Axonfeld-Riegel syndrome

Axenfeld-Rieger syndrome (Axenfeld-Rieger syndrome) refers to a group of developmental diseases with or without systemic abnormalities in the eyes, which are characterized by: ① developmental defects in the eyes; ② May be accompanied by systemic developmental abnormalities; ③ Secondary glaucoma; ④ Autosomal dominant inheritance, family history and reports of sporadic cases; ⑤ Same incidence in men and women. In 1920, Axenfeld reported that a patient had a raised white line near the limbus, and an organized band extending from the peripheral part of the iris to this white line. Axenfeld calls it the Posterior embryotoxon of the cornea. In the mid-1930s, Rieger reported that a group of patients had the same anterior segment abnormalities, but there were other changes on the iris, such as: pupil ectopic, iris atrophy, and hole formation. Some of these patients are also accompanied by dysplasia outside the eyes, especially dysplasia of teeth and facial bones.

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