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Abstract #119944 Published in IGR 25-1
Glaucoma in PAX6-related congenital aniridia: A review of the literature
Bastelica P; Daruich A; Paganelli B; Labbé A; Baudouin C; Bremond-Gignac DJournal Français d'Ophtalmologie 2024; 48: 104300
PAX6-related congenital aniridia is a genetic pan-ocular disease characterized by a partial or total absence of the iris and foveal hypoplasia. The mechanisms involved in the development of ocular hypertension and glaucoma in patients with congenital aniridia are still unknown. Many hypotheses have been proposed and the advent of new anterior segment imaging techniques has allowed the identification of various potential mechanisms: congenital trabecular dysfunction, progressive closure of the iridocorneal angle, postoperative ocular hypertension. The diagnosis must take into account the various obstacles to clinical examination (corneal opacity, obturating cataract, foveolar aplasia, significant nystagmus) and is often considered only upon detection of ocular hypertension. Glaucoma remains, along with limbal insufficiency, one of the major causes of blindness in congenital aniridia. The treatment of glaucoma in congenital aniridia is primarily medical. The benefit/risk ratio of a surgical intervention should always be thoroughly evaluated in order to not underestimate the postoperative complications associated with congenital aniridia.
Service 3, hôpital national de la vision des Quinze-Vingts, IHU FOReSIGHT, 28, rue de Charenton, 75012 Paris, France; Institut de la vision, IHU FOReSIGHT, Sorbonne université, 17, rue Moreau, 75012 Paris, France.
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