AXENFELD RIEGER SYNDROME: A RARE CONGENITAL ANOMALY

Dr. Sejal Katoch, Dr. Mayur Garkar, Dr. Gaurav Sharma and Dr. Bijoyata Reang*

ABSTRACT

The story of Axenfeld-Rieger syndrome (ARS) was first reported in 1920 when German ophthalmologist Dr. Theodor Axenfeld described prominent and displaced Schwalbe's line (posterior embryotoxon) along with adhesions between the iris and corneal endothelium, a condition he termed Axenfeld anomaly.[1] In 1935, Dr. Albert Rieger expanded the understanding of this disorder by identifying more severe ocular features, including iris abnormalities such as hypoplasia, corectopia (pupil displacement), and polycoria (multiple pupils), which he named Rieger anomaly. Systemic manifestations such as dental anomalies, facial bone abnormalities, umbilical defects, hypospadias, and pituitary dysfunction, collectively are referred to as Rieger syndrome. Over time, the overlapping features of Axenfeld anomaly and Rieger anomaly were recognized as part of a broader spectrum, leading to the adoption of the term "Axenfeld-Rieger syndrome" to encompass both ocular and systemic manifestations.[2]

Keywords: .