Purchase this article with an account.
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.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
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.
A retrospective chart review was performed and data was obtained on ocular, neurological and endocrine function
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.
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.
This PDF is available to Subscribers Only