A century ago, Theodor Axenfeld described a series of patients with congenital abnormalities of the anterior segment of the eye, including anterior displacement of Schwalbe’s line, termed posterior embryotoxon, with anterior iris adhesions.
Shortly thereafter, Herwigh Rieger noted more iris abnormalities such as corectopia, polycoria and iris atrophy in these patients along with systemic deformities of teeth and facial bones.
Due to the dysgenesis of the anterior segment, the eye is prone to more pathology such as development of glaucoma due to irregular angle anatomy. Fortunately,