Retinitis Pigmentosa Flashcards

1
Q

What is retinitis pigmentosa?

A

Congenital inherited condition where there is degeneration of the rods and cones in the retina

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2
Q

Cause of retinitis pigmentosa?

A

Genetic
Isolated retinitis pigmentosa
Others result in systemic diseases

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3
Q

Which retinal cells degenerate more?

A

Rods more than cones
|
This leads to night blindness & decreased central & peripheral vision

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4
Q

Presentation of retinitis pigmentosa?

A

Varies on underlying causes

  • Night blindness (often 1st symptom)
  • Peripheral vision lost before central vision
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5
Q

Investigation of retinitis pigmentosa?

A
Fundoscopy: 
Will show- 
-Pigmentation 
-Associated narrowing or arterioles 
-Waxy/pale appearance of optic disc
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6
Q

Associated systemic disease with retinitis pigmnetosa?

A

Usher’s syndrome
Bassen-Kornzwerg syndrome
Refsum’s disease

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7
Q

What does Usher’s syndrome cause?

A

Hearing loss + retinitis pigmentosa

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8
Q

General management of Retinitis Pigmentosa?

A
  • Refer
  • Genetic counselling
  • Vision aids
  • Driving limitations
  • Informing DVLA
  • Regular follow ups
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9
Q

Possible management options to consider for retinitis pigmentosa?

A
  • Vitamin antioxidant supplements
  • Oral acetazolamide
  • Topical dorzolamide
  • Steroid injections
  • Anti-VEGF injections
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