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Refractive development in children with Down's syndrome: a population based, longitudinal study.

AIMS: To study the refractive development in children with Down's syndrome longitudinally. METHODS: An unselected population of 60 children with Down's syndrome was followed with repeated retinoscopies in cycloplegia for 2 years or more (follow up 55 (SD 23) months). Accommodation was assessed with dynamic retinoscopy. RESULTS: From longitudinal spherical equivalent values of the right eye, three main categories of refraction were defined: stable hypermetropia (<1.5 D difference between the first and last visit) (n=34), increasing hypermetropia ("hypermetropic shift"; >/=1.5 D difference) (n=11), and decreasing hypermetropia/development of myopia ("myopic shift"; >/=1.5 D difference) (n=9). Patients with anisometropia (n=6) were evaluated separately. In the stable hypermetropia group three sublevels were chosen: low (</=+2.0 D at the last visit), moderate (+2.25 to + 4.0 D), and high (>+4.0 D). An accommodation weakness was found in 55% of the children. Accommodation weakness was significantly less frequent in the stable, low grade hypermetropia group (22%) than in all the other groups (p=0.008). The frequency of astigmatism >/=1.0 D at the last visit was 57%, the direction of axis being predominantly "with the rule." All the eyes with oblique astigmatism had a side specific direction of axis; the right eyes belonging to the 135 degrees axis group and the left eyes to the 45 degrees axis group. CONCLUSION: A stable, low grade hypermetropia was significantly correlated with a normal accommodation. Accommodation weakness may be of aetiological importance to the high frequency of refractive errors encountered in patients with Down's syndrome. A striking right-left specificity in the oblique astigmatic eyes suggests that mechanical factors on the cornea from the upward slanting palpebral fissures may be a major aetiological factor in the astigmatism.

Accommodation, Ocular↗

Weaning children with accommodative esotropia out of spectacles: a pilot study.

BACKGROUND/AIM: Many children with accommodative esotropia must continue spectacle use throughout life. This study was undertaken to determine which factors are predictive of successfully weaning children with accommodative esotropia out of spectacles. METHODS: A retrospective review of 10 children with accommodative esotropia, who were gradually weaned from their hyperopic correction, and three age matched controls was performed. The main outcome measure was resolution or non-resolution of esotropia following weaning and eventual discontinuation of spectacles. Secondary outcome measures were final refractive error and the final esotropic or esophoric angle without correction. RESULTS: Six patients were successfully weaned from spectacles. At the completion of the weaning period one child was orthophoric and the other five children had well controlled esophorias. The other four patients remained spectacle dependent because of persistent esotropia or decreased vision without spectacles. The baseline and final refractive errors were significantly lower in the children successfully weaned from spectacles (p = 0.014). While the children who were successfully weaned from spectacles were older when initially diagnosed with accommodative esotropia (4.6 v 2.5 years), this difference was not statistically significant (p = 0.09). CONCLUSION: Some children with accommodative esotropia may be weaned out of spectacles during the grade school years with resolution of their esotropia. It is likely that gradual reduction of the hyperopic correction increases divergence amplitudes, but it is unclear whether this facilitates emmetropisation.

Accommodation, Ocular↗

Role of the school eye clinic in modern ophthalmology.

Routine sight tests for children at intervals throughout their school career are clearly important; three-quarters of those referred to the school eye clinics in this area had some ocular defect. It is probably no longer necessary for myopic schoolchildren to be treated by a consultant ophthalmologist after their initial examination.Forty-five per cent. of children referred to the school eye clinics in this area were found to have squint and/or hypermetropic/anisometropic/astigmatic refractive errors. Priority should be given to this group because of the association of amblyopia with these conditions. Their treatment requires closer association with the hospital ophthalmic department, perhaps even complete unity. Transfer of children at present seen in the school eye clinic to a hospital-based "children's eye clinic" would also fit in with a unified health service administration structure and be better placed to indicate, evaluate, and control future developments towards the prevention of amblyopia.

Adolescent↗

Moore-Federman syndrome and acromicric dysplasia: are they the same entity?

Four unrelated patients are reported with short stature, stiffness of the joints, short fingers, inability to make a fist, and thickened skin on the forearms. Investigations have failed to show a lysosomal storage disorder and radiographs show non-specific changes with a delayed carpal bone age. The clinical features in the four children are very similar to the recently described acromicric dysplasia. There are also similarities to Moore-Federman syndrome which has only been described in one family. The case is made that acromicric dysplasia and Moore-Federman syndrome are the same entity.

Abnormalities, Multiple↗

Deterioration of visual acuity associated with growth hormone therapy in a child with extreme short stature and high hypermetropia.

BACKGROUND: Growth hormone (GH) has long been implicated in the pathogenesis of diabetic retinopathy, although its precise role remains ill-defined. In 1998, an association between exogenous human GH and retinal pathology in non-diabetic subjects was described. CASE REPORT: A female child with extreme short stature of unknown aetiology (height -7.38 SD at 11.3 years) and severe hypermetropia developed retinopathy with visual deterioration during two separate empiric trials of GH therapy. On the first occasion, a relatively high dose of GH (10.5 mg/m2/week) administered from age 4.4 to age 4.7 years was associated with the development of central serous retinopathy, resulting in marked reduction in visual acuity. On cessation of GH, the macular oedema resolved, and visual acuity improved. At age 5.6 years, GH therapy was re-introduced at a lower dose (3.9 mg/m2/week) and her vision monitored closely. Bilateral retinal oedema recurred after 3 months, and GH therapy was stopped. Once again, the macular oedema regressed, and visual acuity improved following withdrawal of GH. These ophthalmic changes contra-indicated further GH therapy. CONCLUSION: We suggest that GH may be a risk factor in the development of retinopathy in certain non-diabetic patients, especially in the presence of a severe refractive error.

Child↗

Development of visual line acuity in humans.

An acuity test was developed with defined space between symbols to determine the effect of crowding on visual line acuity. The test differentiates between normalsighted subjects, those with low but uncorrected refractive errors and amblyopes. Furthermore, the test allows a quantitative examination of the crowding phenomenon and a measure of progress during amblyopia therapy. In normal-sighted subjects a maturational effect on visual line acuity was found, reaching the maximum test level at approximately the tenth year of life.

Age Factors↗

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