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  • A developmental abnormality of the anterior uvea that involves multiple congenital ocular anomalies i.e. cornea, anterior chamber angle, lens, retina and optic nerve.
  • Clinical features:
    • Symptoms: markedly decreased visual acuity, photophobia, dry eye symptoms associated with severe ocular surface disease (eg, pain, discomfort, etc)
    • Signs:
      • Total absence of the iris is rare
      • Careful gonioscopic examination usually reveals bilateral rudimentary or hypoplastic iris tissue hidden by the limbal sclera
      • Both open- and closed-angle types of glaucoma can develop: the open-angle variant arises from a dysfunctional or absent aqueous drainage system while the closed-angle form develops secondary to blockage from the hypoplastic stump of peripheral iris tissue
      • Characteristic corneal findings include a superficial grayish haze or opacification with a circumferential corneal epithelial pannus that advances centrally
      • Other related ocular findings include: ptosis secondary to photophobia, lens subluxation, microphakia, cataract, strabismus, nystagmus, amblyopia, optic nerve atrophy or hypoplasia, and foveal hypoplasia
  • Approximately two thirds are familial in origin (autosomal dominant or recessive) and one third are sporadic
  • Recognition of aniridia at an early age is important due to the visual development significance as well as its high association with other ocular and systemic findings
  • Management:
    • Initial medical therapy to control intraocular pressure can be quite difficult since a significant number of patients develop refractory glaucoma
    • Glaucoma surgical procedures are frequently necessary in patients who develop refractory glaucoma
    • Treatment of photophobia and nystagmus
    • Cataract surgery should be approached with caution because of accompanying zonular dehiscence and/or lens subluxation
    • Progressive pannus involving the entire cornea may necessitate penetrating keratoplasty
    • Genetic counseling

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