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Retinitis Pigmentosa

Introduction

Retinitis pigmentosa is a hereditary eye disease where the retinal rods are affected.

Usually it becomes manifest by defective night vision first in childhood or later in early adulthood. The hereditary factor is variably penetrant as it can be autosomal dominant (child has symptoms) or autosomal recessive (symptoms start later in life).

Signs and Symptoms

The initial symptoms are defective night vision, often detected in early childhood. Later a change in vision with a characteristic visual field defect (a so-called”ring scotoma”) becomes evident that can be tested by visual field testing. This becomes eventually so pronounced that there is a loss of peripheral vision to this tunnel vision as shown here.

Diagnostic Tests

Examination by the eye specialist shows the pigmented appearance of the eye background as indicated in this link.

Treatment

Treatment at this time is very restricted. Nevertheless, here is a site that shows attempts of a company to develop a high-tech solution with an implanted microchip  that has already been tested in preliminary trials. However, further testing needs to be done and longterm outcomes and FDA approval have to be awaited.

 Retinitis Pigmentosa

Retinitis Pigmentosa (Tunnel Vision)

 

References:

1. The Merck Manual: Retinitis pigmentosa

2. Ferri: Ferri’s Clinical Advisor: Instant Diagnosis and Treatment, 2004 ed., Copyright © 2004 Mosby, Inc.

3. Rakel: Conn’s Current Therapy 2004, 56th ed., Copyright © 2004 Elsevier

Last modified: December 4, 2016

Disclaimer
This outline is only a teaching aid to patients and should stimulate you to ask the right questions when seeing your doctor. However, the responsibility of treatment stays in the hands of your doctor and you.