Academy Society Spotlight: A Discussion on the Case for Rigid Bracing in Children with Neuropathic Onset Scoliosis

For many years, spinal corrective thoracic-lumbar-sacral orthoses (TLSOs) have been prescribed for children who present with neuropathic onset scoliosis (NOS), as classified by the Scoliosis Research Society, and who also have conditions like cerebral palsy (CP) and Rett syndrome. 33 It has become a common treatment protocol that is as inevitable as spinal surgery, due to the progressive nature of the particular scoliosis curves. In the United Kingdom, this practice is now less common due to evidence questioning the efficacy of rigid orthotic management and a recent review of clinical practice. Discussion Over the years, I, along with many other orthotists, have routinely cast mainly children presenting with CP for rigid spinal corrective braces. These children were seen on an annual cycle for "anniversary" casting, which was done on a Risser traction table to enable good spinal sagittal plane and scoliosis corrective alignment. This situation is not surprising as history indicates that 25 percent of children with CP are likely to develop a spinal deformity. The incidence ranges from 5 percent in children with bilateral presentations to 74 percent in children with quadrilateral presentations, while children who are wheelchair dependent have a 90 percent increased risk of progressive spine deformities due to impaired motor control and postural balance. 32,25 Children who are ambulant typically have a 9-15 percent chance of developing scoliosis. 18 The severity of the curve can be linked to the Gross Motor Functional Classification System (GMFCS) where there is evidence that children with a Level V classification (limited in their ability to maintain antigravity head and trunk postures and control leg and arm movements) will deteriorate faster than children at Levels I-IV. 10 Indeed, historic data suggests that all children with GMFCS Level IV or V will eventually require surgical intervention to their spines. 11 Every consecutive year, the patients with GMFCS Level IV or V typically present with increased Cobb angles and spinal rotation, despite the corrective bracing intervention. The children required progressively more aggressive bracing designs to control the curve and spinal symmetry. As the children grew older, the curves became too large for brace control. Multilevel scoliosis surgery is the only option available in most of these cases. Surgery at this stage is major, invasive, and costly, and carries a 33 percent risk of complications. 6 If the curve angle is too great, or there are significant risks due to a patient's possible reaction to anesthesia, or other prohibitive issues contraindicating surgery, the only treatment option left is to observe the patient and manage his or her pain. To understand why a TLSO intervention can only slow curve progression in this patient population, it is important to grasp the differences between adolescent idiopathic scoliosis (AIS) and NOS. In AIS, there are mechanisms of change in the vertebral growth development where strong pathological pressure can inhibit endochondral longitudinal growth on a segment of the spinal vertebra, coupled with a contrasting reduction in compression causing an increased eccentric growth, resulting in vertebral wedging. 31 In NOS, a disharmonious control of the trunk musculature around the spinal axis occurs, complicated by muscle compensatory mechanisms. There are two curve variants in NOS. The most common is the C-shaped curve (80 percent), which presents as a single thoracolumbar or lumbar curve and has been associated with pelvic obliquity and hip dislocation, however, there is no direct association. 18 The second presentation is a complex S-shaped curve (20 percent) with similar properties to AIS but more aggressive. 32 Children with no or little antigravity head and trunk control have a propensity for scoliosis and it often manifests in the abdominal muscles as "flared ribs," an indication of a lack of abdominal control and poor core stability. 19 The inability to counter gravity is commensurate with the brain injury and is typical of low tone. The resultant kyphotic positioning ensures that an atypical sitting posture is adopted and the child is unable to adjust his or her sitting position. 5 There is some evidence that changes to the central nervous system can be triggered by prolonged kyphotic or lordotic sitting, which can cause compressional changes within the spinal cord. 12 Poor sitting ability can lead to a destructive cycle of events related to proprioceptive learning and positional change. Asymmetrical postural positioning of the spine due to secondary unbalanced muscle tone can lead to proprioceptive learning and spinal decompensation. 7,14 This continues and shows as a mild, low-level C-shaped postural curve. This position is learned as the new normal, resulting in asymmetrical loading on the vertebral growth plates. The high pressure prevents normal growth plate development, initiating an unloading of force on the convex side and increased pressure on the concave side-driving the vicious cycle. Over time, wedge vertebral bodies occur, at which point a structural curve develops. The brain maintains and regularly updates a virtual image of self within the child's memory; it uses this image to prepare for motor end goals by enabling the prediction of a learned movement pattern to initiate the correct limb movement to grasp an object. In children who have CP, this image is distorted because of potential deficits in motor planning, which may cause difficulty in reaching to grasp an object. 