Villaret Syndrome: A Systematic Review

Area of Science:

• Neurology
• Otolaryngology
• Systematic Review

Background:

• Villaret syndrome (VS) is a rare condition involving cranial nerves (CNs) IX-XII and the cervical sympathetic chain.
• It suggests a pathological process within the retrostyloid compartment.

Purpose of the Study:

• To systematically review the etiologies, clinical presentation, management, and outcomes of Villaret syndrome.
• To synthesize current knowledge on this rare neurological disorder.

Main Methods:

• Systematic literature search of PubMed and Scopus databases up to January 2025.
• Inclusion of case reports and case series of clinically/radiologically confirmed VS.
• Data extraction on demographics, etiology, onset, imaging, treatment, and outcomes.

Main Results:

• 13 patients identified across 12 studies; mean age 48.7 years.
• Neoplastic (58.3%), vascular (25%), and infectious/inflammatory (16.7%) causes identified.
• Subacute onset (58.3%) common; MRI preferred imaging.
• 50% recovery, better with vascular/inflammatory causes; neoplastic VS had poorer outcomes.

Conclusions:

• Villaret syndrome requires early recognition and modern imaging to identify retrostyloid compartment pathology.
• Treatment strategies must be individualized and managed by a multidisciplinary team.
• Neoplastic etiology is associated with worse prognosis, highlighting the need for prompt diagnosis and intervention.