Neuromuscular Scoliosis

Wishart, Brian D; Kivlehan, Emily

doi:10.1016/j.pmr.2021.02.007

Cited by 19 publications

(8 citation statements)

References 41 publications

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“…With curves exceeding 50°, surgical intervention is usually considered ( 5 , 6 ). In contrast to adolescent idiopathic scoliosis (AIS), the fusion area in NMS often comprises the whole thoracolumbar spine, which permanently diminishes spinal mobility ( 7 - 10 ).…”

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confidence: 99%

Health-related quality of life after segmental pedicle screw instrumentation: a matched comparison of patients with neuromuscular and adolescent idiopathic scoliosis

Soini

Syvänen

Helenius

et al. 2023

ActaO

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Background and purpose: Progressive neuromuscular scoliosis (NMS) often requires a long instrumented spinal fusion to improve health-related quality of life (HRQoL) and sitting balance. Segmental pedicle screw instrumentation improves HRQoL in patients with adolescent idiopathic scoliosis (AIS), but data on NMS is limited. We aimed to assess the impact of spinal fusion on HRQoL in NMS patients.Patients and methods: We conducted a retrospective case-control study with prospective data collection of NMS patients undergoing posterior spinal fusion at a tertiary level hospital in 2009–2021. 2 controls with AIS matched for sex and age were selected for each NMS patient. The Scoliosis Research Society-24 (SRS-24) questionnaire was utilized for pre- and postoperative HRQoL assessment. Follow-up time was a minimum of 2 years.Results: 60 NMS and 120 AIS patients were included in the analysis, and the mean age (SD) at operation was 14.6 (2.7) in NMS and 15.7 (2.5) in AIS groups. Total SRS score and all domains showed a significant improvement in NMS patients (p < 0.05). Total SRS score improved more (p < 0.001), while pain score improved less (p = 0.04) in NMS (change [95% CI], 0.31 [0.05–0.58] and 0.55 [0.27–0.81]) compared with AIS (0.01 [–0.10 to 0.12] and 0.88 [0.74–1.03]). Postoperative self-image was significantly better in NMS than in AIS at 2-year follow up (p = 0.01). Pelvic instrumentation reduced improvements in the SRS domains.Conclusion: HRQoL in NMS patients improved significantly after spinal fusion, and these benefits are comparable to those of AIS patients.

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confidence: 99%

Health-related quality of life after segmental pedicle screw instrumentation: a matched comparison of patients with neuromuscular and adolescent idiopathic scoliosis

Soini

Syvänen

Helenius

et al. 2023

ActaO

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“…Neuromuscular scoliosis (NMS) is a spinal deformity caused by myopathy or upper or lower motor neuron disease. Severe muscular or neurological involvement, often generating pelvic obliquity, can result in significant progression of the scoliosis 1. Posterior spinal fusion, currently the most common technique for surgical management of NMS, has been shown to have positive clinical outcomes.…”

Section: Introductionmentioning

confidence: 99%

Plastic multilayered closure versus orthopedic surgeon closure after spinal instrumentation in pediatric neuromuscular scoliosis

Ezeokoli

Tamma

Borici

et al. 2023

World Jnl Ped Surgery

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ObjectiveTo compare wound complication rates between orthopedic closure (OC) and plastic multilayered closure (PMC) in patients undergoing primary posterior spinal fusion for neuromuscular scoliosis (NMS). We hypothesize that multilayered closure will be associated with better postoperative outcomes.MethodsWe collected data on pediatric patients diagnosed with NMS who underwent first time spinal instrumentation between 1 January 2018 and 31 May 2021. Patient demographics, length of surgery, spinal levels fused and operative variables, wound complication rate, treatments, and need for wound washout were reviewed in depth and recorded.ResultsIn total, 86 patients were reviewed: 46 with OC and 40 with PMC. There was a significant increase in operating room (OR) time with PMC compared with OC (6.7±1.2 vs 7.3±1.3, p=0.016). There was no difference in complication rate, mean postoperative day of complication or unplanned return to the OR for OC and PMC, respectively. There was a slightly significant increase in the number of patients going home with a drain in the PMC cohort compared with the OC cohort (2.1% vs 15%, p=0.046).ConclusionsPMC demonstrated longer OR times than OC and did not demonstrate a statistically significant reduction in wound complications or unplanned returns to the OR. However, other studies have demonstrated statistical and clinical significance with these variables. Surgical programs should review internal patient volumes and outcomes for spinal fusion in NMS patients and consider if PMC after spinal fusions in pediatric patients with NMS or other scoliosis subtypes is an appropriate option in their institution to minimize postoperative wound complications.

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“…A breakdown of spine morphology occurs in scoliosis, lordosis, and kyphosis. Scoliosis is medically defined as lateral curvatures of the spine greater than 10° ( Cheng et al, 2015 ; Mesiti, 2021 ; Wise et al, 2008 ) and can be caused by congenital defects of vertebral patterning or as a secondary consequence of neuromuscular disease ( Pourquié, 2011 ; Wishart and Kivlehan, 2021 ). However, most cases of scoliosis are idiopathic in nature, with no known etiology: approximately 3% of children are afflicted by idiopathic scoliosis, which most often onsets during adolescence ( Cheng et al, 2015 ; Labrom et al, 2021 ).…”

Section: Introductionmentioning

confidence: 99%

Urotensin II-related peptides, Urp1 and Urp2, control zebrafish spine morphology

Bearce

Irons

O'Hara-Smith

et al. 2022

eLife

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The spine provides structure and support to the body, yet how it develops its characteristic morphology as the organism grows is little understood. This is underscored by the commonality of conditions in which the spine curves abnormally such as scoliosis, kyphosis and lordosis. Understanding the origin of such spinal curves has been challenging in part due to the lack of appropriate animal models. Recently, zebrafish have emerged as promising tools with which to understand the origin of spinal curves. Using zebrafish, we demonstrate that the Urotensin II-related peptides (URPs), Urp1 and Urp2, are essential for maintaining spine morphology. Urp1 and Urp2 are 10-amino acid cyclic peptides expressed by neurons lining the central canal of the spinal cord. Upon combined genetic loss of Urp1 and Urp2, adolescent-onset planar curves manifested in the caudal region of the spine. Highly similar curves were caused by mutation of Uts2r3, an URP receptor. Quantitative comparisons revealed that Urotensin-associated curves were distinct from other zebrafish spinal curve mutants in curve position and direction. Last, we found that the Reissner fiber, a proteinaceous thread that sits in the central canal and has been implicated in the control of spine morphology, breaks down prior to curve formation mutants with perturbed cilia motility but was unaffected by loss of Uts2r3. This suggests a Reissner fiber-independent mechanism of curvature in Urotensin-deficient mutants. Overall, our results show that Urp1 and Urp2 control zebrafish spine morphology and establish new animal models of spine deformity.

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Neuromuscular Scoliosis

Cited by 19 publications

References 41 publications

Health-related quality of life after segmental pedicle screw instrumentation: a matched comparison of patients with neuromuscular and adolescent idiopathic scoliosis

Health-related quality of life after segmental pedicle screw instrumentation: a matched comparison of patients with neuromuscular and adolescent idiopathic scoliosis

Plastic multilayered closure versus orthopedic surgeon closure after spinal instrumentation in pediatric neuromuscular scoliosis

Urotensin II-related peptides, Urp1 and Urp2, control zebrafish spine morphology

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