Predictors for Deformity Progression in a Spinal Muscular Atrophy Cohort After Scoliosis Correction Surgery

This was a single-center, retrospective study.

The objective of this study was to assess the risk factors for deformity progression after scoliosis correction surgery in spinal muscular atrophy (SMA) patients.

Moderate residual postoperative scoliosis curve is common in SMA populations; however, the acceptable postoperative scoliosis curve for preventing deformity progression remains uncertain.

Twenty-nine SMA patients undergoing scoliosis correction surgery were included. Scoliosis progression was defined as an increase of 10 degrees in the major curve of Cobb angle (MCCA); pelvic obliquity (PO) or concave-side hip progression was arbitrarily defined as an increase of ≥1 grade after surgery. Risk factors for deformity progression were examined using Cox proportional hazard models. The cumulative incidence rate of deformity progression was performed by the Kaplan-Meier survival analysis RESULTS:: The mean age at surgery was 13.3 years (range: 8-25 y) and the mean follow-up time was 7 years (range: 2-22.9 y). The mean MCCA was corrected from 69 to 34.6 degrees at initial follow-up and 42.2 degrees at the final follow-up. Postoperative MCCA (P=0.002) and PO (P=0.004) at initial follow-up were the risk factors for scoliosis progression. Postoperative MCCA at initial follow-up (P=0.007) and age at the time of surgery (P=0.017) were the risk factors for PO progression. Different cutoff points of postoperative MCCA at initial follow-up were compared for predicting deformity progression. We found the patient with postoperative MCCA of <30 degrees at initial follow-up had a significantly less cumulative incidence rate of progression than their counterparts for scoliosis (P=0.005), PO (P=0.023), and concave-side hip progressions (P=0.008).

We recommended that MCCA should be corrected to <30 degrees to prevent postoperative scoliosis, PO, and concave-side femoral head coverage percentage progressions. Patients receiving surgery earlier had less postoperative MCCA at initial follow-up but with no increase in the risk of postoperative scoliosis progression.