Congenital Varicella Zoster Infections

Introduction to Congenital Varicella Zoster Infections

Congenital Varicella Zoster Virus (VZV) infection, also known as congenital varicella syndrome (CVS), occurs when a pregnant woman contracts primary VZV infection (chickenpox) and transmits the virus to her fetus. This rare but serious condition can lead to significant morbidity and mortality in affected infants.

  • VZV is a DNA virus of the Herpesviridae family
  • Incidence of CVS: Approximately 1-2% when maternal infection occurs in the first 20 weeks of gestation
  • Risk is highest when maternal infection occurs between 13-20 weeks gestation

Understanding the pathophysiology, clinical presentation, and management of congenital varicella is crucial for healthcare providers to optimize outcomes for affected infants and provide appropriate counseling to pregnant women.



Video Notes



Congenital Varicella Zoster Infections
  1. Q: What causes congenital varicella syndrome? A: Maternal varicella-zoster virus (VZV) infection during pregnancy
  2. Q: During which trimester of pregnancy is the risk of congenital varicella syndrome highest? A: First and early second trimester (before 20 weeks gestation)
  3. Q: What is the approximate risk of congenital varicella syndrome if maternal infection occurs before 20 weeks gestation? A: 0.4-2%
  4. Q: What are the classic triad of symptoms in congenital varicella syndrome? A: Skin lesions, neurological defects, and eye abnormalities
  5. Q: What type of skin lesions are characteristic of congenital varicella syndrome? A: Cicatricial skin lesions in a dermatomal distribution
  6. Q: What neurological abnormalities may be seen in congenital varicella syndrome? A: Microcephaly, cortical atrophy, seizures, and intellectual disability
  7. Q: What ocular abnormalities are associated with congenital varicella syndrome? A: Chorioretinitis, microphthalmia, and cataracts
  8. Q: What limb abnormalities may occur in congenital varicella syndrome? A: Hypoplasia of limbs, especially fingers and toes
  9. Q: How is congenital varicella syndrome diagnosed prenatally? A: Through maternal history, ultrasound findings, and amniocentesis for VZV PCR
  10. Q: What is the mortality rate for infants with congenital varicella syndrome? A: Approximately 30% in the first few months of life
  11. Q: How can congenital varicella syndrome be prevented? A: Vaccination of women of childbearing age before pregnancy
  12. Q: What is the recommended management for VZV-seronegative pregnant women exposed to varicella? A: Administration of varicella-zoster immunoglobulin (VZIG) within 96 hours of exposure
  13. Q: Can the varicella vaccine be given during pregnancy? A: No, it is contraindicated during pregnancy as it contains live attenuated virus
  14. Q: What is neonatal varicella, and how does it differ from congenital varicella syndrome? A: Neonatal varicella occurs when maternal infection is near term, resulting in chickenpox in the newborn
  15. Q: What is the risk period for severe neonatal varicella infection? A: Maternal infection occurring from 5 days before to 2 days after delivery
  16. Q: How is neonatal varicella treated? A: With intravenous acyclovir and supportive care
  17. Q: What long-term complications may occur in survivors of congenital varicella syndrome? A: Developmental delays, learning disabilities, and visual impairment
  18. Q: How does maternal immunity to varicella affect the risk of congenital infection? A: Maternal immunity significantly reduces the risk of congenital varicella syndrome
  19. Q: What is the role of PCR testing in diagnosing congenital varicella syndrome? A: It can detect VZV DNA in amniotic fluid or fetal blood
  20. Q: How does congenital varicella syndrome affect the cardiovascular system? A: It may cause ventricular septal defects and other cardiac abnormalities
  21. Q: What gastrointestinal abnormalities may be associated with congenital varicella syndrome? A: Gastroesophageal reflux, poor feeding, and failure to thrive
  22. Q: How does congenital varicella syndrome affect the skeletal system? A: It may cause bone and muscle hypoplasia, especially in limbs
  23. Q: What is the significance of maternal varicella infection after 20 weeks gestation? A: The risk of congenital varicella syndrome is very low, but the infant may develop herpes zoster in infancy
  24. Q: How does congenital varicella syndrome affect the urogenital system? A: It may cause hydroureter, hydronephrosis, and genital hypoplasia
  25. Q: What is the role of serial ultrasound examinations in pregnancies affected by varicella? A: To monitor for signs of fetal infection and structural abnormalities
  26. Q: How does congenital varicella syndrome differ from fetal varicella infection? A: Congenital syndrome involves permanent malformations, while fetal infection may result in asymptomatic or mild disease
  27. Q: What is the long-term prognosis for children with congenital varicella syndrome? A: Variable, depending on the severity of organ involvement, with many requiring lifelong multidisciplinary care
  28. Q: How does maternal shingles (herpes zoster) during pregnancy affect the fetus? A: Maternal shingles does not typically pose a risk to the fetus due to maternal antibodies
  29. Q: What is the recommended follow-up for infants born to mothers with varicella during pregnancy? A: Close monitoring for signs of infection and developmental assessments




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The notes provided on Pediatime are generated from online resources and AI sources and have been carefully checked for accuracy. However, these notes are not intended to replace standard textbooks. They are designed to serve as a quick review and revision tool for medical students and professionals, and to aid in theory exam preparation. For comprehensive learning, please refer to recommended textbooks and guidelines.





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