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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