Villaret Syndrome: A Systematic Review

Abstract Villaret syndrome (VS) is a rare condition characterized by concurrent involvement of cranial nerves (CNs) IX–XII and the cervical sympathetic chain, suggesting a pathological process in the retrostyloid compartment. We conducted a systematic review to synthesize current knowledge regarding etiologies, clinical presentation, management strategies, and CN outcomes. A systematic search was performed across the PubMed and Scopus databases up to January 2025, in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We included all case reports and case series describing patients with clinically and/or radiologically confirmed VS. Twelve studies met inclusion criteria. Data were extracted regarding demographics, etiology, onset modality, imaging findings, treatment strategies, and clinical outcomes. Among the 13 identified patients (mean age 48.7 years), neoplastic causes were most common (58.3%), followed by vascular (25%) and infectious/inflammatory etiologies (16.7%). The most frequent mode of onset was subacute (58.3%), and MRI was the preferred imaging modality in all cases. Conservative management (including steroids or antithrombotic therapy) was used in 41.6% of cases, while 33.3% underwent surgery. Complete or near-complete recovery was observed in 50% of patients, predominantly those with vascular or inflammatory etiologies. Neoplastic VS was associated with poorer outcomes. VS, although rare, should be recognized early and should prompt the clinician to use modern imaging modalities to search for a pathological process in the retrostyloid compartment. Given its rarity and complexity in most cases, the treatment strategy should be tailored to each specific patient and involve a multidisciplinary team of physicians.