Retinitis Pigmentosa

Question 1

What is the pathophysiology of retinitis pigmentosa?

Answer 1

Congenital inherited condition, degeneration of the rods and cones in the retina.

Question 2

Which degenerates more in most genetic causes of retinitis pigmentosa, rods or cones? What does this degeneration cause?

Answer 2

Rods degenerate more than cones, causes night-blindness and decreased central and peripheral vision.

Question 3

What is this a presentation of:

Child with night-blindness, loss of peripheral vision first, then central vision later?

Answer 3

Retinitis pigmentosa

Question 4

What is this fundoscopy examination indicative of:
‘Bone-spicule’ pigmentation (networking appearance), most concentrated around mid-peripheral area, narrow arterioles and pale appearance of optic disc?

Answer 4

Retinitis pigmentosa

Question 5

Which eye condition are these diseases associated with:

1. Usher’s syndrome
2. Bassen-Kornzweig syndrome
3. Refsum’s disease

Answer 5

Retinitis pigmentosa

Question 6

What is the treatment for retinitis pigmentosa?

Answer 6

1. Referral to ophthalmology.
2. Genetic counselling
3. Vision aids and sunglasses to protect retina
4. Regular follow-up
5. Medications

Question 7

What are the medications considered in retinitis pigmentosa?

Answer 7

1. Vitamin and antioxidant supplements
2. Oral acetazolamide
3. Topical dorzolamide
4. Steroid injections
5. Anti-VEGF injections