Congenital rubella is a viral infection acquired by a fetus in utero. Infection can result in multiple congenital anomalies (including microcephaly, cataracts, cardiac defects, and hearing loss) and fetal death. Diagnosis is by serology, polymerase chain reaction testing, and cell culture. There is no specific treatment. Prevention is by routine vaccination.
(See also Rubella and Overview of Neonatal Infections.)
Congenital rubella typically results from a primary maternal infection. This infection is now rare in the United States because of very successful immunization programs (see Effectiveness and Safety of Childhood Vaccination).
Rubella is believed to invade the upper respiratory tract, with subsequent viremia and dissemination of virus to different sites, including the placenta. The fetus is at highest risk of developmental abnormalities when infected during the first 12 weeks of gestation. Early in gestation, the virus is thought to establish a chronic intrauterine infection. Its effects include endothelial damage to blood vessels, direct cytolysis, and disruption of cellular mitosis.
Symptoms and Signs of Congenital Rubella
In a pregnant patient, rubella may be asymptomatic or characterized by upper respiratory tract symptoms, mild fever, conjunctivitis, lymphadenopathy (especially in the suboccipital and posterior auricular areas), and a maculopapular rash. This illness may be followed by joint symptoms.
In the fetus and neonate, there may be no symptoms or there may be intrauterine fetal demise or multiple congenital abnormalities referred to as congenital rubella syndrome (CRS). The most frequent abnormalities include
Intrauterine growth restriction
Meningoencephalitis
Retinopathy
Cardiac defects (patent ductus arteriosus and pulmonary artery stenosis)
Hepatosplenomegaly
Bone radiolucencies
Less common clinical manifestations include thrombocytopenia with purpura, dermal erythropoiesis resulting in bluish red skin lesions, adenopathy, hemolytic anemia, and interstitial pneumonia.
Ongoing observation is needed to detect subsequent hearing loss, intellectual disability, abnormal behavior, endocrinopathies (eg, diabetes mellitus), or a rare progressive encephalitis.
Infants with congenital rubella infection may develop immune deficiencies such as hypogammaglobulinemia.
Diagnosis of Congenital Rubella
Maternal serum rubella titers
Viral detection in the mother via cell culture and/or polymerase chain reaction (PCR) of amniotic fluid, nose, throat (preferred), urine, cerebrospinal fluid (CSF), or blood specimens
Infant antibody titers (measured serially) and viral detection as above
Pregnant patients routinely have a serum rubella IgG titer measured early in pregnancy. Titer is repeated in people who develop symptoms or signs of rubella; diagnosis is made by a positive serologic test for IgM antibody, IgG seroconversion, or a ≥ 4-fold rise between acute and convalescent IgG titers. PCR or viral culture also can be used to confirm infection.
Fetal infection can be diagnosed in a few centers by detecting the virus in amniotic fluid, detecting rubella-specific IgM in fetal blood, or applying PCR techniques to fetal blood or chorionic villus biopsy specimens.
Infants suspected of having congenital rubella syndrome should have antibody titers and specimens obtained for viral detection. Persistence of or increase in rubella-specific IgG in the infant after 6 to 12 months suggests congenital infection. Detection of rubella-specific IgM antibodies typically also suggests congenital rubella infection, but false-positive IgM results can occur. PCR of specimens obtained from the nasopharynx or throat is the preferred method of detection. Collection of ≥ 1 specimen type (eg, nasopharyngeal, urine, cataract) increases sensitivity. Nasopharyngeal samples usually offer the best sensitivity for culture, and the laboratory should be notified that rubella virus is suspected.
Other tests include a complete blood count (CBC) with differential, CSF analysis, and radiographic examination of the bones to detect characteristic radiolucencies. Thorough ophthalmologic and cardiac evaluations are also useful.
Treatment of Congenital Rubella
Supportive care
No specific therapy is available for maternal or congenital rubella infection.
Pregnant patients who are not immune to rubella (negative rubella IgG) and are exposed to rubella early in pregnancy should be informed of the potential risks to the fetus. Serial antibody testing is performed to detect IgM, which indicates acute infection, followed by the presence of IgG. Routine use of immune globulin for postexposure prophylaxis in nonimmune pregnant patients is not recommended by the American Academy of Pediatrics (1) but is recommended in some countries (2).
Treatment references
1. Committee on Infectious Diseases, American Academy of Pediatrics: Rubella in Red Book: 2021–2024 Report of the Committee on Infectious Diseases, ed. Kimberlin DW, Banerjee R, Barnett ED, Lynfield R, and Sawyer MH. Itasca, American Academy of Pediatrics, 2021.
2. Young MK, Bertolini J, Kotharu P, Maher D, Cripps AW. Rubella antibodies in Australian immunoglobulin products. Hum Vaccin Immunother. 2017;13(8):1952-1955. doi:10.1080/21645515.2017.1327110
Prevention of Congenital Rubella
Rubella can be prevented by vaccination. In the United States, infants should receive a combined measles, mumps, and rubella (MMR) vaccine. The first dose is given at 12 to 15 months of age and the second dose is given at 4 to 6 years (see Childhood Vaccination Schedule).
People capable of becoming pregnant who do not have serologic evidence of immunity should receive at least 1 dose of the MMR vaccine (1). (CAUTION: Rubella vaccination is contraindicated in immunodeficient or pregnant patients.) After vaccination, people should be advised not to become pregnant for 28 days.
Efforts should also be made to screen and vaccinate high-risk groups, such as hospital and child care workers, military recruits, recent immigrants, and college students. Pregnant patients who are found to be susceptible during prenatal screening should be vaccinated after delivery (typically before hospital discharge).
Prevention reference
1. McLean HQ, Fiebelkorn AP, Temte JL, Wallace GS; Centers for Disease Control and Prevention. Prevention of measles, rubella, congenital rubella syndrome, and mumps, 2013: summary recommendations of the Advisory Committee on Immunization Practices (ACIP) [published correction appears in MMWR Recomm Rep. 2015 Mar 13;64(9):259]. MMWR Recomm Rep. 2013;62(RR-04):1-34.
Key Points
Maternal rubella infection, particularly during the first trimester, can cause intrauterine growth restriction and serious developmental abnormalities.
Routine rubella vaccination has made congenital rubella rare in the United States.
Rubella vaccine is contraindicated in pregnancy, so pregnant patients with or exposed to rubella should be informed of the potential risk to the fetus.