Three-Year Observation of Children 3 to 10 Years of Age with Untreated Intermittent Exotropia.

PURPOSE: To describe the course of intermittent exotropia (IXT) in children followed up without treatment for 3 years. DESIGN: Observation arm from randomized trial of short-term occlusion versus observation. PARTICIPANTS: One hundred eighty-three children 3 to 10 years of age with previously untreated IXT and 400 seconds of arc (arcsec) or better near stereoacuity. METHODS: Participants were to receive no treatment unless deterioration criteria were met at a follow-up visit occurring at 3 months, 6 months, or 6-month intervals thereafter for 3 years. MAIN OUTCOME MEASURES: The primary outcome was deterioration by 3 years, defined as meeting motor criterion (constant exotropia ≥10 prism diopters [Δ] at distance and near) or near stereoacuity criterion (≥2-octave decrease from best previous measure). For the primary analysis, participants also were considered to have deteriorated if treatment was prescribed without meeting either deterioration criterion. RESULTS: The cumulative probability of protocol-specified deterioration by 3 years was 15% (95% confidence interval, 10%-22%), but that was likely an overestimate, partly because of misclassification. Among 25 deteriorations, 2 met motor deterioration, 11 met stereoacuity deterioration, and 12 started treatment without meeting either criteria (7 for social concern, 1 for diplopia, 4 for other reasons). Among the 132 participants who completed the 3-year visit and had not been treated during the study, only 1 (<1%) met motor or stereoacuity deterioration criteria at 3 years. Of the 4 participants completing the 3-year visit who met deterioration criteria previously and had not started treatment, none still met deterioration criteria. Between the baseline and 3-year examination for these 132 patients, improvement occurred in distance and near stereoacuity (mean improvement, 0.14 and 0.14 logarithm of arcsec; P ≤ 0.001 and P ≤ 0.001, respectively), distance exotropia control (mean improvement, 0.6 points; P ≤ 0.001), and distance exodeviation magnitude (mean improvement, 2.2 Δ; P = 0.002). CONCLUSIONS: Among children 3 to 10 years of age with IXT for whom surgery was not considered to be the immediately necessary treatment, stereoacuity deterioration or progression to constant exotropia over 3 years was uncommon, and exotropia control, stereoacuity, and magnitude of deviation remained stable or improved slightly.

A new computer-based pediatric vision-screening test.

BACKGROUND: We developed and validated the Jaeb Visual Acuity Screener (JVAS), a computerized visual acuity-based screening program for children that employs a rapid, age-specific, standardized algorithm for vision screening in the medical home that is available for download at no cost. METHODS: A total of 175 children aged 3 to <8 (median, 6) years were screened with the JVAS before undergoing a complete eye examination (gold standard). The JVAS presented 2 large single surround optotypes (20/100 and 20/80) and then 5 optotypes at a predetermined, age-specific normal threshold. Failure on the gold standard examination was determined using recently published referral criteria and published visual acuity norms for age. We evaluated the sensitivity and specificity of the JVAS for detecting reduced visual acuity, amblyopia, and amblyopia risk factors. JVAS pass/fail paradigms evaluated were inability to identify 3 of 4, 3 of 5, and 4 of 5 age-appropriate optotype presentations. RESULTS: Screening testability for the JVAS was high, at 100%. Sensitivity of the JVAS ranged from 88% to 91%, and specificity from 73% to 86%, with positive predictive value ranging from 66% to 79% and negative predictive value from 92% to 93% (ranges reflect different pass/fail paradigms). CONCLUSIONS: The new JVAS provides an effective and practical method for screening 3- to 7-year-olds using any Windows-based computer. Providing the JVAS free-of-charge to pediatricians and school systems would standardize currently fragmented visual acuity-based screening practices.

Associations between health-related quality of life and the decision to perform surgery for childhood intermittent exotropia.

