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BOOK TITLE: Retina: Medical and Surgical Management
Retinitis pigmentosa (RP) refers to a group of hereditary, clinically, and genetically diverse disorders of progressive retinal dysfunction characterized by photoreceptor, retinal pigment epithelium, and eventually diffuse retinal atrophy. RP is termed typical RP if it occurs in isolation. In the presence of associated systemic disease, the terminology used is syndromic RP. Electroretinography (ERG) is an important test in the diagnosis and management of patients with RP. Scotopic and combined responses are often severely diminished even when fundus may seem normal. Sectoral retinitis pigmentosa, unilateral retinitis pigmentosa, pericentral retinitis pigmentosa, and retinitis punctata albescens are the variants of RP. While as of now RP is incurable, it is often possible to help patients by providing useful information and support to improve quality of their life. Syndromic retinitis pigmentosa, artificial vision in retinitis pigmentosa, leber congenital amaurosis, and congenital stationary night blindness are also discussed in this chapter.