - oct imaging of the left eye revealed cystoid macular edema with inner layer schisis . foveal thickness was 77 microns in the right eye and 672 microns in the left eye . microperimetry ( nidek technologies , padova , italy ) showed a reduced mean retinal sensitivity of 0.0 db in the right eye and 5.5 db in the left eye , with a central dense scotoma in both eyes ( fig . the chromosomes were stained by giemsa - trypsin banding and scanned using ikaros software ( metasystems , altltussheim , germany ) . the pedigree showed an autosomal recessive inheritance pattern with one additional affected male in the family . twenty - five plates , which were screened for the proband , revealed normal karyotypes . however , genetic analysis could not be performed . a 41-year - old male presented with decreased vision , haloes , and visual distortion that he had been experiencing for the past 13 years . the patient 's best corrected visual acuity , with + 0.50 ds / -4.50 dc 100 , was 6 / 36 in the right eye , and , with + 0.50 ds / -4.00 dc 80 , was 6 / 15 in the left eye . indirect ophthalmoscopy revealed vitreous floaters , macular schisis , and diffuse rpe alterations in both eyes . also revealed was a peripheral hole in the inferotemporal quadrant in the left eye . the total error score for the farnsworth test was 36 in the right eye and 19 in the left eye . the test results revealed tritanomaly in the right eye and diffuse color defect in the left eye . sd - oct imaging of both eyes showed incomplete posterior vitreous detachment , elevated foveal contours , and foveal schisis . foveal thickness was 292 microns in the right eye and 490 microns in the left eye . microperimetry showed a reduced mean retinal sensitivity of 7.7 db in the right eye and 8.2 db in the left eye , with a central dense scotoma in both eyes ( fig . 2 ) . a 36-year - old male presented with decreased vision , haloes , and visual distortion that he had been experiencing for the past 4 years . ds / -0.50 dc  90 , was 6 / 12 in the right eye , and , with + 5.00 ds / -1.00 dc  90 , was 6 / 24 in the left eye . indirect ophthalmoscopy revealed vitreous floaters , macular schisis , diffuse rpe alterations , and peripheral schisis in both eyes . the patient 's total error score for the farnsworth d-15 color vision test was 28 in the right eye and 31 in the left eye . the test results revealed tritanomaly in the right eye and diffuse color defect in the left eye . foveal thickness was 136 microns in the right eye and 171 microns in the left eye . microperimetry showed a reduced mean retinal sensitivity of 1.0 db in the right eye and 0.2 db in the left eye , with a central dense scotoma in both eyes ( fig . a 21-year - old male presented with a progressive decrease in vision that had begun at the age of 8 years . the patient 's best corrected visual acuity , with + 4.00 ds / -0.50 dc  180 , was counting fingers at 50 cm in the right eye , and , with + 1.00 ds / -1.50 dc  20 , was 6 / 24 in the left eye . indirect ophthalmoscopy revealed multiple vitreous membranes , peripheral vitreous detachment , peripheral retinal epithelial alterations , pigment clumping , equatorial chorioretinal atrophy inferior to the macula , lamellar macular holes , and foveal microcystic spaces in the patient 's right eye . time domain ( stratus ) optical coherence tomography demonstrated confluent macular cystoid changes , as well as foveal retinoschisis in both eyes , with macular holes in the right eye . during electroretinogram evaluation , isolated rod responses were unrecordable for the right eye , and showed delayed implicit time with grossly reduced amplitude in the left eye . there was a gross delay in implicit time in both eyes with 30 hz flicker . fundus fluorescein angiography ( ffa ) revealed window defects corresponding to areas of atrophy of the retinal pigment epithelium ( rpe ) . cataract formation was noted in his right eye . he had developed a bicycle wheel pattern of foveal schisis in his left eye . imaging with spectral domain optical coherence tomography ( sd - oct ; copernicus , optopol technologies , zawierci , poland ) of the right eye revealed lamellar macular holes with macular schisis , microcystic spaces , and vitreo - macular traction . sd - oct imaging of the left eye revealed cystoid macular edema with inner layer schisis . foveal thickness was 77 microns in the right eye and 