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Retinitis Pigmentosa

Esther Bak, M.D. Reviewed 04/2024
 


BASICS

DESCRIPTION

  • A rod-cone dystrophy of the eye in which progressive damage to the retina occurs with gradual loss of peripheral vision that eventually leads to significant visual impairment. Signs ...

DIAGNOSIS

HISTORY

  • Headache and light flashes are the most common initial complaints.

  • Night blindness (nyctalopia)

  • Progressive visual field loss

  • Central visual acuity is usually preserved until the end sta...

TREATMENT

GENERAL MEASURES

  • Supportive care

  • Genetic counseling

  • Low-vision aids

  • Patient education

MEDICATION

  • Vitamin A palmitate 15,000 IU/day (retinal degeneration slowed, as measured by ERG/visual fields); ...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Ophthalmic examinations every 1 to 2 years

  • Check for complications (e.g., cataracts).

DIET

A diet rich in omega-3 fatty acids (primarily docosahexa...

REFERENCES

1
Berson EL, Weigel-DiFranco C, Rosner B, et al. Association of vitamin A supplementation with disease course in children with retinitis pigmentosa. JAMA Ophthalmol. 2018;136(5):490–495.
2
Ber...

CODES

ICD10

H35.52 Pigmentary retinal dystrophy 

SNOMED

  • 28835009 Retinitis pigmentosa (disorder)

  • 232054005 X-linked retinitis pigmentosa (disorder)

  • 232052009 Autosomal dominant retinitis pigmentosa (disord...

CLINICAL PEARLS

  • Characterized by poor night vision, constricted visual fields, bone spicule-like pigmentation of the fundus, and electroretinographic evidence of photoreceptor cell dysfunction

  • Avoid vi...

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