Medical Management Considerations
Congenital rubella syndrome (CRS) is a severe and damaging illness, usually manifesting in infancy, resulting from rubella infection in utero and characterized by a constellation of birth defects. CRS affects virtually all organ systems. The most common manifestation is intrauterine growth retardation. Other common findings include nerve deafness, cataracts, and cardiac anomalies such as ventricular and atrial septal defect. Later sequelae include motor and mental retardation.
A viral illness caused by a togavirus of the genus Rubivirus, rubella infection in children and adults is generally benign. However, maternal infection during the first 12 weeks of pregnancy presents an 80% probability of a fetal infection with serious residual defects, decreasing slightly to 67% probability during weeks 13 and 14, and to 25% by week 26.
Endemic in most countries, no specific treatment for rubella exists. The control of CRS rests on the prevention of maternal viremia by vaccination.
Serology remains the mainstay of diagnosis of CRS, obtained by demonstrating IgM antibody in cord blood or by showing the persistence of IgG antibody during the first year of life rather than its disappearance within three to six weeks.
Since the introduction of rubella vaccine in the United States in 1969, the incidence of rubella and CRS have decreased by approximately 99%. The following number of confirmed cases were reported in the United Sates: 172 (1997); 353 (1998); and 267 (1999). The United States is on the verge of eliminating CRS, having established 2010 as the target year for elimination of the disease.
Since 2001, the number of rubella cases has been the lowest ever recorded in the United States. 23 in 2001, 18 in 2002, 7 in 2003 and 9 in 2004. During 2001-2004, four CRS cases were reported to CDC; the mother of three of the children were born outside the United States.
In the prevaccine era in countries with endemic rubella, the rate of CRS was 1 per 1,000 live births.
The most recent rubella outbreak in the United States occurred during the winter of 1963-1964. As a result most persons with CRS in the United States are in their thirties. Because the togavirus is neuropathic, these individuals are likely to suffer from challenging behaviors, psychiatric disorders, or epilepsy in addition to visual or hearing impairments. Antiepilectic medications such as carbamazepine and valproic acid may be preferred psychotropic therapy for challenging behaviors and psychiatric disorders. In addition, partial epilepsy is often underdiagnosed.
All birth defects in which rubella infection is etiologically suspected should be investigated and reported to the Centers for Disease Control and Prevention through local or state health departments.
At birth, 72% of infants show:
Note: These considerations are in addition to the normal medical care provided to an individual without Congenital Rubella. All recommendations can be addressed through clinical examination by the primary care provider, unless otherwise noted.
Center for Disease Control. (2000). Measles, Rubella, and Congenital Rubella Syndrome — United States and Mexico, 1997-1999. Morbidity and Mortality Weekly Report, 49(46), 1048-1050.
Gold, E. (1996). Almost Extinct Diseases: Measles, Mumps, Rubella, and Pertussis. Pediatrics in Review, 17(4).
Rangel, M. C., Sales, R.M., Valeriano, E.N. (1999). Rubella Outbreaks Among Hispanics in North Carolina: Lessons Learned from a Field Investigation. Ethnicity and Disease, 9(2), 230-236.
Reef, S.E. (1998). Rubella and congenital rubella Syndrome. Bulletin of the World Health Organization, 76(2), 156.
Batshaw, M.L. (1997). Children with Disabilities,4th edition. Baltimore: Paul H. Brookes Publishing.
Behrman, R.E. 2004. NelsonTextbook of Pediatrics, 16th edition. Philadelphia: W. B. Saunders.
Jones, K.L. (1997). Smith’s Recognizable Patterns of Human Malformation, 5th edition. Philadelphia: W. B. Saunders.
Menkes, J.H., Sarnat, H.B. (2000). Child Neurology, 6th edition. Philadelphia: Lippincott Williams & Wilkins.
The Arc (a national organization on mental retardation)
1010 Wayne Avenue, Suite 650
Silver Spring, MD 20910
California Regional Centers
4301 Connecticut Avenue NW,Suite 404
Washington, DC 20008-2304
March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains, NY 10605
National Organization for Rare Disorders
PO Box 8923
New Fairfield, CT 06812-8923
Theodore A. Kastner, M.D., M.S.
Mary Ann Lewis, Dr.P.H., R.N., F.A.A.N.
Funded by a grant from the California Department of Developmental Services
For more information, contact:
Center for Health Improvement
1330 21st Street, Suite 100
Sacramento, CA 95814
This document does not provide advice regarding medical diagnosis or treatment for any individual case, and any opinions or statements contained in this document are not intended to serve as a standard of medical care. Physicians are encouraged to view the considerations presented in this document in light of evolving scientific information. This document is not intended for use by the layperson. Reproduction of this document may be done with proper credit given to California Department of Developmental Services and the Center for Health Improvement.