OBJECTIVE: To assess associations between health-related quality of life (HRQOL) and the decision to perform strabismus surgery for children with intermittent exotropia. DESIGN: Retrospective chart review. PARTICIPANTS: Children with intermittent exotropia. METHODS: Included subjects, identified in a clinical practice, had assessment of HRQOL using the intermittent exotropia questionnaire (IXTQ), comprising child, proxy, and parent components (parent domains: function, psychosocial, and surgery). The IXTQ scores were evaluated for association with surgery, along with standard clinical measures: prism and alternate cover test (PACT), stereoacuity, and control score (mean of the 3 most recent scores). Included data were from preoperative examination (surgical cohort) or from most recent follow-up examination (nonsurgical cohort). Univariate and multivariate logistic regression analyses were performed, and relative risk (RR) ratios were calculated. Spearman rank correlations were calculated to identify highly correlated items. MAIN OUTCOME MEASURES: Association of individual factors with the decision to perform surgery, calculated using RR ratios. RESULTS: One hundred six children with intermittent exotropia (median age, 6 years; range, 2-16 years) were eligible for inclusion. Nineteen (18%) of 106 underwent surgery. Using all available data, the IXTQ proxy score, IXTQ parent function score, IXTQ parent psychosocial score, distance control score, near control score, near PACT, and Randot Preschool stereoacuity (Stereoptical Co, Inc, Chicago, IL) were associated with undergoing surgery (P<0.1). Sixty-nine of 106 patients had complete data on all factors identified in univariate analysis and were included in multivariate analyses. Fourteen (20%) of these 69 patients underwent surgery. In multivariate analyses, poor distance control score (RR, 1.83; 95% confidence interval [CI], 1.25-2.68) and reduced IXTQ parent function score (RR, 0.96; 95% CI, 0.92-0.99) were associated with surgical intervention. Repeat multivariate analyses retaining only 1 of the highly correlated items showed IXTQ proxy, IXTQ parent psychosocial, larger near PACT, and worse near control were also associated with surgery. CONCLUSIONS: After accounting for poorer exodeviation control at distance, reduced parent and proxy HRQOL were associated with undergoing strabismus surgery for childhood intermittent exotropia. Recognizing reduced parental HRQOL may be important, with a possible role for educational or counselling interventions.

Specific health-related quality of life concerns in children with intermittent exotropia.

PURPOSE: To assess the frequency of specific health-related quality of life (HRQOL) concerns in children with intermittent exotropia (XT) using the Intermittent Exotropia Questionnaire (IXTQ). METHODS: Eighty children with intermittent XT (aged 5 to <17 years), plus one parent for each child, completed the Child and Proxy IXTQ respectively. Each item was scored from 0 to 100 (worst to best HRQOL). Mean scores were calculated for each item and items were ranked from lowest to highest impact (worst HRQOL). Analysis was repeated for sub-groups of 5- to <8-year-olds (n = 46) and 8- to <17-year-olds (n = 34). RESULTS: Highest impact Child IXTQ items were: "It bothers me that I have to shut one eye when it is sunny," "I worry about my eyes," and "It bothers me because I have to wait for my eyes to clear up." Highest impact Proxy IXTQ items were: "It bothers my child because he/she has to shut one eye when it is sunny" and "My child worries about his/her eyes." Lowest impact Child and Proxy items were "My eyes make it hard to make friends" and "Kids tease me because of my eyes." Ranking of items was similar for younger and older children. CONCLUSIONS: The greatest HRQOL concerns for children with intermittent XT were shutting one eye when sunny, waiting for their eyes to clear up, and worrying about their eyes. Items related to teasing and making friends were of least concern. Evaluation of HRQOL concerns using the IXTQ may be helpful in the clinical care of individual patients.

Assessing divergence in children with intermittent exotropia.

PURPOSE: To report fusional divergence in children with intermittent exotropia (XT). METHODS: We retrospectively studied 32 children with intermittent XT (aged 4 to 13 years) and 38 visually normal non-strabismic children. Fusional divergence break points were measured using a prism bar, starting from a naturally fused state. Distribution of divergence break points was evaluated. Subnormal fusional divergence was defined as below the 5th percentile in visually normal children. In children with intermittent XT, correlations were calculated between divergence break point and control score, angle of deviation, and convergence break point. RESULTS: The distribution of fusional divergence break points in intermittent XT was normal at near but bimodal at distance. Nine percent had subnormal divergence (<10 prism diopters [pd]) at near, and 48% (<6 pd) at distance. There was a moderate correlation between divergence and convergence break points at near (r = 0.44; P=.01) but only weak inverse correlations between divergence break point and control score (r = -0.29; P = .11) and divergence break point and angle of deviation (r = 0.08; P = . 68) at near. CONCLUSIONS: Most children with intermittent XT have normal near fusional divergence but nearly half have reduced distance fusional divergence. We found the magnitude of near divergence break point correlates with the magnitude of near convergence break point. Measuring divergence may provide useful information about fragility of fusion in patients with intermittent XT. Future studies of fusional divergence are needed to establish whether common measurement methods represent true divergence amplitudes and whether such measures have prognostic significance.

