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Reduced health-related quality of life correlates to diplopia severity in patients with thyroid eye disease
Volume 14 Number 1 / February 2010 70D (mean 53D). Vision in each eye ranged from 20/120 Snellen to light perception, except one patient with Usher syndrome had grossly restricted visual fields and vision of 20/40 and 20/200. The etiology was retinitis pigmentosa in 5 patients, 3 had cone dystrophy, 2 were high myopes. Other etiologies included CMV retinitis in both eyes secondary to HIV, bilateral optic atrophy post craniopharyngioma and cortical visual impairment. All patients were unable to actually see their strabismus but were aware of its presence from family members and friends, and wished improvement. Eight patients underwent botulinum toxin injections; 2 of these then proceeded to surgery. A total of 7 patients had surgery. Postoperative angles ranged from 6D to 25D of esotropia. Five patients continued with maintenance toxin. Follow-up ranged from 3 months to 15 years. Conclusions: This is the first report of patients with bilaterally severely reduced vision requesting treatment for strabismus that they were unable to see for themselves. 056 Vessel Width in Type 1 ROP: Zone I vs Zone II. Emily A. DeCarlo, Gui-shuang Ying, Graham E. Quinn, Diego Fiorin, Alfredo Ruggeri, Karen Karp, Monte D. Mills Introduction: Type 1 retinopathy of prematurity (ROP) as defined by ETROP encompasses disease in zone 1 with or without plus disease and zone II with plus disease. Our hypothesis is that the width of posterior retinal vessels in eyes with Type 1 ROP do not differ when the retinopathy occurs in zone 1 compared to zone 2. Methods: Right eyes of 32 patients identified as Type 1 ROP were included. Images were captured just prior to laser photocoagulation using an NM200D camera (Nidek Inc., Gamagori, Japan). A computerassisted algorithm, ROPnet, developed by D Fiorin and A Ruggeri, University of Padua, was used to measure tortuosity, width, and standard deviation of width of up to 8 vessels from each image. Vessel parameters were compared between the group of Type 1 eyes with zone 1 ROP and Type 1 eyes with zone 2 ROP using two-group t-tests. Results: Of these 32 eyes analyzed, Type 1 ROP was present in zone 1 in 13 eyes and in zone 2 in 19. Gestational age and birth weight were similar between the two groups. As expected, the post-menstrual age at the time of the photographs was significantly different, since Type 1 disease in zone 1 presents earlier than in zone II. All of the 19 zone 2 eyes had plus disease compared with 7 of the 13 zone 1 eyes. The width of venules and arterioles was not significantly different between the Type 1 ROP eyes in zone 1 and in zone 2. Conclusions: Using the digital vessel analysis program ROPnet, width measurements of posterior retinal vessels does not differentiate Type 1 ROP in zone 1 from Type 1 ROP in zone 2. This suggests that vessel width of the group of eyes with Type 1 ROP in zone 1 is not different from the group of eyes with Type 1 ROP in zone 2 using only posterior pole images from the NM200D camera. 057 Anatomy of superior oblique (SO) palsy in primate model: rapid overall atrophy with relative sparing of orbital layer. Joseph L. Demer, Vadims Poukens, Howard Ying, X. Shan, J. Tian, David S. Zee Introduction: We employed magnetic resonance imaging (MRI) and whole orbital histology to study effects of trochlear neurectomy on the monkey SO muscle. Methods: Five normal adult macaque monkeys underwent unilateral or bilateral lesion by removal of 10 mm of subarachnoid trochlear nerve 5-72 weeks before sacrifice. Lesioned animals exhibited typical strabismus. Orbits were imaged bilaterally by MRI, embedded whole, serially sectioned, and stained with Masson trichrome. We compared whole muscle and individual fiber cross sections in ipsilesional and normal SO muscles throughout the orbit.
