October 2008
Volume 49, Issue 10
Free
Clinical and Epidemiologic Research  |   October 2008
The National Eye Institute Visual Function Questionnaire in the Macular Telangiectasia (MacTel) Project
Author Affiliations
  • Traci E. Clemons
    From the The EMMES Corporation, Rockville, Maryland; the
  • Mark C. Gillies
    Department of Clinical Ophthalmology, Save Sight Institute, University of Sydney, Sydney, Australia; the
  • Emily Y. Chew
    National Eye Institute, Bethesda, Maryland; the
  • Alan C. Bird
    Institute of Ophthalmology and Moorfields Eye Hospital, London, United Kingdom.
  • Tunde Peto
    Institute of Ophthalmology and Moorfields Eye Hospital, London, United Kingdom.
  • Maria Figueroa
    From the The EMMES Corporation, Rockville, Maryland; the
  • Molly W. Harrington
    From the The EMMES Corporation, Rockville, Maryland; the
Investigative Ophthalmology & Visual Science October 2008, Vol.49, 4340-4346. doi:10.1167/iovs.08-1749
  • Views
  • PDF
  • Share
  • Tools
    • Alerts
      ×
      This feature is available to authenticated users only.
      Sign In or Create an Account ×
    • Get Citation

      Traci E. Clemons, Mark C. Gillies, Emily Y. Chew, Alan C. Bird, Tunde Peto, Maria Figueroa, Molly W. Harrington; The National Eye Institute Visual Function Questionnaire in the Macular Telangiectasia (MacTel) Project. Invest. Ophthalmol. Vis. Sci. 2008;49(10):4340-4346. doi: 10.1167/iovs.08-1749.

      Download citation file:


      © ARVO (1962-2015); The Authors (2016-present)

      ×
  • Supplements
Abstract

purpose. To describe vision-targeted health-related quality of life (HR-QOL), measured with the National Eye Institute Visual Functioning Questionnaire (NEI-VFQ-25) in a cohort of patients with macular telangiectasia (MacTel) type 2 and to evaluate the relationship between visual acuity and NEI-VFQ-25 scores.

methods. This was an analysis of cross-sectional baseline data from a longitudinal natural history study. Patients with MacTel type 2 were enrolled in the Natural History Study of The Macular Telangiectasia Project (The MacTel Project). NEI-VFQ-25 were completed at enrollment. Linear correlation and regression analyses were used to relate baseline NEI-VFQ-25 overall and subscale scores to visual acuity.

results. Participants reported lower vision-related functioning measured by the NEI-VFQ-25 in most of the domains measured by the NEI VFQ compared with that of a normal reference group (P < 0.001 for all domains except color vision). Visual acuity was found to be associated with the NEI-VFQ-25 in many of the domains measuring degree of difficulty with common visual activities.

conclusions. This is the first cross-sectional cohort study to assess vision targeted HR-QOL in patients with MacTel type 2. Patients with MacTel type 2 reported markedly reduced visual functioning compared to reports of a normal reference group. These findings provide support to the use of the NEI-VFQ-25 in patients with MacTel type 2 to measure the effect of disease and potential therapies on vision-targeted HR-QOL.

