Surgery for atypical scoliosis

Purpose of review: Atypical scoliosis poses challenges for recognition and medical and surgical management. Recent technological advances in spine surgery provide new opportunities for application to atypical deformity. Recent findings: Marfan syndrome must be recognized for proper cardiovascular management. Spinal challenges include early deformity, poor response to bracing, increased blood loss, thin pedicles, risk of cerebrospinal fluid leak, and failure of fixation and adding-on. Neurofibromatosis presents large curves at an early age. Dystrophic apices may present thin pedicles and consequent difficulties of fixation. Anterior surgery should be considered if kyphosis and/or dystrophic curves cannot be corrected. Syringomyelia should be considered in early-onset or left-main thoracic scoliosis, or patients with subtle neurologic findings. Curves less than 45° may stabilize with neurosurgical treatment of the syrinx itself. About 10% of patients with Charcot-Marie-Tooth disorder will have scoliosis. Spinal cord monitoring may be unpredictable. Scoliosis in patients with Ehlers-Danlos syndrome should be approached primarily posteriorly. Summary: Newer principles of spinal deformity can produce significant improvements in management of atypical deformity.