672 microns in the left eye . microperimetry ( nidek technologies , padova , italy ) showed a reduced mean retinal sensitivity of 0.0 db in the right eye and 5.5 db in the left eye , with a central dense scotoma in both eyes ( fig . the chromosomes were stained by giemsa - trypsin banding and scanned using ikaros software ( metasystems , altltussheim , germany ) . the pedigree showed an autosomal recessive inheritance pattern with one additional affected male in the family . twenty - five plates , which were screened for the proband , revealed normal karyotypes . a 41-year - old male presented with decreased vision , haloes , and visual distortion that he had been experiencing for the past 13 years . the patient 's best corrected visual acuity , with + 0.50 ds / -4.50 dc 100 , was 6 / 36 in the right eye , and , with + 0.50 ds / -4.00 dc 80 , was 6 / 15 in the left eye . indirect ophthalmoscopy revealed vitreous floaters , macular schisis , and diffuse rpe alterations in both eyes . also revealed was a peripheral hole in the inferotemporal quadrant in the left eye . the total error score for the farnsworth test was 36 in the right eye and 19 in the left eye . the test results revealed tritanomaly in the right eye and diffuse color defect in the left eye . sd - oct imaging of both eyes showed incomplete posterior vitreous detachment , elevated foveal contours , and foveal schisis . foveal thickness was 292 microns in the right eye and 490 microns in the left eye . microperimetry showed a reduced mean retinal sensitivity of 7.7 db in the right eye and 8.2 db in the left eye , with a central dense scotoma in both eyes ( fig . a 36-year - old male presented with decreased vision , haloes , and visual distortion that he had been experiencing for the past 4 years . the patient 's best corrected visual acuity , with + 4.50 ds / -0.50 dc  90 , was 6 / 12 in the right eye , and , with + 5.00 ds / -1.00 dc  90 , was 6 / 24 in the left eye . indirect ophthalmoscopy revealed vitreous floaters , macular schisis , diffuse rpe alterations , and peripheral schisis in both eyes . the patient 's total error score for the farnsworth d-15 color vision test was 28 in the right eye and 31 in the left eye . the test results revealed tritanomaly in the right eye and diffuse color defect in the left eye . foveal thickness was 136 microns in the right eye and 171 microns in the left eye . microperimetry showed a reduced mean retinal sensitivity of 1.0 db in the right eye and 0.2 db in the left eye , with a central dense scotoma in both eyes ( fig . differential diagnosis includes conditions such as retinitis pigmentosa ( rp ) , stickler 's syndrome , and familiar exudative vitreo - retinopathy . rp is differentiated from goldmann - favre syndrome on the basis of vascular attenuation and electroretinogram . cystoid macular oedema associated with rp is a potentially treatable condition , and can be distinguished from retinoschisis by oct and ffa . ocular management of goldmann - favre syndrome includes scatter photocoagulation of peripheral avascular lesions , as well as vitrectomy for clearance of vitreous hemorrhage and relief of retinal traction if present . genetic counseling is also offered to patients and potential carriers . because the syndrome is associated with mutations in arg311gln nr2e3 in the 15q23 chromosome , it is a potential target for gene therapy . case 1 followed the temporal sequence of events over a period of 18 months in a patient diagnosed with goldmann - favre syndrome . the patients in cases 2 and 3 were clinically diagnosed with goldmann - favre syndrome . we are unaware of previous reports about goldmann - favre syndrome that use sd - oct and microperimetry for analysis . the findings vary with the age of the patient and the severity of the disease . findings from sd - oct imaging included lamellar macular holes , macular schisis , microcystic spaces , alterations of photoreceptor layers , epiretinal membrane with vitreomacular traction , enhanced foveal thickness , and elevated foveal contours . microperimetry revealed reduced foveal sensitivity with dense scotomas . in this series of cases , retinoschisis and macular cystoid changes noted with advanced diagnostic techniques such as sd - oct and microperimetry detect new characteristics , as well as confirm correlations among known features , to elevate our knowledge of the disease .