Korean J Ophthalmol.  2014 Dec;28(6):479-485. 10.3341/kjo.2014.28.6.479.

Congenital Aniridia: Long-term Clinical Course, Visual Outcome, and Prognostic Factors

• Chang JW
• Kim JH
• Kim SJ
• Yu YS

• KMID: 2345825
• DOI: http://doi.org/10.3341/kjo.2014.28.6.479

Abstract

PURPOSE
To describe the clinical course of congenital aniridia and to evaluate prognostic factors for visual outcome after long-term follow-up.
METHODS
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.
RESULTS
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.
CONCLUSIONS
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.

Keyword

Aniridia; Eye abnormalities; Prognosis

MeSH Terms

Adolescent
Adult
Aniridia/*diagnosis/physiopathology
Cataract/diagnosis
Child
Child, Preschool
Corneal Diseases/diagnosis
Eye Abnormalities/diagnosis
Eye Diseases/*diagnosis/physiopathology
Follow-Up Studies
Humans
Infant
Infant, Newborn
Nystagmus, Pathologic/diagnosis
Ocular Hypertension/diagnosis
Prognosis
Retina/abnormalities
Retrospective Studies
Visual Acuity/*physiology
Young Adult

Figure

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  Fig. 1 Changes in spherical equivalent (A) and astigmatism (B) between the initial and final exams in congenital aniridia.

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  Fig. 2 Distribution of final visual acuity in congenital aniridia.

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