Rieger anomaly and Axenfeld-Rieger syndrome: a rare anterior segment dysgenesis

Axenfeld Rieger Syndrome is anterior segment dysgenesis characterized by posterior embryotoxon and of the chamber. Developmental abnormalities angle cause increased resistance to outflow. Ocular hypertension a complication in almost 50% cases. It an autosomal dominant disease its prevalence between 50,000 100,000 newborns per year. The main associated affected genes are FOXC1 PITX2, occurring 40% can be with systemic manifestations such as dental anomalies (hypodontia microdontia), facial (maxillary hypoplasia, telecanthus hypertelorism), redundant paraumbilical skin, hypospadia, auditory cardiac alterations. Within this syndrome, we distinguish different phenotypes, being anomaly one least affectation. We present three cases stages disease, which allow us understand development management disorder.