Journal of AAPOS
e15 Results: Both MRI and histology showed highly significant (p \ 0.000001) , persistent atrophy of the SO belly in all animals complete within 5 weeks post-lesion. Atrophy of 30% to 60% was most severe in global fibers and varied along the SO length, relatively sparing orbital fibers, particularly late. Affected SO bulk shifted anteriorly, consistent with elongation of the muscle belly, and preserving SO volume even after denervation. Microscopy showed ipsilesional fibrosis of all trochlear nerve branches. In one animal, scattered clusters of hypertrophic global layer fibers extending the entire SO length suggested reinnervation from small nerves traveling from the orbital layer. Discussion: MRI detects, and histology confirms, striking but partial and persistent SO belly atrophy fully established by 5 weeks after acquired SO palsy in monkey. This atrophy is mainly in the oculorotary global fibers, relatively sparing orbital fibers that insert on the SO sheath and pulley system. Residual or re-innervating fibers do not travel in the trochlear nerve. Denervated SO muscles elongate as they thin. Conclusions: MRI can confirm partial SO atrophy that becomes complete shortly after acquired SO palsy onset, supporting use of MRI in clinical diagnosis of SO palsy. More extreme SO size reduction probably indicates congenital SO hypoplasia. Elongation of denervated SO muscles would explain ''floppy'' tendons observed at surgery for SO palsy, but would not indicate that the palsy was congenital. 058 Pediatrician experience with the Plusoptix vision screener: An observational clinical trial. Sean P. Donahue, Niraj R. Nathan, Kathryn E. Allen Introduction: To determine the usefulness of the Plusoptix photoscreener in the pediatric office. Methods: Four-hundred fifty seven children were screened using the Plusoptix screener with the manufacturer's referral criteria in five pediatric practices. Referred children received a comprehensive ophthalmic examination. Follow-up information was determined by chart review at the individual pediatric practices or by review of electronic medical record at our institution. Screening results were compared using AAPOS Vision Screening Committee amblyogenic factors and were also evaluated using other proposed referral criteria for the Plusoptix instrument. Results: Of the 457 children screened, 94 were referred by the Plusoptix screener (21%). Only 19% of children had documented follow-up, and the PPV was 27% in this group. Applying the criteria proposed by Arthur1 (Anisometropia $1.5 D, Hyperopia .3.5 D, Myopia .3.0 D, Astigmatism .1.5 D, Anisocoria . 1 mm) decreased referral rate. Modifying the Arthur criteria (increase cylinder to .2.0 D) lowered the referral rate to 10%. Conclusions: The manufacturer's criteria led to an excessively high referral rate and low PPV. The referral criteria proposed by Arthur led to a high referral rate, while the modified Arthur criteria significantly reduced referral rate, suggesting that the Plusoptix screener overestimates astigmatism. Confirmation of follow-up was found in only a minority of referred patients' charts, indicating a concerning problem of low follow-up rates and/or low confirmation of follow-up by the pediatric practices, which has been demonstrated in other studies evaluating vision screening in the pediatric home. 059 Reduced health-related quality of life correlates to diplopia severity in patients with thyroid eye disease. Noha S. Ekdawi, Elizabeth A. Bradley, Sarah R. Hatt, David A. Leske, Jonathan M. Holmes Introduction: Diplopia may be a specific cause of reduced health-related quality of life (HRQOL) in thyroid eye disease (TED). We evaluated the impact of diplopia on HRQOL in patients with TED.
e16 Methods: HRQOL was assessed in 57 patients with TED using a new disease specific questionnaire (Thyroid Eye Disease Patient Reported Outcome, TED-PRO) scored from 0 to 100 (worst to best HRQOL). Diplopia was quantified using 3 previously described methods: the cervical range of motion device (CROM), Goldmann perimeter, and diplopia questionnaire (DQ), each yielding scores from 0 (no diplopia) to 100 (diplopia in all fields). Results: Overall, HRQOL scores ranged from 16 to 89 and diplopia scores ranged from 0 to 100 for each measure of diplopia. Reduced HRQOL was best correlated to increasing severity of diplopia measured by the DQ (r 5 0.38; p 5 0.004) and was also correlated to increased CROM scores (r 5 0.31, p 5 0.02). Overall, HRQOL did not correlate to diplopia scores using the Goldmann (r 5 0.23, p 5 0.09), due to a tendency for the Goldmann to both over- and underestimate diplopia, depending on the clinical scenario. Supporting the construct validity of the TED-PRO, questions specifically addressing diplopia and depth perception showed good correlation with diplopia scores, and questions not addressing diplopia or depth perception were poorly correlated with diplopia scores. Conclusions: In patients with Graves' ophthalmopathy, reduced HRQOL is correlated with severity of diplopia when quantified using the CROM and DQ. The TED-PRO is a useful new questionnaire to assess HRQOL in patients with thyroid eye disease.
