Eye Info

Stargardt's Macular Dystrophy

Summary: Designed to help you understand more about your eye condition, this guide has been written by our experienced eye health team.

What is Stargardt Macular Dystrophy?

Stargardt's Macular Dystrophy is an inherited condition that affects the macula; an area of the central retina. Conditions involving the macula affect central vision. Although there may be considerable sight loss, in some cases to levels where registration would be offered, total loss of sight is rare.

Stargardt's Macular Dystrophy was described in 1909 by Karl Stargardt, a German ophthalmologist, from whom the disorder derives its name. It is the most frequently encountered juvenile onset form of the group of conditions known as “macular dystrophies”.

What is Fundus Flavimaculatus?

Where the eyes show yellow-white spots and flecks of various shapes, and when this feature is accompanied by minimal changes in the macula, Fundus Flavimaculatus will often be used to describe the condition. This term was first coined in 1963 by the ophthalmologist Franceschetti.

Fundus Flavimaculatus is more often associated with later onset and a better prognosis.

What is the link between Stargardt Macular Dystrophy and Fundus Flavimaculatus?

In 1976 Hadden and Gass identified Stargardt's and Fundus Flavimaculatus as manifestations of the same genetic disorder and for all general purposes this view is accepted.

What are the effects of Stargardt's and Fundus Flavimaculatus?

Decreased central vision is usually the first symptom of Stargardt's Macular Dystrophy and visual loss is progressive over a period of years, sometimes to a level of 6/60. Diagnosis is often confirmed by observing retinal changes and by two tests: (i) Fluorescein angiography, which entails the injection of fluorescein into the arm whilst photography of the eye is being carried out and (ii) Electro-oculography which entails application of electrodes to the eye lids whilst the patient looks to left and right.

As a general rule, the condition is first identified after 6 and before 20 years of age but some people may not be aware of any sight problem until they are 30 or even 40 years of age.

As with other macular problems, colour vision is affected but many people do not notice loss of colour vision in the early stages. Most people do not suffer loss of peripheral vision nor do they experience night blindness.

Are there any genetic implications, risk groups and risk factors?

Stargardt's Macular Dystrophy and Fundus Flavimaculatus are usually autosomal recessive, hereditary conditions. Inheritance is from both parents who are carriers but are not themselves affected.

Males and females are affected equally.

As with all genetic problems, it is wise to seek advice from a genetic counsellor in order to identify implications for family members. Your GP or other medical advisor will be able to advise you about genetic counselling.

Is there any treatment?

RNIB is not aware of any effective treatment currently available to control these conditions and until the cause is fully understood, treatment is unlikely to be identified.

The gene responsible for Stargardt’s has recently been discovered and it is hoped that it will lead to some form of testing or treatment in the future.

Other sources of support

Advice and training in making maximum use of peripheral vision will enable sufferers to access useful function and thus improve visual ability. For these reasons it is important to seek referral to a Low Vision Service. The Partially Sighted Society can advise on low vision clinics and services. Contact them at

1. The Partially Sighted Society
7 - 9 Bennetthorpe
Doncaster
South Yorkshire
DN2 6AA
Telephone 0844 477 4966
Fax 0844 477 4969

Advice on low vision aids and techniques is also available from the Hospital Eye Service, which will offer hands-on training, and a loan service of appropriate equipment. More than one appliance is usually required to cover the range of individual needs such as reading, shopping, travelling, close and distant work.

2. LOOK
Queen Alexandra College
Court Oak Rd
Harbourne
Birmingham
B17 9TG
Telephone 0121 428 5038

LOOK is an organisation, which seeks to identify sight-related support groups and put parents in contact with relevant services.

3. Macular Disease Society
Darwin House
13a Bridge St
Andover
Hampshire
SP10 1BE
Telephone 0845 241 2041

Macular Disease Society has a helpline and information service. Contact the Co-ordinator on 0990 14 35 73.

4. Stargardt International (and Juvenile Macular Dystrophies), formed in 1992, is based in West Chicago, USA and offers a self-help network, education, information and referral, conferences and support groups for persons and their families with Stargardt and allied juvenile macular dystrophies. Their address is

Macular Degeneration International
PO Box 136
West Chicago Illinois 60186
USA

This address is shared with Macular Degeneration International, an organisation addressing special lifestyle needs of individuals as well as supporting research. Macular Degeneration is a condition which mostly affects people aged 60 and over.

What's next?

Our Adapting to sight loss page gives information useful for people with a newly diagnosed sight problem.

If you need more information contact our Helpline by email helpline@rnib.org.uk or phone 0845 766 9999 / 020 7388 2525 (some callers may find it cheaper to call a landline, so we have detailed both 08 prefixed numbers and landline equivalents where available).

RNIB does not comment on every trend or development but, when a treatment becomes established / recognised, we revise our website material to include it.

Unfortunately, RNIB can only answer email enquiries from the UK. But you may find an organisation for people with poor sight in your country through our agencies database.

Back to eye conditions

Content author: eyehealth@rnib.org.uk

Last updated: 06/03/2008 15:41

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