Axenfeld-rieger syndrome (posterior embryotoxon; axenfeld syndrome)

General: Dominant inheritance; occasionally sporadic; variable in expression.

Ocular: Posterior embryotoxon: ringli 727e41h ke opacity of cornea; long trabecula; prominent Schwalbe line; iris adhesions to Schwalbe line and cornea with large abnormal iris processes or broad sheets of tissues of varying size and location; anterior layer of iris may appear hypoplastic; ectopia of the pupil not uncommon; polycoria occurs; ringlike opacity of the deep corneal layers extending several millimeters from the limbus in continuity with the sclera; keratoconus.

Alward WLM. Axenfeld-Rieger syndrome in the age of molecular genetics. Am J Ophthalmol 2000; 130: 107-

Axenfeld T. Embryotoxon corneae posterius. Berl Dtsch Ophth Ges 1920; 42:301.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.

Nemet P, et al. Spontaneous filtering blebs in Axenfeld syndrome. Am J Ophthalmol 1973; 76:590.