Macular telangiectasia (MacTel) type 2, a type of idiopathic MacTel, 1 2 3 4 is an uncommon condition of bilateral irregular capillary dilation and incompetence in the macula. Visual acuity at presentation usually ranges from 20/25 to 20/40, although vision as poor as 20/200 may occur. The disease is typically diagnosed in the fifth or sixth decade of life. The maculae of these patients exhibit retinal juxtafoveolar telangiectasia, minimal exudation, superficial retinal crystalline deposits, and right-angle venules. As the disease progresses, intraretinal pigment plaques and subretinal neovascularization may develop. The pathogenesis of the disease is unknown. Its classification was reported originally by Gass et al. 2 3 and recently was revised by Yannuzzi et al. 5 This group of diseases is now referred to as MacTel types 1 and 2. Type 1 MacTel, is considered to be aneurysmal, with dilated retinal capillaries easily detected clinically in the macular area, and fluorescein leakage is readily evident. These patients are primarily male, with unilateral disease. The condition that we evaluated in this study is MacTel type 2, either the nonproliferative type or the proliferative type in which neovascularization is present. 
The MacTel project is an international observational clinical study designed to evaluate the structural and functional changes associated with MacTel type 2 over a 5-year period. In addition, a group of laboratories with complimentary expertise is assessing the pathobiology of the disease to improve the understanding of its pathogenesis and potential treatment. The project includes 22 clinical centers in seven countries with laboratories in three different countries. 
Increasing attention has been given to the assessment of health-related quality of life (HR-QOL) outcome measures in patients with eye disease. 6 The National Eye Institute Visual Function Questionnaire (NEI-VFQ-25) was constructed to evaluate the effect of visual disability on HR-QOL across several common eye conditions. 7 8 The NEI-VFQ-25 has been incorporated into research studies, and findings have been reported for patients with conditions such as AMD, diabetic retinopathy, optic neuritis, glaucoma, uveitis, dry eye, and general low vision. 9 10 11 12 13 14 15 16 17 18  
An assessment of vision-targeted HR-QOL in a cohort of patients with MacTel type 2 is important because it will help in understanding the natural history of the disease and how it may affect the daily lives of patients with MacTel type 2. Nothing is known about the vision-targeted HR-QOL among patients with MacTel type 2. Nearly all patients with MacTel type 2 have some degree of visual impairment in both eyes, and a minority has severe visual loss sometimes due to development of neovascularization that may be intraretinal and choroidal. 
The purpose of this study was to provide the first large-cohort assessment of vision targeted HR-QOL as measured by the NEI-VFQ-25 in patients with MacTel type 2. We also assessed the association between visual function and vision-targeted HR-QOL. 
Methods
Design and Procedures
We studied patients with MacTel Type 2 who were enrolled in the MacTel Project’s Natural History Study, a multicenter observational study conducted in 22 clinical centers in seven countries. Each center was granted approval to conduct the study by their institutional review board or independent ethics committee. The eligibility criteria for the study required the participant be at least 18 years of age with a diagnosis of MacTel type 2 made clinically at each clinical center and confirmed on fundus photographs, ocular coherence tomography, fluorescein angiographs, or autofluorescence image gradings performed by the Fundus Reading Center of Moorfields Eye Hospital. 
A total of 243 participants were enrolled in the study between November 17, 2005, and January 15, 2007. After signing the informed consent, in accordance with the Declaration of Helsinki, each participant had a comprehensive dilated ophthalmic examination and completed a health and family history interview. Best corrected visual acuity 19 was measured by trained examiners using a standardized protocol and Early Treatment for Diabetic Retinopathy Study (ETDRS) visual acuity charts. 20 21  
At the baseline visits, the 25-item NEI-VFQ-25 (http://www.rand.org/health/surveys_tools/vfq/) was administered to participants by the clinical center coordinator. The NEI-VFQ-25 is the short form of the NEI-VFQ Field Test Version that was developed to include general health, general vision, ocular pain, near vision activities, distance vision activities, social functioning, vision-specific role difficulties, vision-specific mental health, dependency because of vision, driving, peripheral vision, and color vision. 9  
Item responses were transformed to a scale of 0 to 100. The overall NEI-VFQ-25 score was calculated as the average of the 25 items, whereas the subscale scores were the averages of the responses to items within each subscale. Both the overall and subscale scores range from 0 to 100, with higher scores indicating better vision-targeted HR-QOL. 
Statistical Methods
Demographic and visual acuity characteristics for participants were summarized with descriptive statistics. The NEI-VFQ-25 subscale scores were computed according to published algorithms. 8 A mean (SD) for the overall NEI-VFQ-25 and for each subscale was computed. The subscale means were compared with those of a reference group of 122 normal subjects derived from the field test of the NEI-VFQ-25. 9 The relation of visual acuity to vision-targeted HR-QOL was evaluated by assessing the magnitude of Spearman’s rank correlation coefficients 22 between the better eye and worse eye visual acuity and the scores from the NEI-VFQ-25. Associations of visual acuity and vision-targeted HR-QOL were also evaluated by comparing the age, sex, and disease type (nonproliferative versus proliferative disease) adjusted mean differences in scores within ordered visual acuity categories (20/32 or better in both eyes, worse than 20/32 in one eye, or worse than 20/32 in both eyes) using a test of trend. The Dunnett multiple-comparisons test 23 was used to identify significant differences in scores within the visual acuity categories by comparing each level with the reference level (20/32 or better in both eyes). Tests for trend in the linear models were performed by using linear contrasts. Internal consistency and reliability were assessed with Cronbach’s α 24 for the eight multi-item subscales of the 25-item NEI-VFQ. All analyses were performed with commercially available statistical software (SAS, ver. 8.02; SAS Institute, Cary, NC). 
Results
