Posters

Bilateral congenital sclerocornea associated with polymalformative syndrome

Poster Details

First Author: F.Boudguigue MOROCCO

Co Author(s): A. Ridallah   Y. Boubnan   K. Madbouhi   N. Boutimzine   W. Cherkaoui        

Abstract Details

Purpose:

Congénital sclerocornea belongs to the group of congénital corneal opacifications. it is a non-progressive, non-inflammatory lésion, peripheral, central or sectoral. It's a genetic pathology that occurs sporadically or according to an autosomal dominant transmission.

Setting:

département A of ophtalmology, hospital of spécialité in rabat

Methods:

A 5-year-old child consults in the Ophthalmology Department for bilateral blindness since birth. He is Child of a non-consanguineous marriage and the second of a sibship of 2. The pregnancy and childbirth were normal. In her antecedents, her mother has a unilateral congenital sclerocornea.

Results:

The examination of the chamber segment was narrow in both eyes. His visual acuity was reduced to a bilateral light perception. Ocular tone was normal in both eyes. Slit lamp examination showed complete opacification of both corneas, more important on the left eye where corneal neovascularization was also found. The fundus was inaccessible. The echography showed a normal posterior segment and axial length. Pediatric examination has be done, in search of associated malformations and revealated a tetralogy of Fallot,velopharynx lesions, growth hormone deficiency and scoliosis.

Conclusions:

Congenital sclerocornea is a rare disease. Screening for abnormal varieties of cleavage of the chamber anterior is essential before any therapeutic management. The Transfixant keratoplasty is the treatment of choice.

Financial Disclosure:

None