Rieger anomaly is a developmental disorder of the anterior segment in which Axenfeld-type angle changes are accompanied by more marked iris abnormalities. It lies on the same spectrum as Axenfeld anomaly but with additional structural changes.
Ocular Features
People with Rieger anomaly may have corectopia where the pupil is displaced, holes or splitting in the iris stroma, and more pronounced adhesions between the iris and angle structures. The cornea is often clear, but the drainage angle is abnormal and glaucoma is common.
- Often bilateral with variable asymmetry
- Pupil distortions can be visible on routine inspection
- Angle anomalies resemble those seen in Axenfeld anomaly
- Visual acuity depends on associated defects and glaucoma control
Systemic Associations and Management
When similar eye changes occur with systemic features such as dental, facial, or umbilical abnormalities, the term Axenfeld-Rieger syndrome is used. Management focuses on early detection and treatment of glaucoma and on coordinated care for any extra-ocular manifestations.
- Intraocular pressure monitoring from childhood is essential
- Medical or surgical glaucoma treatments may be required
- Genetic counselling can be helpful for affected families
- Multidisciplinary review addresses systemic findings when present
