Pediatric Rubella Clinical Presentation
- Author: Elias Ezike, MD; Chief Editor: Russell W Steele, MD more...
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
Physical
Physical findings are based on the type of rubella.
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 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.
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/Uncommon | Early/Delayed | Comment |
| General | |||
| Intrauterine growth retardation | Common | Early | ... |
| Prematurity | Uncommon | Early | ... |
| Stillbirth | Uncommon | Early | ... |
| Abortion | Uncommon | Early | ... |
| Cardiovascular system | |||
| Patent ductus arteriosus | Common | Early | May occur with pulmonary artery stenosis |
| Pulmonary artery stenosis | Common | Early | Caused by intimal proliferation |
| Coarctation of the aorta | Uncommon | Early | ... |
| Myocarditis | Uncommon | Early | ... |
| Ventricular septal defect | Uncommon | Early | ... |
| Atrial septal defect | Uncommon | Early | ... |
| Eye | |||
| Cataract | Common | Early | Unilateral or bilateral |
| Retinopathy | Common | Early | Salt-and-pepper appearance; visual acuity unaffected; frequently unilateral |
| Cloudy cornea | Uncommon | Early | Spontaneous resolution |
| Glaucoma | Uncommon | Early/Delayed | May be bilateral |
| Microphthalmia | Common | Early | Common in patients with unilateral cataract |
| Subretinal neovascularization | Uncommon | Delayed | Retinopathy with macular scarring and loss of vision |
| Ear | |||
| Hearing loss | Common | Early/Delayed | Usually bilateral; mostly sensorineural; may be central in origin; rare when maternal rubella occurs >4 months' gestation; sometimes progressive |
| CNS | |||
| Meningoencephalitis | Uncommon | Early | Transient |
| Microcephaly | Uncommon | Early | May be associated with normal intelligence |
| Intracranial calcifications | Uncommon | Early | ... |
| Encephalographic abnormalities | Common | Early | Usually disappear by age 1 y |
| Mental retardation | Common | Delayed | ... |
| Behavioral disorders | Common | Delayed | Frequently related to deafness |
| Autism | Uncommon | Delayed | ... |
| Chronic progressive panencephalitis | Uncommon | Delayed | Manifest in second decade of life |
| Hypotonia | Uncommon | Early | Transitory defect |
| Speech defects | Common | Delayed | Uncommon in absence of hearing loss |
| Skin | |||
| Blueberry muffin spots | Uncommon | Early | Represents dermal erythropoiesis |
| Chronic rubelliform rash | Uncommon | Early | Usually generalized; lasts several weeks |
| Dermatoglyphic abnormalities | Common | Early | ... |
| Lungs | |||
| Interstitial pneumonia | Uncommon | Delayed | Generalized; probably immunologically mediated |
| Liver | |||
| Hepatosplenomegaly | Common | Early | Transient |
| Jaundice | Uncommon | Early | Usually appears in the first day of life |
| Hepatitis | Uncommon | Early | May not be associated with jaundice |
| Blood | |||
| Thrombocytopenia | Common | Early | Transient; no response to steroid therapy |
| Anemia | Uncommon | Early | Transient |
| Hemolytic anemia | Uncommon | Early | Transient |
| Altered blood group expression | Uncommon | Early | ... |
| Immune system | |||
| Hypogammaglobulinemia | Uncommon | Delayed | Transient |
| Lymphadenopathy | Uncommon | Early | Transient |
| Thymic hypoplasia | Uncommon | Early | Fatal |
| Bone | |||
| Radiographic lucencies | Common | Early | Transient; most common in distal femur and proximal tibia |
| Large anterior fontanel | Uncommon | Early | ... |
| Micrognathia | Uncommon | Early | ... |
| Endocrine glands | |||
| Diabetes mellitus | Common | Delayed | Usually becomes apparent in second or third decade of life |
| Thyroid disease | Uncommon | Delayed | Hypothyroidism, hyperthyroidism, and thyroiditis |
| Growth hormone deficiency | Uncommon | Delayed | ... |
| Genitourinary system | |||
| Cryptorchidism | Uncommon | Early | ... |
| Polycystic kidney | Uncommon | Early | ... |
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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- Table 1. Reported Cases of Rubella, Deaths From Rubella, and Number of Cases of Congenital Rubella Syndrome in the United States From 1969-2007[2, 3, 4, 5]
- Table 2. Clinicopathologic Abnormalities in Congenital Rubella
- Table 3. Age-Specific CD4+ T-lymphocyte Count and Percentage of Total Lymphocytes as a Criteria for Severe Immunosuppression in Persons with HIV
| Year | Number of Cases | Number of Deaths | Cases of Congenital Rubella Syndrome |
| 1969 | 57,686 | 29 | 31 |
| 1970 | 56,552 | 31 | 77 |
| 1971 | 45,086 | 20 | 68 |
