Posterior-only Resection of Single Hemivertebrae With 2-Level Versus >2-Level Fusion: Can We Improve Outcomes?

BACKGROUND: The outcomes of congenital scoliosis (CS) patients undergoing hemivertebra (HV) resection surgery with a 2-level fusion versus a >2-level fusion are unclear. We hypothesized that CS patients undergoing HV resection and a >2-level fusion have decreased curve progression and reoperation rates compared with 2-level fusions. METHODS: Retrospective review of prospectively collected data from a multicenter scoliosis database. Fifty-three CS patients (average age 4.5, range 1.2 to 10.9 y) at index surgery were included. Radiographic and surgical parameters, complications, as well as revision surgery rates were tracked at a minimum of 2-year follow-up. RESULTS: Twenty-six patients had a 2-level fusion while 27 patients had a >2-level fusion with similar age and body mass index between groups. The HV was located in the lumbar spine for 69% (18/26) 2-level fusions and 30% (8/27) >2-level fusions ( P =0.006). Segmental HV scoliosis curve was smaller in 2-level fusions compared to >2-level fusions preoperatively (38 vs. 50 degrees, P =0.016) and at follow-up (25 vs. 34 degrees, P =0.038). Preoperative T2-T12 (28 vs. 41 degrees, P =0.013) and segmental kyphosis (11 vs. 23 degrees, P =0.046) were smaller in 2-level fusions, but did not differ significantly at postoperative follow-up (32 vs. 39 degrees, P =0.22; 13 vs. 11 degrees, P =0.64, respectively). Furthermore, the 2 groups did not significantly differ in terms of surgical complications (27% vs. 22%, P =0.69; 2-level fusion vs. >2-level fusion, respectively), unplanned revision surgery rate (23% vs. 22%, 0.94), growing rod placement or extension of spinal fusion (15% vs. 15%, P =0.95), or health-related quality of life per the EOS-Questionnaire 24 (EOSQ-24). Comparison of patients with or without the need for growing rod placement or posterior spinal fusion revealed no significant differences in all parameters analyzed. CONCLUSIONS: Two-level and >2-level fusions can control congenital curves successfully. No differences existed in curve correction, proximal junctional kyphosis or complications between short and long-level fusion after HV resection. Both short and long level fusions are viable options and generate similar risk of revision. The decision should be individualized by patient and surgeon.

Klotho Is Neuroprotective in the Superoxide Dismutase (SOD1G93A) Mouse Model of ALS.

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by the loss of motor neurons in the brain and spinal cord. ALS neuropathology is associated with increased oxidative stress, excitotoxicity, and inflammation. We and others reported that the anti-aging and cognition-enhancing protein Klotho is a neuroprotective, antioxidative, anti-inflammatory, and promyelinating protein. In mice, its absence leads to an extremely shortened life span and to multiple phenotypes resembling human aging, including motor and hippocampal neurodegeneration and cognitive impairment. In contrast, its overexpression extends life span, enhances cognition, and confers resistance against oxidative stress; it also reduces premature mortality and cognitive and behavioral abnormalities in an animal model for Alzheimer's disease (AD). These pleiotropic beneficial properties of Klotho suggest that Klotho could be a potent therapeutic target for preventing neurodegeneration in ALS. Klotho overexpression in the SOD1 mouse model of ALS resulted in delayed onset and progression of the disease and extended survival that was more prominent in females than in males. Klotho reduced the expression of neuroinflammatory markers and prevented neuronal loss with the more profound effect in the spinal cord than in the motor cortex. The effect of Klotho was accompanied by reduced expression of proinflammatory cytokines and enhanced the expression of antioxidative and promyelinating factors in the motor cortex and spinal cord of Klotho × SOD1 compared to SOD1 mice. Our study provides evidence that increased levels of Klotho alleviate ALS-associated pathology in the SOD1 mouse model and may serve as a basis for developing Klotho-based therapeutic strategies for ALS.