TITLE:
Fatal Neonatal Presentation of Congenital Varicella Syndrome Following First-Trimester Maternal Infection in a Resource Limited Setting: A Case Report
AUTHORS:
Ezioma Anne Alinnor, Chioma Ada Nnah, Nkeiruka Ngozi Iweha, Omoye Winifred Usifo
KEYWORDS:
Congenital Varicella Syndrome, Varicella-Zoster Virus, Maternal Infection, Congenital Anomalies, Neonatal Mortality, Resource-Limited Settings
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.16 No.4,
July
6,
2026
ABSTRACT: Background: Congenital varicella syndrome (CVS) is a rare but severe fetal consequence of primary maternal varicella-zoster virus (VZV) infection during early pregnancy. Characterized by cutaneous, musculoskeletal, ocular, neurologic, and visceral abnormalities, CVS is associated with substantial morbidity and mortality. Although uncommon, the syndrome remains clinically relevant in regions where varicella vaccination and post-exposure prophylaxis are not routinely available. Case Presentation: We report the case of a term male neonate born to a 35-year-old gravida 3 para 3 mother with documented clinical varicella infection at approximately 12 weeks’ gestation. The maternal illness was characterized by a generalized vesicular rash and fever and was managed conservatively without antiviral therapy or varicella-zoster immunoglobulin (VZIG). The infant was delivered by elective cesarean section at term and presented shortly after birth with poor cry, respiratory distress, extensive cicatricial skin lesions, limb contractures, bilateral talipes equinovarus, microphthalmia, bilateral corneal opacities, micropenis, rudimentary scrotum, and bilateral cryptorchidism. The diagnosis of congenital varicella syndrome was made clinically based on the characteristic phenotype and documented maternal history, as virologic confirmation was not available. Despite supportive management, the infant developed recurrent apneic episodes and died within six hours of admission. Discussion: This case illustrates the classical phenotypic spectrum of CVS and the severe consequences of maternal varicella infection during the critical period of fetal organogenesis. The co-existence of extensive cutaneous, ophthalmologic, musculoskeletal, and genital abnormalities, together with early neonatal death, represents a particularly severe presentation. Diagnostic and therapeutic challenges were made worse by limited access to virologic testing, fetal surveillance, antiviral therapy, and VZIG prophylaxis. Conclusion: Congenital varicella syndrome is a potentially fatal yet preventable condition. Improved screening, varicella vaccination strategies, timely post-exposure prophylaxis, and enhanced awareness among healthcare providers are critical to reducing the burden of CVS in low- and middle-income countries.