Diseases & Conditions


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: [email protected]
      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: [email protected]
      Home page: http://www.blindchildrenscenter.org
    • Blind Children's Fund
      311 W. Broadway
      Mt. Pleasant MI 48858
      Phone #: 989-779-9966
      800 #: --
      e-mail: [email protected]
      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: [email protected]
      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: [email protected]
      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: [email protected]
      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: [email protected]
      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: [email protected]
      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: [email protected]
      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: [email protected]
      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: [email protected]
      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: [email protected]
      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: [email protected]
      Home page: http://www.napvi.org
    • National Federation of the Blind
      1800 Johnson Street
      Baltimore MD 21230
      Phone #: 410-659-9314
      800 #: --
      e-mail: [email protected]
      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: [email protected]
      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