In:
Acta Ophthalmologica Scandinavica, Wiley, Vol. 85, No. s240 ( 2007-09), p. 0-0
Abstract:
Purpose: Our purpose was to analyze the various ocular manifestations, treatment options, and the possible outcome of congenital aniridia (CA) a rare and severe developmental disorder. Methods: 52 eyes of 26 patients were regularly checked with full eye examination including anterior segment photography. Electrophysiology, OCT, UBM, and glaucoma testing was done if appropriate. The mean age at the last examination was 17.6 years (range: 2 months – 72 years). Results: The best corrected visual acuity was: 0.2 (ranging from no light perception to 0.6). 20 cases were sporadic and 6 cases showed autosomal dominant inheritance in 3 families. A deletion including band p13 of the short arm of chromosome 11 was found in one familial and one sporadic case. Associated ocular signs were nystagmus (88.5%), congenital cataract (61.5%), and ectopia lentis (3.8%). 14 eyes of 10 cases needed cataract surgery. Secondary glaucoma occurred in 24 eyes of 12 patients, with severe outcome in 15 eyes of 8 patients despite treatment. Aniridic keratopathy occurred in 23 eyes of 12 patients; in 3 cases keratoplasty was performed. One patient had aniridia on one eye, and iris coloboma on the fellow eye confirming the common origin of the two diseases. Interestingly, no Wilms tumor occurred in our series, but mental deficits were found in 4 patients. Conclusions: Secondary glaucoma is the most severe complication of CA. It can appear in relatively young age, is relatively resistant to treatment, and the earlier the onset, the worse the prognosis. Risk factors for vision loss included eye surgeries at young age. Moreover, the outcome of cataract‐surgery is limited by foveal hypoplasia and the progression of glaucoma.
Type of Medium:
Online Resource
ISSN:
1395-3907
,
1600-0420
DOI:
10.1111/aos.2007.85.issue-s240
DOI:
10.1111/j.1600-0420.2007.01063_3339.x
Language:
English
Publisher:
Wiley
Publication Date:
2007
detail.hit.zdb_id:
2466981-7
detail.hit.zdb_id:
2024571-3
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