Normal vision despite narrowing of the optic canal in fibrous dysplasia.
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BACKGROUND: Fibrous dysplasia of bone frequently involves the anterior base of the cranium and results in encasement of the optic-nerve canals. It has been assumed that such encasement leads to constriction and eventual blindness. There is controversy about whether patients should be regularly monitored or whether they should undergo prophylactic decompression of the optic nerve. This question is of particular concern in patients with normal vision, since the risks associated with surgical decompression include blindness. METHODS: We studied 38 patients with fibrous dysplasia of the lesser wing of the sphenoid bone. The patients underwent a detailed neuro-ophthalmologic examination and computed tomography of the face and skull, reformatted to measure the extent of involvement of the optic canal and the area of the canals. The results were compared with those of 38 age- and sex-matched controls. RESULTS: Of the 38 patients, 15 were male and 23 female, and their mean age was 26 years. Twelve had polyostotic fibrous dysplasia, and 26 had the McCune-Albright syndrome. Sixty-seven optic canals were affected by fibrous dysplasia; in 49 of them (73 percent) there was complete encasement. The mean (+/-SD) areas of the right and left canals were 9.6+/-3.8 mm2 and 9.9+/-3.6 mm2, respectively, in the patients, as compared with 12.0+/-2.9 mm2 and 11.9+/-2.7 mm2 in the controls (P=0.009 for the comparison of the right areas and P=0.03 for the comparison of the left areas by the paired t-test). In all but two of the patients, the results of neuro-ophthalmologic examination were normal. In the two patients with monocular visual impairment, the areas of the optic canals were similar on the normal and abnormal sides. CONCLUSIONS: Encasement of the optic canal in fibrous dysplasia causes narrowing of the canal, but that in itself does not result in visual loss. Therefore, prophylactic decompression of the optic nerve does not appear to be indicated on the basis of the presence of fibrous dysplasia on diagnostic images alone, since it does not correlate with visual loss. (+info)
Relation between colour vision loss and occupational styrene exposure level.
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AIMS: To investigate the relation between colour vision loss and the exposure level of styrene. Exposure level included the current exposure concentration, past cumulative exposure, and the maximum exposure level in the past. METHODS: Colour vision was examined by the Lanthony desaturated panel D-15 test for 76 subjects exposed to styrene in a fibreglass reinforced plastics boat plant (as an exposed group) and 102 non-exposed subjects (as a control group). The current exposure level was expressed by the concentration of atmospheric styrene and end shift urinary mandelic acid (MA) and phenylglyoxylic acid (PGA) levels. The individual cumulative exposure index (CEI) was calculated, based on the exposure frequency and urinary MA concentrations measured for the past eight years. RESULTS: The Colour Confusion Index (CCI) of the exposed group showed a significant difference from the age matched controls. However, only a slight significant relation was found between CCI and the concentration of urinary MA plus PGA. In this study, the exposed group was further divided into two subgroups (as sub-MA+PGA groups) by the median of urinary MA plus PGA of each subject. The dividing line between the subgroups was 0.24 g/g creatinine, which was equivalent to an atmospheric concentration of styrene of about 10 ppm. The CCI values of both the sub-MA+PGA groups were significantly higher than that of the control group. The relation between CCI value and the maximum exposure concentration in the past eight years was examined. It was found that the CCI values of the group with the maximum exposure concentration of styrene over 50 ppm were significantly higher than that of the other groups. CONCLUSIONS: Exposure to styrene would impair colour vision even if the exposure concentration was lower than 10 ppm. Furthermore, if the maximum concentration of styrene exposure transiently exceeded 50 ppm in the past, the styrene related damage might remain. Thus, the safe limit of exposure to styrene and the relation between exposure to styrene and the degree of damage to ocular structure, retina, optic nerve, and brain need to be re-examined. (+info)
Measurement error of visual field tests in glaucoma.
