Rubella is an acute viral disease often affecting susceptible children and young adults worldwide. Although it causes only a mild clinical illness in these groups, its public health importance is due to the teratogenic potential of the virus resulting in congenital rubella syndrome (CRS). From just before conception through the first 8–10 weeks of gestation, rubella infection of the pregnant woman can result in multiple fetal abnormalities in up to 90% of cases, and may result in miscarriage or stillbirth. CRS defects can affect any organ system, including ophthalmic, auditory, cardiac, neurologic, hepatic and haemotologic. After 18 weeks of gestation, the risk of CRS is low. The most common defects of CRS are hearing impairment and deafness, eye defects (cataracts, congenital glaucoma or pigmentary retinopathy) and cardiac defects. Infected infants can shed high amounts of rubella virus from body secretions for up to one year, thus potentially causing outbreaks.

1. Congenital Rubella Syndrome

2. Introduction
Congenital rubella syndrome [CRS]
• Congenital Infections
• Caused by Rubella Virus
Infection of nonimmune pregnant
• Spontaneous Abortion
• Stillbirth/Fetal Death
• Birth of an Infant with CRS

3. Introduction
Before Introduction of Vaccine
• Incidence of CRS: 0.1–0.2/1000 live births during endemic periods
• ̴̴0.8–4/1000 live births during rubella epidemics
As per WHO Global Estimates [2008]
• Rubella a leading cause of congenital anomalies
• Highest CRS burden in developing world
• Southeast Asia [~48%] & Africa [~38%]
• ~300 babies are born worldwide with CRS every day

4. Introduction
Pre-vaaccination Era
• Incidence of CRS: >1000 newborn per year
• ~192/100,000 live births per year
As on 3 August 2018 [WHO Data]
• Successfully controlled rubella & CRS
• Achieved 97% reduction of rubella cases [against a target of 95%]

5. Introduction
 Exact estimates are not available
 1%–15% of all infants suspected to have intrauterine infection had laboratory
evidence of CRS
 6 – 47% of School girls aging 11-18 are susceptible to Rubella Infection

6. Introduction
Rubella Virus
• Transmission
• Respiratory Route
• I. Pd.: 14-21days
• Infect Cells of URT, Multiply
• Local LN
• Blood & Systemic Infection

7. Introduction
Rubella [German Measles]
• Prodromal symptoms
• Lymphadenopathy
• Brief appearance of an exanthem
• First on face and spreads down
• Usually lasts ≤ 3 days
• Mostly Mild, Self-limiting Measles-like Disease
• Threat When Nonimmune Pregnant Get Infection Especially In First Trimester

8. Pathogenesis
Obscured due to lack of suitable animal model
Are based on microscopic analyses of aborted infected fetuses
Two possible mechanisms
• A direct cytopathic effect, which may involve rubella virus-induced apoptosis
• A virus-induced inhibition of cell division

9. Pathogenesis
Cellular damage in multiple sites, with non-inflammatory necrosis
• Eyes, Heart, Brain, & Ears
RV-induced cataractous eye lenses from first-trimester fetuses revealed
• Pyknotic Nuclei, Cytoplasmic Vacuoles, & Inclusion Bodies
• Retarded Lens Development

10. Pathogenesis
 Necrosis in endothelial cells within the blood vessels lining the heart
• Thrombosis of small vessels & necrosis of surrounding tissue
• Destruction of myocardium
Necrosis of cerebral blood vessels
• Ischemic brain damage
Damage to the epithelium of the cochlear duct
• Deafness

11. Clinical Features
 Classical Triad
Can’t Hear Bell

12. Clinical Features
 Classical Triad
 Ocular abnormalities [43%]
 Cataract, Pigmentary Retinopathy, & Infantile Glaucoma
 Sensorineural hearing loss [58%]
 Congenital heart ailment [50%]
 Patent Ductus Arteriosus [PDA]
 Pulmonary artery stenosis

13. Teratogenecity
 Infection in first 12 weeks of pregnancy
• CRS [90%], with almost a 100% risk of congenital defects
From 13 to 17 weeks
• Risk is about 60% with risk of defects about 50%
From 18 to 24 weeks
• Risk is about 25%, with hardly any risk of congenital defects

