Priyadarshini Cholera 1 , Ashwattha Shetty 2 , Nita shanbhag 3 , Taran Wanage 4
ABSTRACT: Axenfeld-Rieger syndrome is an uncommon autosomal dominant condition characterized by anterior segment dysgenesis and systemic abnormalities. We report a case of Axenfeld Rieger syndrome associated with secondary developmental glaucoma who remained undiagnosed since childhood. Twenty-one year old male patient presented with progressive dimness of vision and coloured halos. Slit lamp examination showed scleral thinning and megalocornea in both the eyes and iris hypoplasia in the right eye only. Posterior embryotoxon, few tissue bands, high insertion of the iris roots and anterior synechiae were seen on gonioscopy. Intraocular pressure was 32mmHg in the right eye and 38mmHg in the left eye. Glaucomatous disc changes were observed in both eyes on fundus examination. Systemic features such as maxillary hypoplasia, oligodontia, microdontia, taurodontism, micrognathia and redundant periumbilical skin were observed.
