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Retinitis pigmentosa

Retinitis pigmentosa is a rare inherited disease which affects the photoreceptors of the retina. These cells (rods and cones) absorb the light energy and transmit the signal to other retinal cells, until the signal reaches special parts of the brain.

This disease affects mostly one type of photoreceptors, which are called rods and are activated at night and at mesopic conditions (low light conditions). Some gene's mutation leads to gradual death of the photosensitive cells and thereby cause loss of the peripheral visual field initially and later on loss of central vision also.

We can distinguish retinitis pigmentosa in two subcategories:

• Type 1: Night blindness early (children at the age of 10), due to rods cells loss and as a result, it leads to peripheral vision loss.
• Type 2: Night blindness appears at the adulthood and is characterized by loss of rods as well as cones sensitivity.

Retinitis Pigmentosa symptoms

At initial stages, retinitis pigmentosa causes nyctalopia (also called night blindness), which is represented as difficulty in low light conditions, since early age. A common symptom is the loss of the peripheral vision, due to destruction of retinal rods. Rods are located mostly in the peripheral retina, so there is a narrowing of the visual fiend. The patients describe this condition as seeing through a tube.

Later, the disease may affect also the other type of photoreceptors, which are called cones. These cells are located mostly in the central retina, in the macula lutea and are responsible for central and color vision. In most cases, patients retain for a long time adequate central vision. However, there is always a big risk of the cones destruction, resulting in reduction of visual acuity.

Retinitis Pigmentosa prevalence in population

The prevalence of the disease in the general population is very rare. In particular, it is reported that there is 1 patient for each 3000 or 4000 persons. The severity of the disease depends on how is inherited to the patient. It can be inherited by autosomal dominant, recessive, as well as X-linked. In case of recessive inheritance the disease progress is faster and the prognosis worse.

Retinitis Pigmentosa Treatment

Unfortunately, retinitis pigmentosa cannot be compensated with glass prescription or contact lenses. There is no certain management of restoring visual field. However, the recognition of genes and their mutations, which lead to the disease gives us hope that retinitis pigmentosa can soon be coped with effectiveness. Nevertheless, there are some studies which show that the disease progression can be slowed down by takin high doses of vitamin A in combination with omega-3 fatty acids. In addition, patients can be benefit from taking a large amount of oxygen to reduce the phenomenon of hypoxia (lower amounts of oxygen) that the disease provokes.

In any case, the optometrist and ophthalmologist should be very precise on the appropriate prescription of any refractive condition the patient may have and give ergonomic advice on how using the appropriate light conditions. Moreover, low vision aids can help patients with tunnel vision.

Surgically, there are many cases in which retinitis pigmentosa has been associated with cataract. Thus, the removal of the dense crystalline lens can improve the low vision that the patient have. The surgeon should be very careful to avoid the appearance of cystoid macular edema which may happen postoperatively.

Success Rates

Retinal detachment
Final restoration → 98,7%!

Μacular hole, final
restoration → 100%!

Epiretinal Membrane → Final Restoration 100%!
Lameral Hole → research in progress, results will be presented soon

 

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