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Congenital varicella syndrome — Clinical Guidelines

Overview

Congenital varicella syndrome (CVS) results from maternal varicella-zoster virus (VZV) infection during pregnancy, leading to multi-system anomalies in the fetus, including skin lesions, neurological defects, ocular abnormalities, and skeletal malformations 1 9.

Diagnosis

Key Diagnostic Criteria:

- Dermatomal or segmental skin lesions 1 9. - Neurological deficits (e.g., cerebral abnormalities, Horner's syndrome) 2 3 9. - Ocular manifestations (e.g., chorioretinal scarring, microphthalmia) 11. - Skeletal anomalies (e.g., limb hypoplasia, fractures) 5.

Recommended Tests:

- Maternal serology for VZV IgM and IgG 6. - Neonatal serology for VZV IgM 7. - Polymerase Chain Reaction (PCR) for VZV in tissue samples 8 10.

Grading: Diagnosis often clinical, supported by serological and molecular evidence 1 8 10.

Management

First-Line Treatments:

- Supportive care tailored to specific deficits (e.g., physical therapy for motor impairments) 3 5.

Adjunctive Treatments:

- Antiviral therapy (e.g., acyclovir) may be considered for severe complications, though specific dosing is not detailed in the abstracts 4. - Ophthalmic and orthopedic interventions as needed for ocular and skeletal abnormalities 11 12.

Special Populations

Pregnancy:

- Infection in first 20 weeks of gestation carries a higher risk (~2% incidence) of CVS 1 9. - Prenatal screening and monitoring recommended for women contracting VZV during pregnancy 6.

Pediatrics:

- Neonatal resuscitation and intensive care for severe respiratory failure and feeding difficulties 3. - Long-term follow-up for developmental and physical impairments 3 5.

Key Recommendations

Screen and monitor pregnant women for VZV infection early in pregnancy to prevent CVS (Evidence: Moderate 6).

Perform comprehensive neonatal evaluations including neurological, ophthalmological, and skeletal assessments in infants born to mothers with VZV infection during pregnancy (Evidence: Moderate 1 9).

Consider antiviral therapy for severe manifestations of CVS, though specific protocols require further evidence (Evidence: Weak 4).

Provide multidisciplinary care addressing diverse deficits in affected infants (Evidence: Expert opinion 3 5).

References

1 Ahn KH, Park YJ, Hong SC, Lee EH, Lee JS, Oh MJ et al.. Congenital varicella syndrome: A systematic review. Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology 2016. link 2 Neubauer V, Griesmaier E, Trawoger R, Kiechl-Kohlendorfer U. Congenital varicella syndrome in a very low birthweight preterm infant. Archives of disease in childhood. Fetal and neonatal edition 2011. link 3 Rizzardi E, Tagliaferro T, Snijders D, Bertuola F, Spolaore F, Monciotti CM et al.. Unilateral laryngeal paralysis in a newborn with congenital varicella syndrome. International journal of pediatric otorhinolaryngology 2009. link 4 Boussault P, Boralevi F, Labbe L, Sarlangue J, Taïeb A, Leaute-Labreze C. Chronic varicella-zoster skin infection complicating the congenital varicella syndrome. Pediatric dermatology 2007. link 5 Sasidharan CK, Anoop P. Congenital varicella syndrome. Indian journal of pediatrics 2003. link 6 Harger JH, Ernest JM, Thurnau GR, Moawad A, Thom E, Landon MB et al.. Frequency of congenital varicella syndrome in a prospective cohort of 347 pregnant women. Obstetrics and gynecology 2002. link02059-8) 7 Choong CS, Patole S, Whitehall J. Congenital varicella syndrome in the absence of cutaneous lesions. Journal of paediatrics and child health 2000. link 8 Cooper C, Wojtulewicz J, Ratnamohan VM, Arbuckle S. Congenital varicella syndrome diagnosed by polymerase chain reaction--scarring of the spinal cord, not the skin. Journal of paediatrics and child health 2000. link 9 Sauerbrei A, Wutzler P. The congenital varicella syndrome. Journal of perinatology : official journal of the California Perinatal Association 2000. link 10 Sauerbrei A, Müller D, Eichhorn U, Wutzler P. Detection of varicella-zoster virus in congenital varicella syndrome: a case report. Obstetrics and gynecology 1996. link00253-0) 11 Mendívil A, Mendívil MP, Cuartero V. Ocular manifestations of the congenital varicella-zoster syndrome. Ophthalmologica. Journal international d'ophtalmologie. International journal of ophthalmology. Zeitschrift fur Augenheilkunde 1992. link 12 Brice JE. Congenital varicella resulting from infection during second trimester of pregnancy. Archives of disease in childhood 1976. link

Congenital varicella syndrome