RARE DISEASE
Congenital varicella syndrome
Congenital varicella syndrome
Congenital varicella syndrome
Synonyms: Antenatal varicella virus infection, Mother-to-child transmission of varicella syndrome
Synonyms: Antenatal varicella virus infection, Mother-to-child transmission of varicella syndrome
Synonyms: Antenatal varicella virus infection, Mother-to-child transmission of varicella syndrome
Drug discovery
1
drug
With orphan designation
Overview
Congenital varicella syndrome (CVS) is a rare embryopathy caused by maternal varicella-zoster virus (VZV) infection during the first 20 weeks of gestation. It manifests with dermatomal skin scarring, limb hypoplasia, neurological defects, ocular anomalies, and visceral organ damage. Diagnosis combines maternal infection history, fetal ultrasound findings, and VZV PCR testing. Prevention through pre-pregnancy vaccination and post-exposure prophylaxis with varicella-zoster immune globulin (VariZIG) remains critical [1][2][16].
Burden
30% mortality in neonates ≤1 month from pulmonary/GI complications [6][16]
Survivors face lifelong disabilities: 60% neurological deficits, 51% ocular damage, 49% skeletal anomalies [5][13]
Preventable through vaccination programs, with 97% reduction in pediatric varicella cases since 1995 in vaccinated populations [18]
Therapies
Prophylaxis: VariZIG within 10 days of maternal VZV exposure [3][8]
Antivirals: IV acyclovir (30 mg/kg/day) for neonates with active infection; maternal oral acyclovir for third-trimester infections [6][8][13]
Supportive care: Multidisciplinary management of limb defects, neurological complications, and ophthalmic sequelae [1][7]
Categories: rare developmental anomalies during embryogenesis, rare infectious diseases, rare ophthalmic disorders, rare teratologic disorders
Research Papers
93 drug discovery papers about Congenital varicella syndrome, with 2 first-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
93 drug discovery papers about Congenital varicella syndrome, with 2 first-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
2025-12-31 | TORCH – Current state of knowledge as of 2025
The acronym TORCH designates a group of pathogens that can lead to serious pregnancy complications, such as miscarriage, fetal growth restriction, and congenital infections. This review summarizes current insights into these infections, offering practical guidance primarily for obstetricians, infectious disease specialists, and general practitioners involved in prenatal care. The TORCH complex includes Toxoplasma gondii, Rubella virus, Cytomegalovirus (CMV), and Herpes simplex virus (HSV). The definition may be extended to encompass additional (other) pathogens such as Hepatitis B and C viruses (HBV, HCV), Human immunodeficiency virus (HIV), Varicella-zoster virus (VZV), Treponema pallidum (syphilis), Parvovirus B19, and Zika virus. Screening practices for TORCH infections during pregnancy vary significantly across countries. Despite widespread access to medical care and increasing awareness among women planning pregnancy, routine screening for TORCH pathogens is not universally implemented. In Poland, diagnostic procedures during pregnancy are defined by the Standard of Perinatal Care established by the Ministry of Health. This regulation- which replaced earlier recommendations of the Polish Society of Gynecologists and Obstetricians- does not distinguish between ,,mandatory” and ,,recommended” tests but specifies a unified set of investigations to be performed at defined stages of pregnancy. Screening conducted during pregnancy plays a crucial role in detecting previously unrecognized infections. In Poland , a substantial proportion of new diagnoses of HIV, HBV and HCV among young women are made during routine antenatal testing, underscoring the importance of standardized serological screening in prenatal care rather than relying on diagnosis before conception. However, due to their distinct epidemiological and clinical profiles, HIV, HBV, and HCV infections are not discussed in detail in this review.
2025-08-19 | Varicella-zoster virus seroprevalence among reproductive-age women in Iran: a meta-analysis and implications for targeted immunization.
