Retinitis Pigmentosa
Short Summary

41-year-old female noticed some decrease in her vision and was diagnosed with Retinitis Pigmentosa. The progression of her disease is slow but visual fields showed contraction and loss of peripheral vision in both eyes.

Patient's Questions
While she is aware that Retinitis Pigmentosa is incurable, the patient has heard of a surgery performed in Cuba that blocks the progression of the disease.
The attending ophthalmologist is against the surgery, but the patient requests additional information about related risks and real benefits.
Alternatively, she would like to know if other treatments exist that have been shown to be effective
Medical Background

Age: 41, Sex: F

Diagnosis: Retinitis Pigmentosa
 
Female patient, 41 years old, between the ages of 6 and 30, the patient has had regular opthamoligical tests, and has always worn glasses to correct astigmatism.
In 2003, she noticed a deterioration in her sight and had an ophthalmologic exam, during which she was diagnosed with Retinitis Pigmentosa.
Further tests prescribed by the specialist were done, and they confirmed the diagnosis.
The physician who examines her periodically sustains that the progression of the disease is very slow, and that the patient should consider herself 'lucky' compared with the traditional progression of the disease at this age.
The patient however believes that the reduction in sight is significant.
 
At her last test:
-right eye
9/10; SPH +2.25; CYL +1.25; AXS 79
-left eye
9/10; SPH +2.75; CYL +1.25; AXS 88
Transparent diopters, lively reflexes, bilateral tone 15 mm Hg.
Thin vessels, vitreoretinal interface syndrome greater at left. Equatorial pigmented spicula.
 
Normal intraocular pressure in both eyes.
Visual fields in both eyes showed contraction and loss of peripheral vision in both eyes.

Expert's Opinion

Retinitis Pigmentosa is a degenerative disease that significantly and progressively affects the visual function. The rate of progression of the disease can be very variable among different individuals. At the present time, there are no effective treatment regimens for this condition.

 
There have been reports that large daily doses of Vitamin A supplementation (15,000 international units per day) may retard somewhat the deterioration of the electroretinographic abnormalities in certain types of Retinitis Pigmentosa. The results of this study were published in the following article: Berson El et al, Archives of Ophthalmology 111:761-772, 1992.
 
If the patient is interested in this type of treatment, I would urge her to take it under the guidance of her family physician, as these are very large doses of this vitamin. This type of treatment has side effects such as increased risk of lung malignancies and reduced calcium intake and osteoporosis. Furthermore, this type of treatment may not be sufficient in all kinds of Retinitis Pigmentosa.
 
I would strongly urge the patient to see a specialist in retinal degenerative disorders for more detailed evaluation of her condition. There are a number of genetic abnormalities that have been reported to cause this condition and knowledge of the exact genetic abnormality may provide better information about her visual prognosis.
 
For further evaluation, counselling and follow up, I would recommend Dr Bennedetto Falsini, a world renowned specialist in Retinitis Pigmentosa who practises in Rome, Italy.
 
I am aware of ocular surgery that is being performed in Cuba where an attempt is made to bring a new source of blood circulation to the choroidal circulation. The choroids are the vasculature that nourishes the outer part of the retina, the area that degenerates in Retinitis Pigmentosa.
 
I have not seen any serious scientific evaluation of the results of this type of surgery. Therefore, I cannot advise whether this surgery may improve her vision. I would probably rather discourage her from pursuing this avenue of therapy. I am myself an expert on the area of choroidal circulation and I have my doubts that this type of procedure may improve the circulation to the choroid.

 

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