Management of congenital cataract associated with Axenfeld Rieger Anomaly

Ashal Kaisar Pal, Rao Muhammad Arif Khan, Abdul Qayyum


Anterior Segment Dysgenesis(ASD) represents  a spectrum of developmental disease involving neural crest mesenchyme1.Axenfeld anomaly refers to presence of posterior embryotoxon (anteriorly displaced Schwalbe’s line) with attached iris strands1.When these peripheral abnormalities are associated with iris changes such as corectopia or atrophy it is called Rieger’s anomaly1.In association with systemic anomalies  such as abnormal teeth  and facial abnormalities ,particularly hypertelorism, it is referred to as Rieger syndrome1.Families with Axenfeld Rieger anomaly show an autosomal dominant pattern of inheritance1.Gene loci have been mapped to 4q25(PITX2 gene),6p25(FKHL7) and  13q14(RIEG2)2.AxenfeldRieger anomaly has no gender predilection and there is associated glaucoma2.Cataract is not an association with it. We report a case of anterior segment dysgenesis with associated congenital cataract but no congenital glaucoma, managed by lens aspiration, primary posterior capsulotomy and deep anterior vitrectomy. To our knowledge no case of congenital cataract with associated AxenfeldRieger anomaly is reported, so this is probably the first case report.