A síndrome da varicela fetal (CVS) é uma síndrome de anomalia adquirida do desenvolvimento caracterizada por defeitos cutâneos, neurológicos, oculares, dos membros e de crescimento secundários à infecção materna pelo vírus Varicela-Zoster (VZV).
Introdução
O que você precisa saber de cara
A síndrome da varicela fetal (CVS) é uma síndrome de anomalia adquirida do desenvolvimento caracterizada por defeitos cutâneos, neurológicos, oculares, dos membros e de crescimento secundários à infecção materna pelo vírus Varicela-Zoster (VZV).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 2 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 9 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome varicela congênita
Centros de Referência SUS
24 centros habilitados pelo SUS para Síndrome varicela congênita
Centros para Síndrome varicela congênita
Detalhes dos centros
Hospital Universitário Prof. Edgard Santos (HUPES)
R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808
Serviço de Referência
Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
Serviço de Referência
Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
Serviço de Referência
Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)
Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207
Serviço de Referência
Hospital das Clínicas da UFG
Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424
Serviço de Referência
Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
Atenção Especializada
Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
Serviço de Referência
Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
Atenção Especializada
Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
Serviço de Referência
Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
Atenção Especializada
Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
Serviço de Referência
Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
Serviço de Referência
Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
Atenção Especializada
Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
Serviço de Referência
Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
Serviço de Referência
Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)
Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988
Serviço de Referência
Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
Serviço de Referência
Hospital de Clínicas de Porto Alegre (HCPA)
Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601
Serviço de Referência
Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
Serviço de Referência
Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
Serviço de Referência
Hospital de Base de São José do Rio Preto
Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798
Atenção Especializada
Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
Serviço de Referência
Hospital de Clínicas de Ribeirão Preto (HCRP-USP)
R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187
Serviço de Referência
UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
Serviço de Referência
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
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Publicações mais relevantes
Australian Paediatric Surveillance Unit (APSU) Annual Surveillance Report 2024.
Since 1993, the Australian Paediatric Surveillance Unit (APSU) has been conducting prospective national surveillance of rare conditions in Australian children, including communicable diseases and complications of communicable diseases. In 2024, fifteen communicable diseases and complications were under APSU surveillance: acute flaccid paralysis (AFP); congenital cytomegalovirus (cCMV) infection; dengue; severe acute hepatitis; neonatal/infant herpes simplex virus (HSV) infection; perinatal exposure to human immunodeficiency virus (HIV); paediatric HIV infection, juvenile-onset recurrent respiratory papillomatosis (JoRRP); severe complications of influenza (Flu); Japanese encephalitis virus infection; paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS); Q fever; congenital rubella infection/syndrome; congenital varicella syndrome; and neonatal varicella infection. A total of 1,350 paediatricians and other child health specialists received the monthly APSU report card (97% electronically) in 2024. A total of 237 notifications were received, with 174 confirmed as incident cases after excluding duplicates, errors and prevalent (historic) cases not previously reported. The incident cases included: Flu (n = 34) - one child died and only two children had received influenza vaccination; JoRRP (n = 1); NVI (n = 1); cCMV (n = 26); HSV (n = 8) - neurological sequelae were common; perinatal exposure to HIV (n = 15) - no cases of mother-to-child transmission identified; and rare emerging diseases dengue (n = 4) and PIMS-TS (n = 2). The non-polio AFP rate of ≥ 1 case per 100,000 children aged < 15 years was again achieved. The APSU continues to be an important mechanism for obtaining enriched data on rare communicable diseases and their complications in Australian children, to better understand disease burden, and the effects of health interventions, over time.
Congenital Varicella Syndrome and Crossed Nonfused Renal Ectopia in a Neonate: A Case Report.
Congenital varicella syndrome (CVS) is a rare consequence of maternal varicella infection during early pregnancy and is characterized by cicatricial skin lesions, neurological impairment, limb hypoplasia, and ocular abnormalities. Renal anomalies are not classical features but may occur. We report a term male neonate, born to a primigravida mother with a history of varicella infection at 12 weeks of gestation. The neonate presented with perinatal asphyxia, seizures, cicatricial skin lesions, and a rare renal anomaly - crossed nonfused renal ectopia (CNRE). The neonate required resuscitation at birth, developed hypoxic-ischemic encephalopathy, and was managed with therapeutic hypothermia and antiepileptics. Radiological imaging revealed CNRE. Both maternal and neonatal varicella IgG serology were positive. The baby improved with supportive management and was discharged in stable condition. To the best of our knowledge, CVS and CNRE are not correlated and represent coincidental, unrelated findings in our case.