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AIM: Psychophysical strategies designed for clinical visual field testing produce rapid estimates of threshold with relatively few stimulus presentations and so represent a trade-off between test quality and efficiency. The aim of this study was to determine the measurement error of a staircase algorithm similar to full threshold with standard automated perimetry (SAP) and frequency doubling perimetry (FDP) in glaucoma patients. METHODS: Seven patients with early open angle glaucoma (OAG) were prospectively recruited. All were experienced in laboratory based psychophysics. Three matched test locations were examined with SAP (externally driven Humphrey field analyser) and FDP (CRT) in a single arbitrarily selected eye of each subject. Each location was tested twice with a 4-2-2 dB staircase strategy, similar to full threshold, and then with the method of constant stimuli (MOCS). Accuracy (threshold estimation error) was quantified by determination of differences between "true" threshold measurements made by MOCS and single staircase threshold estimates. Precision (repeatability) was quantified by the differences between repeated staircase threshold estimates. RESULTS: Precision was relatively high for both tests, although higher for FDP than SAP at depressed sensitivity levels. The staircase strategy significantly underestimated threshold sensitivity for both test types, with the mean difference (95% CI) between staircase and MOCS thresholds being 4.48 dB (2.35 to 7.32) and 1.35 dB (0.56 to 1.73) for SAP and FDP respectively. Agreement levels (weighted kappa) between MOCS and staircase thresholds were found to be 0.48 for SAP and 0.85 for FDP. Although this "bias" appeared constant for FDP across all sensitivity levels, this was not the case for SAP where accuracy decreased at lower sensitivity levels. CONCLUSION: Estimations of threshold sensitivity made using staircase strategies common to clinical visual field test instrumentation are associated with varying degrees of measurement error according to visual field test type and sensitivity. In particular, SAP significantly overestimates the "true" level of sensitivity, particularly in damaged areas of the visual field, suggesting that clinical data of this type should be interpreted with caution. (+info)
Visual factors should be assessed in older people presenting with falls or hip fracture.
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Visual impairment, although not routinely assessed, is an important risk factor for falls and hip fracture in older people. Impaired vision is highly prevalent and commonly unreported in the elderly population particularly in women and those living in nursing homes. Measurement of visual functions such as visual acuity, contrast sensitivity and depth perception may identify older people at risk of falls and hip fracture. Visual loss in older people is correctable in most cases. Intervention strategies, for example, change of glasses or cataract extraction may have the potential of improving visual function and preventing falls in older people. (+info)
Effect of a patient training video on visual field test reliability.
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AIMS: To evaluate the effect of a visual field test educational video on the reliability of the first automated visual field test of new patients. METHODS: A prospective, randomised, controlled trial of an educational video on visual field test reliability of patients referred to the hospital eye service for suspected glaucoma was undertaken. Patients were randomised to either watch an educational video or a control group with no video. The video group was shown a 4.5 minute audiovisual presentation to familiarize them with the various aspects of visual field examination with particular emphasis on sources of unreliability. Reliability was determined using standard criteria of fixation loss rate less than 20%, false positive responses less than 33%, and false negative responses less than 33%. RESULTS: 244 patients were recruited; 112 in the video group and 132 in the control group with no significant between group difference in age, sex, and density of field defects. A significant improvement in reliability (p=0.015) was observed in the group exposed to the video with 85 (75.9%) patients having reliable results compared to 81 (61.4%) in the control group. The difference was not significant for the right (first tested) eye with 93 (83.0%) of the visual fields reliable in the video group compared to 106 (80.0%) in the control group (p = 0.583), but was significant for the left (second tested) eye with 97 (86.6 %) of the video group reliable versus 97 (73.5%) of the control group (p = 0.011). CONCLUSIONS: The use of a brief, audiovisual patient information guide on taking the visual field test produced an improvement in patient reliability for individuals tested for the first time. In this trial the use of the video had most of its impact by reducing the number of unreliable fields from the second tested eye. (+info)
Variation in stereoacuity: normative description, fixation disparity, and the roles of aging and gender.