30,8 As a result, it will take longer to reach the expected muscle target and to complete sequential movement planning, because these children may have a poor internal model of their musculoskeletal systems. 26,15 This suggests that perhaps we should initiate orthotic spinal management much earlier than current practice norms. Most TLSOs are used to provide stability in wheelchairs, but there is evidence that the rigid spinal TLSOs can cause discomfort due to excessive skin pressure over bony prominence, reduced quality of life, or the inability to move, often resulting in poor compliance. 2,32 In Letts et al.'s clinical practice, the use of semirigid, closed-foam TLSOs solved some of the compliance issues but did not solve the need to fixate the orthosis over the iliac crests to provide the foundation for distraction. 17 It is also possible that fixation within a brace coupled with a wheelchair seated position is painful, with reports of 62 percent of children with CP experiencing pain, 70 percent in the lower limbs/pelvis and 9 percent in the lower back. 27 There is some evidence that TLSOs may reduce the rate of curve progression and can limit the consequences of impaired pulmonary development and function. 1,7,23,24,32,33 However, this needs to be balanced by the possibility of immobility of the spine resulting in muscle atrophy of already weak spinal muscles, similar to the effect seen in children who wear AFOs over an extended period of time. 16 This, therefore, suggests that we should refrain from rigidly bracing small children. Research in the United Kingdom has shown that early intervention for NOS can have encouraging results, and although there are no randomized, controlled trials published to date, there is a growing evidence base for use of a dynamic elastomeric fabric orthosis (DEFO). While there are no empirically proven studies about the efficacy of DEFOs, there are a number of case studies and case-control studies, and a recent review of clinical practice that offers an alternative option. In a small number of clinics in England, DEFOs have been used to control scoliosis since 1997. A paper published in 2006 suggests that a DEFO suit designed to counter scoliosis in a child with a spinal tumor was effective in maintaining a curve for a period of time. A published poster paper describes a child with myotonic dystrophy and a spinal curve of 70 degrees at T8 that was reduced to 35 degrees, coupled with a change in the vertebral rib angle from above 20 degrees to less than 20 degrees, within one year of treatment with a DEFO. 21,20 Further studies include the use of gait laboratories to show the changes in sagittal position and stability when using DEFOs for children with poor core stability. 28,9 DEFOs have been used to stabilize the spine in children, combining the effects of circumferential compression and the facilitation that neurodevelopmental physiotherapists use to initiate spinal extension by downward compression on the shoulders. The pressure was thought to increase proprioceptive input, and that the production of a mechanical compressive effect provides a stabilization effect on the abdomen, improving spinal symmetry and stability; a spinal corset can have a similar localized effect. This concept was discarded a decade ago with one author disregarding dynamic orthoses as "illogical in patients with neuromuscular scoliosis as these orthoses require normal muscle function to be effective." 23 However, the term "dynamic" relates to the elastomeric fabric's ability to be constrained by the linear range of elasticity, where the fabric can stretch and therefore be dynamic until the end of range is reached, whereby the resistance to stretch is increased, causing high pressure in a localized area. The ability of new fabrics to adhere to the skin enables a force to be transferred through the skin to the underlying muscle and bone structure in a local area. The circumferential pressure has been shown to reduce pelvic pain in sport injuries, thereby providing improved sitting comfort. 29 Evidence suggests that children with CP often sit in a posterior sitting position with the center of gravity above or just behind the ischial tuberosity and with approximately 25 percent of the body weight transferred to the feet. 13 If this position is not corrected, then asymmetric loading on the vertebra is possible. A recent paper identifies an increasing body of evidence to support the link between control of individual trunk segments and the resultant effect on gross motor function and mobility. 4 This suggests that by improving alignment and spinal symmetry, DEFOs may be able to improve coordination and function in this population by enhancing hip joint stability, force closure, and proprioception. 