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Pediatric Rubella Clinical Presentation

  • Author: Elias Ezike, MD; Chief Editor: Russell W Steele, MD  more...
 
Updated: Dec 18, 2014
 

History

Postnatal rubella

Rubella virus is transmitted from person to person via the aerosolized particles from the respiratory tract. A history of exposure may not be present. Individuals may acquire the infection from a completely asymptomatic patient or from an individual shedding the virus during the incubation period.

The incubation is usually 14-21 days after exposure to a person with rubella.

Prodromal symptoms are unusual in young children but are common in adolescents and adults.

The following signs and symptoms usually appear 1-5 days before the onset of rash:

  • Eye pain on lateral and upward eye movement (a particularly troublesome complaint)
  • Conjunctivitis
  • Sore throat
  • Headache
  • General body aches
  • Low-grade fever
  • Chills
  • Anorexia
  • Nausea
  • Tender lymphadenopathy (particularly posterior auricular and suboccipital lymph nodes)
  • Forchheimer sign (an enanthem observed in 20% of patients with rubella during the prodromal period; can be present in some patients during the initial phase of the exanthem; consists of pinpoint or larger petechiae that usually occur on the soft palate)

Congenital rubella

Congenital rubella history focuses on the following:

  • The number of weeks of pregnancy when maternal exposure to rubella occurred (The risk of congenital rubella syndrome is higher if maternal exposure occurs during the first trimester.)
  • Maternal history of immunization or medical history of rubella
  • Evidence of intrauterine growth retardation during pregnancy
  • Manifestations suggestive of congenital rubella syndrome in a child
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Physical

Physical findings are based on the type of rubella.

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Postnatal Rubella

Rash

The exanthem of rubella consists of a discrete rose-pink maculopapular rash ranging from 1-4 mm. See the image below.

Image in a 4-year-old girl with a 4-day history ofImage in a 4-year-old girl with a 4-day history of low-grade fever, symptoms of an upper respiratory tract infection, and rash. Courtesy of Pamela L. Dyne, MD.

Rash in adults may be quite pruritic.

The synonym "3-day measles" derives from the typical course of rubella exanthem that starts initially on the face and neck and spreads centrifugally to the trunk and extremities within 24 hours. It then begins to fade on the face on the second day and disappears throughout the body by the end of the third day.

Temperature

Fever is usually not higher than 38.5°C (101.5°F).

Lymph nodes

Enlarged posterior auricular and suboccipital lymph nodes are usually found on physical examination.

Mouth

The Forchheimer sign may still be present on the soft palate.

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Congenital Rubella Syndrome

The classic triad presentation of congenital rubella syndrome consists of the following:

  • Sensorineural hearing loss is the most common manifestation of congenital rubella syndrome. It occurs in approximately 58% of patients. Studies have demonstrated that approximately 40% of patients with congenital rubella syndrome may present with deafness as the only abnormality without other manifestations. Hearing impairment may be bilateral or unilateral and may not be apparent until the second year of life.
  • Ocular abnormalities including cataract, infantile glaucoma, and pigmentary retinopathy occur in approximately 43% of children with congenital rubella syndrome. Both eyes are affected in 80% of patients, and the most frequent findings are cataract and rubella retinopathy. Rubella retinopathy consists of a salt-and-pepper pigmentary change or a mottled, blotchy, irregular pigmentation, usually with the greatest density in the macula. The retinopathy is benign and nonprogressive and does not interfere with vision (in contrast to the cataract) unless choroid neovascularization develops in the macula.
  • Congenital heart disease including patent ductus arteriosus (PDA) and pulmonary artery stenosis is present in 50% of infants infected in the first 2 months' gestation. Cardiac defects and deafness occur in all infants infected during the first 10 weeks of pregnancy and deafness alone is noted in one third of those infected at 13-16 weeks of gestation.