Two hundred twenty-two (91%) of 243 participants enrolled in the Natural History Study completed the baseline 25-item NEI-VFQ and are included in this report. Twenty-one participants were excluded from the analysis because they did not have a confirmatory diagnosis of MacTel type 2 by the Reading Center. A summary of the baseline characteristics is provided in Table 1 . The mean (± SD) age of the population was 61 (±9) years (range, 36–83 years) at the time the questionnaire was administered. Approximately 60% of the participants were women and 88% were Caucasian. According to letter scores, the mean visual acuity for the better eye of the participants was 75 (±11) letters (Snellen equivalent, ∼20/32). Thirteen percent of the participants had proliferative disease defined as neovascularization, as detected on fundus photographs or OCT, or receiving treatment specifically for neovascularization. Fifty-one participants (23%) had received treatment for MacTel type 2. The mean duration of disease in the population was 3.7 (±4.3) years. The mean visual acuity of the worse eye was 61 (±17) letters (Snellen equivalent, ∼20/62.5). Forty percent of the participants had visual acuity worse than 20/32 OU at the time the questionnaire was administered. 
Mean (SD) and median baseline NEI-VFQ-25 scores, proportions of participants with a perfect score (100 points, ceiling) and with the lowest possible score (0 points, floor), and internal consistency estimates for the eight subscales with multiple items are presented in Table 2 . The mean overall score on the NEI-VFQ-25 was 77, with a median of 80. The median score was less than 85 on half of the subscales (general health, general vision, near activities, distance activities, mental health, and role difficulties). Some of the subscales, in particular the ocular pain, social functioning, dependency, color vision and peripheral vision, had large ceiling effects where a substantial percentage of participants (>25%) had the highest possible scores, whereas very few participants had subscale scores of 0. The internal consistency estimates for the NEI-VFQ-25 subscales as measured by Cronbach’s α, ranged from 0.48 to 0.79. With the exception of ocular pain and driving (Cronbach’s α = 0.66 and 0.48, respectively), the subscales had similar internal consistency estimates as those found by the developers 9 (Cronbach’s α ≥ 0.70 for all the eight multi-item subscales). The six of eight subscales with Cronbach’s α ≥ 0.70 demonstrated a moderately strong internal consistency and reliability within this cohort of patients with MacTel type 2. 
To develop a shorter version of the NEI-VFQ-51 item questionnaire consisting of 25 questions, researchers tested participants with various eye diseases and a control/reference group free of eye disease. Mean subscale scores from the MacTel type 2 cohort were compared with those of the reference group. 8 Subscale NEI-VFQ-25 scores were markedly lower in patients with MacTel type 2 than in the reference group from the NEI-VFQ-25 field test (Fig. 1) . More specifically, mean scores of patients with MacTel type 2 from five subscales including role difficulties, mental health, near vision, and distance vision, were ∼20 points below the mean scores of the reference group. Similar mean scores between the reference and MacTel type 2 groups were apparent in only the color vision subscales (98 vs. 97). All differences, except for color vision, were statistically significant (P < 0.001). 
Rank correlations between the NEI-VFQ-25 subscale scores and best and worse visual acuity are provided in Table 3 . Correlations ranged from small (0.14) to moderate (0.38), except for the ocular pain subscale, which showed a negligible correlation (0.04). Correlations with visual acuity in the better eye and scores on the NEI-VFQ-25 were statistically significant (P < 0.01) for all NEI-VFQ-25 subscales except ocular pain and mental health. Moderate correlations corresponded to subscales, such as general vision, near activities, distance activities, and driving, which measure the degree of difficulty with common visual activities. The correlations of NEI-VFQ-25 overall and subscale scores with visual acuity of the worse eye were similar to those with the visual acuity of the better eye, probably because MacTel type 2 is a bilateral disease. 
Mean NEI-VFQ-25 subscale scores by participants’ age, sex, race, and disease type were computed (data not shown). Univariate analyses showed that the mean overall NEI-VFQ-25 score of older participants was significantly higher than that of younger participants. The older age groups (≥70 years) had significantly higher scores on average than did the younger age group (≤55 years) on the subscales relating to near activities, distance activities, mental health, role difficulties, and dependency. This result, showing higher NEI-VFQ-25 scores in older persons with MacTel type 2, differs from that found for other cohorts in which the mean overall NEI-VFQ-25 score was found to be significantly lower in persons who were older. 10 13 The distribution of visual acuity in the worse and better eyes did not show the same phenomenon. The visual acuity in the better eye in the younger and older age groups did not differ (74 letters vs. 73 letters, respectively). In addition, the visual acuity in the worse eye in the two age groups did not differ (61 letters vs. 60 letters, respectively). 
The mean overall NEI-VFQ-25 score differed significantly by sex, with the men having a significantly higher mean score than did the females (81 vs. 75). In addition, the men had higher mean scores on the near activity (76 vs. 65), distance activity (82 vs. 72), mental health (75 vs. 66), dependency (92 vs. 85), driving (83 vs. 66), and peripheral vision (90 vs. 83) subscales. The mean overall NEI-VFQ-25 score differed significantly by disease type. Participants with proliferative disease had a significantly lower mean score than did participants with nonproliferative disease (67 vs. 79). In addition, participants with proliferative disease had significantly lower mean scores on all subscales except general health, ocular pain, color vision, and peripheral vision. Caucasian participants had a higher, yet not significant, mean overall NEI-VFQ-25 score than did non-Caucasians (78 vs. 73, respectively; P = 0.034). Caucasians had significantly higher scores than did non-Caucasians on subscales relating to distant activities (77 vs. 67), social functioning (94 vs. 88), and peripheral vision (87 vs. 76). 
