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Retinitis Pigmentosa

Richard W. Allinson, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • An eye disease in which progressive damage to the retina occurs with gradual loss of peripheral vision that eventually leads to significant visual impairment; signs often seen in chi...

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 15,000 IU/day (retinal degeneration slowed, as measured by ERG/visual fields); β-carotene...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Caution should be exercised because of reduced peripheral vision and poor night vision. 

Patient Monitoring

  • Ophthalmic examinations every 1 to 2 years

  • Check for comp...

REFERENCES

1
Sujirakul T, Lin MK, Duong J, et al. Multimodal imaging of central retinal disease progression in a 2-year mean follow-up of retinitis pigmentosa. Am J Ophthalmol.  2015;160(4):786.e4...

ADDITIONAL READING

Berson EL, Rosner B, Sandberg MA, et al. ω-3 intake and visual acuity in patients with retinitis pigmentosa receiving vitamin A. Arch Ophthalmol.  2012;130(6):707–711. 

CODES

ICD10

H35.52 Pigmentary retinal dystrophy 

ICD9

362.74 Pigmentary retinal dystrophy 

SNOMED

  • 28835009 Retinitis pigmentosa (disorder)

  • 232054005 X-linked retinitis pigmentosa (disorder)

  • 232052009 Autosom...

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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