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Bilateral congenital aniridia: 3 case reports

Poster Details


First Author: B.Yousra MOROCCO

Co Author(s): A. Ridallah   F. Boudguigue   K. Madbouhi   N. Boutimzine   O. Cherkaoui        

Abstract Details

Purpose:

Aniridia is a rare genetic disorder, by mutation of the Pax6 gene. Aniridia is an eye disorder characterized by a complete or partial absence of the iris. It can be isolated or associated with systemic abnormalities. Our purpose is to report 3 cases of familial congenital aniridia.

Setting:

Department A of ophtalmology, Mohammed V University, Faculty of medicine, Rabat, Morocco

Methods:

40-year-old women who complained of visual loss and exotropia of the right eye presented bilateral congenital aniridia, corneal dystrophy with vascular pannus and Bilateral congenital cataract. Her 18-year-old also presented visual loss, exotropia of the left eye, bilateral congenital aniridia associated with congenital dislocated cataract and ocular hypertension. The other daughter 18 months had nystagmus with bilateral congenital aniridia and congenital lens dislocation.

Results:

The Optical Coherence Tomography document foveal hypoplasia. The renal ultrasound doesn’t find abnormalities in this family. Our patients was scheduled for a lensectomy without intraocular implantation and the aphakia was corrected by glasses.

Conclusions:

Aniridia can present several clinical aspects as demonstrated by our observation. It is a disorder affecting not only the iris but also cornea, angle structures, lens, optic nerve and fovea. The management of this disease is difficult. In fact, the delay of diagnosis in our regions, promotes amblyopia installation, and the surgical treatment becomes challenging

Financial Disclosure:

None

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