Posters

Corneal manifestations of congenital ichthyosis: a case report

Poster Details

First Author: S.Homaide MOROCCO

Co Author(s): Z. Cheikh   S. Boussetta   A. Siati   A. Mchachi   L. Benhmidoune   M. Elbelhadji     

Abstract Details

Purpose:

We present a case of congenital  ichthyosis to highlight the ocular features and management of this condition.

Setting:

Ichthyosis is an inherited group of skin disorders characterized by the skin thickening and scale formation.The main ocular associations of congenital ichthyosis are corneal opacities and ectropion of the lids.

Methods:

A 5 year old female child was complaining of a burning sensation, and tearing in both eyes.She had been diagnosed with congenital ichtyosis since her birth. In her ocular examination,the child’s visual acuity was 6/10 in both eyes. Her ocular motility and pupil reflex were normal in both eyes. The external examination revealed diffuse dry skin and cicatricial ectropion in both eyes.  The Biomicroscopy revealed that she had corneal bilateral inferior opacities without any staining of fluorescein . Her fundoscopy was normal in both eyes.she was managed conservatively with lid emollients, and topical eye lubricants,with Improvement of her ocular symptoms.

Results:

The ichthyoses are a group of heterogeneous,mainly hereditary disorders characterised clinically byexcessive scaling of the skin. Ichthyosis maybe congenital or acquired.Primary corneal opacities are found in the x-linkedtype. They do not cause reduced visual acuity but can lead to recurrent corneal erosions.Secondary corneal opacities may occur from exposure caused by ectropion.Ectropion is described only in the lamellar type of ichthyosis.In the early stages of the disease, frequent lubrication of the lids with emollients and keratolytics has been reported to reverse the ectropion and prevent corneal exposure,but In cases where corneal exposure occurs, surgicalintervention is indicated.

Conclusions:

Early detection and timely treatment or referral to specialized ophthalmology centers is crucial to avoid the risk of serious ocular complications of ichthyosis

Financial Disclosure:

None