Multistage surgical treatment of early-onset scoliosis in patients with Ehlers-Danlos syndrome: A series of observations

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.