Other findings in congenital rubella syndrome include the following:

  • Intrauterine growth retardation, prematurity, stillbirth, and abortion
  • CNS abnormalities, including mental retardation, behavioral disorders, encephalographic abnormalities, hypotonia, meningoencephalitis, and microcephaly
  • Hepatosplenomegaly
  • Jaundice
  • Hepatitis
  • Skin manifestations, including blueberry muffin spots that represent dermal erythropoiesis and dermatoglyphic abnormalities
  • Bone lesions, such as radiographic lucencies
  • Endocrine disorders, including late manifestations in congenital rubella syndrome usually occurring in the second or third decade of life (eg, thyroid abnormalities, diabetes mellitus)
  • Hematologic disorders, such as anemia and thrombocytopenic purpura

Table 2. Clinicopathologic Abnormalities in Congenital Rubella (Open Table in a new window)

Abnormality Common/UncommonEarly/DelayedComment
General
Intrauterine growth retardationCommonEarly...
PrematurityUncommonEarly...
StillbirthUncommonEarly...
AbortionUncommonEarly...
Cardiovascular system
Patent ductus arteriosusCommonEarlyMay occur with pulmonary artery stenosis
Pulmonary artery stenosisCommonEarlyCaused by intimal proliferation
Coarctation of the aortaUncommonEarly...
MyocarditisUncommonEarly...
Ventricular septal defectUncommonEarly...
Atrial septal defectUncommonEarly...
Eye
CataractCommonEarlyUnilateral or bilateral
RetinopathyCommonEarlySalt-and-pepper appearance; visual acuity unaffected; frequently unilateral
Cloudy corneaUncommonEarlySpontaneous resolution
GlaucomaUncommonEarly/DelayedMay be bilateral
MicrophthalmiaCommonEarlyCommon in patients with unilateral cataract
Subretinal neovascularizationUncommonDelayedRetinopathy with macular scarring and loss of vision
Ear
Hearing lossCommonEarly/DelayedUsually bilateral; mostly sensorineural; may be central in origin; rare when maternal rubella occurs >4 months' gestation; sometimes progressive
CNS
MeningoencephalitisUncommonEarlyTransient
MicrocephalyUncommonEarlyMay be associated with normal intelligence
Intracranial calcificationsUncommonEarly...
Encephalographic abnormalitiesCommonEarlyUsually disappear by age 1 y
Mental retardationCommonDelayed...
Behavioral disordersCommonDelayedFrequently related to deafness
AutismUncommonDelayed...
Chronic progressive panencephalitisUncommonDelayedManifest in second decade of life
HypotoniaUncommonEarlyTransitory defect
Speech defectsCommonDelayedUncommon in absence of hearing loss
Skin
Blueberry muffin spotsUncommonEarlyRepresents dermal erythropoiesis
Chronic rubelliform rashUncommonEarlyUsually generalized; lasts several weeks
Dermatoglyphic abnormalitiesCommonEarly...
Lungs
Interstitial pneumoniaUncommonDelayedGeneralized; probably immunologically mediated
Liver
HepatosplenomegalyCommonEarlyTransient
JaundiceUncommonEarlyUsually appears in the first day of life
HepatitisUncommonEarlyMay not be associated with jaundice
Blood
ThrombocytopeniaCommonEarlyTransient; no response to steroid therapy
AnemiaUncommonEarlyTransient
Hemolytic anemiaUncommonEarlyTransient
Altered blood group expressionUncommonEarly...
Immune system
HypogammaglobulinemiaUncommonDelayedTransient
LymphadenopathyUncommonEarlyTransient
Thymic hypoplasiaUncommonEarlyFatal
Bone
Radiographic lucenciesCommonEarlyTransient; most common in distal femur and proximal tibia
Large anterior fontanelUncommonEarly...
MicrognathiaUncommonEarly...
Endocrine glands
Diabetes mellitusCommonDelayedUsually becomes apparent in second or third decade of life
Thyroid diseaseUncommonDelayedHypothyroidism, hyperthyroidism, and thyroiditis
Growth hormone deficiencyUncommonDelayed...
Genitourinary system
CryptorchidismUncommonEarly...
Polycystic kidneyUncommonEarly...
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Causes

Rubella and congenital rubella syndrome are caused by rubella virus. Only one antigenic type of rubella virus is available, and humans are the only natural hosts. The virus is spherical with a diameter of 50-70 nm, has a central core (ie, nucleocapsid), and is covered externally by a lipid-containing envelope. The nucleocapsid is composed of polypeptide (C protein) and a single-stranded RNA.