060 Binocular visual function following boston type 1 keratoprostheses. Noa Ela-Dalman, Stacy L. Pineles, Arthur L. Rosenbaum, Anthony J. Aldave, Federico G. Velez Purpose: To report the postoperative binocular function of patients with Boston type I keratoprosthesis implantation for unilateral visual impairment. Methods: All patients who underwent implantation of a Boston type I keratoprosthesis, and had a best-corrected visual acuity $20/50 in the contralateral eye prior to surgery, were identified through a retrospective chart review. Patients prospectively underwent sensory testing of binocular function including Bagiolini lenses, Worth-4dot test, stereoacuity at distance and near, and double Maddox rods. In addition, assessments of ocular alignment and ocular rotations were performed on each of the subjects. Results: Twenty patients with best-corrected visual acuity $ 20/50 in the contralateral eye at the time of keratoprosthesis surgery were identified. Seventeen patients underwent binocular visual testing, with 16/17 (94%) demonstrating binocular function. Second-degree fusion at near was demonstrated via the Worth-4-dot test in 13/17 (76%) of the patients. Third degree fusion at near was demonstrated in 7/17 (41%) of patients. Patients with better post-operative sensory binocular function tended to be of younger age (p 5 0.05) and have better post-operative visual acuity (p 5 0.006). Five patients were found to have some degree of ocular misalignment. Overall, patients with strabismus had worse binocularity (p 5 0.04). Discussion: Implantation of the Boston type I keratoprosthesis in patients with good preoperative visual acuity in the fellow eye is associated with recovery of useful binocular function in greater than 90% of patients in this series. Conclusions: Keratoprosthesis implantation should be considered in this population of patients, and should not be reserved only for patients with binocular visual impairment. 061 Reproducibility of OCT in children with glaucoma. Mays A. ElDairi, Susana Noval, Dana M. Hornbeak, Sandra Holgado, Sanjay Asrani, Sharon F. Freedman
Volume 14 Number 1 / February 2010 Introduction: Optical Coherence Tomography(OCT) has been proven to be a useful clinical tool in the follow-up of adult glaucoma(IOVS2008;49:4886-4892.). Its use in pediatric glaucoma is limited because reproducibility has been assessed only in normal children and on the same visit (AJO,2007;143:484–488). This study's purpose is to evaluate the reproducibility of OCT in children with stable glaucoma in a clinical setting. Methods: OCT is used as part of standard care for childhood glaucoma patients treated at XXX pediatric ophthalmology clinic. Subjects with stable disease and who could perform OCT were enrolled prospectively and OCT-3 measures of both the Fast Macular Thickness and the Fast Retinal Nerve Fiber Layer (RNFL) 3.4 Thickness protocols were collected annually. Reproducility measures were assessed on subjects with .3 sequential scans. Results: Eighteen subjects with childhood glaucoma had 3-5 reliable fast macular thickness scans (age at enrollment, 4-16 years; mean, 9.5 SD 3.1). Mean macular volume was 6.8 0.55 mm3. Intraclass correlation coefficient was 95.8% with intervisit error of 0.1 mm3. Six subjects had 3 reliable fast RNFL scans (mean age 10.2 2.8 (6-13) years). Mean RNFL thickness was 90.92 39.51. Intraclass correlation coefficient 90.9% with intervisit error of 13.4 mm. None of the subjects enrolled had a consistent trend towards RNFL or macular thinning or thickening. Conclusions: OCT has an acceptable reproducibility rate for clinical monitoring of children with glaucoma. Macular scans are easier to obtain and are more reproducible than RNFL scans. Further study comparing variability between normal children and those with glaucoma is underway.
