Objective This study aimed to establish a novel grading system, based on the craniovertebral junction compression severity index (CVJCSI) for multidirectional quantification at the foramen magnum plane for Chiari malformation type I (CMI). Methods The CVJCSI grading system was established to stratify patients based on the ventral (modified clivoaxial angle < 138 & DEG;), dorsal (tonsil herniation), and central (brainstem herniation) CVJ (craniovertebral junction) compression, the CVJCSI grading system was established to stratify patients. The optimal surgical method for each grade was recommended by intragroup comparisons regarding the efficacy of the three operations. Finally, according to the CVJCSI grading system, a prospective validation trial was performed and surgically treated for internal validation. Results Based on the retrospective study ( n = 310), the CVJCSI included six grades: I: syrinx alone without compression; II: dorsal compression; III: dorsal and central compression; IV: ventral compression; V: dorsal and ventral compression; and VI: ventral, dorsal, and central compression. Among all available variables, only the CVJCSI and surgical methods significantly affected the CCOS. The CCOS scores, overall and for each CVJCSI grade, increased in the prospective cohort ( n = 42) compared with that in the retrospective analysis. Conclusions The CVJCSI can be used to stratify CMI patients. The higher the CVJCSI grade, the more severe the CVJ compression and the worse posterior fossa deformity. Meanwhile, the CVJCSI was negatively correlated with the CCOS. The lower the CVJCSI grade, the better the response to surgery, and the less-invasive surgical procedures were warranted. Finally, the prospective cohort study validated the proposed CVJCSI-based surgical protocols.