14. Clinicopathological Defects
Abnormality Common/Uncommon Early/Delayed
General
IUGR Common Early
Prematurity, Stillbirth, Abortion Uncommon Early
CVS
PDA, Pulmonary artery stenosis Common Early
Coarctation of the aorta, Myocarditis, Septal defects Uncommon Early
Ear
Hearing loss Common Early/Delayed
Eye
Cataract, Retinopathy, Microphthalmia Common Early
Cloudy cornea [With Spontaneous Resolution] Uncommon Early
Glaucoma Uncommon Early/Delayed

15. Clinicopathological Defects
Abnormality Common/Uncommon Early/Delayed
Skin
Blueberry muffin spots, Chronic rubelliform rash Uncommon Early
Dermatoglyphic abnormalities Common Early
Lungs
Interstitial pneumonia Uncommon Delayed
Liver
Hepatosplenomegaly Common Early
Hepatitis, Jaundice Uncommon Early
Bone Uncommon Early
Radiographic lucencies Common Early
Large anterior fontanel, Micrognathia Uncommon Early

16. Clinicopathological Defects
Abnormality Common/Uncommon Early/Delayed
Immune system
Hypogammaglobulinemia Uncommon Delayed
Lymphadenopathy, Thymic hypoplasia Uncommon Early
Blood
Anemia, Hemolytic anemia, Altered blood group expression Uncommon Early
CNS
Meningoencephalitis, Microcephaly, Intracranial
Calcifications, Hypotonia
Uncommon Early
Encephalographic abnormalities Common Early
Mental retardation, Behavioral disorders, Speech defects Common Delayed
Autism, Chronic progressive panencephalitis Uncommon Delayed

17. Clinicopathological Defects
Abnormality Common/Uncommon Early/Delayed
Endocrine glands
Diabetes mellitus Common Delayed
Hypothyroidism, Hyperthyroidism,Thyroiditis, Growth
hormone deficiency
Uncommon Delayed
Genitourinary system
Cryptorchidism, Polycystic kidney Uncommon Early

18. WHO Case Definition- Suspected Case
 Any infant < 12 months of age that presents with any of the following:
• Congenital Heart Disease
• Suspicion of hearing impairment
• One or more of the following eye signs:
• Cataract
• Congenital glaucoma
• Pigmentary retinopathy
 Any infant < 12 months of age in whom a health worker suspects CRS, even
without apparent signs of CRS, including maternal history of suspected or
confirmed rubella during pregnancy

19. WHO Case Definition- Final Classification
 Depends, in part, on identifying Group A or Group B clinical signs of CRS :
Group A Group B
Cataract(s)
Congenital Glaucoma
Pigmentary Retinopathy
Congenital Heart Disease
Hearing Impairment
Purpura
Splenomegaly
Microcephaly
Developmental Delay
Meningoencephalitis
Radiolucent Bone Disease
Jaundice within the First 24H after birth
 Using these Clinical Signs, final classifications are made

20. WHO Case Definition- Final Classification
 Laboratory-confirmed CRS:
• A suspected CRS case with at least one sign from group A
• Meets the laboratory criteria for confirmation of CRS
 Clinically Compatible CRS:
• A suspected CRS case without an adequate specimen
• At least two of the complications from group A OR
• One from group A and one from group B

21. WHO Case Definition- Final Classification
 Congenital Rubella Infection [CRI]:
• An infant who has none of the clinical signs of CRS from group A
• Meets the laboratory criteria for CRS
 Discarded:
• A suspected CRS case with an adequate specimen not meeting the laboratory-
confirmed case definition OR
• A suspected case without an adequate laboratory specimen and not meeting
the clinically compatible case definition

22. Laboratory Diagnosis
Specimen
• For Detection of IgM /IgG by ELISA
• Serum
• Specimens for viral detection by Viral Culture or RT-PCR
• Throat Swabs, Oral fluids, Nasopharyngeal Secretions
• Cataract Tissue, Urine, Dried Blood Spots

23. Laboratory Diagnosis-Confirmation 0f CRI/CRS
 For infants < 6 months
• Rubella IgM detected
For infants ≥ 6 months but < 12 months
• Rubella IgM & IgG detected, OR
• Sustained rubella IgG antibody level OR
• Rubella virus detected

24. Immunization
 Live Attenuated Viral Rubella Vaccine
• Route: Intramuscular/Subcutaneous
• Monovalent
• MR [measles-rubella]
• MMR [mumps-measles-rubella]

25. Prevention
 Immunization
ANC
• TORCH Test

26. Thank you