Congenital Conditions Pregnancy-Related Conditions (Viral Infection In Fetus)
Congenital Varicella Syndrome (CVS)
IMPORTANT INFORMATION
The information on the Rare Awareness Rare Education (RARE) Portal is intended for educational purposes only and does not replace professional advice.
Rare diseases typically display a high level of symptom complexity and variability. Individuals diagnosed with the same rare disease may be impacted differently and each person’s experience is unique. Please seek support from qualified healthcare professionals to learn more about the most suitable care and support options for you.
For further information on CVS, please refer to the Genetic and Rare Diseases (GARD) Information Center – Congenital Varicella Syndrome1 and The Australian Immunisation Handbook – Varicella (chickenpox).2
There are currently no known organisations in Australia specifically for this rare disease. If you know of any rare disease organisation/s supporting people living with CVS in Australia, please let us know via the Contribute page. If you are interested in starting an Australian organisation for people living with CVS, please see Engaged, Ethical and Effective: A Guide for Rare Disease Organisation Leaders in Australia.
If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.
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Emergency Management | Clinical Care Guidelines | Synonyms | Summary | Personal Stories | Symptoms | Cause/Inheritance | Diagnosis | Treatment | Clinical Care | Research | Rare Disease Organisation(s) | Support Services/Resources | Mental Health | Other | References
Emergency Management
There may be special considerations for the emergency management of individuals living with CVS or pregnant women with chickenpox (maternal varicella zoster infection) presenting to emergency departments.
Clinical Care Guidelines
There are guidelines for management of varicella infection (chickenpox) in pregnancy; however some of them are from other countries and may not necessarily be in line with Australian guidelines:
- South Australia (SA) Health: Varicella Zoster (chicken pox) in Pregnancy Clinical Guidelines; please note that this was last updated in 2015
- Society of Obstetricians and Gynaecologists of Canada: Management of Varicella Infection (Chickenpox) in Pregnancy; please note that this was published in 2012
- Royal College of Obstetrics and Gynecology UK: Chickenpox in Pregnancy; please note that this was published in 2015
Synonyms
Antenatal varicella virus infection; Mother-to-child transmission of varicella syndrome; Fetal varicella syndrome
ORPHA:291
ICD-11: KA62.2 Congenital Varicella Zoster virus infection
Summary
Congenital varicella syndrome (CVS) is a very rare disorder that presents from birth, as a result of the mother being infected with chickenpox (maternal varicella zoster) early during pregnancy (usually in the first 20 weeks).1,3 Infants with CVS often have low birth weight and may be born with abnormalities of the skin, eyes, brain, arms, legs, hands and feet. The symptoms of this disorder can be severe, but varies between individuals.1,3
Women who are planning pregnancy are recommended to be vaccinated against the varicella zoster virus (VZV), or to do a blood test to check if they have immunity to VZV.2,4 Vaccination during pregnancy is not advised.4 Pregnant women who have not had chickenpox, or have been tested to not have immunity to chickenpox (seronegative), are advised to avoid any contact with those who have chickenpox or shingles, throughout their pregnancy. If they have come into such contact, they should seek medical advice from doctors immediately.5 In addition to the risk of CVS during early pregnancy, a maternal infection during late pregnancy (perinatal varicella infection) can also pose risks to the child – newborns can develop chickenpox (neonatal varicella infection), which in some cases can be severe and fatal.6
Useful Links for Professionals
Orphanet: Congenital varicella syndrome
Human Phenotype Ontology (HPO): Congenital varicella syndrome
Symptoms
Congenital varicella syndrome (CVS) may result in low birth weight, scarring of the skin as well as defects of the eyes, arms, legs and brain.1,2 The symptoms and presentation of CVS may vary between individuals.
Please speak to your medical team to learn more about the symptoms and complications of CVS.
