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Stargardt desease about one case

Poster Details


First Author: M.Bouchaar MOROCCO

Co Author(s): S. Bajjouk   S. Haddougui   F. Elasri   K. Reda   A. Oubaaz        

Abstract Details

Purpose:

Stardardt's disease is a rare hereditary macular dystrophy characterized by progressive bilateral loss of central vision, but with intact peripheral vision , and dyschromatopsia .We also note macular atrophy with or without paramacular  flecks and degeneration of the retinal pigment epithelium. We report a rare case of stargart's disease

Setting:

International University Hospital Sheikh Khalifa Ibn Zaid

Methods:

A 19-year-old patient consults for a progressive visual acuity decline for 3 years. The patient has no significant past, personal, systemic and family history.

Results:

In Ophthalmologic Examination , the best-corrected visual acuity of both eyes was 1/10, with normal anterior  segment  and normal intraocular pressure. The examination of the fundus revealed the existence of a yellowish macular lesion rounded with macular atrophy. The glazed is transparent. Auto fluorescent images describe a granular aspect of maculas with alternating hypoautofluorescent and hyperautofluorescent dots. Fluorescein angiography confirms the presence of macular atrophy accompanied by remodeling of the pigment epithelium. OCT shows bilateral macular atrophy. The electroretinogram is normal. All of these paraclinical examinations led to the conclusion of the diagnosis of Stargardt's disease.

Conclusions:

Stargardt's disease is the most common form of juvenile macular dystrophy with autosomal recessive inheritance. the disease is progressive and bilateral. The diagnosis is based on the interrogation, the fundus and the complementary examinations

Financial Disclosure:

None

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