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Retinitis pigmentosa

RP; Vision loss - RP; Night vision loss - RP; Rod Cone dystrophy; Peripheral vision loss - RP

 

Retinitis pigmentosa is an eye disease in which there is damage to the retina. The retina is the layer of tissue at the back of the inner eye. This layer converts light images to nerve signals and sends them to the brain.

Causes

 

Retinitis pigmentosa can run in families. The disorder can be caused by several genetic defects.

The cells controlling night vision (rods) are most likely to be affected. However, in some cases, retinal cone cells are damaged the most. The main sign of the disease is the presence of dark deposits in the retina.

The main risk factor is a family history of retinitis pigmentosa. It is a rare condition affecting about 1 in 4,000 people in the United States.

 

Symptoms

 

Symptoms often first appear in childhood. However, severe vision problems do not often develop before early adulthood.

  • Decreased vision at night or in low light. Early signs may include having a harder time moving around in the dark.
  • Loss of side (peripheral) vision, causing "tunnel vision."
  • Loss of central vision (in advanced cases). This will affect the ability to read.

 

Exams and Tests

 

Tests to evaluate the retina:

  • Color vision
  • Exam of the retina by ophthalmoscopy after the pupils have been dilated
  • Fluorescein angiography
  • Intraocular pressure
  • Measurement of the electrical activity in the retina (electroretinogram)
  • Pupil reflex response
  • Refraction test
  • Retinal photography
  • Side vision test (visual field test)
  • Slit lamp examination
  • Visual acuity

 

Treatment

 

There is no effective treatment for this condition. Wearing sunglasses to protect the retina from ultraviolet light may help preserve vision.

Some studies suggest that treatment with antioxidants (such as high doses of vitamin A palmitate) may slow the disease. However, taking high doses of vitamin A can cause serious liver problems. The benefit of treatment has to be weighed against risks to the liver.

Clinical trials are in progress to assess new treatments for retinitis pigmentosa, including the use of DHA, which is an omega-3 fatty acid.

Other treatments, such as microchip implants into the retina that act like a microscopic video camera, are in the early stages of development. These treatments may be useful for treating blindness associated with RP and other serious eye conditions.

A vision specialist can help you adapt to vision loss. Make regular visits to an eye care specialist, who can detect cataracts or retinal swelling. Both of these problems can be treated.

 

Outlook (Prognosis)

 

The disorder will continue to progress slowly. Complete blindness is uncommon.

 

Possible Complications

 

Peripheral and central loss of vision will occur over time.

People with retinitis pigmentosa often develop cataracts at an early age. They may also develop swelling of the retina (macular edema). Cataracts can be removed if they contribute to vision loss.

 

When to Contact a Medical Professional

 

Contact your health care provider if you have problems with night vision or you develop other symptoms of this disorder.

 

Prevention

 

Genetic counseling and testing may help determine whether your children are at risk for this disease.

 

 

References

Berson EL. Retinitis pigmentosa and allied retinal diseases. In: Tasman W, Jaeger EA, eds. Duane's Clinical Ophthalmology. 2013 ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2013:vol 3, chap 24.

Cukras CA, Xein WM, Caruso RC, Sieving PA. Progressive and 'stationary' inherited retinal degenerations. In: Yanoff M, Duker JS, eds. Ophthalmology. 4th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 6.13.

National Eye Institute (NEI). Facts about retinitis pigmentosa.Updated May 2014. nei.nih.gov/health/pigmentosa/pigmentosa_facts. Accessed April 13, 2016.

Olitsky SE, Hug D, Plummer LS, Stahl ED, et al. Disorders of the retina and vitreous. In: Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 630.

Stingl K, Bartz-Schmidt KU, Besch D, et al. Artificial vision with wirelessly powered subretinal electronic implant alpha-IMS. Proc Biol Sci. 2013;280(1757):1471-2954. PMID: 23427175 www.ncbi.nlm.nih.gov/pubmed/23427175.

Yanoff M, Cameron D. Diseases of the visual system. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 423.

 
  • Eye

    Eye - illustration

    The eye is the organ of sight, a nearly spherical hollow globe filled with fluids (humors). The outer layer or tunic (sclera, or white, and cornea) is fibrous and protective. The middle tunic layer (choroid, ciliary body and the iris) is vascular. The innermost layer (the retina) is nervous or sensory. The fluids in the eye are divided by the lens into the vitreous humor (behind the lens) and the aqueous humor (in front of the lens). The lens itself is flexible and suspended by ligaments which allow it to change shape to focus light on the retina, which is composed of sensory neurons.

    Eye

    illustration

  • Slit-lamp exam

    Slit-lamp exam - illustration

    A slit-lamp, which is a specialized magnifying microscope, is used to examine the structures of the eye (including the cornea, iris, vitreous, and retina). The slit-lamp is used to examine, treat (with a laser), and photograph (with a camera) the eye.

    Slit-lamp exam

    illustration

    • Eye

      Eye - illustration

      The eye is the organ of sight, a nearly spherical hollow globe filled with fluids (humors). The outer layer or tunic (sclera, or white, and cornea) is fibrous and protective. The middle tunic layer (choroid, ciliary body and the iris) is vascular. The innermost layer (the retina) is nervous or sensory. The fluids in the eye are divided by the lens into the vitreous humor (behind the lens) and the aqueous humor (in front of the lens). The lens itself is flexible and suspended by ligaments which allow it to change shape to focus light on the retina, which is composed of sensory neurons.

      Eye

      illustration

    • Slit-lamp exam

      Slit-lamp exam - illustration

      A slit-lamp, which is a specialized magnifying microscope, is used to examine the structures of the eye (including the cornea, iris, vitreous, and retina). The slit-lamp is used to examine, treat (with a laser), and photograph (with a camera) the eye.

      Slit-lamp exam

      illustration

    A Closer Look

     

      Tests for Retinitis pigmentosa

       

         

        Review Date: 3/15/2016

        Reviewed By: Franklin W. Lusby, MD, ophthalmologist, Lusby Vision Institute, La Jolla, CA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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