Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

all patients

1st line – assessment of refractive ability ± visual aids

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ACUTE
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all patients
1st line – 

assessment of refractive ability ± visual aids

An assessment with a low-vision consultant (ophthalmologist or optometrist) is recommended to accurately determine and optimise visual ability. Visual aids such as glasses, magnifiers, or telescopes may be helpful.

Consider – docosahexaenoic acid (DHA)

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ACUTE
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all patients
Consider – 

docosahexaenoic acid (DHA)

Additional treatment recommended for SOME patients in selected patient group

DHA is an omega-3 fatty acid and key component of fish oils. It is present in high concentrations in the photoreceptors and may be a precursor for neuroprotective factors.[8]

Three randomised studies in patients with retinitis pigmentosa did not show a significant benefit, but many centres still recommend supplementation due to the low risk and potential benefit.[44][45][46][47]

Consider – lutein

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ACUTE
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all patients
Consider – 

lutein

Additional treatment recommended for SOME patients in selected patient group

One randomised controlled trial examined the efficacy of lutein (a dietary carotenoid, found in the human retina and dark green leafy vegetables) to slow visual field loss in patients with retinitis pigmentosa who were taking vitamin A.[48] The study showed a reduction in the loss of mid-peripheral visual fields.[48] However, others have challenged the conclusions of this study.[49]

Consider – surgery

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ACUTE
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all patients
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with cataracts
Consider – 

surgery

Additional treatment recommended for SOME patients in selected patient group

Cataract extraction can benefit many patients, especially if the degeneration has not involved the central macula. It is important to rule out the presence of cystoid macular oedema before cataract extraction because this can worsen after surgery. Occult weak zonules require appropriate surgical precautions to minimise the risks of complications during cataract surgery.

Consider – carbonic anhydrase inhibitor

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ACUTE
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all patients
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with cystoid macular oedema
Consider – 

carbonic anhydrase inhibitor

Additional treatment recommended for SOME patients in selected patient group

Carbonic anhydrase inhibitors such as topical dorzolamide or oral acetazolamide are effective at treating cystoid macular oedema in some patients. May need several months of treatment before an effect is seen.[50][51]

Effects can wear off with time, and some patients do not benefit. Furthermore, many patients cannot tolerate the adverse effects of these drugs such as paraesthesias and frequent urination.

Primary options

dorzolamide ophthalmic: (2%) 1 drop into the affected eye(s) three times daily

OR

acetazolamide: 500 mg orally (extended-release) once daily initially, adjust dose according to response

Consider – voretigene neparvovec

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ACUTE
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all patients
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with biallelic RPE65 mutations
Consider – 

voretigene neparvovec

Additional treatment recommended for SOME patients in selected patient group

Voretigene neparvovec, an adeno-associated virus vector carrying a normal copy of the RPE65 gene, has been shown to improve functional vision in patients with biallelic RPE65-mutation associated retinal dystrophy, and is approved for treatment in this patient group.[58] It is administered as a subretinal injection. Studies have also demonstrated a consistent safety profile and longer-term efficacy.[59][60]​ ​Patients must have sufficient viable retinal cells to be considered for this treatment.[58]

Primary options

voretigene neparvovec subretinal: consult specialist for guidance on dose

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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