TYPICAL RETINITIS PIGMENTOSA (TYPICAL RP)
DESCRIPTION:Is a genetic disorder of the eyes that causes loss of vision. There are two types of photoreceptors: rods (which are responsible for vision in low light) and cones (which are responsible for color vision and detail in high light). In RP, the photoreceptors progressively lose function.
Symptoms include trouble seeing at night and decreased peripheral vision (side vision). Onset of symptoms is generally gradual. As peripheral vision worsens, people may experience “tunnel vision.
CLINICAL TRIAD OF RP:
In children medications will be given depending on the body weight of the child. Medications will either be given in divided dosages or single dose per day. Given medications are:
OXYNAS improves the blood supply in retina and COMPLINAS prevents further degeneration.
Hence forth, both will aid in improvement of vision and further prevention of vision loss and also will stabilize the vision.
RETINITIS PIGMENTOSA WITH CATARACT
Patients with RP often develop a type of cataract called subcapsular cataracts. When this occurs, the lens becomes cloudy and vision is impaired.
Advice given: In this condition first treatment is given to strengthen the optic nerve and then advised forextraction of the cataract based on the stage of the cataract when necessary. With these medications retina will be strengthened to give a positive result.
RETINITIS PIGMENTOSA WITH DIABETIC RETINOPATHY
The incidence of diabetic retinopathy (DR) in patients with RP, in which the photoreceptor cells are apoptotic due to mutations of rhodopsin and other proteins, is very interesting from clinical and pathophysiological points of view
In this condition both treatment for DR as well as RP has to be given.
Treatment is given with medications to improve blood supply as well as to treat the diabetic related changes in individual case.
RETINITIS PIGMENTOSA WITH CYSTOID MACULAR EDMEA
RP may be complicated by cystoid macular oedema (CMO), causing a reduction of central vision. The underlying pathogenesis of RP-associated CMO (RP-CMO) remains uncertain, however, several mechanisms have been proposed,
(1) Breakdown of the blood-retinal barrier,
(2) Failure (or dysfunction) of the pumping mechanism in the retinal pigment epithelial,
(3) Müller cell oedema and dysfunction,
(4) Antiretinal antibodies and (5) vitreous traction.
RETINITS PIGMENTOSA WITH DEAFNESS – USHER SYNDROME
Retinitspigmentosa with deafness – Usher syndrome: Is a condition characterized by partial or total hearing loss and vision loss that worsens over time. The loss of vision is caused by an eye disease called retinitis pigmentosa (RP), which affects the layer of light-sensitive tissue at the back of the eye (the retina).
LMBBS SYNDROME – LAURENCE MOON BARDET–BIEDL SYNDROME
Laurence–Moon Bardet–Biedlsyndrome (LMS) is a rare autosomal recessive genetic disorder associated with retinitis pigmentosa, spastic paraplegia, and mental disabilities.