How to rectify the convex coronal imbalance in patients with unstable dystrophic scoliosis secondary to type I neurofibromatosis: experience from a case series

如何纠正I型神经纤维瘤病继发的不稳定型营养不良性脊柱侧弯患者的凸冠状面失衡:病例系列研究经验

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Abstract

BACKGROUND: There was a paucity of valid information on how to rectify the convex coronal imbalance effectively in dystrophic scoliosis secondary to Type I neurofibromatosis (DS-NF1), while postoperative inadvertent aggravation of CCI occurred regularly resulting in poor patient satisfaction. We aimed to identify the risk factors for persistent postoperative CCI in DS-NF1, and to optimize the coronal rebalancing strategies based on the lessons learned from this rare case series. METHODS: NF1-related scoliosis database was reviewed and those with significant CCI (> 3 cm) were identified, sorted and the outcomes of surgical coronal rebalance were analyzed to identify the factors being responsible for failure of CCI correction. RESULTS: CCI with dystrophic thoracolumbar/lumbar apex was prone to remain uncorrected (7 failure cases in 11) when compared to those with thoracic apex (0 failure cases in 4) (63.6% vs. 0.0%, p = 0.077). Further comparison between those with and without post-op CCI showed a higher correction of main curve Cobb angle (65.9 ± 9.1% vs. 51.5 ± 37.3%, p = 0.040), more tilted instrumentation (10.3 ± 3.6° vs. 3.2 ± 3.1°, p = 0.001) and reverse tilt and translation of upper instrumented vertebra (UIV) to convex side (8.0 ± 2.3° vs. -3.4 ± 5.9°, p < 0.001; 35.4 ± 6.9 mm vs. 12.3 ± 13.1 mm, p = 0.001) in the uncorrected imbalanced group. Multiple linear regression analysis revealed that △UIV translation (pre- to post-operation) (β = 0.832; p = 0.030) was significantly correlated with the correction of CBD. CONCLUSION: Thoracolumbar/lumbar CCI in dystrophic scoliosis was prone to suffer high risk of persistent post-op CCI. Satisfying coronal rebalance should avoid UIV tilt and translation to the convex side, tilted morphology of instrumentation and over correction maneuvers for main curve, the upper hemi-curve region in particular.

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