Surgical Treatment of Spinal Deformity in Patients with CLOVES Syndrome: A Report of 4 Cases

Background: CLOVES syndrome is a rare genetic condition manifested by lipomatous overgrowth of the trunk, vascular, and epidermal abnormalities, and skeletal anomalies including spinal deformity. The treatment of spinal deformity in these patients has not been described in the literature. Methods: A retrospective review of the clinical and radiographic data on patient with CLOVES syndrome. Results: Four patients were operated on at our institutions with CLOVES syndrome and resultant spinal deformity. One patient had postlaminoplasty cervical kyphosis and 3 patients had thoracic spinal deformities. All patients had paraspinal/intraspinal infiltration with high-flow vascular lesions and 3 of the patients had previous neurosurgical treatment for intraspinal disease. All patients obtained fusion with no neurological sequelae from deformity surgery. There was 1 complication, a patient had an intraoperative pulmonary embolus despite having an inferior caval filter requiring termination of surgery. The patient survived and underwent posterior fusion 4 months later, after recovery and placement of a superior caval filter. The unique characteristics of this disorder and resultant challenges encountered during care are described. Conclusions: CLOVES syndrome is a rare and challenging disorder with unique characteristics placing patients at risk for complications related to surgery for spinal deformity. Multidisciplinary approaches along with understanding of the potential pitfalls in treatment are paramount to successful treatment. Level of Evidence: Level IV.