Congenital Varicella Syndrome
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Congenital Varicella Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms Back to top
- Fetal Effects of Chickenpox
- Fetal Varicella Infection
- Fetal Varicella Zoster Syndrome
- Fetal Effects of Varicella Zoster Virus
- Varicella Embryopathy
Disorder Subdivisions Back to top
General Discussion Back to top
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.
Resources Back to top
HRSA Information Center
PO Box 2910
Merrifield, VA 22116
Feingold Center for Children
52 Second Avenue 1st Floor
Waltham, MA 02451
Center for Disease Control and Prevention
1600 Clifton Road NE
Atlanta, GA 30333
NIH/National Eye Institute
31 Center Dr
Bethesda, MD 20892-2510
NIH/National Institute of Allergy and Infectious Diseases
Office of Communications and Government Relations
6610 Rockledge Drive, MSC 6612
Bethesda, MD 20892-6612
NIH/National Institute of Neurological Disorders and Stroke
P.O. Box 5801
Bethesda, MD 20824
World Health Organization (WHO)
Avenue Appia 20
Geneva 27, 1211
Tel: + 41 22 791 21 11
Fax: + 41 22 791 31 11
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
For a Complete Report Back to top
This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email firstname.lastname@example.org
Last Updated: 4/25/2008
Copyright 1997, 2005 National Organization for Rare Disorders, Inc.
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