062 Vision-related quality-of-life in children with albinism from 0 to 10 years of age. Joost Felius, Ann M. Holleschau, C. Gail Summers, Andrew C. Bowman, Pooja V. Patel Introduction: Individuals with albinism show mild to severe visual acuity (VA) deficits from an early age. Compromised visual functioning beyond the VA deficit results from poor foveal development, cortical changes, nystagmus and photosensitivity. Because these effects on functioning are difficult to assess in children, we evaluated vision-related quality-of-life (VR-QOL). Methods: Parents of 91 children with albinism (ages 4 months-10 years) and 104 age-similar normals were given the Children's Visual Function Questionnaire (CVFQ). Z-scores (number of standard deviations below normal) were derived for the Competence, Personality, Family, and Total scales. Binocular VA was tested with Teller cards or optotypes and categorized using published age-specific norms. General Linear Model analysis was performed to assess the associations between CVFQ Z-scores, VA deficit category, age, and type of albinism. Results: Overall, VA was moderately reduced, averaging 0.55 0.30 logMAR below normal limits. Mean CVFQ z-scores were 0.7 (ie, within normal limits) for the Personality scale, 4.2 for the Family scale and 8.6 for the Competence scale, with no significant age effects. Children with OCA1 (n 5 40) showed larger VA deficits (p 5 0.02) and scored poorer on the Competence scale (z 5 10.5 vs z 5 7.1, p 5 0.002) than other patients in this cohort. There was an association between VA deficit category and Z-scores only for the Competence (p 5 0.001) and Total (p 5 0.02) scales. Conclusions: From early age, deficits in binocular VA were accompanied by severe reductions in VR-QOL as measured by the Family scale and most notably the Competence scale. Patients with OCA1 generally scored poorer on both VA and VR-QOL.
Journal of AAPOS
Journal of AAPOS
e15 Results: Both MRI and histology showed highly significant (p \ 0.000001) , persistent atrophy of the SO belly in all animals complete within 5 weeks post-lesion. Atrophy of 30% to 60% was most severe in global fibers and varied along the SO length, relatively sparing orbital fibers, particularly late. Affected SO bulk shifted anteriorly, consistent with elongation of the muscle belly, and preserving SO volume even after denervation. Microscopy showed ipsilesional fibrosis of all trochlear nerve branches. In one animal, scattered clusters of hypertrophic global layer fibers extending the entire SO length suggested reinnervation from small nerves traveling from the orbital layer. Discussion: MRI detects, and histology confirms, striking but partial and persistent SO belly atrophy fully established by 5 weeks after acquired SO palsy in monkey. This atrophy is mainly in the oculorotary global fibers, relatively sparing orbital fibers that insert on the SO sheath and pulley system. Residual or re-innervating fibers do not travel in the trochlear nerve. Denervated SO muscles elongate as they thin. Conclusions: MRI can confirm partial SO atrophy that becomes complete shortly after acquired SO palsy onset, supporting use of MRI in clinical diagnosis of SO palsy. More extreme SO size reduction probably indicates congenital SO hypoplasia. Elongation of denervated SO muscles would explain ''floppy'' tendons observed at surgery for SO palsy, but would not indicate that the palsy was congenital. 058 Pediatrician experience with the Plusoptix vision screener: An observational clinical trial. Sean P. Donahue, Niraj R. Nathan, Kathryn E. Allen Introduction: To determine the usefulness of the Plusoptix photoscreener in the pediatric office. Methods: Four-hundred fifty seven children were screened using the Plusoptix screener with the manufacturer's referral criteria in five pediatric practices. Referred children received a comprehensive ophthalmic examination. Follow-up information was determined by chart review at the individual pediatric practices or by review of electronic medical record at our institution. Screening results were compared using AAPOS Vision Screening Committee amblyogenic factors and were also evaluated using other proposed referral criteria for the Plusoptix instrument. Results: Of the 457 children screened, 94 were referred by the Plusoptix screener (21%). Only 19% of children had documented follow-up, and the PPV was 27% in this group. Applying the criteria proposed by Arthur1 (Anisometropia $1.5 D, Hyperopia .3.5 D, Myopia .3.0 D, Astigmatism .1.5 D, Anisocoria . 