EXPORT CITATION

Chapter-06 Retinitis Pigmentosa and Allied Disorders

BOOK TITLE: Retina: Medical and Surgical Management

Author
1. Kumar Atul
2. Kumar Vinod
3. Ravani Raghav
4. R Karthikeya
5. Chohan Annu
ISBN
9789352702947
DOI
10.5005/jp/books/18054_7
Edition
1/e
Publishing Year
2018
Pages
11
Author Affiliations
1. Dr RP Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India, All India Institute of Medical Sciences, New Delhi, India, Women’s Health Center; Anil Nursing Home, Jaipur, Rajasthan, India, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India, Jaipur, Rajasthan, India, Dr RP Center for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India, Anil Hospital, Jaipur, India, All India Institute of Medical Sciences New Delhi, India, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, Dr. RP Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
3. Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India, All India Institute of Medical Sciences, New Delhi, India
4. Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India, Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of edical Sciences, New Delhi, India, All India Institute of Medical Sciences, New Delhi, India
5. Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India, All India Institute of Medical Sciences, New Delhi, India
Chapter keywords
Retinitis pigmentosa, RP, sectoral retinitis pigmentosa, Usher syndrome, Bardet-Biedl syndrome, Refsum syndrome, Leber congenital amaurosis

Abstract

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.

© 2019 Jaypee Brothers Medical Publishers (P) LTD.   |   All Rights Reserved