Retinitis Pigmentosa

Question 1

What is retinitis pigmentosa?

Answer 1

Genetic condition involving degeneration of mostly rods but also cones

Question 2

Signs and symptoms of retinitis pigmentosa

Answer 2

Symptoms:
- night blindness as 1st sx
- peripheral vision loss and eventually central vision loss

Signs:
- Fundoscopy shows bone-spicule pigmentation around mid peripheral area of retina, arterioles narrowing, or pale/waxy optic disc

Question 3

What diseases are associated with retinitis pigmentosa?

Answer 3

Usher syndrome
Bassen kornzweig syndrome
Refsum disease

Question 4

Management of retinitis pigmentosa?

Answer 4

Opthalmology referral
Genetic counselling
Vision aids
Sunglasses to protect retina from further damage
Driving limitations and informing DVLA