22,29 This concept is supported by a review of clinical practice in southern England, wherein five National Health Service pediatric clinics identified 121 children in DEFO suits used to manage scoliosis from a subject group of 180. The retrospective review suggests that dynamic orthoses could have a place in the prevention and management of NOS. 3 The study also confirms a 33 percent complication rate within the group of 16 children who had surgery; complications included rod breakage, excessive movement in fixations, infections, reduced head control, respiratory arrest, and deep wound infection. Conclusion The question of the efficacy of the use of rigid TLSO management of NOS is open to discussion. Semirigid, closed cell TLSOs may be of use to enable growth to continue, while slowing the higher curves prior to surgical intervention. The use of DEFO scoliosis suits can provide a further option for the orthotist in the treatment of children with neuropathic onset scoliosis if used as an early intervention. However, should the orthotist wait until the scoliosis is structural and progressing? Early intervention is starting to demonstrate that possibly prophylactic options may be an alternative to rigid TLSOs. Martin Matthews, MPhil, Dip OPTEC, MBAPO, is the orthotic external examiner for the University of Salford, England, Orthotic and Prosthetic degree course. He is also an associate lecturer at the University of East Anglia, United Kingdom. Matthews is responsible for research and development at DM Orthotics, Cornwall, United Kingdom, and is a member of the Spinal Orthotics Society of the American Academy of Orthotists and Prosthetists. References Editor's note: The references are listed in alphabetical order, per the author's original manuscript. The footnotes are listed in non-numerical order to correspond accordingly. 2ND, S. 2000. Second Skin Dynamic Lycra Splints for Neurological Dysfunction. Allam, A. M., and A. L. Schwabe. 2013. Neuromuscular Scoliosis. PM&R, The Journal of Injury, Function and Rehabilitation 5 (11):957-63. Blandford, S., J. Marsden, M. Matthews, and J. Freeman. 2014. Dynamic elastomeric fabric orthoses in neuropathic scoliosis management: An audit of the frequency and characteristics of use. Scoliosis 9 (Suppl. 1):11. Curtis, D. J., P. Butler, S. Saavedra, J. Bencke, T. Kallemose,S. Sonne-Holm, and M. Woolacott. 2015. The central role of trunk control in the gross motor function of children with cerebral palsy: A retrospective cross-sectional study. Developmental Medicine & Child Neurology 57 (4):351-7. Damiano, D. 2006. Activity, activity, activity: Rethinking our physical therapy approach to cerebral palsy. Physical Therapy 86 (11):1534-40. Diefenbach, C., M. N. Ialenti, B. S. Lonner, J. R. Kamerlink, K. Verma, and T. J. ErricoJ. 2013. Hospital cost analysis of neuromuscular scoliosis surgery. Bulletin of the Hospital for Joint Diseases 71 (4):272-7. Driscoll, S. W. and J. Skinner. 2008. Musculoskeletal complications of neuromuscular disease in children. Physical Medicine and Rehabilitation Clinics of North America 19 (1):163-94. Duff, S. V., and A.M. Gordon. 2003. Learning of grasp control in children with hemiplegic cerebral palsy. Developmental Medicine & Child Neurology 45 (11):746-57. Edwards, K., and M. Cramp. 2004. Using motion analysis to investigate whether wearing dynamic Lycra garments changes posture and movement in children with cerebral palsy: A pilot study. University of East London. Garg, S,. G. Engelman, H. Yoshihara, B. McNair, and F. Chang. 2013. The relationship of gross motor functional classification scale level and hip dysplasia on the pattern and progression of scoliosis in children with cerebral palsy. Spine Deformity 1 (4):266-71. Graham, K. H. 2013. The right treatment for the right child. American Academy of Cerebral Palsy and Developmental Medicine, October 18; Milwaukee. Elsevier. Harrison, D. E, R. Cailliet, D. D. Harrison, S. J. Troyanovich, and S. O. Harrison. 1999 A review of biomechanics of the central nervous system-Part I: Spinal canal deformations resulting from changes in posture. Journal of Manipulative and Physiological Therapeutics 22 (4):227-34. Harrison, D. D., S. O. Harrison, A. C. Croft, D.E. Harrison, and S. J. Troyanovich. 1999. Sitting biomechanics Part 1: Review of the literature. Journal of Manipulative and Physiological Therapeutics 22 (9):594-609. Kouwenhoven, J. M., P. M. Van Ommeren, H. E. Pruijs, and R. M. Castelein. 2006. Spinal decompensation in neuromuscular disease. Spine 31 (7): E188-91. Kurz, M. J., K. M. Becker, E. Heinrichs-Graham, and T. W. Wilson. 2014. Neurophysiological abnormalities in the sensorimotor cortices during the motor planning and movement execution stages of children with cerebral palsy. Developmental Medicine & Child Neurology 56 (11):1072-7. Lam, W. K., J. C. Y. Leong, Y. H. Li, and W. W. Lu. 2005. Biomechanical and electromyographic evaluation of ankle foot orthosis and dynamic ankle foot orthosis in spastic cerebral palsy. Gait & Posture , 22 (3):189-97. Letts, M., D. Rathbone, T. Yamashita, B. Nichol, and A. Keeler, A. 1992. Soft Boston orthosis in management of neuromuscular scoliosis: A preliminary report. Journal of Pediatric Orthopaedics 12 (4):470-4. Loeters, M.J., C. 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Neuromuscular scoliosis. Orthopaedics & Traumatology: Surgery & Research , 99S:S124-39.

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