Improvement in academic behaviors after successful treatment of convergence insufficiency.

PURPOSE: To determine whether treatment of symptomatic convergence insufficiency (CI) has an effect on Academic Behavior Survey (ABS) scores. METHODS: The ABS is a six-item survey developed by the Convergence Insufficiency Treatment Trial Group that quantifies the frequency of adverse school behaviors and parental concern about school performance on an ordinal scale from 0 (never) to 4 (always) with total scores ranging from 0 to 24. The ABS was administered at baseline and after 12 weeks of treatment to the parents of 218 children aged 9 to 17 years with symptomatic CI, who were enrolled in the Convergence Insufficiency Treatment Trial and randomized into (1) home-based pencil push-ups; (2) home-based computer vergence/accommodative therapy and pencil push-ups; (3) office-based vergence/accommodative therapy with home reinforcement; and (4) office-based placebo therapy with home reinforcement. Participants were classified as successful (n = 42), improved (n = 60), or non-responder (n = 116) at the completion of 12 weeks of treatment using a composite measure of the symptom score, nearpoint of convergence, and positive fusional vergence. Analysis of covariance methods were used to compare the mean change in ABS between response to treatment groups while controlling for the ABS score at baseline. RESULTS: The mean ABS score for the entire group at baseline was 12.85 (SD = 6.3). The mean ABS score decreased (improved) in those categorized as successful, improved, and non-responder by 4.0, 2.9, and 1.3 points, respectively. The improvement in the ABS score was significantly related to treatment outcome (p < 0.0001), with the ABS score being significantly lower (better) for children who were successful or improved after treatment as compared to children who were non-responders (p = 0.002 and 0.043, respectively). CONCLUSIONS: A successful or improved outcome after CI treatment was associated with a reduction in the frequency of adverse academic behaviors and parental concern associated with reading and school work as reported by parents.

Health-related quality of life in parents of children with intermittent exotropia.

PURPOSE: To assess the health-related quality of life (HRQOL) of parents of children with intermittent exotropia with use of the newly developed condition-specific Parent Intermittent Exotropia Questionnaire (Parent IXTQ) and the generic PedsQL Family Impact Module (PedsQL FIM) and to compare the performance of both instruments. METHODS: One parent was recruited for each of 59 children with intermittent exotropia (age range, 3-16; median, 7 years) and for each of 29 visually normal children (age range, 5-13; median, 8 years). The parent completed the Parent IXTQ and the PedsQL FIM. For each questionnaire, we compared median HRQOL scores between the intermittent exotropia group and visually normal group. We also calculated normal thresholds, which were defined as the 5th percentile score in the cohort of parents of visually normal children. The proportion of subnormal scores between questionnaires in parents of children with intermittent exotropia was compared. RESULTS: The Parent IXTQ score was worse in the intermittent exotropia group than in the visually normal group (70.6 vs 94.1, p < 0.0001), whereas the PedsQL FIM scores were similar (97.9 vs 95.8, p = 0.8). More parents scored below normal when the Parent IXTQ was used than when the PedsQL FIM was used (31% vs 12%; p = 0.008, McNemar's test). CONCLUSIONS: The Parent IXTQ detects subnormal HRQOL in parents of children with intermittent exotropia more often than the PedsQL FIM. It is possible that parental worry may influence management decisions in children with intermittent exotropia and therefore parental HRQOL is worthy of further study.

Spectacle wear in children reduces parental health-related quality of life.

