Aniridia: causes, symptoms. How to treat

Aniridium Is the absence of the iris of the eye. At a scrupulous inspection from time to time reveal small parts of roots of an iris. Such congenital deficiency may be accompanied by other pathologies in development: microphthalmia, subluxation of the lens, nystagmus. In addition aniridii congenital type accompany: amblyopia, hypermetropia, at times - secondary glaucoma. In addition to an aniridia of the congenital type, there is an acquired form of it.

The cause of this type can serve as a strong blow, while the iris comes off at the base.

Heredity



  • Autosomal dominant origin. It is the most common form of inheritance. The disease has a two-sided nature and is expressed in different intensity.

  • Autosomal recessive origin. Diagnosed less often, according to the symptoms is part of the Gillespie syndrome (Gillespie).


  • Origin, which is shown from case to case. Such aniridia is diagnosed in one case out of three. Sometimes there are cases of finding deletions of 13p. Aniridia may appear along with Wilms' tumor; with mental retardation; violation of the complex of organs of the reproductive and urinary system; with other malformations. Patients suffering from this type of aniridia undergo a thorough examination of the karyotype or fluorescein in situ hybridization. To diagnose uncomplicated forms of deletion, conduct a microscopic genetic study. Always, with an aniridia of a random character, for every 3 months during the first 5 years of life, do abdominal palpation and ultrasound examination to confirm the absence of a Wilms tumor. Sometimes there are isolated cases of general family involvement of this tumor.


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    The process of occurrence of anisidium



    Disease always accompanies deterioration of visual acuity. Patients have to protect their eyes from excessive sunlight.

    Full aniridium

  • Presence of residual parts of the roots of the iris.

  • Anterior polar cataract.

  • Peripheral vascularized anomaly of the cornea.

  • Lens shift.

  • Glaucoma disease.

  • Hypoplasia of the macular zone.

  • Nystagmus.

  • Hypoplasia of the nerve responsible for visual acuity.

  • The magnitude of visual acuity is in the range of 6 /36-3 /60 (005-016).


  • Incomplete kind of disease with
    aniridium.
    It is due to the partial presence of the iris. Symptomatic corresponds to the full form of aniridia, but it does not have such a severe form. The disease can be hardly expressed, as, for example, an easy form of hypoplasia of the iris stroma.

    An aniridium, which is accompanied by other deviations

  • The totality of symptoms characterizing the Gillespie disease, as well as aniridia, together with cerebellar ataxia and mental disability.

  • An aniridia accompanying the absence of the patella.

  • Other combinations.


  • The treatment of aniridium



    Existing medicine is currently able to cure this disease quite successfully by installing an artificial iris made of hydrogel coated with a dye. Such an iris in the center has a 3-millimeter lumen reproducing the pupil. If the patient has a one-sided anerydia, the color of the iris is selected according to the color of the healthy eye.

    Installation of artificial iris is a very difficult operating process. To sew it up, a transscleral surgical method is used in the limb zones located at diametrical distances. In case aniridia is combined with cataract, the latter is removed and a prosthesis is installed, which replaces both the iris and the lens at once.

    Additional information on the treatment of the disease



  • Refraction: often pronounced ametropia is diagnosed.

  • The vision is corrected with glasses or contact lenses.

  • The use of devices for patients with poor eyesight.

  • Creation of educational system.

  • Diagnosis of glaucoma at the initial stages of its occurrence.

  • For the treatment of small children, anesthesia may be necessary.

  • Most of the forms of cataracts together with aniridia are not of functional significance, but after a while visual functions may worsen due to the development of cataracts or amblyopia.