Abstract
CLOVES syndrome is a condition characterized by congenital lipomatous overgrowth, vascular malformations, epidermal nevi and skeletal anomalies. The association of cervical arteriovenous fistula with CLOVES syndrome is extremely rare. To the best of our knowledge, only two cases were reported in the literature. We hereby report an additional case and review the literature on this condition. A 12-year-old girl, known to have CLOVES syndrome, presented to the emergency department with a 2-week history of bilateral, progressive lower extremity and left arm weakness. Radiological imaging demonstrated a C3–C6 left extradural lesion, containing multiple enhancing vessels, causing marked compression on the spinal cord. Urgent spinal decompression was performed, with C3–C6 instrumentation, followed by a two-stage embolization of the fistula. Cervical arteriovenous fistula can be rarely identified in patients with CLOVES syndrome. High index of suspicion is required to promptly evaluate patients with CLOVES syndrome presenting with new-onset neurological deficits.