Participants treated for MacTel type 2 had significantly lower social functioning subscale scores than did participants not treated for MacTel type 2 (88 vs. 94), whereas there was a significant linear relationship between duration of disease and NEI-VFQ-25 score for the near activities and role difficulties (data not shown). The near activities score for participants newly diagnosed with MacTel type 2 at enrollment (duration equal to 0 years) was 73 compared with 76 in participants with a duration of disease equal to 3 years before enrollment, whereas the role difficulties score of participants newly diagnosed with MacTel type 2 at enrollment was 75 compared with 79 in participants with a duration of disease equal to 3 years before enrollment (data not shown). The overall mean NEI-VFQ-25 score and subscale scores were compared across sites located in North America (United States), Europe (Germany, France, and the United Kingdom), Israel, India, and Australia (data not shown). There was no significant difference in the overall NEI-VFQ-25 mean score and only the mean scores from the general health and mental health subscales were found to be significantly different across the five regions (P < 0.01). 
The NEI-VFQ-25 overall and subscale mean scores adjusted for age, sex, race, and disease type are presented in Table 4 . The test for trend indicated a progressive trend (P < 0.01) toward dysfunction, as reflected by the overall NEI-VFQ-25 score and most of the subscales, between participants with the unilateral and bilateral forms of vision impairment. The differences between the group with vision worse than 20/32 in both eyes and the group with 20/32 or better in both eyes were primarily responsible for this association, as demonstrated by the significant results of the Dunnett multiple comparisons test. Participants with vision worse than 20/32 in at least 1 eye had moderately lower scores (yet did not reach statistical significance) for the overall NEI-VFQ-25 and all subscales except ocular pain compared with participants with vision 20/32 or better in both eyes. Scores of participants with vision worse than 20/32 in both eyes were markedly lower (P < 0.01) on the overall NEI-VFQ-25 and the general vision, near activity, dependency, and driving subscales compared with participants with vision 20/32 or better in both eyes. Participants with worse than 20/32 vision in both eyes reported the most difficulty (mean score ≤70 and significantly different from the reference mean) with general vision, near activities, and driving. Scores for participants with vision worse than 20/32 in both eyes remained significantly lower after further adjustment for treatment for MacTel type 2 and duration of disease for the overall score and the general vision, near activities, dependency, and driving subscales. 
Table 5provides the mean NEI-VFQ-25 subscale scores for MacTel type 2 patients for comparison with published scores for other cohorts of patients with eye diseases. 9 10 15 18 The mean age, percentage who were women, and the mean visual acuity in the better eye are provided if available within the cited publication. NEI-VFQ-25 subscale scores for this cohort of patients with MacTel type 2 are similar to those found for participants with preoperative age-related cataract (column F, Table 5 ). For most subscales, patients with MacTel type 2 had worse scores than those of patients with acute optic neuritis, early age-related macular degeneration in the Complications of Age-Related Macular Degeneration Prevention Trial (CAPT), 12 glaucoma, cytomegalovirus retinitis, and dry eye (columns A, B, E, G, H, Table 5 ). The last column of Table 5provides a ranking of the MacTel type 2 mean subscale scores (from worse score to better score) among mean scores from other cohorts with various eye diseases. On most subscales, the patients with MacTel type 2 had scores that ranked within one, two, or three steps from the worst score. This may be because MacTel type 2 is one of the few clinical retinal diagnoses in which visual impairment is usually present in both eyes and thus reflects a reduced vision-related quality of life. 
Discussion
This report provides the first data regarding vision-targeted HR-QOL collected using a standard instrument for patients with MacTel type 2. Significant correlation coefficients were found between most of the NEI-VFQ-25 subscale scores and visual acuity. It was also shown that the NEI-VFQ-25 showed moderately strong internal consistency within the MacTel cohort. This demonstrates that the NEI-VFQ-25 is sensitive to the effect of MacTel type 2 and supports the construct validity of the questionnaire. Further evidence of the validity of the questionnaire within this cohort is provided by the decrease in the overall score and several subscale scores with the progressive degrees of visual impairment. Vision-targeted HR-QOL mean subscale scores assessed with the NEI-VFQ-25 were lower compared with the reference group free from eye disease. Furthermore, compared with other cohorts with AMD and diabetic retinopathy, the MacTel cohort showed lower mean scores for most subscales and ranked among the lowest scores across other cohorts with various eye diseases. This result is consistent with the impression of study investigators that MacTel type 2 has a significant impact on visual functioning, in particular vision-targeted HR-QOL, even when visual acuity is only modestly impaired. A plausible explanation for this is that the most markedly affected region of the macula is not the fovea, but rather the inferotemporal perifoveal zone. 25  
The results of this study confirm results in previous studies of cohorts with eye disease that showed the influence of visual function on vision-targeted HR-QOL. 8 9 11 12 15 18 However, relationships between mean NEI-VFQ-25 scores and demographics in the MacTel cohort revealed some results not demonstrated in other studies—for example, the linear relationship between the MacTel cohort’s age and NEI-VFQ-25 mean scores. Age was not associated with previous treatment or whether the disease was the proliferative type (data not shown). A significantly higher percentage of men were in the older age group and had higher scores than did the women. This difference may be one explanation for the higher scores in the older cohort, yet it is important to note that the linear relationship between MacTel cohort’s age and NEI-VFQ-25 mean scores remained after adjustment for sex and other covariates. Previous studies have reported lower scores in older individuals than in younger ones. 11 14 26  
The previous studies consisted of participants with age-related macular degeneration and type I diabetes and had similar proportions of women. All mean subscale scores except for color vision were higher in the AMD and diabetes cohorts than in participants with MacTel type 2. Participants with MacTel type 2 were on average younger but had a lower mean visual acuity in both the better and worse eye than did the AMD cohort. Correlations between visual acuity and subscale scores were lower in the MacTel cohort than were correlations found in the other studies. 
