Aniridia 6
General: Hereditary, recessive (two thirds of cases), can be dominant, sporadic, or traumatic; absence of the iris; rare; usually bilateral unless due to trauma.
Ocular: Absence of iris; subluxed lens; iridodialysis; cataract; glaucoma; corneal scarring, vascularization, and edema; iris colobomata; round eccentric pupils; keratoconus.
Clinical: Cerebellar ataxia; mental retardation; Wilms tumor.
Francois J, et al. Aniridia Wilms' tumor syndrome. Ophthalmologica 1977; 174:35-39.
Johns KJ, O'Day DM. Posterior chamber intraocular lenses after extracapsular extraction in patients with aniridia. Ophthalmology 1991; 98:1698-l702.
Kremer I, et al. Results of penetrating keratoplasty in aniridia. Am J Ophthalmol 1993; 115:317-320.
Magalini SI, Scrascia E. Dictionary
of Medical Syndromes, 2nd ed.
Mintz-Hittner HA, et al. Criteria to detect minimal expressivity within families with autosomal dominant aniridia. Am J Ophthalmol 1992; 114:700-707.
Nelson LB, Spaeth GL, Nowinski TS, et al. Aniridia. A review. Surv Ophthalmol 1984; 28:621-642.
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