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
- Q: What causes congenital varicella syndrome? A: Maternal varicella-zoster virus (VZV) infection during pregnancy
- Q: During which trimester of pregnancy is the risk of congenital varicella syndrome highest? A: First and early second trimester (before 20 weeks gestation)
- Q: What is the approximate risk of congenital varicella syndrome if maternal infection occurs before 20 weeks gestation? A: 0.4-2%
- Q: What are the classic triad of symptoms in congenital varicella syndrome? A: Skin lesions, neurological defects, and eye abnormalities
- Q: What type of skin lesions are characteristic of congenital varicella syndrome? A: Cicatricial skin lesions in a dermatomal distribution
- Q: What neurological abnormalities may be seen in congenital varicella syndrome? A: Microcephaly, cortical atrophy, seizures, and intellectual disability
- Q: What ocular abnormalities are associated with congenital varicella syndrome? A: Chorioretinitis, microphthalmia, and cataracts
- Q: What limb abnormalities may occur in congenital varicella syndrome? A: Hypoplasia of limbs, especially fingers and toes
- Q: How is congenital varicella syndrome diagnosed prenatally? A: Through maternal history, ultrasound findings, and amniocentesis for VZV PCR
- Q: What is the mortality rate for infants with congenital varicella syndrome? A: Approximately 30% in the first few months of life
- Q: How can congenital varicella syndrome be prevented? A: Vaccination of women of childbearing age before pregnancy
- 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
- Q: Can the varicella vaccine be given during pregnancy? A: No, it is contraindicated during pregnancy as it contains live attenuated virus
- 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
- Q: What is the risk period for severe neonatal varicella infection? A: Maternal infection occurring from 5 days before to 2 days after delivery
- Q: How is neonatal varicella treated? A: With intravenous acyclovir and supportive care
- Q: What long-term complications may occur in survivors of congenital varicella syndrome? A: Developmental delays, learning disabilities, and visual impairment
- Q: How does maternal immunity to varicella affect the risk of congenital infection? A: Maternal immunity significantly reduces the risk of congenital varicella syndrome
- 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
- Q: How does congenital varicella syndrome affect the cardiovascular system? A: It may cause ventricular septal defects and other cardiac abnormalities
- Q: What gastrointestinal abnormalities may be associated with congenital varicella syndrome? A: Gastroesophageal reflux, poor feeding, and failure to thrive
- Q: How does congenital varicella syndrome affect the skeletal system? A: It may cause bone and muscle hypoplasia, especially in limbs
- 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
- Q: How does congenital varicella syndrome affect the urogenital system? A: It may cause hydroureter, hydronephrosis, and genital hypoplasia
- 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
- 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
- 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
- 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
- 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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