Spectrum of optic nerve hypoplasia.

Optic nerve hypoplasia is a non-progressive condition characterised by subnormal vision and a subnormal number of optic nerve axons. It may be unilateral or bilateral, isolated or combined with other defects. Analysis of fundus photographs from a series of 7 patients with a stationary abnormality of different degrees showed that the functional defects could be closely correlated with defects in the retinal nerve fibre layer. Our observations show that the condition has a wide range of both functional and anatomical defects and that a subnormal diameter of the optic disc is not a requisite for the diagnosis. Presumably, there is also a wide variety of causes, not only a primary failure of development of retinal ganglion cells. We suggest that optic nerve hypoplasia can be viewed as a non-specific manifestation of damage to the visual system, sustained any time before its full development.