1 mm) decreased referral rate. Modifying the Arthur criteria (increase cylinder to .2.0 D) lowered the referral rate to 10%. Conclusions: The manufacturer's criteria led to an excessively high referral rate and low PPV. The referral criteria proposed by Arthur led to a high referral rate, while the modified Arthur criteria significantly reduced referral rate, suggesting that the Plusoptix screener overestimates astigmatism. Confirmation of follow-up was found in only a minority of referred patients' charts, indicating a concerning problem of low follow-up rates and/or low confirmation of follow-up by the pediatric practices, which has been demonstrated in other studies evaluating vision screening in the pediatric home. 059 Reduced health-related quality of life correlates to diplopia severity in patients with thyroid eye disease. Noha S. Ekdawi, Elizabeth A. Bradley, Sarah R. Hatt, David A. Leske, Jonathan M. Holmes Introduction: Diplopia may be a specific cause of reduced health-related quality of life (HRQOL) in thyroid eye disease (TED). We evaluated the impact of diplopia on HRQOL in patients with TED.
e16 Methods: HRQOL was assessed in 57 patients with TED using a new disease specific questionnaire (Thyroid Eye Disease Patient Reported Outcome, TED-PRO) scored from 0 to 100 (worst to best HRQOL). Diplopia was quantified using 3 previously described methods: the cervical range of motion device (CROM), Goldmann perimeter, and diplopia questionnaire (DQ), each yielding scores from 0 (no diplopia) to 100 (diplopia in all fields). Results: Overall, HRQOL scores ranged from 16 to 89 and diplopia scores ranged from 0 to 100 for each measure of diplopia. Reduced HRQOL was best correlated to increasing severity of diplopia measured by the DQ (r 5 0.38; p 5 0.004) and was also correlated to increased CROM scores (r 5 0.31, p 5 0.02). Overall, HRQOL did not correlate to diplopia scores using the Goldmann (r 5 0.23, p 5 0.09), due to a tendency for the Goldmann to both over- and underestimate diplopia, depending on the clinical scenario. Supporting the construct validity of the TED-PRO, questions specifically addressing diplopia and depth perception showed good correlation with diplopia scores, and questions not addressing diplopia or depth perception were poorly correlated with diplopia scores. Conclusions: In patients with Graves' ophthalmopathy, reduced HRQOL is correlated with severity of diplopia when quantified using the CROM and DQ. The TED-PRO is a useful new questionnaire to assess HRQOL in patients with thyroid eye disease.
060 Binocular visual function following boston type 1 keratoprostheses. Noa Ela-Dalman, Stacy L. Pineles, Arthur L. Rosenbaum, Anthony J. Aldave, Federico G. Velez Purpose: To report the postoperative binocular function of patients with Boston type I keratoprosthesis implantation for unilateral visual impairment. Methods: All patients who underwent implantation of a Boston type I keratoprosthesis, and had a best-corrected visual acuity $20/50 in the contralateral eye prior to surgery, were identified through a retrospective chart review. Patients prospectively underwent sensory testing of binocular function including Bagiolini lenses, Worth-4dot test, stereoacuity at distance and near, and double Maddox rods. In addition, assessments of ocular alignment and ocular rotations were performed on each of the subjects. Results: Twenty patients with best-corrected visual acuity $ 20/50 in the contralateral eye at the time of keratoprosthesis surgery were identified. Seventeen patients underwent binocular visual testing, with 16/17 (94%) demonstrating binocular function. Second-degree fusion at near was demonstrated via the Worth-4-dot test in 13/17 (76%) of the patients. Third degree fusion at near was demonstrated in 7/17 (41%) of patients. Patients with better post-operative sensory binocular function tended to be of younger age (p 5 0.05) and have better post-operative visual acuity (p 5 0.006). Five patients were found to have some degree of ocular misalignment. Overall, patients with strabismus had worse binocularity (p 5 0.04). Discussion: Implantation of the Boston type I keratoprosthesis in patients with good preoperative visual acuity in the fellow eye is associated with recovery of useful binocular function in greater than 90% of patients in this series. Conclusions: Keratoprosthesis implantation should be considered in this population of patients, and should not be reserved only for patients with binocular visual impairment. 