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Is the Future of Spinal Bracing for the Child with Neuropathic Onset Scoliosis Rigid - A Timeline of Development?


Introduction

For many years, orthotists have been involved in corrective casting and manufacturing spinal braces to contain and correct the scoliosis of children presenting with neurological onset scoliosis. In many cases, casting frames have been used to enable corrective positioning and distraction, to ensure good correction of the scoliotic curves. Safe in the knowledge that the curve and the child's quality of life have improved. In the short term this is achieved, however, is this, the case in the long term?

It is known that there are two types of scoliosis presentations in neuropathic presentations; one originating from low core tone due to lack of core stability seen in children with cerebral palsy, and the second caused by other neurological presentations (SOSORT 2012). As young children, low muscle tone spines are often easy to position, however, they present with very flaccid trunks, which often lean to one side or the other if unsupported. These children, however, become the most difficult to control in later life as natural maturation occurs, often requiring surgery to reduce pain, subluxed hips and high Cobb angle scoliosis. Surgery endeavours to provide some stability and comfort.

It is known that 62% of the cerebral palsy population experiences pain; of which 70% is experienced in the lower limbs and pelvis and 9% is reported in the lower back1. The pain is often a result of poor balance and spinal alignment, coupled with long durations of fixed sitting positions. The pressures provided by rigid orthotic interventions to this client group also do not assist in the long term, even if well designed and constructed. As infants the braces are tolerated well, however, as the child grows and the low muscle tone enables postural curves to become structural. Once structural, bracing can at best reduce migration.

The experienced clinician will recognise a typical annual cyclic presentation of seeing the same patient return year on year for new spinal braces, with the Cobb angle continuing to worsen each year. This is due to a vicious cycle, caused by the response pattern to a neurological insult, where the central nervous system lesion initiates an abnormal postural muscle tone reaction. This often shows as weakness in one of the muscles providing spinal balance. The resultant unbalanced posture will ensure abnormal quality of movement which provides abnormal sensory feedback and feed forward to the brain. The brain recognises this as normal and compensates. This continues as worsening abnormal quality of movement and worsening abnormal postural tone2 resulting in unbalanced body awareness and postural scoliosis.

The resultant typical long "C" curve continues to develop, despite the best efforts of the clinical team to stretch out and counter-rotate the spine. Even when aggressive cast rectification over the Iliac crests to ensure a good pelvic fixation to counter the pelvic obliquity is incorporated. This fixation also enables spinal distension. In adolescence, the muscle pull becomes stronger and the curve develops unrestrained eventually requiring surgery to improve positioning and alignment, coupled with all of the resultant surgical risks.

It is known that the first stages of correction are easily maintained by sitting systems and sleeping systems now known generically as 24 hour postural management. Simple soft spinal brace using circumferential pressure also can provide some midline experience. However, even soft spinal braces become the scaffolding around the spine encouraging the spinal muscles atrophy. The same muscles are already affected by imbalance of muscle tone due to the central nervous system infarct. Even with a supportive bracing, the imbalance is uncontrolled and over time the postural curve develops into a structural curve with the wedged vertebra and rotation over the long "C" curve typical of this client group. It has been suggested that in adolescent idiopathic scoliosis, changes occur in the molecular structure of the intervertebral disc leading to an unloading of the vertebral body. This initiates a reduction of pressure on the convex side enabling the bone to grow and resulting in a wedging of the vertebra.3 This could be the same mechanism in neuropathic onset cases. The muscle imbalance appears to continue even when braced. This suggests that the brace is treating the result (scoliosis) and not the cause (muscle imbalance).4

Method

In the 2003, a suit was used to control a spinal curve of a child with scoliosis caused by a cystic tumour which caused a T9 apex curve of 33°.5 As the patient , a 7 year old girl did not want further spinal bracing, a Dynamic Elastomeric Fabric Orthosis (DEFO)scoliosis suit was designed based on the blueprinting of the Boston Brace system6 using derotational and compressive translation panels7 to laterally shift the thoracic curve, historically used in the treatment of neuropathic onset scoliosis (Fig. 1 ). It was found that the close fit of the suit and corrective effects of the panels enabled a change in Cobb angle, reducing the curve down to 15° within a short period of time.

Initially the client felt off-balance, but quickly adapted to the new position, suggesting that the suit had affected spatial awareness. Prior to this case, the use of DEFO suits in children as young as 2 year of age had been developed in the Jenny Lind Children's physiotherapy department within the Norfolk & Norwich University Hospital to stabilise children with low tone cerebral palsy based on work done elsewhere in the UK.8

The clinical team discovered that if the child was placed into a suit for a period of 3-4 years the child appeared to learn body and spatial awareness and therefore no longer required orthotic intervention. Previous studies reported high compliance (9), which has been mirrored with children preferring to wear the suits. Extra Lycra based reinforcement panels could be placed on one side or the other to stiffen up the suit to prevent and encourage improved spinal alignment, reducing development of postural curves. Innovation through research into the reinforcement panel designs, has enabled different client presentations to be successfully treated.

