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Corneal lattice dystrophy

Poster Details


First Author: J.Chaakouk MOROCCO

Co Author(s): J. Chaakouk   Z. Filali   M. El Mhadi   H. Boui   A. Bouzidi   S. Iferkhas     

Abstract Details

Purpose:

Corneal lattice dystrophy, also known as "Biber-Haab-Dimmer dystrophy", in its type I variant, is a stromal dystrophy characterized by predominant inter-linear linear opacities in the anterior stroma. It is a bilateral corneal dystrophy, inherited from autosomal dominant inheritance.

Setting:

we report a case of a 32 year-old patient we received in our formation for progressive visual decrease.

Methods:

• We received in consultation a 32-year-old patient, with no known defects, with a family history of low vision, who presented for a progressive decrease in visual acuity in both eyes.  • The examination found visual acuity limited to 07/10 in the right eye and 04/10 in the left eye. Examination of the anterior segment objective at both eyes a network of  filamentous opacities bound in the cornea with decreased corneal sensitivity. • the attack is more marked on the left.

Results:

• Type I corneal lattice dystrophy belongs to the group of amyloid corneal dystrophies, caused by mutation of the BIGH-3 gene. It is transmitted in an autosomal dominant fashion. Bilateral and symmetrical, it appears at an early age, manifested by recurrent erosions and a progressive decline in visual acuity. In the typical form, the cornea takes a yellowish color and is crossed by crystalline lines that can form a true toasting.  • Histologically, stromal anastomotic cracked oval deposits exist in the stroma that separate the stromal lamellae. These deposits may result in the disappearance and rupture of Bowman's membrane.

Conclusions:

• Type 1 muscular dystrophy is a rare entity. Although his clinical diagnosis is easy, the risk of his recurrence after treatment remains high.

Financial Disclosure:

None

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