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

Abstract

PurposeTo describe the clinical course of congenital aniridia and to evaluate prognostic factors for visual outcome after long-term follow-up.MethodsThe 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.ResultsAniridic 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.ConclusionsIdentification 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.

Outer retinal layers as predictors of visual acuity in retinitis pigmentosa: a cross-sectional study

“…Visual acuity was converted to the logarithm of minimum angle of resolution (logMAR) units for statistical analyses [19]. Hand movement, light perception, and no light perception were classified as + 2, + 3 and + 4, respectively [20][21][22]; tables and figures were used to summarize data as appropriate. Observational agreement was evaluated by kappa test.…”

Efficacy of carbonic anhydrase inhibitors in management of cystoid macular edema in retinitis pigmentosa: A meta-analysis

“…If the VA was reported in Snellen VA form in original publication, the data was converted to logarithm of minimal angle of resolution (logMAR) for statistical analysis. Based on a previous report, “counting fingers” (CF), “hand motion” (HM), “light perception” (LP), and “no light perception (NLP)” were assigned a logMAR value of 1.85, 2.3, 2.7 and 3.0 respectively [21,22]. The mean changes of VA or CMT from baseline to final follow-up points were pooled and calculated using inverse variance methods.…”

Congenital aniridia: etiology, manifestations and management

“…Most of the studies show the presence of foveal hypoplasia to be as high as 90% in these eyes [63]. Clinically, there is absence of the macular reflex and foveal pit, and presence of abnormal retinal vessels crossing the fovea (Figure 4) [12].…”

“…There has been no proven correlation between the optic nerve and foveal hypoplasia, but most cases show nystagmus. Incidence of nystagmus is as high as 90%, where the majority of these eyes show horizontal pendular nystagmus [1, 63]. Vertical nystagmus as a phenotype variant with foveal hypoplasia, presenile cataract and intact irides has been recently described [68].…”