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E. Gramer, C. Reiter, G. Gramer; Prognosis and Riskprofile of Patients With Aniridia. Invest. Ophthalmol. Vis. Sci. 2009;50(13):4039.
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To evaluate in patients with aniridia: 1)Frequency of congenital cataract, lens dislocation, nystagmus, corneal opacification, poor foveal development, family history of aniridia. 2)Frequency of glaucoma (GL) or ocular hypertension (OH) and maximum intraocular pressure (IOP max). 3)Visual acuity prognosis.
Retrospective analysis of ophthalmologic, paediatric and internal findings in 30 consecutively examined patients with aniridia and photo documentation. Age of the patient at first clinical examination, family history and IOP max were requested.
1)Mean age at first clinical examination in 30 patients with bilateral aniridia (17 male, 13 female)was 28±19,3 years (range 2 month-62 years). Age distribution at first clinical examination: up to 9 years: 23,3%, 10-19: 13,3%, 20-29: 13,3%, 30-39: 10%, 40-49: 26,7%, 50-59: 3,3%, 60-69: 6,7%. Congenital cataract had 76,7% (23 of 30 patients), in 20 patients bilateral, in 3 unilateral. 5 patients (16,7%) had bilateral dislocation of the crystalline lens upwards. 8 had corneal opacifications, 3 unilateral, 5 bilateral. One patient had poor foveal development, one patient (3,3%) had a Wilms' Tumor. 2 patients (6,7%)had optic nerve hypoplasia. 53,3% presented nystagmus. Family history of aniridia was found in 10 patients (33,3%) with 1-4 relatives. 2) Frequency of glaucoma was 66,7% (20 patients), 10% unilateral, 90% bilateral. Mean IOP max of 20 glaucoma patients was 35,9 mmHg in the right and 32,6 mmHg in the left eye. 3)A decreased visual acuity in the better eye of 20/100 or less was found in 60% of all patients.
Inspite of pseudophakia patients with aniridia had severely decreased visual acuity related to nystagmus, glaucomatous damage and as a result of the other associated various ocular abnormalities. Elevated IOP occurs very infrequently in childhood. Buphthalmus was only found in one patient. Most commonly IOP starts to increase not before adolescence. Schlemm's canal may be absent in many patients. Due to a highly variable expression of aniridia in relatives a thorough examination of family members is required to provide early diagnosis, amblyopia prophylaxis and a correct genetic counceiling.
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