061 Reproducibility of OCT in children with glaucoma. Mays A. ElDairi, Susana Noval, Dana M. Hornbeak, Sandra Holgado, Sanjay Asrani, Sharon F. Freedman
Volume 14 Number 1 / February 2010 Introduction: Optical Coherence Tomography(OCT) has been proven to be a useful clinical tool in the follow-up of adult glaucoma(IOVS2008;49:4886-4892.). Its use in pediatric glaucoma is limited because reproducibility has been assessed only in normal children and on the same visit (AJO,2007;143:484–488). This study's purpose is to evaluate the reproducibility of OCT in children with stable glaucoma in a clinical setting. Methods: OCT is used as part of standard care for childhood glaucoma patients treated at XXX pediatric ophthalmology clinic. Subjects with stable disease and who could perform OCT were enrolled prospectively and OCT-3 measures of both the Fast Macular Thickness and the Fast Retinal Nerve Fiber Layer (RNFL) 3.4 Thickness protocols were collected annually. Reproducility measures were assessed on subjects with .3 sequential scans. Results: Eighteen subjects with childhood glaucoma had 3-5 reliable fast macular thickness scans (age at enrollment, 4-16 years; mean, 9.5 SD 3.1). Mean macular volume was 6.8 0.55 mm3. Intraclass correlation coefficient was 95.8% with intervisit error of 0.1 mm3. Six subjects had 3 reliable fast RNFL scans (mean age 10.2 2.8 (6-13) years). Mean RNFL thickness was 90.92 39.51. Intraclass correlation coefficient 90.9% with intervisit error of 13.4 mm. None of the subjects enrolled had a consistent trend towards RNFL or macular thinning or thickening. Conclusions: OCT has an acceptable reproducibility rate for clinical monitoring of children with glaucoma. Macular scans are easier to obtain and are more reproducible than RNFL scans. Further study comparing variability between normal children and those with glaucoma is underway.
062 Vision-related quality-of-life in children with albinism from 0 to 10 years of age. Joost Felius, Ann M. Holleschau, C. Gail Summers, Andrew C. Bowman, Pooja V. Patel Introduction: Individuals with albinism show mild to severe visual acuity (VA) deficits from an early age. Compromised visual functioning beyond the VA deficit results from poor foveal development, cortical changes, nystagmus and photosensitivity. Because these effects on functioning are difficult to assess in children, we evaluated vision-related quality-of-life (VR-QOL). Methods: Parents of 91 children with albinism (ages 4 months-10 years) and 104 age-similar normals were given the Children's Visual Function Questionnaire (CVFQ). Z-scores (number of standard deviations below normal) were derived for the Competence, Personality, Family, and Total scales. Binocular VA was tested with Teller cards or optotypes and categorized using published age-specific norms. General Linear Model analysis was performed to assess the associations between CVFQ Z-scores, VA deficit category, age, and type of albinism. Results: Overall, VA was moderately reduced, averaging 0.55 0.30 logMAR below normal limits. Mean CVFQ z-scores were 0.7 (ie, within normal limits) for the Personality scale, 4.2 for the Family scale and 8.6 for the Competence scale, with no significant age effects. Children with OCA1 (n 5 40) showed larger VA deficits (p 5 0.02) and scored poorer on the Competence scale (z 5 10.5 vs z 5 7.1, p 5 0.002) than other patients in this cohort. There was an association between VA deficit category and Z-scores only for the Competence (p 5 0.001) and Total (p 5 0.02) scales. Conclusions: From early age, deficits in binocular VA were accompanied by severe reductions in VR-QOL as measured by the Family scale and most notably the Competence scale. Patients with OCA1 generally scored poorer on both VA and VR-QOL.
Journal of AAPOS