Diseases & Conditions


A B C D E F G H I J K L M N O P Q R S T U V W X Y Z

Congenital Varicella Syndrome


Synonyms of Congenital Varicella Syndrome
  • Fetal Effects of Chickenpox
  • Fetal Effects of Varicella Zoster Virus
  • Fetal Varicella Infection
  • Fetal Varicella Zoster Syndrome
  • Varicella Embryopathy

Disorder Subdivisions



    General Discussion
    Congenital Varicella Syndrome is an extremely rare disorder in which affected infants have distinctive abnormalities at birth (congenital) due to the mother's infection with chickenpox (maternal varicella zoster) early during pregnancy (i.e., up to 20 weeks gestation). Affected newborns may have a low birth weight and characteristic abnormalities of the skin; the arms, legs, hands, and/or feet (extremities); the brain; the eyes; and/or, in rare cases, other areas of the body. The range and severity of associated symptoms and physical findings may vary greatly from case to case depending upon when maternal varicella zoster infection occurred during fetal development.

    In many cases, newborns with Congenital Varicella Syndrome may be abnormally small and have a low birth weight due to abnormal growth delays during fetal development (intrauterine growth retardation). In addition, distinctive skin abnormalities are often present. Certain areas of the skin may consist of thickened, overgrown (hypertrophic) scar tissue (cicatrix), and surrounding skin may appear abnormally hardened (indurate), red, and inflamed (erythema). Such cicatrix scarring typically occurs on one or more of the arms and/or legs, which may also be malformed, underdeveloped (hypoplastic), and abnormally shortened (reduction deformities). Affected infants may also exhibit incomplete development (hypoplasia) of certain fingers and/or toes (rudimentary digits).

    In some cases, newborns with Congenital Varicella Syndrome may have abnormalities of the brain such as degeneration of the outer portion of the brain (cortical atrophy) and/or abnormal enlargement of cavities of the brain (dilated ventricles [ventriculomegaly]). There may also be abnormalities of the part of the nervous system that controls involuntary functions (autonomic nervous system) such as damage to or abnormalities of certain nerve fibers (sympathetic nerve fibers) that pass from the spinal cord to the neck and/or pelvic area. Some affected infants and children may also exhibit abnormal smallness of the head (microcephaly), delays in the acquisition of skills requiring the coordination of mental and physical activities (psychomotor retardation), varying degrees of mental retardation, and/or learning disabilities. In some cases, characteristic eye (ocular) abnormalities may also be present including loss of transparency of the lenses of the eyes (cataracts); abnormal smallness of one or both eyes (unilateral or bilateral microphthalmia); involuntary, rapid, side-to-side movements of the eyes (pendular nystagmus); and/or inflammation and scarring of certain membranes of the eyes (chorioretinitis and chorioretinal scarring). Such ocular abnormalities may result in varying degrees of visual impairment. In rare cases, newborns with Congenital Varicella Syndrome may have additional abnormalities associated with the disorder.

    Organizations related to Congenital Varicella Syndrome
    • Centers for Disease Control and Prevention
      1600 Clifton Road NE
      Atlanta GA 30333
      Phone #: 404-639-3534
      800 #: 800-311-3435
      e-mail: http://www.cdc.gov/netinfo.htm
      Home page: http://www.cdc.gov/
    • HRSA Information Center
      PO Box 2910
      Merrifield VA 22116
      Phone #: 703-442-9051
      800 #: 888-275-4772
      e-mail: ask@hrsa.gov
      Home page: http://www.ask.hrsa.gov/
    • NIH/National Eye Institute
      Building 31 Rm 6A32
      Bethesda MD 20892-2510
      Phone #: 301-496-5248
      800 #: --
      e-mail: 2020@nei.nih.gov
      Home page: http://www.nei.nih.gov/
    • NIH/National Institute of Allergy and Infectious Diseases
      6610 Rockledge Drive
      Bethesda MD 20892-6612
      Phone #: 301-496-5717
      800 #: --
      e-mail: N/A
      Home page: http://www.niaid.nih.gov/
    • National Birth Defects Center
      40 Second Avenue
      Waltham MA 02451
      Phone #: 781-466-9555
      800 #: --
      e-mail: nbdc@thegenesisfund.org
      Home page: http://www.thegenesisfund.org/nbdc.htm
    • National Institute of Neurological Disorders and Stroke (NINDS)
      31 Center Drive
      Bethesda MD 20892-2540
      Phone #: 301-496-5751
      800 #: 800-352-9424
      e-mail: braininfo@ninds.nih.gov
      Home page: http://www.ninds.nih.gov/
    • World Health Organization (WHO) Regional Office for the Americas (AMRO)
      Pan American Health Organization (PAHO)
      Washington DC 20037
      Phone #: 202-974-3000
      800 #: --
      e-mail: postmaster@paho.org
      Home page: http://www.who.ch/



    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