Korean J Ophthalmol Vol.26, No.2, 2012 and 0.2 db in the left eye, with a central dense scotoma in both eyes (Fig. 3). Discussion Goldmann-Favre syndrome is characterized by foveal and/or peripheral retinoschisis. Differential diagnosis in- cludes conditions such as retinitis pigmentosa (RP), Stick- ler’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 poten- tially treatable condition, and can be distinguished from retinoschisis by OCT and FFA. Ocular management of Goldmann-Favre syndrome includes scatter photocoagula- tion 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 as- sociated with mutations in Arg311Gln NR2E3 in the 15q23 chromosome, it is a potential target for gene therapy [7]. Case 1 followed the temporal sequence of events over a period of 18 months in a patient diagnosed with Gold- mann-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 analy- sis. 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, micro- cystic 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 SD-OCT matched the scotomas shown with microperimetry. Advanced diagnostic techniques such as SD-OCT and microperimetry detect new char- acteristics, as well as confirm correlations among known features, to elevate our knowledge of the disease. Conflict of Interest No potential conf lict of interest relevant to this article was reported. References 1. Fishman GA, Jampol LM, Goldberg MF. Diagnostic fea- tures of the Favre-Goldmann syndrome. Br J Ophthalmol 1976;60:345-53. 2. Ikaheimo K, Tuppurainen K, Mantyjarvi M. Clinical features of Goldmann-Favre syndrome. Acta Ophthalmol Scand 1999;77:459-61. 3. Khairallah M, Ladjimi A, Ben Yahia S, et al. Elevated macular retinoschisis associated with Goldmann-Favre syndrome successfully treated with grid laser photocoagu- lation. Retina 2002;22:234-7. 4. Theodossiadis PG, Koutsandrea C, Kollia AC, Theodos- siadis GP. Optical coherence tomography in the study of the Goldmann-Favre syndrome. Am J Ophthalmol 2000;129:542-4. 5. Chavala SH, Sari A, Lewis H, et al. An Arg311Gln NR2E3 mutation in a family with classic Goldmann-Favre syn- drome. Br J Ophthalmol 2005;89:1065-6. 6. Vingrys AJ, King-Smith PE. A quantitative scoring tech- nique for panel tests of color vision. Invest Ophthalmol Vis Sci 1988;29:50-63. 7. Haider NB, Jacobson SG, Cideciyan AV, et al. Mutation of a nuclear receptor gene, NR2E3, causes enhanced S cone syndrome, a disorder of retinal cell fate. Nat Genet 2000;24:127-31. 146