RETINITIS PIGMENTOSA
Description
- Retinitis pigmentosa or primary pigmentary retinal dystrophy is a progressive hereditary dystrophy of the retina or of the photoreceptors and the retinal pigment epithelium
- It is a hereditary disorder predominantly affecting the rods more than the cones
- Difficulty with night vision, slow adaptation to the dark, and gradual loss of peripheral vision (tunnel vision) which often begin in childhood, but commonly diagnosed during adolescence with a positive family history is characteristic of RP
Etiology
Retinitis pigmentosa (RP) may occur as Sporadic disorder, isolated without family history due to mutation of multiple genes (>50%) including rhodopsin gene (40%)
Types
- The disease is almost invariably bilateral and both eyes are equally affected
- In typical retinitis pigmentosa, rod-cone dystrophy is seen, in which rods have degenerated early and cones are involved late
Visual symptoms
- Night blindness. It is the characteristic and earliest feature
- Dark adaptation
- Tubular vision-loss of peripheral vision with preservation of central vision
- Central vision is also lost ultimately after many years.
Fundus changes
- Retinal pigmentary changes - jet black spots resembling bone corpuscles in shape.
- Retinal arterioles are attenuated (narrowed).
- The optic disc becomes pale and waxy
Visual field changes
- Annular or ring-shaped scotoma
Investigation
- Visual acuity–Vision decreased associated with Night blindness
- Ophthalmoscopy - Jet black spots resembling bone corpuscles seen
- Visual field- Loss of peripheral vision with preservation of central vision
Treatments
It is most unsatisfactory
- Measures to stop progression - vasodilators, placental Extracts
- Vitamin A (15000 IU, PO, qd of palmitate form) - to check its progression.
- Correct any refractive error
- Low vision aids (LVA) in the form of ‘magnifying glasses’ and ‘night vision device
- Prophylaxis- Genetic counselling for no consanguineous Marriages
Ayurvedic Treatment
- Consider as a Yapya / Krichasadya disease
- Diet- Agastyapatra ( Agatticheera) is found to be beneficial
Internal medicines
- Patoladi gritha
- Triphala gritha
- Rasna Dashamoola gritha
- Sapthamrutha lauha
External procedures
- Snehapana – Rasna dashamoola ghrita
- Virechana – Gandharva eranda
- Jaloukavacharana- eyelid and forehead
- Nasya - Agastya gritha /Agasyapatra swarasa +honey , ksheerabala
- Anjana – Maricha + Dhadi , yakrut pippali
- Tarpana - Jeevaneeya gritha
- Putapaka
Department
Salakya - Netra
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