Scoliosis Treatment With Growth-Friendly Spinal Implants (GFSI) Relates to Low Bone Mineral Mass in Children With Spinal Muscular Atrophy

BACKGROUND Children with spinal muscular atrophy (SMA) frequently develop neuromuscular scoliosis at an early age, requiring surgical treatment with growth-friendly spinal implants (GFSI), such as magnetically controlled growing rods. This study investigated the effect of GFSI on the volumetric bone mineral density (vBMD) of the spine in SMA children. METHODS Seventeen children (age 13.2±1.2 y) with SMA and GFSI-treated spinal deformity were compared with 25 scoliotic SMA children (age 12.9±1.7 y) without prior surgical treatment as well as age-matched healthy controls (n=29; age 13.3±2.0). Clinical, radiologic, and demographic data were analyzed. For the calculation of the vBMD Z-scores of the thoracic and lumbar vertebrae, phantom precalibrated spinal computed tomography scans were analyzed using quantitative computed tomography (QCT). RESULTS Average vBMD was lower in SMA patients with GFSI (82.1±8.4 mg/cm3) compared with those without prior treatment (108.0±6.8 mg/cm3). The difference was more prominent in and around the thoracolumbar region. The vBMD of all SMA patients was significantly lower in comparison with healthy controls, especially in SMA patients with previous fragility fractures. CONCLUSIONS The results of this study support the hypothesis of reduced vertebral bone mineral mass in SMA children with scoliosis at the end of GFSI treatment in comparison with SMA patients undergoing primary spinal fusion. Improving vBMD through pharmaceutical therapy in SMA patients could have a beneficial effect on the surgical outcome of scoliosis correction while reducing complications. LEVEL OF EVIDENCE Therapeutic Level III.

[1]  N. Pandis,et al.  The effect of scoliosis surgery on pulmonary function in spinal muscular atrophy patients: review of the literature and a meta-analysis , 2022, European Spine Journal.

[2]  A. Hell,et al.  Children with spinal muscular atrophy have reduced vertebral body height, depth and pedicle size in comparison to age-matched healthy controls. , 2022, World neurosurgery.

[3]  W. Lu,et al.  A biomechanical study on the effect of lengthening magnitude on spine off-loading in magnetically controlled growing rod surgery: Implications on lengthening frequency , 2021, Journal of orthopaedic surgery.

[4]  A. Hell,et al.  Smaller Intervertebral Disc Volume and More Disc Degeneration after Spinal Distraction in Scoliotic Children , 2021, Journal of clinical medicine.

[5]  A. Hell,et al.  Vertebral body changes after continuous spinal distraction in scoliotic children , 2021, European Spine Journal.

[6]  Y. Qu,et al.  Bone mineral density and its influencing factors in Chinese children with spinal muscular atrophy types 2 and 3 , 2021, BMC Musculoskeletal Disorders.

[7]  A. Hell,et al.  Continuous lengthening potential after four years of magnetically controlled spinal deformity correction in children with spinal muscular atrophy , 2020, Scientific Reports.

[8]  P. Claus,et al.  Altered bone development with impaired cartilage formation precedes neuromuscular symptoms in Spinal Muscular Atrophy (SMA). , 2020, Human molecular genetics.

[9]  L. Hornung,et al.  Intravenous bisphosphonate therapy in children with spinal muscular atrophy , 2019, Osteoporosis International.

[10]  A. Samdani,et al.  Pedicle stress shielding following growing rod implantation: case report. , 2019, Journal of neurosurgery. Spine.

[11]  D. Kieser,et al.  A six‐year observational study of 31 children with early‐onset scoliosis treated using magnetically controlled growing rods with a minimum follow‐up of two years , 2018, The bone & joint journal.

[12]  A. Hell,et al.  Combining Bilateral Magnetically Controlled Implants Inserted Parallel to the Spine With Rib to Pelvis Fixation: Surgical Technique and Early Results , 2018, Clinical spine surgery.

[13]  N. Endo,et al.  Bone Mineral Density After Spinal Fusion Surgery for Adolescent Idiopathic Scoliosis at a Minimum 20-Year Follow-up , 2018, Spine deformity.

[14]  S. Ohtori,et al.  Bone Mineral Density and Physical Performance of Female Patients 27 Years or Longer after Surgery for Adolescent Idiopathic Scoliosis , 2017, Asian spine journal.