The mean overall NEI-VFQ-25 score was found to be significantly higher in the men than in the women with MacTel type 2. Other studies have found no differences in the overall composite mean score among the men and the women. 11 14 26 As mentioned previously, participants with MacTel type 2 were enrolled from seven countries, another difference from other studies reporting NEI-VFQ-25 results in participants with eye diseases. The studies we looked at enrolled only participants from a single country or region. 8 9 11 12 15 18 26  
This study has some limitations. We did not compare the results with a similarly collected control group, because the objective of this MacTel Project is to evaluate the clinical features and natural history of the disease in patients with MacTel type 2. Although differences in the normal control subjects and our cohort may have an influence on the results, we believe this unlikely given the large differences found between our cohort and the field test reference group. It is important to note that the ranking of MacTel type 2 NEI-VFQ-25 scores in Table 5compared with other ocular conditions did not take into account known and unknown confounders. Therefore, some caution should be used in interpreting the relative rankings. 
In summary, these results show that MacTel type 2 is associated with significant reductions in vision-targeted HR-QOL and may also signify that these reductions are more severe than in cohorts with other eye diseases with similar levels of visual acuity. This study confirms clinical observations from the MacTel Project that MacTel type 2 has a significant effect on vision-specific HR-QOL. 
Appendix 1
The MacTel Research Group
Centre Hospitalier National d’Ophtalmologie des Quinze-Vingts (Paris, France): Jose-Alain Sahel, Jean-Francois Girmens, and Saddek Mohand-Said. 
Centre for Eye Research (Melbourne, Australia): Robyn Guymer, Rebecca Maxwell, Khin Z. Aung, Lisa Breayley, Rebecca Davies, Andrew Newton, and Richard Smallwood. 
Clinique Ophthalmolgie de Creteil (Creteil, France): Gisele Soubrane, Karim Atmani, Aline Kunsch, Marc Lasnier, Vincent Parier, and Helene Poussard. 
Hôpital Lariboisiere (Paris, France): Alain Gaudric, Valerie Krivosic, Salomon Cohen, Ali Erginay, and Pascale Massin. 
Jules Stein Eye Institute, University of California (Los Angeles, CA): Steven Schwartz, Juewon Khwarg, Christine Gonzales, Anurag Gupta, Rosaleen Ostrick, and Malou Rosman. 
Lions Eye Institute (Perth, Australia): Ian Constable, Milada Zlatnik, Chris Barry, Joanne Cerruti, Max Cuypers, Tim Isaacs, Ian McAllister, Esperan Pather, Frank Shilton, and Lynne Smithies. 
Manhattan Eye, Ear, and Throat Hospital (New York, NY): Michael J. Cooney, Michelle Cimino, Eugene Agresta, K. Bailey Freund, Namrata Saroj, Nancy Gonzalez, and Lawrence Yannuzi. 
Moorfields Eye Hospital (London, UK): Cathy Egan, Sally Falk, Rebecca Black, Andrew C. Browning, Mina Devani-Gokaldas, Hannah Dunbar, Felicia Ikeji, Richard Poynter, Matthew Richardson, and Sonal Rughani. 
Retina Associates of Cleveland (Cleveland, OH): Lawrence Singerman, Stephanie Schura, John DuBois, Gregg Greanoff, David Miller, and Scott Pendergast. 
Save Sight Institute (Sydney, Australia): Mark Gillies, Meidong Zhu, Haipha Ali, Nicky Cunningham, Christine Gaston, Grace Hunt, and Maria Williams. 
Scripps Research Institute (La Jolla, CA): Martin Friedlander, Jennifer Trombley, and Martin Cervantes. 
St. Franziskus Hospital (Münster, Germany): Daniel Pauleikhoff, Bjorn Padge, Britta Heimes, Andreas Henschel, Kristina Kazior, George Spital, and Meike Trieschmann. 
The Goldschleger Eye Institute (Tel-Hashomer, Israel): Joseph Moisseiev, Vicky Dai, Nirit Atias, Zvi Friedman, Irit Hadas, Inesa Kelener, and Iris Moroz. 
The New York Eye and Ear Infirmary (New York, NY): Richard Rosen, Anita Ou, Kenneth Boyd, Ronald Gentile, Robert Masini, Juan Romero, Katy Tai, and Paul Whitten. 
The Retina Group of Washington (Fairfax, VA; Chevy Chase, MD): Robert Murphy, Lisa Byank, Debbie Oliver, Aida Ayyad, Courtney Dunn, Mike Flory, and Robert Frantz. 
University of Bonn (Germany): Frank Holz, Peter Charbel Issa, Boris Airo, Kerstin Bartsch, Robert Finger, Susanne Hinzmann, Cevriye Kocyigit, Rana Lee-Scholer, Hendrik Scholl, and Gero Tietz. 
University of Michigan, Kellogg Eye Center (Ann Arbor, MI): Andrew Vine, Pam Titus, Richard Hackel, Robert Prusak, and Tom Zbeda. 
University of Wisconsin (Madison, WI): Barbara Blodi, Michelle Olson, Justin Gottlieb, Gene Knutson, Denise Krolnik, and John Peterson. 
The Wilmer Eye Institute, The Johns Hopkins University (Baltimore, MD): Diana Do, Lisa Greer, Judy Belt, Dennis Cain, David Emmert, Janis Graul, Julia Haller, Jackie MacDonald, and Jennifer Simmons. 
Scheie Eye Institute, University of Pennsylvania (Philadelphia, PA): Alexander J. Brucker, Sheri Grand Drossner, Jim Berger, Cheryl Devine, Joshua L. Dunaief, Joan DuPont, Albert Mahler MaGuier, Bill Nyberg, and Laurel Weeney. 
L. V. Prasad Eye Institute (Hyderabad, India): Raja Narayanan, Savitha Viswanathan, Vibha Choudhary, Taraprasad Das, and G. Muneeshwar Gupta. 
Coordinating Center, The EMMES Corporation (Rockville, MD): Traci E. Clemons, Maria Figueroa, Traci Scheer, Trudy Chandler, Molly Harrington, Michael Frasketi, Glenn Tucker, and Lisa Greene. 
Moorfields Eye Hospital Reading Center (London, UK): Tunde Peto, Richard Seeberan. 
Columbia University Eye Research Institute (New York, NY): Rando Allikmets, Smitha Gopakumar. 
Executive Committee Members: Mark Gillies, Save Sight Institute (Sydney, Australia); Alan C. Bird, Moorfields Eye Hospital (London, UK); Emily Y. Chew, National Eye Institute/National Institutes of Health (Bethesda, MD); Marty Friedlander, Scripps Research Institute (La Jolla, CA); Robert M. Graham, Victor Chang Cardiac Research Institute (Sydney, Australia): Stephen Johns, Westfield Group Limited (Sydney, Australia); and Frank Lowy, Stephen Lowy, and Frank Martin, The Lowy Medical Research Institute Limited (Sydney, Australia). 
Laboratory Science Group: Marty Friedlander, Scripps Research Institute (La Jolla, CA); Rando Allikmets, Columbia University (New York, NY); Marcus Fruttiger, John Greenwood, and Steven Moss, University College London, Institute of Ophthalmology (London, UK); Mark Gillies, Save Sight Institute (Sydney, Australia); and Lois E. H. Smith, Harvard Medical School, Childrens Hospital (Boston, MA). 
Oversight Committee: Brad Straatsma, Dean Bok, David Geffen School of Medicine University of California (Los Angeles, CA); Marie Diener-West, The Johns Hopkins University, Bloomberg School of Public Health (Baltimore, MD); Stuart L. Fine, Scheie Eye Institute, University of Pennsylvania (Philadelphia, PA); and Larry Sherman, Oregon National Primate Research Center, Division of Neuroscience (Beaverton, OR).  
Sponsor: The Lowy Medical Research Institute Limited (Sydney, Australia).  
 