Primary varicella-zoster virus (VZV) infection during pregnancy poses significant risks to both mother and fetus, including congenital varicella syndrome (CVS) and serious maternal complications. In Iran, the absence of varicella vaccination in the national immunization program leaves many women susceptible. This systematic review and meta-analysis aimed to determine the overall seroprevalence of VZV antibodies among reproductive-age women in Iran. A comprehensive search was conducted in both international and Iranian databases for studies published up to November 15, 2024. Eligible studies reporting VZV seroprevalence among Iranian women aged 15-49 years were identified. Screening, data extraction, and risk of bias assessment were independently performed by two reviewers. Pooled seroprevalence was estimated using a random-effects meta-analysis in STATA version 18. Heterogeneity was evaluated with the Cochrane Q test and I² statistic. Subgroup analyses and meta-regression explored sources of heterogeneity. Sensitivity analyses (leave-one-out) and publication bias (Doi plot, LFK index) were also performed. The protocol was registered in PROSPERO (CRD42025647813). Data from 20 studies, including 5,629 participants, were analyzed. A pooled VZV seroprevalence of 81% (95% CI: 77-85%) was found, indicating that approximately 19% of reproductive-age women in Iran remain susceptible. Higher seroprevalence was observed in pregnant women (88%) compared with non-pregnant women (79%), with the lowest rates among medical students (74%). Despite subgroup and meta-regression analyses, substantial heterogeneity remained unexplained. Sensitivity analyses supported the robustness of the results, while possible publication bias was suggested. Nearly one in five reproductive-age women in Iran lack immunity to VZV. Targeted vaccination, especially among non-pregnant women and students, may reduce susceptibility. Preconception screening for VZV immunity could help prevent maternal and fetal complications. However, given the high unexplained heterogeneity, results should be interpreted with caution.
2025-02-26 | Varicella Zoster Virus Infection and Pregnancy: An Optimal Management Approach.
Varicella-zoster virus is an α-herpes virus with a double-stranded DNA genome, which causes two main clinical pictures: varicella or chickenpox and herpes zoster. Chickenpox is the primary infection, predominantly affecting children, and it presents with fever and a cutaneous eruption consisting of a vesicular, pruritic, and painful rash. Herpes zoster is a viral infection that typically develops in adulthood as a result of the reactivation of the varicella-zoster virus. If acquired during pregnancy, chickenpox may be responsible for serious complications for the mother, the fetus, or the newborn. The most frequent complication of primary varicella-zoster virus infection in mothers is varicella pneumonia, while encephalitis and hepatitis are rare. The effects on the fetus due to chickenpox infection depend on the stage of pregnancy when the mother becomes infected. If the infection occurs during the first trimester, it does not increase the risk of miscarriage. However, if the infection occurs during the first or second trimester, it may cause fetal varicella syndrome or congenital varicella syndrome. During pregnancy, if the varicella-zoster virus reactivates, it usually does not cause harm to the fetus or lead to any birth defects. However, it may increase maternal morbidity due to herpes zoster and its complications. In the case of primary varicella-zoster virus infection in pregnant women, about 20% of newborns may get neonatal or infantile herpes zoster without any complications. However, it is recommended to start early treatment of herpes zoster in pregnant women as it is believed to accelerate the healing process of skin lesions and alleviate pain, reducing both its duration and severity. Through this narrative review, we discuss the approach to the optimal management of varicella-zoster virus infection during pregnancy.
2025-12-31 | TORCH – Current state of knowledge as of 2025
The acronym TORCH designates a group of pathogens that can lead to serious pregnancy complications, such as miscarriage, fetal growth restriction, and congenital infections. This review summarizes current insights into these infections, offering practical guidance primarily for obstetricians, infectious disease specialists, and general practitioners involved in prenatal care. The TORCH complex includes Toxoplasma gondii, Rubella virus, Cytomegalovirus (CMV), and Herpes simplex virus (HSV). The definition may be extended to encompass additional (other) pathogens such as Hepatitis B and C viruses (HBV, HCV), Human immunodeficiency virus (HIV), Varicella-zoster virus (VZV), Treponema pallidum (syphilis), Parvovirus B19, and Zika virus. Screening practices for TORCH infections during pregnancy vary significantly across countries. Despite widespread access to medical care and increasing awareness among women planning pregnancy, routine screening for TORCH pathogens is not universally implemented. In Poland, diagnostic procedures during pregnancy are defined by the Standard of Perinatal Care established by the Ministry of Health. This regulation- which replaced earlier recommendations of the Polish Society of Gynecologists and Obstetricians- does not distinguish between ,,mandatory” and ,,recommended” tests but specifies a unified set of investigations to be performed at defined stages of pregnancy. Screening conducted during pregnancy plays a crucial role in detecting previously unrecognized infections. In Poland , a substantial proportion of new diagnoses of HIV, HBV and HCV among young women are made during routine antenatal testing, underscoring the importance of standardized serological screening in prenatal care rather than relying on diagnosis before conception. However, due to their distinct epidemiological and clinical profiles, HIV, HBV, and HCV infections are not discussed in detail in this review.
