Congenital Rubella


Article Author:
Samarth Shukla


Article Editor:
Nizar Maraqa


Editors In Chief:
Eric Flake


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Trevor Nezwek
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
6/4/2019 2:56:20 PM

Introduction

Rubella, also known as German Measles, is a viral illness characterized by maculopapular rash, lymphadenopathy, and fever. It is a highly contagious but generally mild disease, without consequences in most cases. However, maternal infection during the first trimester of pregnancy can cause a fetal malformation syndrome called congenital rubella syndrome.

Etiology

The enveloped, positive-stranded RNA virus known as Rubella virus is classified as a Rubivirus in the Togaviridae family.

Epidemiology

Congenital rubella syndrome is a global public health concern with more than 100,000 cases reported annually worldwide. Natural rubella infection during pregnancy is one of the few known causes of autism. 

Before the rubella vaccine was licensed in the United States in 1969, rubella was a common disease that occurred primarily among young children. However, rubella was eliminated from the United States in 2004. Since elimination, fewer than 10 cases have been reported annually in the United States, and most cases were imported from outside the country.[1]

Rubella continues to be a commonly transmitted infection in many parts of the world. Many rubella cases are not recognized, as the rash resembles many other illnesses and up to half of all infections may be subclinical.

Humans are the only source of infection. Transmission is through direct or droplet contact from nasopharyngeal secretions. Once inhaled, the virus replicates in the respiratory mucosa and cervical lymph nodes before reaching the target organs via systemic circulation. The infectious period extends approximately 8 days before to 8 days after the rash onset.[2]

Maternal rubella during pregnancy can cause miscarriage, fetal death or congenital rubella syndrome.[2] Few infants with congenital rubella continue to spread the virus in nasopharyngeal secretions and urine for a year or more. Rubella virus also has been recovered from lens aspirates in children with congenital cataracts for several years.

Pathophysiology

Pathogenesis of congenital rubella syndrome is multifactorial[2] and include the following:

  1. Non-inflammatory necrosis of chorionic epithelium and in endothelial cells which are then transported to fetal circulation and fetal organs.
  2. Intracellular actin assembly is inhibited by rubella infection, leading to inhibition of mitosis and restricted development of precursor cells.
  3. Upregulation of cytokines and interferon in infected cells which could contribute to congenital defects.

History and Physical

Few or no obvious clinical manifestations occur at birth with mild forms of the disease. The incidence of congenital infection with rubella is high during the early and late weeks of gestation (U-shaped distribution), with chances of birth defects much higher if the infection occurs early in pregnancy.

Congenital defects occur in up to 85% of neonates if maternal infection occurs during the first 12 weeks of gestation, in 50% of neonates if infection occurs during the first 13 to 16 weeks of gestation, and 25% if infection occurs during the latter half of the second trimester.

Serious birth defects include the following[3][4]:

  • Congenital heart defects (patent ductus arteriosus, peripheral pulmonary artery stenosis, ventricular septal defects, atrial septal defects)
  • Auditory (sensorineural hearing impairment)
  • Ophthalmologic (cataracts, pigmentary retinopathy, microphthalmos, chorioretinitis)
  • Neurologic (microcephaly, cerebral calcifications, meningoencephalitis, behavioral disorders, mental retardation)
  • Hematologic (thrombocytopenia, hemolytic anemia, petechiae/purpura, dermal erythropoiesis causing “blueberry muffin” rash)
  • Neonatal manifestations (low birth weight, interstitial pneumonitis, radiolucent bone disease leading to “celery stalking” of long bone metaphyses, hepatosplenomegaly)
  • Delayed onset of insulin-dependent diabetes and thyroid disease.

Evaluation

Maternal screening with rubella titers in early pregnancy is considered standard of care in the United States. Rubella-like illness in early pregnancy should be evaluated to confirm the diagnosis. Laboratory diagnosis is based on observation of seroconversion with use of RV-IgG and IgM titers. Maternal counseling and termination of pregnancy are options in such cases.

Prenatal fetal diagnosis is based on detection of viral genome in amniotic fluid, fetal blood or chorionic villus biopsies.

Postnatal diagnosis of congenital rubella infection is done by detecting RV-IgG antibodies in neonatal serum using ELISA. This approach has sensitivity and specificity of nearly 100% in infants less than three months of age. Confirmation of infection is made by detection of rubella virus in nasopharyngeal swabs, urine and oral fluid using polymerase chain reaction (PCR).[2]

Congenital infection can also be confirmed by stable or increasing serum concentrations of rubella-specific IgG over the first year of life. It is difficult to diagnose congenital rubella in children older than one year of age.

Postnatal confirmation of congenital infection is important despite the absence of clinical features of congenital rubella syndrome. This is to develop a specific follow-up care plan for early detection of long-term neurological and ocular complications.[5]

Treatment / Management

Prenatal management of the mother and fetus depends on gestational age at onset of infection. If infection happens before 18 weeks gestation, the fetus is at high risk for infection and severe symptoms. Termination of pregnancy could be discussed based on local legislation. Detailed ultrasound examination and assessment of viral RNA in amniotic fluid is recommended.

For infections after 18 weeks of gestation, pregnancy could be continued with ultrasound monitoring followed by neonatal physical examination and testing for RV-IgG.[2]

Limited data suggest a benefit of intramuscular Immune Globulin (IG) for maternal rubella infection leading to decrease in viral shedding and risk of fetal infection.

Although symptoms associated with congenital rubella syndrome can be treated, there is no cure for the syndrome; hence, prevention should be the goal.

