Change in visual acuity in albinism in the early school years

J Pediatr Ophthalmol Strabismus. 2012 Mar-Apr;49(2):81-6; quiz 87. doi: 10.3928/01913913-20110628-02. Epub 2011 Jul 6.

Abstract

Purpose: To determine whether binocular best-corrected visual acuity (B-BCVA) improves in the early school years in patients with albinism and whether this is related to type of albinism, ocular pigment, or appearance of the macula.

Methods: Patients with albinism seen between 5.5 and 9 years (Visit A) and 9.5 and 14 years of age (Visit B), with visits separated by at least 2.5 years, were included. Type of albinism, B-BCVA, glasses wear, iris pigment and macular transparency grade, and presence or absence of an annular reflex and melanin in the macula were recorded.

Results: Mean B-BCVA was 20/84 at Visit A and 20/61 at Visit B (P < .001). B-BCVA improved in 80%. Improvement in B-BCVA and glasses wear, iris grade, macular grade, macular melanin, and annular reflex were weakly correlated. However, a moderate correlation was found between measured B-BCVA and iris grade at Visit A (r = 0.485, P < .001) and Visit B (r = 0.467, P < .001), and the presence of macular melanin at Visit A (r = 0.436, P < .001) and Visit B (r = 0.482, P < .001).

Conclusions: B-BCVA often improves in albinism in the early school years and this observation should be included in counseling. The etiology is unknown but may be related to change in nystagmus, use of precise null point, developmental maturation, and/or some of the ocular characteristics evaluated in this study.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Albinism, Ocular / metabolism
  • Albinism, Ocular / physiopathology*
  • Albinism, Oculocutaneous / metabolism
  • Albinism, Oculocutaneous / physiopathology*
  • Chediak-Higashi Syndrome / metabolism
  • Chediak-Higashi Syndrome / physiopathology
  • Child
  • Child, Preschool
  • Electroretinography
  • Evoked Potentials, Visual
  • Female
  • Hermanski-Pudlak Syndrome / metabolism
  • Hermanski-Pudlak Syndrome / physiopathology
  • Humans
  • Male
  • Melanins / metabolism
  • Retina / metabolism
  • Vision, Binocular / physiology*
  • Visual Acuity / physiology*

Substances

  • Melanins

Supplementary concepts

  • Oculocutaneous albinism type 1
  • Oculocutaneous albinism type 1B
  • Oculocutaneous albinism type 2