Topographical Retinal Function in Oculocutaneou Albinim

Purpoe Macular hypoplaia i the major ocular finding in albinim becaue it underlie reduced acuity and congenital nytagmu. Anatomic tudie have hown a lack of foveal differentiation. However, functional anatomy of the macula in albinim i not known. Deign Obervational cae report. Method We meaured the topology of the retinal repone in two patient with oculocutaneou albinim by uing imultaneou fundu monitoring and multifocal electroretinograph (ERG). timuli conited of a 103-hexagon array centered on the macula. Recording condition eliminated nytagmu artifact. Reult ERG amplitude were reduced only in the central 5 to 10 degree compared with adult norm. When the repone wa caled by timulation area, multifocal ERG amplitude were contant acro all retinal eccentricitie. Concluion The reult ugget a homogeneou denity of cone photoreceptor acro the central retina in albinim, conitent with anatomic tudie howing arret of potnatal macular development. Further normative data are needed to fully quantify macular development in young children.