PURPOSE: To investigate whether spectacle wear in children affects responses on the Intermittent Exotropia Questionnaire (IXTQ) or the Pediatric Quality of Life Inventory (PedsQL). METHODS: We recruited 49 children, median age 8 years (range, 5-13 years), presenting with visual acuity 20/40 or better and an otherwise-normal eye examination (no strabismus), who either had no refractive error (n=29) or had refractive error corrected with spectacles (n=20). The IXTQ and PedsQL were completed, each comprising a child report (Child IXTQ, Child PedsQL) and a parent proxy report (Proxy IXTQ, Proxy PedsQL). In addition, the IXTQ contains a parent self-report (Parent IXTQ). Each questionnaire is scored from 0 (worst health-related quality of life [HRQOL]) to 100 (best HRQOL). Median scores were compared using Wilcoxon rank-sum tests. RESULTS: Child IXTQ and Proxy IXTQ scores were similar between groups; nevertheless, Parent IXTQ scores were lower for spectacle wearers (90.4 vs 97.1, p=0.01). Parent IXTQ questions that scored lower in the spectacle group were related to parental worry about permanent damage to their child's eyes, long-term eyesight, surgery, self-consciousness, and teasing. The PedsQL composite scores and all subscale scores were similar between spectacle wearers and nonspectacle wearers, both for Child PedsQL and Proxy PedsQL. CONCLUSIONS: Parental HRQOL, measured with the use of the Parent IXTQ, was lower for children wearing spectacles than for children who did not wear spectacles. There was no difference between spectacle and no-spectacle groups when either Child IXTQ, Proxy IXTQ, Child PedsQL, or Proxy PedsQL was used.

Development and initial validation of quality-of-life questionnaires for intermittent exotropia.

PURPOSE: The development and initial validation of patient-derived, health-related quality-of-life (HRQOL) questionnaires for intermittent exotropia (IXT). DESIGN: Cross-sectional study. PARTICIPANTS: In a development phase, 27 children (age, 2-17 years) with IXT and 1 of their parents. In an initial validation phase, 33 children with IXT and 49 control children (age, 5-17 years), along with 1 parent for each child. Children in the control group had no strabismus or amblyopia. METHODS: Individual patient interviews generated 35 items for child and proxy (parental assessment of child's HRQOL) questionnaires and 46 items for a parent questionnaire. To reduce to a feasible number of items, questionnaires were administered to 5- to 17-year-old children with IXT (n = 15) and parents of 2- to 17-year-old children with IXT (n = 27). Responses were analyzed using standard item reduction methodology. Three final derived IXT questionnaires (IXTQ): child, proxy, and parent (12, 12, and 17 items, respectively) were administered to children with IXT and control children and to parents of IXT and control children. Likert-type scales ranging from never (100, best HRQOL) to almost always (0, worst HRQOL) were used. MAIN OUTCOME MEASURES: Median scores for IXT and control groups, compared using Wilcoxon tests. RESULTS: Median child scores were significantly lower (worse HRQOL) in the IXT group compared with the control group: 85 (quartiles, 73-92) versus 92 (quartiles, 79-96; P = 0.04). Median proxy IXTQ scores were significantly lower for IXT children than controls: 83 (quartiles, 75-94) versus 98 (quartiles, 92-100; P<0.0001). Median parent IXTQ scores also were significantly lower in the IXT group compared with the control group: 68 (quartiles, 56-79) versus 93 (quartiles, 87-99; P<0.0001). CONCLUSIONS: A new 3-part patient-derived HRQOL questionnaire for children with IXT and their parents has been developed and validated, comprising child, proxy, and parent questionnaires. These questionnaires detect reduced HRQOL in children with IXT as reported by the children themselves and perceived by their parents (proxy report). Childhood IXT also seems to affect parent HRQOL. The IXTQ HRQOL questionnaires may prove useful in the clinical assessment of IXT and for clinical trials. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Adaptive radiation of gobies in the interstitial habitats of gravel beaches accompanied by body elongation and excessive vertebral segmentation.