Cause/Inheritance
Whilst it is rare, a chicken pox infection during early pregnancy (maternal varicella zoster) can result in a baby being born with congenital varicella syndrome (CVS). 1,2
Diagnosis
Diagnosis of a varicella zoster virus (VZV) infection can be made through testing for antibodies against VZV in the blood or viral detection in the blood, fluid or tissue samples.6 More information about these tests can be found at Pathology Tests Explained: Varicella zoster virus.
Congenital varicella syndrome (CVS) is usually diagnosed through confirmation of a maternal VZV infection early in pregnancy and presentation of clinical features of CVS in the newborn.3 In some cases, antenatal testing may be used to diagnosis CVS before the child is born. Please consult your medical team find out more information about the possibility of antenatal testing and the risks involved.
Treatment
It is best to speak with your medical team to learn more about the possible treatment or management of congenital varicella syndrome (CVS) and its associated symptoms. Treatment will depend on an individual’s specific symptoms and disease presentation.
Clinical Care
Healthcare professionals involved in the treatment of congenital rubella syndrome (CRS) may include general practitioners (GP), paediatricians, infectious disease specialists, ophthalmologists (eye specialists), neurologists, surgeons and physical therapists.3 The need for different healthcare professionals may change over a person’s lifetime and may extend beyond those listed here.
Research
Please visit Australian Clinical Trials to learn about any clinical trials for CVS in Australia; there may not be any currently available.
Information regarding clinical trials for CVS in other countries can be found at ClinicalTrials.gov; there may not be any currently available.
It is best to discuss your interest in any clinical trials with your medical team to determine if the available clinical trials are suitable for you.
Rare Disease Organisation(s)
There are currently no known rare disease organisations/s supporting people living with CVS in Australia. If you know of any CVS organisations in Australia, please reach out via the Contribute page.
Support Services/Resources
See the National and State Services pages.
Mental Health
See the ‘Mental Health’ sections listed on the National and State Services pages.
Other
Varicella is a notifiable disease in most states and territories in Australia.2 State and territory public health authorities should be informed or consulted about cases of varicella.
Information about varicella (chickenpox) vaccination, including other contraindications, can be found at The Australian Immunisation Handbook: Varicella (chickenpox) vaccine information.
Further information about congenital varicella syndrome can be found at:
- Healthdirect: Chickenpox (varicella) (including a section on chickenpox and pregnancy)
- National Organization for Rare Disorders (NORD): Congenital Varicella Syndrome
References
- Genetic and Rare Diseases (GARD) Information Center. Congenital varicella syndrome. Accessed August 16, 2023. https://rarediseases.info.nih.gov/diseases/45/congenital-varicella-syndrome
- Australian Technical Advisory Group on Immunisation (ATAGI). Varicella (chickenpox). In: Australian Immunisation Handbook, Australian Government Department of Health and Aged Care. Canberra; 2022. Accessed August 16, 2023. https://immunisationhandbook.health.gov.au/contents/vaccine-preventable-diseases/varicella-chickenpox
- National Organization for Rare Disorders (NORD). Congenital Varicella Syndrome. Accessed August 16, 2023. https://rarediseases.org/rare-diseases/congenital-varicella-syndrome/
- Australian Technical Advisory Group on Immunisation (ATAGI). Vaccination for women who are planning pregnancy, pregnant or breastfeeding. In: Australian Immunisation Handbook, Australian Government Department of Health and Aged Care. Canberra; 2022. Accessed August 21, 2023. https://immunisationhandbook.health.gov.au/contents/vaccination-for-special-risk-groups/vaccination-for-women-who-are-planning-pregnancy-pregnant-or-breastfeeding
- Communicable Diseases Factsheet: Chickenpox and Shingles. 2022. NSW Government. 3p. Available from: https://www.health.nsw.gov.au/Infectious/factsheets/Factsheets/chickenpox.pdf
- Pathology Tests Explained. Varicella zoster virus. Accessed August 16, 2023. https://pathologytestsexplained.org.au/ptests-pro.php?q=Varicella+zoster+virus
Page Last Updated
12/10/2023 13:50
