Rieger anomaly

This code represents a rare, inherited developmental disorder affecting the anterior segment of the eye, characterized by malformations of the iris, cornea, and trabecular meshwork. It is often associated with systemic abnormalities, particularly dental and umbilical defects, and has a high risk of developing secondary glaucoma.

Assign this code for patients diagnosed with Rieger anomaly, a specific form of anterior segment dysgenesis. Documentation should clearly state the diagnosis of Rieger anomaly, often identified through ophthalmologic examination detailing iris hypoplasia, corectopia, or posterior embryotoxon.

• Differentiate from Axenfeld anomaly, which is a milder form without systemic involvement.
• Consider additional codes for associated conditions like glaucoma (H40.1x) or systemic manifestations.
• Ensure the medical record specifies "Rieger anomaly" rather than a more general anterior segment dysgenesis.