Summary about Disease
Fetal varicella syndrome (FVS) is a rare condition that can occur when a pregnant woman contracts varicella (chickenpox) during pregnancy. It can lead to a range of birth defects in the developing fetus. The severity of FVS depends on the gestational age at which the mother contracts the virus, with the highest risk occurring during the first and early second trimester.
Symptoms
Symptoms of FVS in the infant can include:
Skin scarring (cicatricial skin lesions), often in a dermatomal distribution
Limb hypoplasia (underdeveloped limbs)
Eye abnormalities (e.g., chorioretinitis, microphthalmia, cataracts)
Neurological abnormalities (e.g., microcephaly, cortical atrophy, intellectual disability, seizures)
Gastrointestinal or genitourinary abnormalities.
Causes
FVS is caused by the varicella-zoster virus (VZV) crossing the placenta from the mother to the fetus during maternal varicella infection. This infection can disrupt normal fetal development, leading to the various symptoms associated with the syndrome.
Medicine Used
There is no specific medicine to cure FVS after birth. Treatment focuses on managing the individual symptoms and complications.
Antivirals: If the mother contracts chickenpox late in pregnancy, the newborn might receive varicella-zoster immune globulin (VZIG) or acyclovir.
Symptomatic treatment: Treatment for specific defects, such as surgery for cataracts or physical therapy for limb hypoplasia, is provided as needed.
Is Communicable
FVS itself is not communicable from the infant to others. However, if the infant is actively shedding the varicella-zoster virus (e.g., if the mother had chickenpox shortly before delivery and the infant is born with congenital varicella infection), then contact precautions may be necessary.
Precautions
Preventing maternal varicella infection during pregnancy is the primary precaution. This includes:
Vaccination: Women of childbearing age should be vaccinated against varicella if they are not immune.
Avoiding exposure: Pregnant women who are not immune should avoid contact with individuals who have chickenpox or shingles.
VZIG: If a pregnant woman who is not immune is exposed to varicella, varicella-zoster immune globulin (VZIG) can be administered to reduce the severity of the infection.
How long does an outbreak last?
FVS is not an "outbreak" but a congenital syndrome. Chickenpox outbreaks in a person typically last 5-10 days. The consequences of the maternal infection during pregnancy, resulting in FVS, are lifelong for the affected child.
How is it diagnosed?
Diagnosis of FVS is usually based on the presence of characteristic clinical findings in the infant, coupled with a history of maternal varicella infection during pregnancy.
Physical examination: Assessing the infant for the characteristic symptoms of FVS.
Maternal history: Obtaining a detailed history of maternal varicella infection during pregnancy, including the gestational age at the time of infection.
Laboratory tests: PCR testing of infant samples (e.g., blood, skin lesion swabs) can detect VZV DNA. Serological tests in the infant can be difficult to interpret due to maternal antibodies.
Imaging studies: Brain imaging (MRI or CT) can help identify neurological abnormalities.
Timeline of Symptoms
The timeline of symptom development is prenatal. The maternal infection during pregnancy leads to developmental abnormalities in the fetus. These abnormalities are present at birth and can become more apparent as the child grows.
Important Considerations
Genetic Counseling: Genetic counseling may be helpful for families affected by FVS, especially regarding recurrence risks.
Multidisciplinary Care: Infants with FVS often require multidisciplinary care from specialists such as pediatricians, neurologists, ophthalmologists, and physical therapists.
Support Groups: Support groups can provide emotional support and resources for families caring for children with FVS.
Prognosis: The prognosis for children with FVS varies depending on the severity of the condition. Some children may have mild disabilities, while others may have more significant impairments.