May 2004
Volume 45, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2004
DEVELOPMENT OF SPHERICAL EQUIVALENT AND ACUITY IN CHILDREN WITH OCULOCUTANEOUS ALBINISM (OCA)
Author Affiliations & Notes
  • N. Couture
    Ophthalmology,
    Children's Hospital, Boston, MA
  • R.M. Hansen
    Ophthalmology,
    Children's Hospital, Boston, MA
  • A. Moskowitz
    Ophthalmology,
    Children's Hospital, Boston, MA
  • A. Gee
    Ophthalmology,
    Children's Hospital, Boston, MA
  • J. LeMoine
    Ophthalmology,
    Children's Hospital, Boston, MA
  • B.–L. Wu
    Laboratory Medicine,
    Children's Hospital, Boston, MA
  • A.B. Fulton
    Ophthalmology,
    Children's Hospital, Boston, MA
  • Footnotes
    Commercial Relationships  N. Couture, None; R.M. Hansen, None; A. Moskowitz, None; A. Gee, None; J. LeMoine, None; B. Wu, None; A.B. Fulton, None.
  • Footnotes
    Support  NIH Grant EY10597
Investigative Ophthalmology & Visual Science May 2004, Vol.45, 4319. doi:
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      N. Couture, R.M. Hansen, A. Moskowitz, A. Gee, J. LeMoine, B.–L. Wu, A.B. Fulton; DEVELOPMENT OF SPHERICAL EQUIVALENT AND ACUITY IN CHILDREN WITH OCULOCUTANEOUS ALBINISM (OCA) . Invest. Ophthalmol. Vis. Sci. 2004;45(13):4319.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Abstract: : Purpose: Study the development of spherical equivalent and acuity in infants and young children with OCA. Broad distributions of refractive error and letter acuity are recognized in mature subjects with OCA Methods: Longitudinal measures of refractive error and acuity were obtained in 16 children starting at age 1 month. Each were followed for up to 8 years. Each demonstrated physical features of OCA and had tyrosinase gene and P–gene testing. Before age 3 years, binocular grating acuities were measured with the Teller Acuity Card procedure. At older ages, symbol acuities were obtained using established clinical tests (including LEA, HOTV, EDTRS). Spherical equivalents were derived from results of cycloplegic retinoscopy. Spherical equivalents and grating acuities were compared to the 95% prediction interval (PI) for healthy infants and children. Data within the PI was defined as normal, and outside the PI as abnormal. Results: The course of grating acuity was abnormal in 9 and normal in 7 of the 16 subjects. The course of spherical equivalent was abnormal in 8 and normal in 8 subjects, but no significant association between grating acuity and spherical equivalent was found. The abnormal courses of spherical equivalent were characterized by persistent, moderate (N=2) or high (N=6) hyperopia, or significant myopia that increased until 2 years of age (N=2). Six of the seven subjects with normal grating acuity developed abnormal spherical equivalents and low symbol acuity (20/100 or poorer). Among subjects with normal refractive development and good symbol acuity (20/50 or better), only one had a history of abnormal grating acuity. Genotype did not predict acuities or refraction. Consistent with previous reports, Summer’s indices of ocular hypopigmentation did not predict acuity, nor did the indices predict refractive error. Conclusions: Abnormal refractive development was associated with low symbol acuity in these subjects with OCA. The determinants of refractive development and acuity in OCA remain to be elucidated.

Keywords: refractive error development • visual acuity • visual development: infancy and childhood 
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