Table 1.
 
Natural History Study Participant Characteristics
Table 1.
 
Natural History Study Participant Characteristics
Participants, n (%) 222 (100)
Age (y)
 Mean (SD) y, n (%) 61 (9)
 <55 47 (22)
 55–59 45 (20)
 60–64 44 (20)
 65–69 50 (23)
 ≥70 36 (16)
Female, n (%) 134 (60)
Caucasian, n (%) 188 (88)
Proliferative disease, n (%)* 28 (13)
Received treatment for MacTel type 2, n (%) 51 (23)
Duration of disease, mean years (SD) 3.7 (4.3)
Visual acuity score of better eye, mean (SD) 75 (11)
Visual acuity score of worse eye, mean (SD) 61 (17)
Visual acuity category
 Both eyes 20/32 or better, n (%) 50 (23)
 One eye worse than 20/32, n (%) 84 (38)
 Both eyes worse than 20/32, n (%) 88 (40)
Table 2.
 
NEI-VFQ-25 Results in Patients with MacTel Type 2
Table 2.
 
NEI-VFQ-25 Results in Patients with MacTel Type 2
NEI-VFQ-25 Subscale Mean ± SD Median Cronbach’s α* Ceiling n (%) Floor n (%)
General health 56 ± 21 50 N/A, † 18 (8) 3 (1)
General vision 65 ± 17 60 N/A, † 7 (3) 0 (0)
Ocular pain 81 ± 20 88 0.66 85 (38) 0 (0)
Near activities 70 ± 21 75 0.75 24 (11) 0 (0)
Distance activities 76 ± 20 83 0.70 36 (17) 0 (0)
Social functioning 93 ± 14 100 0.70 164 (74) 0 (0)
Mental health 70 ± 25 75 0.75 19 (9) 1 (<1)
Role difficulties 70 ± 26 75 0.74 52 (23) 6 (3)
Dependency 88 ± 20 100 0.79 134 (60) 1 (<1)
Driving, ‡ 74 ± 26 88 0.48 41 (18) 9 (4)
Color vision 97 ± 10 100 N/A, † 201 (91) 0 (0)
Peripheral vision 86 ± 21 100 N/A, † 140 (63) 0 (0)
NEI-VFQ-25 (overall) 77 ± 13 80 0.90 1 (<1) 0 (0)
Figure 1.
 
Comparison of NEI-VFQ-25 Subscale Means (Reference group versus MacTel Cohort). * Two tailed t-test P < 0.0001.
Figure 1.
 
Comparison of NEI-VFQ-25 Subscale Means (Reference group versus MacTel Cohort). * Two tailed t-test P < 0.0001.
Table 3.
 
Correlations between Visual Acuity and NEI-VFQ-25 Subscales
Table 3.
 
Correlations between Visual Acuity and NEI-VFQ-25 Subscales
NEI-VFQ-25 Subscale Visual Acuity
Better Eye Worse Eye
General health 0.20* 0.19
General vision 0.34* 0.33*
Ocular pain 0.05 0.04
Near activities 0.38* 0.32*
Distance activities 0.32* 0.27*
Social functioning 0.24* 0.18*
Mental health 0.15 0.16
Role difficulties 0.24* 0.22*
Dependency 0.23* 0.25*
Driving 0.32* 0.27*
Color vision 0.20* 0.14
Peripheral vision 0.29* 0.32*
NEI-VFQ-25 (overall) 0.37* 0.34*
Table 4.
 
Adjusted NEI-VFQ-25 Subscale Scores by Visual Acuity Group*
Table 4.
 
Adjusted NEI-VFQ-25 Subscale Scores by Visual Acuity Group*
NEI-VFQ-25 Subscales 20/32 or Better in Both Eyes Worse Than 20/32 in One Eye Worse Than 20/32 in Both Eyes P for Linear Trend
General health 68 (4.0) 58 (3.4) 57 (3.3) <0.01
General vision 63 (2.9) 62 (2.4) 53 (2.4), † <0.01
Ocular pain 78 (3.7) 82 (3.2) 80 (3.1) 0.75
Near activities 72 (3.6) 68 (3.0) 58 (2.9), † <0.01
Distance activities 73 (3.5) 71 (2.9) 64 (2.8) 0.01
Social functioning 88 (2.5) 88 (2.1) 82 (2.1) 0.02
Mental health 66 (4.3) 65 (3.6) 57 (3.5) 0.04
Role difficulties 67 (4.6) 67 (3.9) 57 (3.8) 0.03
Dependency 90 (3.6) 86 (3.1) 79 (3.0), † <0.01
Driving 77 (4.6) 75 (4.0) 58 (4.1), † <0.01
Color vision 97 (1.9) 94 (1.6) 94 (1.5) 0.08
Peripheral vision 87 (3.9) 82 (3.3) 78 (3.2) 0.02
NEI-VFQ-25 (overall) 77 (2.3) 75 (1.9) 69 (1.9), † <0.01
Table 5.
 
Comparisons of Mean NEI-VFQ-25 Scores of MacTel Type 2 Patients with Other Ocular Disease Cohorts
Table 5.
 