Its outer envelope is made up of glycosylated lipoprotein, which contains 2 virus-specific polypeptides (E1, E2) and a host-cell–derived lipid. These 2 envelope proteins comprise the spiked 5-nm to 6-nm surface projections that are observed on the outer membrane of rubella virus and are important for the virulence of the virus.

Monoclonal antibodies directed against epitopes of E1 and E2 have neutralizing activity. Protein E1 is the viral hemagglutinin that binds both hemagglutination-inhibiting and hemolysis-inhibiting antibodies.

Rubella virus is rapidly inactivated by 70% alcohol, ethylene oxide, formalin, ether, acetone, chloroform, free chlorine, deoxycholate, beta-propiolactone, ultraviolet light, extreme pH (< 6.8 or >8.1), heat (>56°C), and cold (from -10°C to -20°C). It is resistant to thimerosal and is stable at temperatures of -60°C or less.

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Contributor Information and Disclosures
Author

Elias Ezike, MD Consulting Staff, Beaumont Pediatric Center, PLLC

Elias Ezike, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Coauthor(s)

Jocelyn Y Ang, MD, FAAP, FIDSA Associate Professor, Department of Pediatrics, Wayne State University School of Medicine; Consulting Staff, Division of Infectious Diseases, Children's Hospital of Michigan

Jocelyn Y Ang, MD, FAAP, FIDSA is a member of the following medical societies: American Academy of Pediatrics, Infectious Diseases Society of America, Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Chief Editor

Russell W Steele, MD Clinical Professor, Tulane University School of Medicine; Staff Physician, Ochsner Clinic Foundation

Russell W Steele, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American Pediatric Society, American Society for Microbiology, Infectious Diseases Society of America, Louisiana State Medical Society, Pediatric Infectious Diseases Society, Society for Pediatric Research, Southern Medical Association

Disclosure: Nothing to disclose.

Additional Contributors

Leonard R Krilov, MD Chief of Pediatric Infectious Diseases and International Adoption, Vice Chair, Department of Pediatrics, Winthrop University Hospital; Professor of Pediatrics, Stony Brook University School of Medicine

Leonard R Krilov, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, Infectious Diseases Society of America, Pediatric Infectious Diseases Society, Society for Pediatric Research

Disclosure: Nothing to disclose.

Acknowledgements

Leslie L Barton, MD Professor Emerita of Pediatrics, University of Arizona College of Medicine

Leslie L Barton, MD is a member of the following medical societies: American Academy of Pediatrics, Association of Pediatric Program Directors, Infectious Diseases Society of America, and Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