[15]  T. Hurd,et al.  Osteoclast stimulation factor 1 (Ostf1) KNOCKOUT increases trabecular bone mass in mice , 2017, Mammalian Genome.

[16]  F. Rauch,et al.  Muscle-Bone Interactions in Pediatric Bone Diseases , 2017, Current Osteoporosis Reports.

[17]  Nehal A. Shah,et al.  ACR Appropriateness Criteria® Osteoporosis and Bone Mineral Density. , 2017, Journal of the American College of Radiology : JACR.

[18]  B. Wong,et al.  Low bone mineral density and fractures are highly prevalent in pediatric patients with spinal muscular atrophy regardless of disease severity , 2017, Neuromuscular Disorders.

[19]  L. Morandi,et al.  Bone and Spinal Muscular Atrophy. , 2015, Bone.

[20]  S. Milz,et al.  Comparison of the Immature Sheep Spine and the Growing Human Spine: A Spondylometric Database for Growth Modulating Research , 2010, Spine.

[21]  John J. Carey,et al.  T-Scores and Z-Scores , 2010 .

[22]  T. Hangartner,et al.  Height adjustment in assessing dual energy x-ray absorptiometry measurements of bone mass and density in children. , 2010, The Journal of clinical endocrinology and metabolism.

[23]  S. Iannaccone,et al.  Low Bone Mineral Density in Spinal Muscular Atrophy , 2008, Journal of clinical neuromuscular disease.

[24]  Sharmila Majumdar,et al.  Volumetric quantitative CT of the spine and hip derived from contrast-enhanced MDCT: conversion factors. , 2007, AJR. American journal of roentgenology.

[25]  Y. Qiu,et al.  The accumulation of bone mineral content and density in idiopathic scoliotic adolescents treated with bracing. , 2006, Studies in health technology and informatics.

[26]  B. Snyder,et al.  Bone Density Accumulation Is Not Affected by Brace Treatment of Idiopathic Scoliosis in Adolescent Girls , 2005, Journal of pediatric orthopedics.

[27]  E. Vuorio,et al.  Bone defect repair in immobilization-induced osteopenia: a pQCT, biomechanical, and molecular biologic study in the mouse femur. , 2005, Bone.

[28]  P. Vestergaard,et al.  Fracture risk in patients with muscular dystrophy and spinal muscular atrophy. , 2001, Journal of rehabilitation medicine.

[29]  E. Vuorio,et al.  Expression Profiles of mRNAs for Osteoblast and Osteoclast Proteins as Indicators of Bone Loss in Mouse Immobilization Osteopenia Model , 1999, Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research.

[30]  B. Snyder,et al.  Does Bracing Affect Bone Density in Adolescent Scoliosis? , 1995, Spine.

[31]  M. Asher,et al.  The effect of a stiff spinal implant and its loosening on bone mineral content in canines. , 1993, Spine.

[32]  S. Cook,et al.  Lumbar Spine and Femoral Neck Bone Mineral Density in Idiopathic Scoliosis: A Follow‐up Study , 1992, Journal of pediatric orthopedics.

[33]  L. Merlini,et al.  Fractures in myopathies. , 1991, La Chirurgia degli organi di movimento.

[34]  M. Leppert,et al.  Genetic mapping of chronic childhood-onset spinal muscular atrophy to chromosome 5q1 1.2–13.3 , 1990, Nature.

[35]  B. Cunningham,et al.  1989 Volvo Award in Basic Science: Device-Related Osteoporosis with Spinal Instrumentation , 1989, Spine.

[36]  H. Genant,et al.  Models of spinal trabecular bone loss as determined by quantitative computed tomography , 1989, Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research.

[37]  C. Cann,et al.  Quantitative CT for determination of bone mineral density: a review. , 1988, Radiology.

[38]  M. Boechat,et al.  Vertebral bone density in children: effect of puberty. , 1988, Radiology.

[39]  S. Cook,et al.  Trabecular Bone Mineral Density in Idiopathic Scoliosis , 1987, Journal of pediatric orthopedics.

[40]  C. Cann,et al.  Quantitative spinal mineral analysis in children. , 1986, Annales de radiologie.

[41]  H K Genant,et al.  Quantitative computed tomography for prediction of vertebral fracture risk. , 1985, Bone.

[42]  A. Campbell A survey of 190 cases of motor neurone disease. , 1965, Rivista di patologia nervosa e mentale.