BACKGROUND: The seacoasts of the Japanese Arc are fringed by many gravel beaches owing to active tectonic uplift and intense denudation caused by heavy rainfall. These gravel beaches are inhabited by gobies of the genus Luciogobius that burrow into the gravel sediment and live interstitially. Although their habitat and morphology (e. g., reduced fins, elongated, scale-less body, and highly segmented vertebral column) are highly unusual among fishes, little is known on how their morphological evolution has facilitated the colonization of interstitial habitats and promoted extensive diversification. We conducted thorough sampling of Luciogobius and related species throughout Japan, and performed molecular phylogenetic analysis to explore the patterns of morphological evolution associated with gravel beach colonization. RESULTS: An analysis of the mitochondrial cytochrome b gene suggested a remarkable diversity of previously unrecognized species. The species-level phylogeny based on six protein-coding nuclear genes clearly indicated that interstitial species cluster into two distinct clades, and that transitions from benthic or demersal habits to interstitial habits are strongly correlated with an increase in vertebral number. Colonization of gravel beach habitats is estimated to have occurred ca. 10 Ma, which coincides with the period of active orogenesis of the Japanese landmass. Different species of interstitial Luciogobius inhabit sediments with different granulometric properties, suggesting that microhabitat partitioning has been an important mechanism facilitating speciation in these fishes. CONCLUSION: This is the first study to document the adaptation to interstitial habitats by a vertebrate. Body elongation and excessive vertebral segmentation had been the key aspects enhancing body flexibility and fishes' ability to burrow into the gravel sediment. The rich diversity of coastal gravel habitats of the Japanese Arc has likely promoted the adaptive radiation of these unique gravel-dwelling fishes.

Validity of the convergence insufficiency symptom survey: a confirmatory study.

PURPOSE: The objectives of the present study were to evaluate whether investigator bias influenced the Convergence Insufficiency Symptom Survey (CISS) scores of children with normal binocular vision (NBV) in our original validation study, reevaluate the usefulness of the cutoff score of 16, and reexamine the validity of the CISS. METHODS: Six clinical sites participating in the Convergence Insufficiency Treatment Trial (CITT) enrolled 46 children 9 to <18 years with NBV. Examiners masked to the child's binocular vision status administered the CISS. The mean CISS score was compared with that from the children with NBV in the original, unmasked CISS study and also to that of the 221 symptomatic convergence insufficiency (CI) children enrolled in the CITT. RESULTS: The mean (+/-standard deviation) CISS score for 46 subjects with NBV was 10.4 (+/-8.1). This was comparable with our prior unmasked NBV study (mean = 8.1 (+/-6.2); p = 0.11) but was significantly different from that of the CITT CI group (mean = 29.8 +/- 9.0; p < 0.001). Eighty-three percent of these NBV subjects scored <16 on the CISS, which is not statistically different from the 87.5% found in the original unmasked study (p = 0.49). CONCLUSIONS: Examiner bias did not affect the CISS scores for subjects with NBV in our prior study. The CISS continues to be a valid instrument for quantifying symptoms in 9 to <18-year-old children. These results also confirm the validity of a cut-point of > or = 16 in distinguishing children with symptomatic CI from those with NBV.

A comparison of stereopsis testing between red/green targets and polarized targets in children with normal binocular vision.

BACKGROUND: Measurement of stereopsis is important in assessing a patient's binocular status. Several measurement methods are available, most commonly using polarized targets. Recently, less expensive red/green targets have become available. In this study, we compare polarized versus red/green methods, using random dot and contour targets. METHODS: Sixty children with no strabismus, amblyopia, or high refractive error and normal ocular health were recruited. Stereopsis measurements were taken using the red/green and polarized versions of the Random Dot Letter "E"/RDE Test, Random Dot Butterfly/Stereo Butterfly Test, Stereo Circles/Wirt Circles, Stereo Numbers, and Stereo Animals tests. Observed agreement was used to assess agreement between results. Wilcoxon signed-rank tests were used to compare an individual's response with both targets. RESULTS: There was greater than 95% agreement using any of the Random Dot-based tests and the Stereo Animals tests. However, agreement was less than 60% with the Stereo Numbers test and less than 35% with the Stereo Circles/Wirt Circles test. CONCLUSION: The red/green versions of the Random Dot-based tests and the Stereo Animals test appear to be a cost-effective alternative to their polarized equivalents. Our data, however, show that the red/green versions tend to underestimate the level of stereopsis when using the Stereo Numbers and Stereo Circles/Wirt Circles tests compared to their polarized equivalents.