Comparisons of Mean NEI-VFQ-25 Scores of MacTel Type 2 Patients with Other Ocular Disease Cohorts
NEI-VFQ-25 Subscales MacTel Type 2 Patients Other Ophthalmology Patients* MacTel Type 2 Ranking, †
A B C D E F G H
General health 56 72 71 65 46 62 55 45 74 4
General vision 65 79 79 53 62 71 60 76 84 4
Ocular pain 81 87 89 87 88 89 86 90 70 2
Near activities 70 90 85 54 63 79 73 84 86 3
Distance activities 76 89 86 56 66 77 73 84 89 4
Social functioning 93 97 97 73 81 89 87 96 97 5
Mental health 70 85 85 58 66 81 77 74 88 3
Role difficulties 70 89 87 61 69 84 76 78 86 3
Dependency 88 97 97 72 77 92 88 89 98 3
Driving 74 84 85 39 55 75 63 80 86 4
Color vision 97 95 95 85 90 93 90 98 97 7
Peripheral vision 86 89 93 77 78 76 87 78 94 5
Participants, n 222 244 1052 108 123 77 93 37 75
Mean age, y 61 40 71 76 62 67 73 39 46, ‡
% Female 60 79 61 63 66 54 66 5 71
Median visual acuity in better eye, § 32 <20 20 63 40 25 40 20 N/A
GassJDM. A fluorescein angiographic study of macular dysfunction secondary to retinal vascular disease. V. Retinal telangiectasis. Arch Ophthalmol. 1968;80:592–605. [CrossRef] [PubMed]
GassJD, OyakawaRT. Idiopathic juxtafoveolar retinal telangiectasis. Arch Ophthalmol. 1982;100:769–780. [CrossRef] [PubMed]
GassJDM, BlodiBA. Idiopathic juxtafoveolar retinal telangiectasis: update of classification and follow-up study. Ophthalmology. 1993.1536–1546.
GassJDM. Stereoscopic atlas of macular disease: diagnosis and treatment. 1987;Mosby St. Louis.
YannuziJA, BardalAMC, FreundKB, ChenKJ, EandiCM, BlodiB. Idiopathic macular telangiectasia. Arch Ophthalmol. 2006;124:450–460. [CrossRef] [PubMed]
SpilkerB eds. Quality of Life Assessments in Clinical Trials. 1990;Raven Press New York.
MangioneCM, BerryS, LeePP, et al. Identifying the content area for the National Eye Institute Vision Function Questionnaire (NEI-VFQ): results from focus groups with visually impaired persons. Arch Ophthalmol. 1998;116:227–238. [PubMed]
MangioneCM, LeePP, PittsJ, et al. Psychometric properties of the National Eye Institute Visual Function Questionnaire (NEI VFQ). NEI-VFQ Field Test Investigators. Arch Ophthalmol. 1998;116:1496–1504. [CrossRef] [PubMed]
MangioneCM, LeePP, GutierrezPR, et al. Development of the 25-Item National Eye Institute Visual Function Questionnaire. Arch Ophthalmol. 2001;119:1050–1058. [CrossRef] [PubMed]
BrodyBL, GamstAC, WilliamsRA, et al. Depression, visual acuity, comorbidity, and disability associated with age-related macular degeneration. Ophthalmology. 2001;108:1893–1900. [CrossRef] [PubMed]
ClemonsTE, ChewEY, BresslerSB, McBeeW, for the AREDS Research Group. National Eye Institute Visual Function Questionnaire in the Age-Related Eye Disease Study (AREDS) Report no. 10. Arch Ophthalmol. 2003;121:211–217. [CrossRef] [PubMed]
The Complications of Age-Related Macular Degeneration Prevention Trial Research Group. Baseline characteristics, the 25-item National Eye Institute Visual Functioning Questionnaire, and their associations in the Complications of Age-Related Macular Degeneration Prevention Trial (CAPT). Ophthalmology. 2004;111:1307–1316. [CrossRef] [PubMed]
Submacular Surgery Trials Research Group. Health-and vision-related quality of life among patients with ocular histoplasmosis or idiopathic choroidal neovascularization at enrollment in a randomized trial of submacular surgery: Submacular Surgery Trials report no 5. Arch Ophthalmol. 2005;123:78–88. [CrossRef] [PubMed]
KleinR, MossSE, KleinBE, GuiterrezMA, MangioneCM. The NEI-VFQ-25 in people with long-term type I diabetes mellitus. The Wisconsin Epidemiologic Study of Diabetic Retinopathy. Arch Ophthalmol. 2001;119:733–740. [CrossRef] [PubMed]
ColeSR, BeckRW, MokePS, GalRL, LongDT. The National Eye Institute Visual Function Questionnaire: experience of the ONTT: Optic Neuritis Treatment Trial. Invest Ophthalmol Vis Sci. 2000;41:1017–1021. [PubMed]
ParrishRK, GeddeSJ, ScottIU, et al. Visual function and quality of life among patients with glaucoma. Arch Ophthalmol. 1997;115:1447–1455. [CrossRef] [PubMed]
SchiffmanRM, JacobsenG, WhitcupSM. Visual functioning and general health status in patients with uveitis. Arch Ophthalmol. 2001;119:841–849. [CrossRef] [PubMed]
NicholsKK, MitchellGL, ZadnikK. Performance and repeatability of the NEI-VFQ-25 in patients with dry eye. Cornea. 2002;21:578–583. [CrossRef] [PubMed]
BaileyIL, LovieJE. New design principles for visual acuity letter charts. Am J Optom Physiol Opt. 1976;53:740–745. [CrossRef] [PubMed]
Early Treatment Diabetic Retinopathy Study (ETDRS) Research Group. Early treatment diabetic retinopathy study design and baseline patient characteristics. ETDRS report number 7. Ophthalmology. 1991;98:741–756. [CrossRef] [PubMed]
FerrisFL, KassoffA, BresnickGH, BaileyI. New visual acuity charts for clinical research. Am J Ophthalmol. 1982;94:91–96. [CrossRef] [PubMed]
SnedecorGW, CochranWC. Statistical Methods. 1967;Iowa State University Press Ames, IA.
HsuJC. Multiple Comparisons. 1996;Chapman and Hall New York.
CronbachLJ. Coefficient alpha and the internal structure of tests. Psychometrika. 1951;16:297–334. [CrossRef]
Charbel IssaP, HelbHM, RohrschneiderK, HolzFG, SchollHP. Microperimetric assessment of patients with type 2 idiopathic macular telangiectasia. Invest Ophthalmol Vis Sci. 2007;48:3788–3795. [CrossRef] [PubMed]
ChiaE-M, MitchellP, OjaimiE, RochtchinaE, WangJJ. Assessment of vision-related quality of life in an older population subsample: The Blue Mountains Eye Study. Ophthalmic Epidemiol. 2006;13:371–377. [CrossRef] [PubMed]
Figure 1.
 
Comparison of NEI-VFQ-25 Subscale Means (Reference group versus MacTel Cohort). * Two tailed t-test P < 0.0001.
Figure 1.
 
Comparison of NEI-VFQ-25 Subscale Means (Reference group versus MacTel Cohort). * Two tailed t-test P < 0.0001.
Table 1.
 
Natural History Study Participant Characteristics
Table 1.
 
