Aniridia Treatment & Management

Updated: Sep 08, 2017
  • Author: Michael Ross, MD; Chief Editor: Hampton Roy, Sr, MD  more...
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Treatment

Medical Care

Prophylaxis in patients with aniridia is directed toward the prevention of glaucoma, which includes the following:

  • Medical treatment with miotics
  • Surgical separation of the iris from the trabecular meshwork in selected cases

Limbal stem cell deficiency associated with aniridia can be treated with the following:

  • Lubricating drops
  • Topical steroid pulses
  • Vitamin A ointments
  • Autologous serum drops
  • Topical bevacizumab drops have been reported in one study. [18]
  • Limbal stem cell transplantation

The medical treatment of aniridia is directed toward control of intraocular pressure, which includes the topical use of the following:

  • Miotics
  • Beta-blockers
  • Sympathomimetics
  • Carbonic anhydrase inhibitors
  • Prostaglandin analogues

The chances of failure with local antiglaucoma treatment are high.

Treatment of photophobia and nystagmus in patients with aniridia is as follows:

  • Tinted or iris contact lenses
  • Tinted spectacle lenses
  • Tinted intraocular lenses (IOLs) [19]
  • By the above measures, reducing the amplitude and frequency of nystagmus is possible.

Refractive errors are treated with careful refraction and complete correction.

Treatment of amblyopia and strabismus in patients with aniridia is as follows:

  • Usually, the potential visual acuity in both eyes should be symmetrical.
  • When the vision is unequal without structural difference, vigorous amblyopia exercises should be performed in the worst eye.
  • Binocularity can be achieved if macular hypoplasia is not severe.
  • Strabismus surgery is indicated at an early age.
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Surgical Care

Management of corneal opacification in patients with aniridia is as follows:

  • Limbal stem cell transplantation
  • Keratoplasty
  • Keratoprosthesis [20]

Management of cataract in patients with aniridia is as follows:

  • In-the-bag lens implantation in cases without lens dislocation
  • Use of opaque intracapsular rings to produce an artificial pupil or use of a large intraocular lens with a clear central optic and an opaque periphery
  • In-the-bag IOL placement with intracapsular rings, when there is slight lens displacement
  • Lens extraction followed by tinted/artificial pupil scleral-fixated posterior chamber lens
  • Lens extraction followed by contact lens correction, if the lens is grossly out of place

Management of glaucoma in patients with aniridia is as follows:

  • Goniotomy: This includes prophylactic and early surgical therapy. In early surgical therapy, some risk to the crystalline lens and the zonules exists because the surgery is performed from the anterior approach. [21]
  • Trabeculotomy is safer than goniotomy. The tissues can be defined more clearly, and accurate surgery can be performed. However, failures are common.
  • Filtering procedures: Greater danger of injury to the crystalline lens and disturbance of the vitreous exists because the iris is absent. Nonperforating filtration surgery techniques are safer, because the anterior chamber remains undisturbed. Since glaucoma develops in young patients with aniridia, the intraoperative use of mitomycin is justified. Fugo blade can be used to produce a transconjunctival microfiltration track of 200-300 µm in any part of the angle, away from the lens and the vitreous.
  • Laser therapy to angle abnormalities
  • Cyclocryotherapy: Endocyclophotocoagulation in selected cases
  • Glaucoma valve procedure: The choice of the techniques and the order in which they are used depends upon the peculiarities of the case and the perception of the surgeon.
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Consultations

See the list below:

  • Banded chromosome analysis on the patient and both parents
  • Linkage analysis when large families are available
  • Genetic counseling
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