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PURPOSE: Variation in stereoacuity was examined in a large group of observers with Snellen acuity of 20/30 or less. METHODS: Threshold retinal disparity for 2.78 degrees x 2.28 degrees rectangular test stimuli was determined as a function of the retinal disparity (varied from 55 arcmin uncrossed to 55 arcmin crossed) of a 5.57 degrees x 4.8 degrees rectangular pedestal stimulus in 160 observers 15 to 79 years of age. In most cases, data were collected during viewing of random dot stereograms (RDSs) presented for 100-ms, which prevents involvement of vergence or monocular depth cues. RESULTS: When plotted logarithmically, 100-ms thresholds in 106 observers less than 60 years of age approximated a normal distribution (mean, 1.57 +/- 0.227 [SD] log arcsec [37 linear arcsec]). Among these, one observer was supernormal, 88% were within the normal range (+/-2 SD of the log mean), 2% had elevated thresholds, and 8% failed testing with 100-ms stimuli but had residual binocular depth discrimination; 1 observer was stereoblind. In contrast, only 37% of the observers aged 60 to 69 and 25% of the observers aged 70 to 79 had stereoacuity within the normal range. Moreover, the extent of the stereo deficiencies became more pronounced with age. Fixation disparity was operationally defined as optimal stereoscopic threshold with a nonzero retinal disparity pedestal. Of the 151 normal observers tested, 89% were maximally sensitive to disparities within 11 arcmin of fixation: all males were maximally sensitive to pedestals within 22 arcmin of fixation, whereas 8% of females had fixation disparities of more than 22 arcmin. Males were more likely to be sensitive with uncrossed-disparity pedestals, whereas females were more likely to be sensitive with crossed disparity. CONCLUSIONS: Age-related deterioration in stereoacuity is reflected not only by a linear correlation between age and threshold but also by a catastrophic factor that produces more marked deterioration after age 60. Both factors are probably cerebral and not specifically related to stereopsis. The prevalence of fixation disparity in the normal population is probably more common than previously reported. (+info)
Functional and structural recovery of the retina after gene therapy in the RPE65 null mutation dog.
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PURPOSE: To assess the efficacy of AAV-mediated gene therapy to restore vision in a large number of RPE65(-/-) dogs and to determine whether systemic and local side effects are caused by the treatment. METHODS: Normal RPE65 dog cDNA was subcloned into an rAAV vector under control of a cytomegalovirus promoter, and an AAV.GFP control vector was also produced with the titers 2 x 10(12) particles/mL and 2 x 10(10) transducing U/mL, respectively. RPE65(-/-) dogs, aged 4 to 30 months were treated with subretinal injections of the AAV.RPE65 and control vectors, respectively, in each eye, and three 24- to 30-month-old normal control dogs with the latter. Baseline and postoperative systemic and ophthalmic examinations, blood screenings, vision testing, and electroretinography (ERG) were performed. Two RPE65(-/-) dogs were killed at 3 and 6 months after treatment for morphologic examination of the retinas. RESULTS: RPE65(-/-) dogs were practically blind from birth with nonrecordable or low-amplitude ERGs. Construct injections or sham surgeries were performed in 28 eyes; 11 were injected subretinally with the AAV.RPE65 construct. ERGs at 3 months after surgery showed that in the latter eyes, dark-adapted b-wave amplitudes recovered to an average of 28% of normal, and light adapted b-wave amplitudes to 32% of normal. ERG amplitudes were not reduced during a 6- to 9-month follow-up. No systemic side effects were observed, but uveitis developed in nine AAV.RPE65-treated eyes. No uveitis was observed in the eyes treated with the control vector. Immunocytochemistry showed expression of RPE65 in the retinal pigment epithelium (RPE) of AAV.RPE65-treated eyes. Fluorescence microscopy showed expression of green fluorescent protein (GFP) in the RPE and, to a lesser extent, in the neural retinas of AAV.GFP-treated eyes. Ultrastructurally, a reversal of RPE lipid droplet accumulation was observed at the AAV.RPE65 transgene injection site, but not at the site of injection of the control vector. CONCLUSIONS: In 10 of 11 treated RPE65(-/-) eyes, gene transfer resulted in development of vision, both subjectively apparent by loss of nystagmus, and objectively recorded by ERG. Structurally, there was reversal of lipid droplet accumulation in the RPE. Uveitis developed in 75% of the transgene-treated eyes, a complication possibly due to an immunopathogenic response to the RPE65 molecule. (+info)
Amblyopia in astigmatic preschool children.
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Best-corrected acuity was measured for vertical and horizontal gratings and for recognition acuity optotypes (Lea Symbols) in a group of three- to five-year-old children with a high prevalence of astigmatism. Results showed meridional amblyopia (MA) among children with simple/compound myopic or mixed astigmatism, due to reduced acuity for horizontal gratings. Children with simple/compound hyperopic astigmatism showed no MA, but did show reduced acuity for both grating orientations. Reduced best-corrected recognition acuity was shown by both myopic/mixed and hyperopic astigmats. These results suggest that optical correction of astigmatism should be provided prior to age three to five years, to prevent development of amblyopia. (+info)