Corneal involvement in congenital aniridia

Area of Science:

• Ophthalmology
• Corneal Diseases

Background:

• Congenital aniridia is a rare genetic disorder characterized by the absence of the iris.
• Patients with aniridia are prone to developing various ocular complications, including keratopathy.

Purpose of the Study:

• To investigate the prevalence and severity of keratopathy in patients with congenital aniridia.
• To determine the correlation between keratopathy and factors such as age, intraocular surgery, and corneal sensitivity.

Main Methods:

• A comprehensive search identified 181 patients with aniridia in Sweden and Norway; 124 were clinically examined.
• Ophthalmologic examinations, including slit lamp assessment and medical history review, were performed to evaluate keratopathy.
• Eyes with artificial implants or transplants were excluded from the analysis.

Main Results:

• Visible keratopathy was present in 80% of examined eyes, with 26% experiencing visual impairment.
• A significant correlation was found between keratopathy severity and patient age.
• Intraocular surgery and reduced corneal sensitivity were significantly correlated with increased keratopathy, independent of age.

Conclusions:

• Congenital aniridia is strongly associated with keratopathy, affecting 80% of patients and impairing vision in 26%.
• Keratopathy progression is linked to advancing age, intraocular surgical procedures, and diminished corneal sensitivity.
• Careful patient selection for intraocular surgery is crucial to mitigate the risk of triggering or exacerbating keratopathy.