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Number of rubella cases per year.
Number of congenital rubella syndrome cases per year.
Deaths from rubella per year.
Image in a 4-year-old girl with a 4-day history of low-grade fever, symptoms of an upper respiratory tract infection, and rash. Courtesy of Pamela L. Dyne, MD.
Table 1. Reported Cases of Rubella, Deaths From Rubella, and Number of Cases of Congenital Rubella Syndrome in the United States From 1969-2007[4, 5, 6, 7]
YearNumber of CasesNumber of DeathsCases of Congenital Rubella Syndrome
196957,6862931
197056,5523177
197145,0862068
197225,5071442
197327,8041635
197411,9171545
197516,6522130
197612,4911230
197720,3951723
197818,2691030
197911,795162
19803,904150
19812,077519
19822,32547
1983970322
198475215
198563010
198655115
198730605
198822516
198939643
19901,125811
19911,401147
1992160111
199319205
199422707
199512816
199623804
199718105
199836407
199926709
200017609
20012323
200218N/A1
20037N/A1
200410N/A0
200511N/A1
200611N/A1
200712N/A0
Table 2. Clinicopathologic Abnormalities in Congenital Rubella
Abnormality Common/UncommonEarly/DelayedComment
General
Intrauterine growth retardationCommonEarly...
PrematurityUncommonEarly...
StillbirthUncommonEarly...
AbortionUncommonEarly...
Cardiovascular system
Patent ductus arteriosusCommonEarlyMay occur with pulmonary artery stenosis
Pulmonary artery stenosisCommonEarlyCaused by intimal proliferation
Coarctation of the aortaUncommonEarly...
MyocarditisUncommonEarly...
Ventricular septal defectUncommonEarly...
Atrial septal defectUncommonEarly...
Eye
CataractCommonEarlyUnilateral or bilateral
RetinopathyCommonEarlySalt-and-pepper appearance; visual acuity unaffected; frequently unilateral
Cloudy corneaUncommonEarlySpontaneous resolution
GlaucomaUncommonEarly/DelayedMay be bilateral
MicrophthalmiaCommonEarlyCommon in patients with unilateral cataract
Subretinal neovascularizationUncommonDelayedRetinopathy with macular scarring and loss of vision
Ear
Hearing lossCommonEarly/DelayedUsually bilateral; mostly sensorineural; may be central in origin; rare when maternal rubella occurs >4 months' gestation; sometimes progressive
CNS
MeningoencephalitisUncommonEarlyTransient
MicrocephalyUncommonEarlyMay be associated with normal intelligence
Intracranial calcificationsUncommonEarly...
Encephalographic abnormalitiesCommonEarlyUsually disappear by age 1 y
Mental retardationCommonDelayed...
Behavioral disordersCommonDelayedFrequently related to deafness
AutismUncommonDelayed...
Chronic progressive panencephalitisUncommonDelayedManifest in second decade of life
HypotoniaUncommonEarlyTransitory defect
Speech defectsCommonDelayedUncommon in absence of hearing loss
Skin
Blueberry muffin spotsUncommonEarlyRepresents dermal erythropoiesis
Chronic rubelliform rashUncommonEarlyUsually generalized; lasts several weeks
Dermatoglyphic abnormalitiesCommonEarly...
Lungs
Interstitial pneumoniaUncommonDelayedGeneralized; probably immunologically mediated
Liver
HepatosplenomegalyCommonEarlyTransient
JaundiceUncommonEarlyUsually appears in the first day of life
HepatitisUncommonEarlyMay not be associated with jaundice
Blood
ThrombocytopeniaCommonEarlyTransient; no response to steroid therapy
AnemiaUncommonEarlyTransient
Hemolytic anemiaUncommonEarlyTransient
Altered blood group expressionUncommonEarly...
Immune system
HypogammaglobulinemiaUncommonDelayedTransient
LymphadenopathyUncommonEarlyTransient
Thymic hypoplasiaUncommonEarlyFatal
Bone
Radiographic lucenciesCommonEarlyTransient; most common in distal femur and proximal tibia
Large anterior fontanelUncommonEarly...
MicrognathiaUncommonEarly...
Endocrine glands
Diabetes mellitusCommonDelayedUsually becomes apparent in second or third decade of life
Thyroid diseaseUncommonDelayedHypothyroidism, hyperthyroidism, and thyroiditis
Growth hormone deficiencyUncommonDelayed...
Genitourinary system
CryptorchidismUncommonEarly...
Polycystic kidneyUncommonEarly...
Table 3. Age-Specific CD4+ T-lymphocyte Count and Percentage of Total Lymphocytes as a Criteria for Severe Immunosuppression in Persons with HIV
 Age Range
< 12 mo1-5 y6-12 y≥13 y
Total CD4+ T-lymphocytes< 750/mcl< 500/mcl< 200/mcl< 200/mcl
CD4+ T-lymphocytes (as % of total lymphocytes)< 15%< 15%< 15%< 14%
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