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# Retinitis pigmentosa

An inherited disease of the [retina](https://durgical.com/en/anatomy/retina/) 's rods: night blindness first, then a slowly narrowing field. Rehabilitation and genetic counselling help.

Updated Nov 5, 2026

On this page

- The basics What it is
- Who gets it
- Causes
- Risk factors
- Pathophysiology
- Why it happens
- In ophthalmology

- Clinical Symptoms and signs
- Red flags
- Diagnosis
- Differential diagnosis
- Complications
- Prognosis

- Treatment Goal of treatment
- Treatment
- Prevention

- Study Test yourself

- Connections Connections

## What it is

A group of inherited retinal dystrophies in which rod photoreceptors die first and cones later. It causes night blindness in youth, a progressive loss of peripheral field (tunnel vision) and, late, loss of central vision. It can be isolated or part of a syndrome such as Usher syndrome (with deafness).

See it in 3D

- [Retina](https://durgical.com/en/anatomy/retina/)
- [Optic disc](https://durgical.com/en/anatomy/optic-disc/)
- [Macula](https://durgical.com/en/anatomy/macula/)
- [Lens](https://durgical.com/en/anatomy/lens/)

## Who gets it

The most common inherited retinal dystrophy; symptoms usually begin in childhood or early adulthood.

## Causes

- Mutations in one of many genes for photoreceptor or pigment epithelium proteins
- Autosomal dominant, autosomal recessive or X-linked inheritance
- Syndromic forms (Usher, Bardet-Biedl)

## Risk factors

- Family history
- Parental consanguinity

## Pathophysiology

A genetic defect makes the rods degenerate, beginning in the mid-periphery. Dying photoreceptors release pigment that migrates into the retina as bone-spicule clumps; cones then fail and the retinal vessels and [optic nerve](https://durgical.com/en/anatomy/optic-nerve/) atrophy.

## Symptoms and signs

- Poor vision in the dark (night blindness)
- Gradual loss of side vision
- Glare and slow adaptation to light changes
- Late loss of central vision

## Red flags

- Sudden drop in central vision (treatable cataract or macular oedema)
- Hearing loss in a child with night blindness (Usher syndrome)

## Diagnosis

**Clinical**
Fundus shows bone-spicule pigmentation in the mid-periphery, narrowed arterioles and a pale, waxy disc; visual fields show a ring scotoma or tunnel vision.

**Tests**
Electroretinography (reduced or absent responses) and genetic testing.

**Imaging**
OCT and fundus autofluorescence to follow the [macula](https://durgical.com/en/anatomy/macula/) and detect cystoid macular oedema.

## Differential diagnosis

- Vitamin A deficiency
- Drug toxicity (thioridazine, chloroquine)
- Syphilis or other inflammatory retinopathies
- Cancer-associated retinopathy

## Goal of treatment

Preserve remaining vision, treat complications, support rehabilitation and offer genetic counselling.

## Treatment

**Rehabilitation**
Low vision rehabilitation, mobility training and educational support.

**Surgical**
Cataract surgery when [lens](https://durgical.com/en/anatomy/lens/) opacity worsens vision.

**Medical**
Treatment of cystoid macular oedema with carbonic anhydrase inhibitors under ophthalmology.

**Medical**
Gene therapy for some specific mutations in specialised centres.

## Complications

- Tunnel vision and legal blindness
- Posterior subcapsular cataract
- Cystoid macular oedema

## Prognosis

Slowly progressive over decades; many patients keep useful central vision until middle age or later.

## Prevention

- Genetic counselling for affected families

## Test yourself

Think of the answer, then tap the question to see it.

## What is usually the first symptom of retinitis pigmentosa?

Night blindness.

## What are the classic fundus signs?

Bone-spicule pigmentation, narrowed arterioles and a pale, waxy optic disc.

## Which syndrome combines retinitis pigmentosa and deafness?

Usher syndrome.

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## Why it happens

- [Macular degeneration and retinitis pigmentosa](https://durgical.com/en/general-pathology/macular-degeneration-and-retinitis-pigmentosa/)

## In ophthalmology

- [Visual electrophysiology: ERG, EOG and VEP](https://durgical.com/en/ophthalmology/visual-electrophysiology-erg-eog-and-vep/)

## Connections

## Related conditions 2

- [Cataract](https://durgical.com/en/conditions/cataract/)
- [Vitamin A deficiency (night blindness and xerophthalmia)](https://durgical.com/en/conditions/vitamin-a-deficiency/)

Educational information, not medical advice. For any symptom, see a health professional.

Sources: [Retinitis Pigmentosa](https://www.ncbi.nlm.nih.gov/books/NBK519518/) (StatPearls Publishing (NCBI Bookshelf), 2024).

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Representative model, for educational purposes only.

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Source: https://durgical.com/en/conditions/retinitis-pigmentosa/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/conditions/retinitis-pigmentosa/