Congenital aniridia: long-term clinical course, visual outcome, and prognostic factors

To describe the clinical course of congenital aniridia and to evaluate prognostic factors for visual outcome after long-term follow-up. The medical records of 120 eyes from 60 patients with congenital aniridia were retrospectively reviewed. The prevalence and clinical course of ophthalmic characteri...

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Bibliographic Details
Published in:Korean journal of ophthalmology 2014, 28(6), , pp.479-485
Main Authors: Chang, Ji Woong, Kim, Jeong Hun, Kim, Seong-Joon, Yu, Young Suk
Format: Article
Language:English
Subjects:
Adolescent
Adult
Aniridia - diagnosis
Aniridia - physiopathology
Cataract - diagnosis
Child
Child, Preschool
Corneal Diseases - diagnosis
Eye Abnormalities - diagnosis
Eye Diseases - diagnosis
Eye Diseases - physiopathology
Follow-Up Studies
Humans
Infant
Infant, Newborn
Nystagmus, Pathologic - diagnosis
Ocular Hypertension - diagnosis
Original
Prognosis
Retina - abnormalities
Retrospective Studies
Visual Acuity - physiology
Young Adult
안과학
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Summary:To describe the clinical course of congenital aniridia and to evaluate prognostic factors for visual outcome after long-term follow-up. The medical records of 120 eyes from 60 patients with congenital aniridia were retrospectively reviewed. The prevalence and clinical course of ophthalmic characteristics, systemic disease, refractive errors, and visual acuity were assessed. Prognostic factors for final visual outcomes were analyzed. Aniridic keratopathy developed in 82 (69%) of 119 eyes. Macular hypoplasia was observed in 70 eyes of 35 patients (91%). Cataract was observed in 63 of 120 eyes (53%). Nystagmus was present in 41 patients (68% of 60 patients) at the initial visit but decreased in five patients (8% of 60 patients). Ocular hypertension was detected in 19 eyes (20% of 93 eyes), six (32% of 19 eyes) of which developed secondarily after cataract surgery. The mean changes in spherical equivalent and astigmatism during the follow-up period were -1.10 and 1.53 diopter, respectively. The mean final visual acuity was 1.028 logarithm of minimal angle of resolution. Nystagmus and ocular hypertension were identified as prognostic factors for poor visual outcome. Identification of nystagmus and ocular hypertension was important to predict final visual outcome. Based on the high rate of secondary ocular hypertension after cataract surgery, careful management is needed.
ISSN:1011-8942
2092-9382
DOI:10.3341/kjo.2014.28.6.479