A Natural History of the Progression of Stargardt Disease: Retrospective and Prospective Studies

Inclusion Criteria:

- Provide a signed informed consent form and authorization allowing the disclosure and
use of protected health information.

- The designated primary study eye must have at least one well-demarcated area of
atrophy as imaged by fundus autofluorescence with a minimum diameter of 300 microns
and all lesions together must add to less than or equal to 12 mm2 (equivalent to no
more than 5 disc areas in a least one eye) and a BCVA of 20 ETDRS letters (20/400
Snellen equivalent) or better.

- Two (2) pathogenic mutations confirmed present, in the ABCA4 gene. If only one ABCA4
allele contains a pathogenic mutation, the patient shall have a typical Stargardt
phenotype, namely at least one eye must have flecks at the level of the retinal
pigment epithelium typical for STGD.

- The primary study eye must have clear ocular media and adequate pupillary dilation to
permit good quality FAF and sd-OCT imaging in the opinion of the investigator.

- Be able to cooperate in performing the examinations.

- Be willing to undergo ocular examinations once every 6 months for up to 24 months.

- Be at least six years old.

- Both eyes can be included if inclusion criteria are fulfilled for both eyes.

Exclusion Criteria:

- Ocular disease, such as choroidal neovascularization, glaucoma and diabetic
retinopathy, in either eye that may confound assessment of the retina morphologically
and functionally.

- Intraocular surgery in the primary study eye within 90 days prior to baseline visit.

- Current or previous participation in an interventional study to treat STGD such as
gene therapy or stem cell therapy. Current participation in a drug trial or previous
participation in a drug trial within six months before enrollment. The use of oral
supplements of vitamins and minerals are permitted although the current use of Vitamin
A supplementation shall be documented.

- The site Principal Investigator may declare any patient at their site ineligible to
participate in the study for a sound medical reason prior to the patient's enrollment
into the study.

- Any systemic disease with a limited survival prognosis (e.g. cancer, severe/unstable
cardiovascular disease).

- Any condition that would interfere with the patient attending their regular follow-up
visits every 6 months for up to 24 months, e.g. personality disorder, use of major
tranquilizers such as Haldol or Phenothiazine, chronic alcoholism, Alzheimer's Disease
or drug abuse.

- Evidence of significant uncontrolled concomitant diseases such as cardiovascular,
neurological, pulmonary, renal, hepatic, endocrine or gastro-intestinal disorders.