This chapter provides a background on several retinal dystrophies – retinitis pigmentosa, Stargardt disease, Best’s vitelliform dystrophy, and pattern dystrophy – and surveys each condition’s principal microperimetric features. Diagnostic devices discussed include the Nidek MP-1 (Nidek Technologies; Padova, Italy) and the OPKO optical coherence tomography/scanning laser ophthalmoscope microperimetry (OPKO Health, Miami, FL). Retinal dystrophies often strike at a young age and cause increasing visual loss with time. Therefore, monitoring disease progression and improvement with modalities, such as microperimetry, that can integrate evaluation of structural integrity and functional status will be essential in determining the efficacy of future therapies and directing treatment. Obstacles to optimal imaging include unstable fixation, which is often seen in patients with retinal dystrophies, as well as the lack of perfect correlation of maps created by various imaging modalities. Microperimetry is a useful method for detecting functional decline and may be an integral accompaniment to anatomical evaluation of patients with inherited retinal dystrophies. Additionally, it offers a unique tool for the detection of subtle functional vision changes and will be especially useful in the evaluation of newly developed experimental therapies that aim to slow the progression of structural and functional visual loss.
ASJC Scopus subject areas