Septo Optic Dysplasia

Septo Optic Dysplasia

Septo Optic Dysplasia (SOD), also know as DeMorsier’s Syndrome, is a congenital birth defect that affects the optic disc, optic nerve and brain of the patient. With Septo Optic Dysplasia, the brain is missing the tissue that separates the ventricles of the brain (septum pellucidum).

Individuals with SOD will have visual problems ranging from decreased vision to total blindness. They may also suffer from involuntary movement of the eyeballs called nystagmus and deviation of the eyes (both inward and outward). SOD individuals will also have low muscle tone (hypotonia), hormone deficiencies, diabetes insipidus and jaundice at birth. People with SOD may be shorter and heavier than normal. This is due to the deficiency of growth hormones produced in the brain.

No one actually knows what causes SOD, but it does occur when babies are born to very young mothers (middle to late adolescence). SOD effects male and females equally. Children with SOD are more often born in areas that food is grown. There is a possible connection to the pesticides used on the farms and how they effect the development of the optic nerve. Treatments for SOD include hormone treatment for pituitary issues. However, there is no treatment for the optical problems that one may have. Research is being done in the area of neurogenetics. This research is focused on the study of genes and how they are involved in the development of a “normal brain”. When doctors finally understand what happens in normal brain development, it is believed that they will be able to understand how disorders such as SOD affect the brain.

Some SOD children will have learning disabilities ranging from perceptual problems to mental retardation. School age children could be educated in a functional academic class (depending on severity of learning disability) and will have related services such as VI, occupational therapy and/ or physical therapy. Depending on severity of vision loss, students could use a cane to help maneuver in their environments, CCTV’s to help enlarge reading materials and use a monocular telescope to help with visualizing items from a distance.

References:

California School for the Blind. (n.d.) Septo Optic Dysplasia (DeMorsier’s Syndrome). Retrieved June 17,2011 from http//.

Kauffman, Francine R, M.D and Kauffman, Neal, M.D.(2002). Septo Optic Dysplasia-General Information. The Magic Foundation for Children. Retrieved June 17,2011 from http//

Office of Communications and Public Liaison; National Institute of Neurological Disorders and Stroke. National Institutes of Health. (August 1, 2008) NINDS-Septo Optic Dysplasia Information Page. Retrieved June 17, 2011 from

Robinson, Jean. ( Fall 1997, revised 2002) Septo Optic Dysplasia. Texas School for the Blind and Visually Impaired (See/Hear). Retrieved June 17, 2011 from

Smith, Phillip M.,M.D. and Rismondo, Vivian, M.D. (2009). Diagnosing Septo Optic Dysplasia. EyeNet Magazine; American Academy of Ophthalmology.Retrieved June 17, 2011 from

Prepared by: Jody Harris DeLeon