Natural History Study Participant Characteristics
Participants, n (%) 222 (100)
Age (y)
 Mean (SD) y, n (%) 61 (9)
 <55 47 (22)
 55–59 45 (20)
 60–64 44 (20)
 65–69 50 (23)
 ≥70 36 (16)
Female, n (%) 134 (60)
Caucasian, n (%) 188 (88)
Proliferative disease, n (%)* 28 (13)
Received treatment for MacTel type 2, n (%) 51 (23)
Duration of disease, mean years (SD) 3.7 (4.3)
Visual acuity score of better eye, mean (SD) 75 (11)
Visual acuity score of worse eye, mean (SD) 61 (17)
Visual acuity category
 Both eyes 20/32 or better, n (%) 50 (23)
 One eye worse than 20/32, n (%) 84 (38)
 Both eyes worse than 20/32, n (%) 88 (40)
Table 2.
 
NEI-VFQ-25 Results in Patients with MacTel Type 2
Table 2.
 
NEI-VFQ-25 Results in Patients with MacTel Type 2
NEI-VFQ-25 Subscale Mean ± SD Median Cronbach’s α* Ceiling n (%) Floor n (%)
General health 56 ± 21 50 N/A, † 18 (8) 3 (1)
General vision 65 ± 17 60 N/A, † 7 (3) 0 (0)
Ocular pain 81 ± 20 88 0.66 85 (38) 0 (0)
Near activities 70 ± 21 75 0.75 24 (11) 0 (0)
Distance activities 76 ± 20 83 0.70 36 (17) 0 (0)
Social functioning 93 ± 14 100 0.70 164 (74) 0 (0)
Mental health 70 ± 25 75 0.75 19 (9) 1 (<1)
Role difficulties 70 ± 26 75 0.74 52 (23) 6 (3)
Dependency 88 ± 20 100 0.79 134 (60) 1 (<1)
Driving, ‡ 74 ± 26 88 0.48 41 (18) 9 (4)
Color vision 97 ± 10 100 N/A, † 201 (91) 0 (0)
Peripheral vision 86 ± 21 100 N/A, † 140 (63) 0 (0)
NEI-VFQ-25 (overall) 77 ± 13 80 0.90 1 (<1) 0 (0)
Table 3.
 
Correlations between Visual Acuity and NEI-VFQ-25 Subscales
Table 3.
 
Correlations between Visual Acuity and NEI-VFQ-25 Subscales
NEI-VFQ-25 Subscale Visual Acuity
Better Eye Worse Eye
General health 0.20* 0.19
General vision 0.34* 0.33*
Ocular pain 0.05 0.04
Near activities 0.38* 0.32*
Distance activities 0.32* 0.27*
Social functioning 0.24* 0.18*
Mental health 0.15 0.16
Role difficulties 0.24* 0.22*
Dependency 0.23* 0.25*
Driving 0.32* 0.27*
Color vision 0.20* 0.14
Peripheral vision 0.29* 0.32*
NEI-VFQ-25 (overall) 0.37* 0.34*
Table 4.
 
Adjusted NEI-VFQ-25 Subscale Scores by Visual Acuity Group*
Table 4.
 
Adjusted NEI-VFQ-25 Subscale Scores by Visual Acuity Group*
NEI-VFQ-25 Subscales 20/32 or Better in Both Eyes Worse Than 20/32 in One Eye Worse Than 20/32 in Both Eyes P for Linear Trend
General health 68 (4.0) 58 (3.4) 57 (3.3) <0.01
General vision 63 (2.9) 62 (2.4) 53 (2.4), † <0.01
Ocular pain 78 (3.7) 82 (3.2) 80 (3.1) 0.75
Near activities 72 (3.6) 68 (3.0) 58 (2.9), † <0.01
Distance activities 73 (3.5) 71 (2.9) 64 (2.8) 0.01
Social functioning 88 (2.5) 88 (2.1) 82 (2.1) 0.02
Mental health 66 (4.3) 65 (3.6) 57 (3.5) 0.04
Role difficulties 67 (4.6) 67 (3.9) 57 (3.8) 0.03
Dependency 90 (3.6) 86 (3.1) 79 (3.0), † <0.01
Driving 77 (4.6) 75 (4.0) 58 (4.1), † <0.01
Color vision 97 (1.9) 94 (1.6) 94 (1.5) 0.08
Peripheral vision 87 (3.9) 82 (3.3) 78 (3.2) 0.02
NEI-VFQ-25 (overall) 77 (2.3) 75 (1.9) 69 (1.9), † <0.01
Table 5.
 
Comparisons of Mean NEI-VFQ-25 Scores of MacTel Type 2 Patients with Other Ocular Disease Cohorts
Table 5.
 
Comparisons of Mean NEI-VFQ-25 Scores of MacTel Type 2 Patients with Other Ocular Disease Cohorts
NEI-VFQ-25 Subscales MacTel Type 2 Patients Other Ophthalmology Patients* MacTel Type 2 Ranking, †
A B C D E F G H
General health 56 72 71 65 46 62 55 45 74 4
General vision 65 79 79 53 62 71 60 76 84 4
Ocular pain 81 87 89 87 88 89 86 90 70 2
Near activities 70 90 85 54 63 79 73 84 86 3
Distance activities 76 89 86 56 66 77 73 84 89 4
Social functioning 93 97 97 73 81 89 87 96 97 5
Mental health 70 85 85 58 66 81 77 74 88 3
Role difficulties 70 89 87 61 69 84 76 78 86 3
Dependency 88 97 97 72 77 92 88 89 98 3
Driving 74 84 85 39 55 75 63 80 86 4
Color vision 97 95 95 85 90 93 90 98 97 7
Peripheral vision 86 89 93 77 78 76 87 78 94 5
Participants, n 222 244 1052 108 123 77 93 37 75
Mean age, y 61 40 71 76 62 67 73 39 46, ‡
% Female 60 79 61 63 66 54 66 5 71
Median visual acuity in better eye, § 32 <20 20 63 40 25 40 20 N/A
×
×

This PDF is available to Subscribers Only

Sign in or purchase a subscription to access this content. ×

You must be signed into an individual account to use this feature.

×