Posterior spinal fusion for severe kyphoscoliosis in a Loeys-Dietz syndrome patient with a large syringomyelia.

Spinal deformity is a characteristic feature of Loeys-Dietz syndrome (LDS). Surgical correction in LDS is indicated when the deformity is progressive to avoid neurological deficits, respiratory impairment, and back pain. However, few reports exist on the surgical treatment of spinal deformity in LDS, and no therapeutic standards have been established. We described the clinical and radiological outcomes of a patient with LDS receiving surgery for severe kyphoscoliosis. A 21-year-old male patient with LDS underwent posterior spinal fusion with an all-pedicle screw construct from T10 to L5 for a preoperative main curve Cobb angle of 70 degrees and kyphotic angle of 49 degrees. The postoperative Cobb angle of the main curve and kyphotic angle improved to 36 and 8 degrees, respectively. Correction surgery was performed with frequent motor evoked potential testing, taking care not to cause motor paralysis. Ameliorated low back pain and improvements in clinical questionnaire scores were noted at 21 months after surgery. No perioperative complications were reported. Based on the present case, posterior spinal fusion represents a good correction option for severe spinal deformity in LDS with syringomyelia. Careful preoperative examination and treatment for neurovascular and neurological lesions is advised to prevent severe complications.