Optic Nerve Hypoplasia / Disease

Diseases & Conditions

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Optic Nerve Hypoplasia

Synonyms of Optic Nerve Hypoplasia

DeMorsier syndrome
ONH
septooptic dysplasia
SOD

Disorder Subdivisions

General Discussion
The syndrome of optic nerve hypoplasia (ONH), also known as septooptic dysplasia (SOD) or DeMorsier syndrome, is a congenital disorder characterized by underdevelopment (hypoplasia) of the optic nerves. The optic nerves transmit impulses from the nerve-rich membranes lining the retina of the eye to the brain. Children with ONH may have brain and pituitary malformations. Abnormalities of midline structures of the brain may include absence of the septum pellucidum and hypoplasia of the corpus callosum (nerve fibers that connect the two hemispheres of the brain). The pituitary gland is a hormone-producing gland at the base of the brain that controls hormones in the body that are necessary for growth, energy, and sexual development.

Most people with ONH have abnormal eye movements (nystagmus) and vision can range from no light perception to good functional vision, or even full vision in one eye. Some affected children have normal intelligence and others have learning disabilities and developmental delays. Deficiencies of certain hormones may result in growth retardation, poor development, and may be life-threatening without treatment. Hormone deficiencies can be controlled with daily hormone replacement therapy and close monitoring by an endocrinologist (hormone doctor). The cause of ONH is not fully understood.

Organizations related to Optic Nerve Hypoplasia

American Foundation for the Blind
11 Penn Plaza
New York NY 10001
Phone #: 212-502-7600
800 #: 800-232-5463
e-mail: afbinfo@afb.org
Home page: http://www.afb.org
Blind Children's Center
4120 Marathon Street
Los Angeles CA 90029-3584
Phone #: 323-664-2153
800 #: 800-222-3566
e-mail: info@blindchildrenscenter.org
Home page: http://www.blindchildrenscenter.org
Blind Children's Fund
311 W. Broadway
Mt. Pleasant MI 48858
Phone #: 989-779-9966
800 #: --
e-mail: bcf@blindchildrensfund.org
Home page: http://www.blindchildrensfund.org
Chromosome
11435 Cronhill Drive
Owings Mills MD 21117-2220
Phone #: 410-568-0150
800 #: 800-683-5555
e-mail: info@blindness.org
Home page: http://www.fightblindness.org
Council of Families with Visual Impairment
1155 15th St. NW
Washington DC 20005
Phone #: 202-465-5081
800 #: --
e-mail: info@acb.org
Home page: http://www.acb.org/
Diabetes Insipidus Foundation, Inc.
5203 New Prospect Court
Ellicott City MD 21043
Phone #: 410-480-0880
800 #: --
e-mail: info@diabetesinsipidus.org
Home page: http://www.diabetesinsipidus.org
FOCUS Families--For Our Children's Unique Sight
2453 Emerald Street
San Diego CA 92109
Phone #: 858-273-1473
800 #: 866-362-8750
e-mail: support@focusfamilies.org
Home page: http://www.focusfamilies.org
Human Growth Foundation
997 Glen Cove Avenue
Glen Head NY 11545
Phone #: 516-671-4041
800 #: 800-451-6434
e-mail: hgf1@hgfound.org
Home page: http://www.hgfound.org/
MAGIC Foundation for Children's Growth
6645 W. North Avenue
Oak Park IL 60302
Phone #: 708-383-0808
800 #: 800-362-4423
e-mail: mary@magicfoundation.org
Home page: http://www.magicfoundation.org
MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
150 Custer Court
Green Bay WI 54301-1243
Phone #: 920-336-5333
800 #: 877-336-5333
e-mail: mums@netnet.net
Home page: http://www.netnet.net/mums/
Madisons Foundation
PO Box 241956
Los Angeles CA 90024
Phone #: 310-264-0826
800 #: N/A
e-mail: getinfo@madisonsfoundation.org
Home page: http://www.madisonsfoundation.org
NIH/National Digestive Diseases Information Clearinghouse
2 Information Way
Bethesda MD 20892-3570
Phone #: 301-654-3810
800 #: 800-891-5389
e-mail: nddic@info.niddk.nih.gov
Home page: http://www.niddk.nih.gov
National Association for Parents of Children with Visual Impairments (NAPVI)
P.O. Box 317
Watertown MA 02472
Phone #: 617-972-7441
800 #: 800-562-6265
e-mail: napvi@perkins.org
Home page: http://www.napvi.org
National Federation of the Blind
1800 Johnson Street
Baltimore MD 21230
Phone #: 410-659-9314
800 #: --
e-mail: nfb@nfb.org
Home page: http://www.nfb.org
The Arc (a national organization on mental retardation)
1010 Wayne Ave
Silver Spring MD 20910
Phone #: 301-565-3842
800 #: 800-433-5255
e-mail: info@thearc.org
Home page: http://www.thearc.org/

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html