Macular Degeneration: Anderson RJ

Hajali M, Fishman GA, Anderson RJ, McAnany JJ. · Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois, USA. · Invest Ophthalmol Vis Sci. · Pubmed #19234345 No free full text.

Abstract: PURPOSE: To determine the extent of interocular difference in visual acuity (VA) and the time to at least double the minimal angle of resolution (MAR) in a cohort of patients with Stargardt disease. METHODS: One hundred fifty patients with Stargardt disease who were examined at least four times over a minimum period of 3 years were identified and their VA and age at each visit recorded. The maximum interocular difference of VA was determined by whether the MAR between the two eyes differed by less than a factor of 2 or by a factor of 2 or greater. Differences in maximum VA between the two eyes were also examined according to a Bland-Altman-type approach. One hundred thirty-one eyes from 76 patients were subjected to survival analysis to determine whether the time to at least double the MAR was dependent on age at baseline or starting VA. RESULTS: Of the 150 patients, 48% had interocular MAR that differed maximally by a factor of less than 2. Thirty-five percent showed a maximum interocular difference in their Snellen VA of less than one line. The Bland-Altman- type analysis showed that maximum interocular acuity difference was dependent on the mean acuity of the two eyes. The hazard for at least doubling the MAR was related to baseline vision and patient age. CONCLUSIONS: This information has clinical significance for patient counseling and for monitoring possible benefits and patient selection in future treatment trials.

Hajali M, Fishman GA, Anderson RJ. · Department of Ophthalmology and Visual Sciences (MC 648), Room 3.85, Eye and Ear Infirmary, 1855 W Taylor Street, Chicago, IL 60612-7234, USA. · Br J Ophthalmol. · Pubmed #18653601 No free full text.

Abstract: AIMS: To determine the prevalence of cystoid macular oedema (CMO) in retinitis pigmentosa (RP) patients of various genetic subtypes using optical coherence tomography (OCT). METHODS: We performed a complete ocular examination on 124 RP patients including best corrected visual acuity, intraocular pressure measurement, anterior segment and a detailed fundus exam. OCT images were then acquired using two different units. The presence of hypo-reflective lacunae was used to diagnose CMO. RESULTS: Of the 124 patients, 47 showed CMO in at least one eye (38%), while 34 showed CMO in both eyes (27%). The prevalence of CMO in at least one eye for autosomal dominant (AD) patients was 52%, for autosomal recessive (AR) 39%, isolated 39%, Usher II 35% and none in the X linked recessive (XL) group. Using a chi-square analysis, no statistical significant difference was found for the prevalence of "bilateral CMO" (p = 0.60) or "CMO in at least one eye" (p = 0.59) among the AD, AR, isolated and Usher II genetic subtypes. CONCLUSION: Because of its notable prevalence, it would seem prudent to screen RP patients by OCT for the possible presence of CMO, to identify those amenable to treatment and also for future treatment trials when monitoring visual acuity.

Rotenstreich Y, Fishman GA, Anderson RJ. · Department of Ophthalmology and Visual Science, University of Illinois at Chicago, 60612, USA. · Ophthalmology. · Pubmed #12799240 No free full text.

Abstract: PURPOSE: To assess visual acuity impairment in Stargardt disease. DESIGN: Retrospective clinic-based cross-sectional study. PARTICIPANTS: Three-hundred sixty-one patients with Stargardt disease. METHODS: Clinical findings in 361 patients were analyzed as part of a cross-sectional evaluation. Visual acuity at their most recent visit, fundus photographs, and electroretinographic findings were reviewed, and patients were categorized into four clinical phenotypes. Seventy-three patients with 20/40 or better vision and 38 patients with 20/50 to 20/100 vision in the better seeing eye at their initial visit who were followed for at least 1 year were included in a survival analysis. For analysis purposes, these latter patients were categorized into four 20-year age groups according to their age at initial visit. MAIN OUTCOME MEASURES: Best-corrected visual acuity from the eye with better vision on the most recent visit was used in the cross-sectional analysis. For the survival analysis, best-corrected visual acuity was used from the eye with better vision on the initial visit. RESULTS: Eighty-two of the 361 patients (23%) had 20/40 or better acuity in at least one eye, 64 (18%) 20/50 to 20/100, and 199 (55%) 20/200 to 20/400, whereas 16 (4%) had worse than 20/400 in each eye at their most recent visit. In the patients with visual acuity of 20/40 or better, 59 (72%) had foveal sparing visible on ophthalmoscopic examination. The median time to develop visual acuity of 20/200 or worse was 22 years for the patients with 20/40 or better visual acuity at their initial visit. Those seen initially in the first two decades of life with this level of acuity showed a median time of 7 years to reach a visual acuity of 20/200 or worse compared with 22 years and 29 years for those who were initially seen at ages 21 to 40 or 41 to 60, respectively. Analyzing by the four 20-year age groups, the log rank statistic indicated significant differences in the survival experience among the four groups (P = 0.004). The median time to develop 20/200 vision or worse was 6 years for the patients with 20/50 to 20/100 visual acuity at their initial visit, and this result, based on the log rank statistic, was independent of age group at initial visit (P = 0.852). CONCLUSIONS: In a large cohort of Stargardt patients, a cross-sectional analysis showed that almost a quarter had vision of 20/40 or better, whereas 4% had acuity of worse than 20/400. The presence of foveal sparing ophthalmoscopically was associated with a higher prevalence of 20/40 or better visual acuity. Survival analysis showed that the prognosis of patients who initially were seen with visual acuity of 20/40 or better is related to age at initial visit.