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Post-Varicella Arteriopathy as a Cause of Pediatric Arterial Ischemic Stroke: A Systematic Review and Case Report
Martina Testaì1, Silvia Marino2, Giovanna Russo3
1Postgraduate School of Pediatrics, University of Catania, 95100 Catania, Italy.
Insights
Post-varicella arteriopathy (PVA) is a rare cause of pediatric stroke, often occurring within a year of chickenpox. While treatments vary, most children recover with some residual effects, highlighting the need for standardized management guidelines.
Area of Science:
- Neurology
- Pediatrics
- Infectious Diseases
Background:
- Post-varicella arteriopathy (PVA) is a significant cause of arterial ischemic stroke (AIS) in children.
- It typically affects previously healthy children within 12 months of varicella infection, often with a monophasic course.
- Current diagnosis relies on clinical and imaging findings, with cerebrospinal fluid analysis for confirmation, and treatment remains empirical.
Purpose of the Study:
- To describe a typical case of PVA.
- To conduct a systematic review of clinical, radiological, therapeutic, and outcome features of PVA.
- To provide insights into the diagnosis and management of pediatric stroke secondary to varicella infection.
Main Methods:
- Systematic review following PRISMA 2020 and AMSTAR-2 guidelines.
- Data extraction from PubMed, Embase, and Scopus on demographics, clinical presentation, imaging, laboratory confirmation, treatment, and outcomes.
- Inclusion of 47 studies comprising 312 pediatric patients diagnosed with PVA.
Main Results:
- The mean age of affected children was 4 years, with a median latency of 3.82 months from varicella infection to neurological symptoms.
- Common presentations included hemiparesis, language impairment, and seizures, with imaging showing unilateral anterior circulation artery involvement.
- Cerebrospinal fluid (CSF) VZV-DNA PCR and anti-VZV IgG were positive in 39% and 48% of tested patients, respectively. Treatment commonly involved low-dose aspirin (77%), with 43% achieving full recovery and 45% experiencing residual deficits.
Conclusions:
- PVA is a rare but distinct cause of pediatric stroke with a generally favorable prognosis.
- Standardized guidelines and prospective studies are crucial for establishing evidence-based management strategies.
- Clinicians should maintain a high index of suspicion for PVA in pediatric stroke cases following varicella infection.
Background/Objectives:
Post-varicella arteriopathy (PVA) is a significant cause of pediatric arterial ischemic stroke (AIS) that typically involves previously healthy children within 12 months of primary varicella infection, mostly with a monophasic course. Diagnosis is based on clinical and imaging findings, and cerebrospinal fluid analysis may confirm it; treatment is empirical and heterogeneous. We describe a typical case of PVA and present a systematic review of its clinical, radiological, therapeutic, and outcome features.
Methods:
Following PRISMA 2020 and AMSTAR-2 guidelines, data on demographics, clinical presentation, imaging, laboratory confirmation, treatment, and outcomes were extracted across databases (PubMed, Embase, Scopus).
Results:
Forty-seven studies, encompassing 312 pediatric patients, were included. Mean age was 4 years with a median latency of 3.82 months from varicella to neurologic symptoms. Common presentation included hemiparesis, language impairment, and seizures. Imaging findings showed unilateral focal involvement of anterior circulation arteries, basal ganglia infarctions, and, rarely, bilateral or posterior circulation involvement. CSF VZV-DNA PCR and anti-VZV IgG were positive in 39% and 48% of tested patients. Treatment included intravenous acyclovir (34%), corticosteroids (20%), and low-dose aspirin (77%); two patients underwent acute reperfusion therapy (rt-PA or thrombectomy). Outcomes tended to be moderately favorable: 43% achieved full recovery, 45% had residual deficit, and 11% experienced recurrence. Prothrombotic state was reported, and it may influence disease severity.
Conclusions:
PVA is a rare distinct cause of pediatric stroke, with a generally favorable prognosis quoad vitam. Standardized guidelines and prospective studies are needed to establish evidence-based management. Clinicians should maintain a high suspicion for its diagnosis.
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