Are craniocervical angulations or syrinx risk factors for the initiation and progression of scoliosis in Chiari malformation type I?

The pathophysiology behind the instigation and progression of scoliosis in Chiari malformation type I (CMI) patients has not been elucidated yet. This study aims to explore the initiating and progressive factors for scoliosis secondary to CMI. Pediatric patients with CMI were retrospectively reviewed for radiological characteristics of tonsillar herniation, craniocervical anomaly, syrinx morphology, and scoliosis. Subgroup analyses of the presence of syrinx, scoliosis, and curve progression were also performed. A total of 437 CMI patients were included in the study; 62% of the subjects had syrinx, and 25% had scoliosis. In the subgroup analysis of 272 CMI patients with syrinx, 78 of them (29%) had scoliosis, and multiple logistic regression analysis showed that tonsillar herniation ≥ 10 mm (OR 2.13; P = 0.033) and a clivus canal angle ≤ 130° (OR 1.98; P = 0.025) were independent risk factors for scoliosis. In the subgroup analysis of 165 CMI patients without syrinx, 31 of them (19%) had scoliosis, and multiple logistic regression analysis showed that a clivus canal angle ≤ 130° (OR 3.02; P = 0.029) was an independent risk factor for scoliosis. In the subgroup analysis of curve progression for 97 CMI patients with scoliosis, multiple logistic regression analysis showed that anomalies of the craniocervical junction and syrinx were not risk factors for curve progression. Many complex factors including craniocervical angulation, tonsillar herniation, and syrinx might participate in the instigation of scoliosis for CMI patients, and the relationship between craniocervical angulation and scoliosis deserves further study.