Optic nerve hypoplasia is a congenital malformation consisting of small, underdeveloped optic nerves that cause impaired vision from birth. It involves faulty connection of the axons within the optic nerve, which is often accompanied by faulty wiring elsewhere in the brain, particularly in the hypothalamus and the midline structures.
Hypoplasia is not progressive and its severity varies considerably. Functional impairment ranges from near-normal central visual acuity to blindness in severe cases. It usually occurs in both eyes, but it may also be present in one eye only.
Because it is linked to abnormal development of the central nervous system, this diagnosis adversely affects the visual, neurological and hormonal systems. Treatment calls for a multidisciplinary approach and careful management, given the endocrinopathy, brain malformations and developmental delay that frequently accompany it. Although there is no cure for optic nerve hypoplasia, early intervention can minimise the impact of sight loss and improve these children's development in general.
Any child who potentially shows poor visual function, nystagmus or strabismus should undergo an eye examination by 3 months of age. Where optic nerve hypoplasia is diagnosed, magnetic resonance imaging and an endocrinological assessment are required.
Electrostimulation can improve sight by natural means. Because a child's brain is more readily shaped, this treatment is directly beneficial in improving their visual acuity and contrast vision and in widening the visual field.