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Retrospective case series.To analyze the efficacy and safety of posterior vertebral column resection performed on a consecutive series of patients with severe congenital spinal deformity.The treatment of severe congenital spinal deformities is a demanding and difficult surgical challenge. Conventional procedures, such as posterior and anterior instrumentation or combined anteroposterior instrumentation provide limited correction in rigid neglected or maltreated (fused) deformities.Forty-four patients with severe deformity and managed by posterior vertebral column resection between years 1997 and 2007 having more than 2 years of follow-up were included. Mean age was 8 (range, 2–28) years at the time of operation. The hospital charts were reviewed for demographic data and etiology of deformity. Measurements of curve magnitude and balance were made on 36-in. standing anteroposterior and lateral radiographs obtained before surgery and at most recent follow-up to assess deformity correction, spinal balance, complications related to the instrumentation, and any evidence of pseudarthrosis.Preoperative coronal plane major curve of 106° (range, 90°–132°) with flexibility of less than 30% was corrected to 41.4° (range, 20°–72°), showing a 61% scoliosis correction at the final follow-up. Coronal imbalance was improved by 79% at the most recent follow-up assessment. Preoperative thoracic kyphosis of 87° (range, 67°–103°) in patients with kyphosis was corrected to 36° (range, 25°–48°) at the most recent follow-up evaluation. Lumbar lordosis of 27° (range, 8°–35°) in patients with hypolordotic deformity was corrected to 45°. Complications included postoperative infection in 2 patients, dural laceration in 2 patients, and hemopneumothorax in 1 patient.Posterior vertebral column resection is an effective technique providing a successful correction of stiff complex congenital deformities. However, it is a technically demanding procedure, with possible risks for major complications.