April 2009
Volume 50, Issue 13
Free
ARVO Annual Meeting Abstract  |   April 2009
Ocular, Neurological and Endocrine Abnormalities in Children With Unilateral Optic Nerve Hypoplasia
Author Affiliations & Notes
  • L. L. Cooper
    Department of Surgery, University of Calgary, Calgary, Alberta, Canada
  • W. F. Astle
    Department of Surgery, University of Calgary, Calgary, Alberta, Canada
  • K. G. Romanchuk
    Department of Surgery, University of Calgary, Calgary, Alberta, Canada
  • Footnotes
    Commercial Relationships  L.L. Cooper, None; W.F. Astle, None; K.G. Romanchuk, None.
  • Footnotes
    Support  None.
Investigative Ophthalmology & Visual Science April 2009, Vol.50, 4030. doi:
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    • Get Citation

      L. L. Cooper, W. F. Astle, K. G. Romanchuk; Ocular, Neurological and Endocrine Abnormalities in Children With Unilateral Optic Nerve Hypoplasia. Invest. Ophthalmol. Vis. Sci. 2009;50(13):4030.

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

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Abstract

Purpose: : Children with bilateral optic nerve hypoplasia usually present with bilateral nystagmus and require investigation for neurological and endocrine abnormalities. Fewer children present with unilateral optic nerve hypoplasia and less has been reported on this disorder. The aim of this study is to examine the ocular features as well as the neurological and endocrine abnormalities of children with unilateral optic nerve hypoplasia.

Methods: : A retrospective chart review was performed and data was obtained on ocular, neurological and endocrine function

Results: : Of the 9 patients (7 girls; 2 boys) with unilateral optic nerve hypoplasia, 5 had right optic nerve hypoplasia and 4 had left optic nerve hypoplasia.Two patients had bilateral nystagmus, 1 patient had nystagmus in the affected eye and the remainder of the patients did not have nystagmus. Vision in the eye with optic nerve hypoplasia varied from 20/40 to no light perception. Four patients attempted occlusion therapy and improvement in vision was seen in 1 patient. Strabismus was present in 8 patients (5 esotropia; 3 exotropia). Strabismus surgery was performed in 4 patients and all patients had improvement in eye alignment. One patient had a metabolic abormality in glucose management and 1 patient had neurological abnormalities.

Conclusions: : Children with unilateral optic nerve hypoplasia have different ocular presentation than children with bilateral optic nerve hypoplasia. Children with unilateral optic nerve hypoplasia have a lower frequency of neurological and endocrinological abnormalities. These finds may suggest a different mechanism for the development of unilateral optic nerve hypoplasia than bilateral optic nerve hypoplasia.

Keywords: optic nerve • strabismus • visual acuity 
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