Immediate effects could be seen in children, presenting with low muscle tone and identified by flared inferior, dorsal ribs and typical patterning of protraction of the shoulders; a mechanism children use to stabilise their head. This patterning also severely affects the child's range of upper limb movement; therefore affect their schooling and quality of life, both current and in the future. It was noted that once the DEFO suits were worn, the children did not need to support themselves by holding on to chairs or benches. This is clearly shown in Fig. 2 .

Initially it was thought that DEFO scoliosis suits could only be effective on curves up to 30°, however a 5 year old child presenting with myotonic dystrophy, coupled with pectus carinatum, required a nonrigid intervention. The thoracic T8 apex curve measured 70° with over 20° of vertebral rib angle difference. Using the x-ray to provide a blueprint the DEFO scoliosis orthosis was designed. One year later the child was routinely x-rays and had a reduced scoliosis curve of 35° and vertebral rib angle (Fig. 3 ). This confirmed that the orthosis could cope with curves in excess of 45°, the current recommended cut off for rigid bracing.

Discussion

The use of this type of scoliosis orthosis does question whether rigid bracing is appropriate for the treatment of the neuropathic onset scoliosis. Clinician's long term experiences must question the situations where rigid bracing will be the best option. In the earlier stages there is now limited evidence for the use of dynamic spinal bracing in various guises. The use of strapping systems to initiate corrective responses and some localised effects can have mild effects in the early stages.10

Repeatability, however, often rely on the clinician and carer expertise. By fixing down the reinforcement panelling, the suits are able to be truly dynamic in that they will continue to provide a local deeper pressure gradient. This enables the body segments to be "encouraged" to move laterally to a more symmetrical position. Compression, coupled with designed counter rotation bands, also have a similar effect that would explain the results we have seen to date.

Conclusion

The use of DEFOs have been proven in the treatment of other neurological presentations11, 12 and have become an important orthotic option in the developmental training in young children with cerebral palsy. As orthotists we need to gain a better understanding of the neurophysiological effects of deep pressure on muscle tone and corrective positioning. Although all orthotists are trained in the skills of alignment of body segments, perhaps the profession should investigate using other less rigid methods of realignment. Looking to the future, more scientific studies into this area are required, but one thing is certain the future is definitely not rigid.

Acknowledgements

The physiotherapy services of Norfolk & Norwich University Hospital NHS Foundation trust, Cambridge University Hospital NHS Trust and Pace Centre, Aylesbury. UK

Photos reprinted by kind permission of Orthopaedic & Spinal News ŠNovember 2011

Orthotic Clinical Specialist (DM Orthotics Ltd) / Associate Lecturer (University of East Anglia), UK

Certified Orthotist (Korthotics), Australia

References:


  1. Ramstad KJ, R; Skejeldal,O; Diseth,T;. Characteristics of recurrent musculoskeletal pain in children with cerebral palsy aged 8 to 18 years. Developmental Medicine & Child Neurology. 2011;53(11):1013-8.

  2. Edwards S. Abnormal Tone and movement as a result of neurological impairment:considerations for treatment. In: Edwards S, editor. Neurological Physiotherapy. London: Churchill Livingstone; 2003. p. 89-114.

  3. Stokes A, editor. Scoliosis: discs or vertebrae. Cobb angle friend or foe? 7th International Conference on Conservative Management of Spinal Deformities Montreal, Canada 20-22 May 2010; 2010; Montreal, Canada: Biomed Central.

  4. Matthews MR, AS; Chatterjee, S, editor. Does rigid bracing provide the best outcome for children with neurological onset scoliosis. 9th International Conference on Conservative Management of Spinal Deformities; 2012; Milan, Italy: Scoliosis Journal.

  5. Matthews M, The use of Dynamic Lycra Garments in the treatment of Scoliosis. 2005; Glasgow: British Association of Prosthetists and Orthotics.

  6. Hall JM, ME; Cassella, MC. Manual for the Boston Brace Workshop. Boston: Boston : Children's Hospital Medical Centre; 1976.

  7. Matthews M, Crawford R. The use of dynamic Lycra orthosis in the treatment of scoliosis. A treatment case study. Journal of International Society of Prosthetics and Orthotics. 2006;30(2):174-81.

  8. Edmondson J. How effective are lycra suits in the management of children with cerebral palsy? APCP Journal. 1999;March 1999:49-57.

  9. Bridges S, Mayston M, Peirson J. The effects of dynamic socks in ambulant children with cerebral palsy:a pilot study: University College London; 2004.

  10. Maguire CS, JM; Frank,M; Romkes,J;. Hip abductor control in walking following stroke- the immediate effects of canes, taping and TheraTogs on gait. Clin Rehab. 2009(November). Epub November 11th 2009.

  11. Elliott CR, S.; Hamer, P.; Alderson, J.; Elliott, B.;. Lycra arm splints improve movement fluency in children with cerebral palsy. Gait Posture. 2011;33:214-9.

  12. Matthews MW, M.; Richardson, B.;. Effects of dynamic elastomeric fabric orthoses on children with cerebral palsy. Prosthetics & Orthotics International. 2009;33 (4):339-47.