2025-08-19 | Varicella-zoster virus seroprevalence among reproductive-age women in Iran: a meta-analysis and implications for targeted immunization.
Primary varicella-zoster virus (VZV) infection during pregnancy poses significant risks to both mother and fetus, including congenital varicella syndrome (CVS) and serious maternal complications. In Iran, the absence of varicella vaccination in the national immunization program leaves many women susceptible. This systematic review and meta-analysis aimed to determine the overall seroprevalence of VZV antibodies among reproductive-age women in Iran. A comprehensive search was conducted in both international and Iranian databases for studies published up to November 15, 2024. Eligible studies reporting VZV seroprevalence among Iranian women aged 15-49 years were identified. Screening, data extraction, and risk of bias assessment were independently performed by two reviewers. Pooled seroprevalence was estimated using a random-effects meta-analysis in STATA version 18. Heterogeneity was evaluated with the Cochrane Q test and I² statistic. Subgroup analyses and meta-regression explored sources of heterogeneity. Sensitivity analyses (leave-one-out) and publication bias (Doi plot, LFK index) were also performed. The protocol was registered in PROSPERO (CRD42025647813). Data from 20 studies, including 5,629 participants, were analyzed. A pooled VZV seroprevalence of 81% (95% CI: 77-85%) was found, indicating that approximately 19% of reproductive-age women in Iran remain susceptible. Higher seroprevalence was observed in pregnant women (88%) compared with non-pregnant women (79%), with the lowest rates among medical students (74%). Despite subgroup and meta-regression analyses, substantial heterogeneity remained unexplained. Sensitivity analyses supported the robustness of the results, while possible publication bias was suggested. Nearly one in five reproductive-age women in Iran lack immunity to VZV. Targeted vaccination, especially among non-pregnant women and students, may reduce susceptibility. Preconception screening for VZV immunity could help prevent maternal and fetal complications. However, given the high unexplained heterogeneity, results should be interpreted with caution.
2025-02-26 | Varicella Zoster Virus Infection and Pregnancy: An Optimal Management Approach.
Varicella-zoster virus is an α-herpes virus with a double-stranded DNA genome, which causes two main clinical pictures: varicella or chickenpox and herpes zoster. Chickenpox is the primary infection, predominantly affecting children, and it presents with fever and a cutaneous eruption consisting of a vesicular, pruritic, and painful rash. Herpes zoster is a viral infection that typically develops in adulthood as a result of the reactivation of the varicella-zoster virus. If acquired during pregnancy, chickenpox may be responsible for serious complications for the mother, the fetus, or the newborn. The most frequent complication of primary varicella-zoster virus infection in mothers is varicella pneumonia, while encephalitis and hepatitis are rare. The effects on the fetus due to chickenpox infection depend on the stage of pregnancy when the mother becomes infected. If the infection occurs during the first trimester, it does not increase the risk of miscarriage. However, if the infection occurs during the first or second trimester, it may cause fetal varicella syndrome or congenital varicella syndrome. During pregnancy, if the varicella-zoster virus reactivates, it usually does not cause harm to the fetus or lead to any birth defects. However, it may increase maternal morbidity due to herpes zoster and its complications. In the case of primary varicella-zoster virus infection in pregnant women, about 20% of newborns may get neonatal or infantile herpes zoster without any complications. However, it is recommended to start early treatment of herpes zoster in pregnant women as it is believed to accelerate the healing process of skin lesions and alleviate pain, reducing both its duration and severity. Through this narrative review, we discuss the approach to the optimal management of varicella-zoster virus infection during pregnancy.
Access all drug discovery articles and probability of success in trials forecasts:
Access all drug discovery articles and probability of success in trials forecasts:
Drug Discovery Landscape
1 orphan drug designation for Congenital varicella syndrome, including 1 approved therapy.
1 orphan drug designation for Congenital varicella syndrome, including 1 approved therapy.
Drug | Therapy type | Regulator | Orphan designation | Approval | Sponsor |
|---|---|---|---|---|---|
Varicella Zoster Immune Globulin (Human) [VARIZIG] | antibodies | FDA | 2006-11-07 | 2012-12-20 | Cangene bioPharma, Inc. |
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