Introduction: Surgical treatment of early onset scoliosis (EOS) is one of the most challenging problems of spine surgery and includes staged distraction and final fusion at the end of skeletal maturity that remains debatable. Aim: The objective of the review is to evaluate the efficacy of final fusion following staged distraction with VEPTR instrumentation in patients with EOS. Materials and methods: Outcomes of multi-staged operative treatment of 37 patients with EOS of different etiology were reviewed. Medical records and radiographs of the patients were retrospectively analyzed. Standing postero-anterior and lateral spine radiographs were used for the spinal radiologic assessment before and after each stage of distraction-based treatment, before and after final fusion and at the last follow-up. Results: The mean age of patients at baseline was 5.2 years and the mean age at final fusion was 13.9 years. All patients demonstrated decrease in the angle of primary (from 81.5° to 51.6°) and secondary (from 59.3° to 37.8°) curves, increase of the height and normalized body balance. The mean height increased from 104.8 cm to 141.0 cm, and the mean weight increased from 15 kg to 35 kg throughout the treatment period. The height of the thoracic and lumbar vertebra (Th1-S1) increased from 245 mm to 340 mm, and that of the thoracic vertebra – from 136 mm to 193 mm. There was a mean of 2.3 complications per patient during distraction performed in a staged manner, and they were arrested during elective procedures. There were 7 (19%) complications after final fusion that required 6 (16%) unplanned revisions. Radiologic evidence of spontaneous autofusion was seen in the lumbar spine of the patients with the inferior anchor at the lumbar vertebra. Conclusions: Multi-staged pediatric surgeries performed in the first decade of life facilitate radical changes in the natural history of progressive scoliosis and ensure satisfactory functional and cosmetic results despite multiple difficulties and complications. The VEPTR instrumentation used for the thoracic curve is unlikely to result in the spinal fusion of the major arch and this is the cause for the use of third-generation instrumented final spinal fusion in the patients.
BACKGROUND: EhlersDanlos syndrome (EDS) is a group of hereditary pathological conditions caused by various disorders of collagen biosynthesis. The study analyzed the results of multistage surgical treatment of early scoliosis in patients with severe spinal deformities due to EDS. No similar observations have been found in the literature. CLINICAL CASES: Four patients with a verified diagnosis of EDS and progressive spinal deformities were subjected to multistage surgical treatment using the VEPTRII instrumentation, which included periodic distractions and final spinal fusion with segmental instrumentation. Stage-by-stage surgical treatment was initiated from the age of 3 to 6 years. In 3 of 4 cases, the kyphotic component prevailed over the scoliotic one (86140 vs. 21110). The number of staged distractions ranged from 6 to 10. The age of the final stage (correction and dorsal fusion) was 914 years (surgery was performed in three of four cases). The primary correction was 3056, the loss of correction before the final stage was 1435, and the correction during the final stage was 2240. A significant correction of the frontal and sagittal imbalances of the spine was noted. Blood loss during the final fusion was 540750 mL, and the operation time was 310350 min. Ten complications occurred, of which 9 were associated with implants and disappeared during staged distractions. No neurological and vascular complications occurred. DISCUSSION: Scoliosis occurring in the first decade of life in patients with EDS is characterized by early-onset, rapid progression, and tendency to form a significant kyphotic component of spinal deformity. CONCLUSIONS: Multistage treatment of early scoliosis in patients with EDS using VEPTRII tools allows for obtaining quite satisfactory results and has not severe complications. The final fusion gives a significant corrective effect; however, new research and accumulation of data are needed to optimize the treatment process.
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