Abstract
Between July, 1982 and December 1986, 36 patients with Williams syndrome were evaluated. The mean age at examination was 8.2 years with a range of 0.5-43 years. There were 30 whites, 2 blacks, 2 Hispanics, and 1 Asian. There was a tendency toward ocular hypotelorism with short palpebral fissures; the mean inner canthal distance was 38% for age, while the outer canthal distance was 27%. Of the white patients, 23 had blue irides, 6 hazel and 1 brown. All 6 non-white pateitns had brown irides. All patients with brown irides and, in addition, six patients with blue and three patients with hazel irides did not have stellate iris patterns. Thirty-one percent of the patients had strabismus, similar to previous observations. Three types of optic disc and vascular changes not previously described in the syndrome were noted. Hypermetropic discs were noted in 13 patients. Seventeen patients had simplex vertical branching and three had situs inversus vasorum. Although the latter two findings are considered normal variants, they seem more common in Williams syndrome patients. Accentuated retinal vascular tortuosity has been previously reported, but was not present in any of our patients. In addition, no patients had evidence of ocular manifestations of hypercalcemia. Because of the high Incidence of ocular manifestations in Williams syndrome, we recommend ophthalmologic evaluation of all such patients.
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Greenberg, F., Lewis, R. AN OPHTHALMOLOGICAL STUDY OF PATIENTS WITH WILLIAMS SYNDROME. Pediatr Res 21 (Suppl 4), 227 (1987). https://doi.org/10.1203/00006450-198704010-00367
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DOI: https://doi.org/10.1203/00006450-198704010-00367