Retinitis pigmentosa in children   

The group of retinal disorders responsible for gradual vision loss is called retinitis pigmentosa. Child Specialist in Lahore diagnoses retinitis pigmentosa when children complain of decreased peripheral vision or inability to see properly in the dark. Read on to know more about retinitis pigmentosa:

What is retinitis pigmentosa?

Retinitis pigmentosa can present in all age groups with progression loss of vision, but commonly presents in teenage. This inherited group of disorders affects the light sensitive or sensory layer of the eye called retina, and contains photoreceptor cells which convert incoming light energy into electrical signals. These signals are thereafter carried to the brain and interpreted as images.

With retinitis pigmentosa, there is degeneration of the photoreceptors of the retina. Photoreceptors are of two types: rods and cones. Of these, the rods are present in the peripheral or outer layer of the retina and are responsible for vision in low light. Conversely, the cones are present in the central portion of the retina and give more detailed images with color perception.

In retinitis pigmentosa, there are degenerative change in the rods, initially, and this is followed by deterioration of the cones. This process of degenerative changes progresses slowly and can lead to total visual loss.

In the United States alone, there are close to 0.1 million diagnosed cases of retinitis pigmentosa. However, it is rare for people to lose complete vision. With time, these adolescents meet the definition of legally blind in their adulthood and can retain central vision and vision for reading.

How does retinitis pigmentosa present?

The symptoms of retinitis pigmentosa include:

  • Inability to see at night
  • Tunnel vision, with loss of peripheral vision
  • Photophobia and inability to see bright lights

How is retinitis pigmentosa diagnosed?

The diagnosis of retinitis pigmentosa is made through the following investigations:

  • Visual field exam: in this exam, the peripheral or side vision of the patient is tested. This is performed in the doctor’s office with the help of a machine. The patient looks through a machine at a point in the center of the vision. Thereafter, objects appear at the sides and the patient presses a button when they see the object. The machine then makes a map of how much the patient can see. With retinitis pigmentosa, there is loss of peripheral vision but retention of central vision.
  • Fundoscopy: the pupil of the patient is dilated or expanded with the help of eye drops, and using a handheld device called direct ophthalmoscope, the retina of the patient is viewed by the healthcare provider. During this exam the optic disc and fundus of the patient are examined. In retinitis pigmentosa, there are specific spots that appear on the retina.
  • Genetic testing: in this test, the DNA sample is sent for investigation to diagnose the form of retinitis pigmentosa.  
  • Electroretinogram: in this examination, there is placement of a special contact lens on the eye which measures how the retina responds to the flashes of light.   

What are the treatment options?

There is presently, no cure for retinitis pigmentosa. There are, however, some options that can slow the progression of vision loss but nothing can reverse the visual damage. These treatment options include:

  • Acetazolamide: this drug helps to reduce a condition called macular edema, in which there is swelling of a tiny area of the retina. With use of acetazolamide, the excessive fluid is pushed out of the macula and the swelling eases. This helps to improve the vision.
  • Retinal implant: this treatment is available in the US, and provides partial sight. The implant is called argus II, and it is put in one eye alone. With camera-equipped-glasses this implant helps to convert images to electric impulses which are sent to the retina.  
  • Sunglasses: UV radiation can speed up vision loss, and can be prevented with glasses.
  • Vitamin A supplements: high doses of vitamin A slow down the progression of retinitis pigmentosa. However, too much vitamin A can be toxic, therefore only doses recommended by Islamabad Specialists Clinic must be consumed.