Septo-Optic Dysplasia

Septo-optic dysplasia (SOD) is a rare disorder that is a result of the optic nerve not developing all the way. The optic nerve is the main nerve that carries impulses from the eye to the brain to allow vision. SOD is also known as De Morsier syndrome. People are born with this disorder (it is a congenital disorder). People with SOD often lack an important part of the brain (the septum pellucidum) and often have problems with the pituitary gland, a gland that controls hormone levels. Intellectual ability can vary from normal intelligence to severe mental retardation. There may be episodes of uncontrollable shaking (seizure). In a few cases, yellow skin coloring (jaundice) may be present at birth. Some children with SOD are also born with abnormal fingers and toes.

CAUSES

The cause of SOD is not known. However, since it can happen more than once in a single family, it may be partly inherited. It happens more often to babies born to very young mothers.

SYMPTOMS

  • Vision in SOD may be normal. However, more often there are different degrees of poor vision and even complete blindness.

  • A rapid shaking of the eyes (nystagmus) may happen in infants between the ages of 1 and 8 months.

DIAGNOSIS

An eye care specialist (ophthalmologist) will often suspect SOD based on the way the the eyes move and the way the optic nerve looks when examined.

TREATMENT

  • There is no treatment for SOD.

  • Hormones are sometimes prescribed to treat the hormone imbalances that can result from problems with the pituitary gland.