Хирургическая коррекция деформаций позвоночника на почве синдрома Элерса - Данло

Objective. To analyze surgical treatment results in patients with severe progressive spinal deformities owing to Ehlers-Danlos syndrome. Material and Methods. A total of nine patients with spinal deformities owing to Ehlers-Danlos syndrome were operated on during the period of 2003-2013. Mean age of patients was 12.5 years. One patient undergoes multistage treatment with VEPTR instrumentation, and eight patients underwent corrective posterior surgery using third-generation segmental instrumentation. Posterior correction was preceded by anterior stage including discectomy and interbody fusion with bone autograft in six cases. Results. The mean primary curve was 95.2° before surgery, 50.9° immediately after surgery, and 59.1° at final follow-up. The kyphotic component was 93.7°, 53.9°, and 59.6°, respectively. The coronal trunk imbalance was 28.3 mm before surgery, 39.4 mm immediately after surgery, and 22.0 mm at final follow-up. Complications were observed in three patients: right-sided pneumothorax in one case, and instability of the cranial anchor of instrumentation system, which required reoperation in two cases. There were neither pyogenic or neurologic complications, nor massive bleeding. Conclusion. Spinal deformities caused by Ehlers-Danlos syndrome are rare, though rapidly progressive disorders, which attain significant magnitude. Timely surgical treatment allows achieving satisfactory correction of both scoliosis and kyphosis, though can cause vascular and neuriological complications, especially with the anterior approach. Hir. Pozvonoc. 2014; (3):29-35.