Control Measures

Children with congenital rubella syndrome should be considered contagious until at least one year of age unless two negative cultures are obtained one month apart after 3 months of age. Neonates should be isolated. Hand hygiene is of utmost importance for reducing disease transmission from the urine of children with congenital rubella infection.[6][7][8]

Pearls and Other Issues

Significant progress has been reported in the prevention of congenital rubella syndrome since the identification of teratogenic potential of rubella virus. However, more efforts are required to eliminate it. Since no treatment is available for congenital rubella syndrome, it is important for women to get vaccinated before they get pregnant. MMR vaccine is a live attenuated vaccine, and due to its theoretical teratogenic risk, women who are pregnant and not vaccinated should wait to get MMR until after they have given birth. Although, several publications have reported the absence of congenital rubella syndrome after vaccination during pregnancy.

The antibody response rate to a single dose is more than 95%; after two doses, the response rate is almost 100%. Rubella vaccine is generally well tolerated with benign adverse effects like fever, rash, and transient lymphadenopathy.

Even though rubella has become a rare disease in developed countries, lack of vaccination may lead to significant public health problems.

Enhancing Healthcare Team Outcomes

Once an infant with congenital rubella is diagnosed, a multidisciplinary team of nurses and clinicians should be involved in its care because of the diverse manifestations and family education requirements.  Children with congenital rubella syndrome should be considered contagious until at least one year of age unless two negative cultures are obtained one month apart after 3 months of age. A birth the Neonate should be isolated. The nurse should emphasize the importance of hand hygiene for reducing disease transmission from the urine of children with congenital rubella infection. More important, all healthcare workers should encourage parents to have their children vaccinated against rubella.


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Congenital Rubella - Questions

Take a quiz of the questions on this article.

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A blueberry muffin baby classically is seen in infants with which congenital infection?

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A newborn is small for gestational age and has purpura, thrombocytopenia, jaundice, and hepatosplenomegaly. The patient also has a grade 2/6 continuous murmur at the left upper sternal border, microphthalmia, and cataracts. What is the most likely cause?

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What is the cardiac lesion most commonly found in an infant with congenital rubella?



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Rubella can cause a serious infection in what population?



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A multiparous woman, with methyldopa controlled chronic hypertension, delivers a growth-restricted infant with cataracts, anemia, patent ductus arteriosus, and sensorineural deafness. She had a viral syndrome with rash in early pregnancy. What is the most likely causative agent?



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What is the time period that a maternal infection with rubella virus carries the greatest risk for congenital rubella syndrome in the fetus?



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Which congenital infection presents with cataracts, deafness, and heart defects?



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Which of the following most commonly causes sensorineural hearing loss secondary to inner ear direct invasion?



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Cataracts and heart defects are most commonly associated with which of the following congenital infections?



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Which of the following congenital infections is most often associated with glaucoma in children?



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Which of the following is true about rubella infection during pregnancy?



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Which of the following is true regarding congenital rubella?



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Select the congenital heart disease most often associated with congenital rubella.



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When is the congenital rubella syndrome most likely to affect a fetus?



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What is not a potential complication to the fetus that develops congenital rubella syndrome?



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A female presents in labor after only having one prenatal visit. The neonate has bilateral cataracts, microcephaly, hepatosplenomegaly, and a heart murmur. Further evaluation shows hemolytic anemia, thrombocytopenia, peripheral pulmonary stenosis, and patent ductus arteriosus. Laboratories from the prenatal visit become available. Select the most probable finding.



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Maternal infection with rubella is most likely to cause blindness in the baby if the infection occurs how many weeks after conception?



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Which of the following is the most frequent cardiovascular anomaly following maternal rubella?



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Infection of the mother with rubella during the first trimester of pregnancy is a frequent cause of congenital malformations. Which of the following has not been attributed to this infection?



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Infection of the pregnant mother with rubella during the seventh to eighth weeks may cause severe damage in the offspring to the:



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Congenital Rubella - References

References

Imported Congenital Rubella Syndrome, United States, 2017., Al Hammoud R,Murphy JR,Pérez N,, Emerging infectious diseases, 2018 Apr     [PubMed]
Fetal and neonatal abnormalities due to congenital rubella syndrome: a review of literature., Yazigi A,De Pecoulas AE,Vauloup-Fellous C,Grangeot-Keros L,Ayoubi JM,Picone O,, The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, 2017 Feb     [PubMed]
Rubella and pregnancy: diagnosis, management and outcomes., Bouthry E,Picone O,Hamdi G,Grangeot-Keros L,Ayoubi JM,Vauloup-Fellous C,, Prenatal diagnosis, 2014 Dec     [PubMed]
Congenital rubella syndrome: A brief review of public health perspectives., Kaushik A,Verma S,Kumar P,, Indian journal of public health, 2018 Jan-Mar     [PubMed]
Standardization of rubella immunoassays., Vauloup-Fellous C,, Journal of clinical virology : the official publication of the Pan American Society for Clinical Virology, 2018 May     [PubMed]
Dinede G,Wondimagegnehu A,Enquselassie F, Rubella outbreak in the school children, Addis Ababa, Ethiopia: February-April 2018. BMC infectious diseases. 2019 Mar 18;     [PubMed]
Obam Mekanda FM,Monamele CG,Simo Nemg FB,Sado Yousseu FB,Ndjonka D,Kfutwah AKW,Abernathy E,Demanou M, First report of the genomic characterization of rubella viruses circulating in Cameroon. Journal of medical virology. 2019 Mar 1;     [PubMed]
Dewan P,Gupta P, 50 Years Ago in The Journal of Pediatrics: Maternal and Congenital Rubella before 1964: Frequency, Clinical Features, and Search for Isoimmune Phenomena. The Journal of pediatrics. 2019 Feb;     [PubMed]

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