What is Optic Nerve Hypoplasia?

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  • Written By: J.L. Drede
  • Edited By: Melissa Wiley
  • Last Modified Date: 12 May 2020
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Optic nerve hypoplasia is a congenital condition in which a person's optic, or eye, nerves are smaller than normal. This leads to poorly developed eyesight, the range of which can vary from slightly decreased vision in just one eye to complete blindness. Additional hormonal and developmental abnormalities are frequently associated with the condition.

Optic nerve hypoplasia is typically diagnosed in children around the ages of three or four years old. Early signs of the condition before that can include unusual eye movements called nystagmus, which is when the eye moves without focusing or purpose. This can be a sign for poor vision in children no matter what the condition. Damage caused by the condition is consistent and does not worsen over time.

Problems with optic nerve hypoplasia will often extend past the eye and into the brain. Many with the condition also suffer from abnormal brain function that can range from very minor to severe. The abnormal brain function is usually caused by De Morsier's syndrome. This disorder, also referred to as septo-optic dysplasia, is caused when the septum pellucidum of the brain is not entirely formed.

When this section of the brain is underdeveloped, it can lead to minor or major problems with motor function, speech, and intelligence. Seizures are also common in children with this condition, but they can usually be controlled with special medication.

Children suffering from optic nerve underdevelopment may also have a poorly functioning pituitary gland. The pituitary gland is responsible for the body releasing hormones that control everything from the body's metabolism to sexual development later in life. If the pituitary gland is not producing these hormones, then the child may be prescribed hormone shots to compensate for their absence.

There is no way to improve the eyesight of a child with optic nerve hypoplasia. Because of the wide variety of conditions and disabilities that are associated with the condition, affected persons usually have to be under the regular care of a ophthamalogist, endocrinologist, and neurologist. Sometimes social workers and other aides are also brought in to make sure the child is getting the best care possible.

Children with optic nerve hypoplasia can sometimes go to school with non-disabled children, but if their brain function is severely effected by the condition this might not be possible. Regardless of the kind of schooling the child receives, he or she will need additional tools such as large-print books or magnifiers that can make small text easier to read. They also may need to attend speech therapy and other additional services to improve their general state of living.

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