| 1972 | 25,507 | 14 | 42 |
| 1973 | 27,804 | 16 | 35 |
| 1974 | 11,917 | 15 | 45 |
| 1975 | 16,652 | 21 | 30 |
| 1976 | 12,491 | 12 | 30 |
| 1977 | 20,395 | 17 | 23 |
| 1978 | 18,269 | 10 | 30 |
| 1979 | 11,795 | 1 | 62 |
| 1980 | 3,904 | 1 | 50 |
| 1981 | 2,077 | 5 | 19 |
| 1982 | 2,325 | 4 | 7 |
| 1983 | 970 | 3 | 22 |
| 1984 | 752 | 1 | 5 |
| 1985 | 630 | 1 | 0 |
| 1986 | 551 | 1 | 5 |
| 1987 | 306 | 0 | 5 |
| 1988 | 225 | 1 | 6 |
| 1989 | 396 | 4 | 3 |
| 1990 | 1,125 | 8 | 11 |
| 1991 | 1,401 | 1 | 47 |
| 1992 | 160 | 1 | 11 |
| 1993 | 192 | 0 | 5 |
| 1994 | 227 | 0 | 7 |
| 1995 | 128 | 1 | 6 |
| 1996 | 238 | 0 | 4 |
| 1997 | 181 | 0 | 5 |
| 1998 | 364 | 0 | 7 |
| 1999 | 267 | 0 | 9 |
| 2000 | 176 | 0 | 9 |
| 2001 | 23 | 2 | 3 |
| 2002 | 18 | N/A | 1 |
| 2003 | 7 | N/A | 1 |
| 2004 | 10 | N/A | 0 |
| 2005 | 11 | N/A | 1 |
| 2006 | 11 | N/A | 1 |
| 2007 | 12 | N/A | 0 |
| Abnormality | Common/Uncommon | Early/Delayed | Comment |
| General | |||
| Intrauterine growth retardation | Common | Early | ... |
| Prematurity | Uncommon | Early | ... |
| Stillbirth | Uncommon | Early | ... |
| Abortion | Uncommon | Early | ... |
| Cardiovascular system | |||
| Patent ductus arteriosus | Common | Early | May occur with pulmonary artery stenosis |
| Pulmonary artery stenosis | Common | Early | Caused by intimal proliferation |
| Coarctation of the aorta | Uncommon | Early | ... |
| Myocarditis | Uncommon | Early | ... |
| Ventricular septal defect | Uncommon | Early | ... |
| Atrial septal defect | Uncommon | Early | ... |
| Eye | |||
| Cataract | Common | Early | Unilateral or bilateral |
| Retinopathy | Common | Early | Salt-and-pepper appearance; visual acuity unaffected; frequently unilateral |
| Cloudy cornea | Uncommon | Early | Spontaneous resolution |
| Glaucoma | Uncommon | Early/Delayed | May be bilateral |
| Microphthalmia | Common | Early | Common in patients with unilateral cataract |
| Subretinal neovascularization | Uncommon | Delayed | Retinopathy with macular scarring and loss of vision |
| Ear | |||
| Hearing loss | Common | Early/Delayed | Usually bilateral; mostly sensorineural; may be central in origin; rare when maternal rubella occurs >4 months' gestation; sometimes progressive |
| CNS | |||
| Meningoencephalitis | Uncommon | Early | Transient |
| Microcephaly | Uncommon | Early | May be associated with normal intelligence |
| Intracranial calcifications | Uncommon | Early | ... |
| Encephalographic abnormalities | Common | Early | Usually disappear by age 1 y |
| Mental retardation | Common | Delayed | ... |
| Behavioral disorders | Common | Delayed | Frequently related to deafness |
| Autism | Uncommon | Delayed | ... |
| Chronic progressive panencephalitis | Uncommon | Delayed | Manifest in second decade of life |
| Hypotonia | Uncommon | Early | Transitory defect |
| Speech defects | Common | Delayed | Uncommon in absence of hearing loss |
| Skin | |||
| Blueberry muffin spots | Uncommon | Early | Represents dermal erythropoiesis |
| Chronic rubelliform rash | Uncommon | Early | Usually generalized; lasts several weeks |
| Dermatoglyphic abnormalities | Common | Early | ... |
| Lungs | |||
| Interstitial pneumonia | Uncommon | Delayed | Generalized; probably immunologically mediated |
| Liver | |||
| Hepatosplenomegaly | Common | Early | Transient |
| Jaundice | Uncommon | Early | Usually appears in the first day of life |
| Hepatitis | Uncommon | Early | May not be associated with jaundice |
| Blood | |||
| Thrombocytopenia | Common | Early | Transient; no response to steroid therapy |
| Anemia | Uncommon | Early | Transient |
| Hemolytic anemia | Uncommon | Early | Transient |
| Altered blood group expression | Uncommon | Early | ... |
| Immune system | |||
| Hypogammaglobulinemia | Uncommon | Delayed | Transient |
| Lymphadenopathy | Uncommon | Early | Transient |
| Thymic hypoplasia | Uncommon | Early | Fatal |
| Bone | |||
| Radiographic lucencies | Common | Early | Transient; most common in distal femur and proximal tibia |
| Large anterior fontanel | Uncommon | Early | ... |
| Micrognathia | Uncommon | Early | ... |
| Endocrine glands | |||
| Diabetes mellitus | Common | Delayed | Usually becomes apparent in second or third decade of life |
| Thyroid disease | Uncommon | Delayed | Hypothyroidism, hyperthyroidism, and thyroiditis |
| Growth hormone deficiency | Uncommon | Delayed | ... |
| Genitourinary system | |||
| Cryptorchidism | Uncommon | Early | ... |
| Polycystic kidney | Uncommon | Early | ... |
| Age Range | ||||
| < 12 mo | 1-5 y | 6-12 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% |