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.
Australian Paediatric Surveillance Unit (APSU) Annual Surveillance Report 2023.
The Australian Paediatric Surveillance Unit (APSU) has been conducting prospective national surveillance of rare communicable diseases, and complications of communicable diseases, of childhood and infancy for more than three decades. In 2023, there were 15 communicable diseases and complications of communicable diseases under APSU surveillance, which included: acute flaccid paralysis (AFP), congenital cytomegalovirus (cCMV), dengue, severe acute hepatitis (SAH), neonatal and infant herpes simplex virus (HSV) infection, perinatal exposure to human immunodeficiency virus (HIV) and paediatric HIV infection, severe complications of influenza, juvenile-onset recurrent respiratory papillomatosis (JoRRP), Q fever, congenital rubella infection/syndrome, congenital varicella syndrome (CVS) and neonatal varicella infection (NVI), as well as two new communicable diseases, which were paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) and Japanese encephalitis virus (JEV) infection. The results of 2023 APSU surveillance show a marked increase in severe influenza cases for the first time in five years, with more complications associated with influenza type B. Moreover, one child died and only 6% of children received a seasonal influenza vaccine. The APSU also received reports of cases of rare emerging diseases: dengue, Q fever and PIMS-TS. Furthermore, our results show a persistence of vaccine-preventable JoRRP, mother-to-child transmission of HIV, and deaths from neonatal HSV.
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.
Publicações recentes
Neuroimaging features of incontinentia pigmenti: prompting genetic confirmation.
Australian Paediatric Surveillance Unit (APSU) Annual Surveillance Report 2024.
Congenital Varicella Syndrome and Crossed Nonfused Renal Ectopia in a Neonate: A Case Report.
Varicella-zoster virus seroprevalence among reproductive-age women in Iran: a meta-analysis and implications for targeted immunization.
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Australian Paediatric Surveillance Unit (APSU) Annual Surveillance Report 2024.
Communicable diseases intelligence (2018)Congenital Varicella Syndrome and Crossed Nonfused Renal Ectopia in a Neonate: A Case Report.
CureusVaricella-zoster virus seroprevalence among reproductive-age women in Iran: a meta-analysis and implications for targeted immunization.
BMC infectious diseasesAustralian Paediatric Surveillance Unit (APSU) Annual Surveillance Report 2023.
Communicable diseases intelligence (2018)Varicella Zoster Virus Infection and Pregnancy: An Optimal Management Approach.
Pathogens (Basel, Switzerland)Varicella in the 21st Century.
NeoReviews[Varicella vaccination, pregnancy and breastfeeding: The current situation].
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Communicable diseases intelligence (2018)Active prospective national surveillance for congenital and neonatal varicella in Australia shows potential prevention opportunities.
Vaccine: XCongenital Varicella Syndrome with Isolated Limb Hypoplasia and Scarring: A Rare Fascinating Case.
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Newborn (Clarksville, Md.)Australian Paediatric Surveillance Unit (APSU) Annual Surveillance Report 2021.
Communicable diseases intelligence (2018)Merck/Centers for Disease Control and Prevention Varicella Vaccine Pregnancy Registry: 19-Year Summary of Data From Inception Through Closure, 1995-2013.
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Journal of clinical virology : the official publication of the Pan American Society for Clinical VirologyAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Australian Paediatric Surveillance Unit (APSU) Annual Surveillance Report 2024.
- Congenital Varicella Syndrome and Crossed Nonfused Renal Ectopia in a Neonate: A Case Report.
- Varicella-zoster virus seroprevalence among reproductive-age women in Iran: a meta-analysis and implications for targeted immunization.
- Australian Paediatric Surveillance Unit (APSU) Annual Surveillance Report 2023.
- Varicella Zoster Virus Infection and Pregnancy: An Optimal Management Approach.
- Neuroimaging features of incontinentia pigmenti: prompting genetic confirmation.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:291(Orphanet)
- MONDO:0017372(MONDO)
- GARD:45(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55787014(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
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