Congenital rubella syndrome (CRS) occurs when a human fetus is infected with the rubella virus (German measles) via maternal-fetal transmission and develops birth defects. The most common congenital defects affect the ophthalmologic, cardiac, auditory, and neurological systems.

Rubella infection in pregnancy can result in various outcomes ranging from asymptomatic infection to congenital defects to miscarriage and fetal death. If infection occurs 0–11 weeks after conception, the infant has a 90% risk of being affected.

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Signs and symptoms

thumb|Infant with skin lesions from congenital rubella

right|thumb|"Salt-and-pepper" retinopathy is characteristic of congenital rubella.

The classic triad for congenital rubella syndrome is:

  • Sensorineural deafness (58% of patients)
  • Eye abnormalities—especially retinopathy, cataract, (43% of patients)
  • Congenital heart disease—especially pulmonary artery stenosis and patent ductus arteriosus (50% of patients)

Other manifestations of CRS may include:

  • Spleen,
  • Small head size (microcephaly)
  • Thrombocytopenic purpura, leading to easy or excessive bleeding or bruising
  • Extramedullary hematopoiesis (presents as a characteristic blueberry muffin rash)
  • Schizophrenia
  • Growth retardation
  • Learning disabilities
  • Diabetes mellitus

Diagnosis

thumb|Congenital rubella serology timeline

Diagnosis of congenital rubella syndrome is made based on clinical findings and laboratory criteria. With the introduction of the rubella vaccine in 1969, the number of cases of rubella in the United States has decreased 99%, from 57,686 cases in 1969 to 271 cases in 1999.

Infants with birth defects suspected to be caused by congenital rubella infection should be investigated thoroughly. Confirmed cases should be reported to the local or state health department to assess control of the virus, and isolation of the infant should be maintained.

Management

Infants with known rubella exposure during pregnancy or those with a confirmed or suspected infection should receive close follow-up and supportive care. There are no medications or antivirals that will shorten the clinical course of the virus.

Auditory care

Many infants with CRS may be born with sensorineural deafness and thus should undergo a newborn hearing evaluation. Hearing loss may not be apparent at birth and thus requires close auditory follow-up. Infants with confirmed hearing impairment may require hearing aids and may benefit from an early intervention program.