Retinitis pigmentosa Flashcards
What is retinitis pigmentosa
Congenital inherited condition of degeneration of rods and cones of the retina
Genetic causes of retinitis pigmentosa
Some are isolated retinitis, whereas others rseult in systemic disease associated with the condition
Which degenerate more - rods or cones
Most cases the rods degenerate more than cones, leading to night blindness
Presentation of retinitis pigmentosa
Night blindness is often the first symptoms, and peripheral vision is lost before the central vision
When do most symptoms start
Childhood
What does fundoscopy show
Pigmentation (bone-spicule) looking similar to bone matrix. Concentrated around mid-peripheral area of the retina
What can the optic disc look like in retinitis pigmentosa
waxy or pale appearance
Associated systemic diseases with retinitis pigmentosa
Usher’s syndrome, BAssen-Kornweig syndrome, Refsum’s disease
Management of retinitis pigmentosa
Genetic counselling, vision aids, sunglasses to decrease accerleration of damage, driving limitations, regular follow ups
