CONGENITAL GLAUCOMA IN AXENFELD-RIEGER SYNDROME : A CASE REPORT

Published 2023 - 27th ESCRS Winter Meeting

Reference: P161 | Type: Presented Poster & Poster Submission | DOI: 10.82333/pcn7-ce81

Authors: Boutayna Azarkan* ¹ , salma Moutamani ¹ , Nihal El Arari ¹ , Zineb Hilali ¹ , L.ouafaa Cherkaoui ¹

¹ophthalmology,Hsr,Rabat,Morocco

Purpose

Axenfeld-Rieger syndrome is a genetic disease affecting multiple organ systems. In the eye, this condition manifests with varying degrees of anterior segment dysgenesis and carries a high risk of glaucoma. This case highlights the importance of management of individuals affected by Axenfeld-Rieger syndrome by a multidisciplinary approach and dedicated surveillance of glaucoma, sensorineural hearing loss, as well as other parts of the body : cardiac, endocrinological, craniofacial and orthopaedic abnormalities.

Setting

ARS is the result of abnormal embryonic development, usually diagnosed during infancy. Congenital glaucoma is a serious consequence of ARS, usually developped later in childhood , is often due to increased fluid pressure within the eyeball and can lead to complete blindness if left untreated. Therefore, the main course of treatment is the effective management of glaucoma, medically or surgically.

Methods

We received a 1year old child in the ophthalmological emergency department. The parents comlain about tearing, photophobia and enlargement of the globe appearing since the first months of life.

Results

On clinical examination, the right eye is characterized by elevated intraocular pressure with thinning of the anterior sclera. We had found a buphthalmus, corneal edema with rupture of Descemet’s membrane, posterior embryotoxon, an anomalously deep anterior chamber and atrophy of the iris with multiple pupils, an ectopia lentis with zonular fragility, the posterior segment was inaccessible. The IOP control was achieved with surgery : trabeculectomy with anti-fibrotics. we have acquired a greate success in obtaining IOP control for the follow-up.

Conclusion

Typically, the diagnosis is made in the first year of life. Depending on when treatment is instituted, visual acuity may be reduced and/or visual fields may be restricted. The management of individuals affected by Axenfeld-Rieger syndrome requires a multidisciplinary approach and would include dedicated surveillance of glaucoma.