**2. Scoliosis presentations**

Scoliosis presentations are due to numerous causes and include genetic changes and omissions to the bony structures; neurological dysfunction to the muscles that support and stabilize the spinal structure; hypo-mobility disorders (Ehler Danlos Syndrome); degenerative scoliosis and dysfunctional learnt patterns of movement. All learnt functions like sitting and walking is a learned pattern of movement, which rely on movement through error based learning to recalibrate a particular movement and altered position awareness [7]. In children, with cerebral palsy (CP) their image of self is different to reality due to the difference in their internal model of self [8]. This shows in sitting, where they feel they are sitting straight, however in reality they are sitting to one side. This loads one side of the spine and sets up a dysfunctional pattern, which they learn and understand as normal. This results in the complex scoliosis cases we see in adolescents and adults- particularly when requiring wheelchairs. Most of these issues are generally identified during childhood, however, they can affect adolescents and adults of all ages with scoliosis, whether idiopathic or neuropathic in origin. Bracing is therefore a major intervention in adolescents and adults who cannot or do not want to follow the surgical route. Although there are several different scoliosis causes, this section will concentrate on the two main causes of scoliosis in adolescents and adults, which require bracing: idiopathic onset scoliosis (IOS) and neuropathic onset scoliosis (NOS).

Idiopathic onset scoliosis is defined as a lateral curvature of the spine, that is greater than or equal to 10 degrees with no known cause. It is further defined by the age of initial presentation, infantile (zero to 3 years), juvenile (3–9 years of age) and, adolescent (10–17 years of age). Progression is related to growth, so patients are most vulnerable to curve progression during periods of rapid growth as occurs during the adolescent years. The Boston Brace, which introduced the concept of using a symmetrical form to fabricate the original modules which then, depending on the patient's x-ray analysis (blueprint) and physical presentation, is trimmed and had strategically placed pads added to make it asymmetrical. Numerous studies have shown this method to be effective [3, 9]. Recently, through the advent of scanning and CADCAM technologies, a custom fabricated asymmetric model of the patient is used to fabricate the brace rather than the symmetrical form. The Boston Brace 3D (**Figure 4**), the ARTbrace and Rigo Cheneau - type braces, are examples that utilise three point triangular sideways movement (known as translation) viewed from the posterior using x-rays of the back, re-aligning a small number of vertebra causing a localised positional change {segmental moulding [10]}. They included the combination of forces for regional and local de-rotation to achieve corrective balance and physiological alignment in the sagittal plane [11] **Figure 5**.

These coupled with physiotherapeutic scoliosis specific exercises (PSSE) like Schroth physiotherapy work to keep the spine mobile [12], but are also held physically by a brace, which encourage the re-alignment of the spine during wear.

Neuropathic onset scoliosis (NOS) typically appears in early childhood, particularly in children with cerebral palsy (CP), and continues into adulthood due to the dysfunctional loading on the spine which causes vertebral and rib remodeling. Twenty five percent of people with CP will develop scoliosis curves and range from 5% incidence for bilateral spastic to 74% in people with quadrilateral- spastic presentations due to the imbalance of muscle pull, and brain damage [13]. Most curves *The Use of a Dynamic Elastomeric Fabric Orthotic Intervention in Adolescents and Adults… DOI: http://dx.doi.org/10.5772/intechopen.96391*

### **Figure 5.**

*Diagrammatic representation of X-ray blueprinting for dynamic elastomeric fabric scoliosis suit- identifying the three points of pressure (yellow arrows), distraction (orange arrow), downward compressive force (pink arrow) and de-rotational shoulder compression force (Red thin arrows).*

present as a single thoracolumbar or lumbar "C" shaped curve associated with pelvic obliquity and hip dislocations, often seen in gross motor function classification scale (GMFCS) [14] Level 4/5 presentations.

The gross motor functional scale is a way of identifying what level of function a child with cerebral palsy can achieve and is divided into 5 levels.

**Level 1** identifies children who can walk at home, school and in the community including outdoors with limited coordination, speed and balance when running and jumping.

**Level 2** identifies children who can climb stairs holding on to a support rail, however they can experience difficulties in balancing and walking when on slopes and rough ground particularly when in crowded or confined areas.

**Level 3** identifies children who need hand-held walking and mobility aids in doors and need supervision when climbing stairs. They will also tend to use selfpropelled or powered wheelchairs for longer distances.

**Level 4** identifies children with require physical assistance or powered mobility in most settings If physically assisted they can walk short distances around their homes and will often require powered wheelchairs and body weight support walkers. A self-propelled wheelchair or powered chair will be used whilst at school, outdoors and in the community.

**Level 5** identifies children who are transported in manual wheelchairs in all areas. The children have a limited ability in maintaining head control and trunk posture [15]. They also have trouble with arm and leg control A classification at the age of 6 is unlikely to change level during adolescence into adulthood.

Therefore level 4 and 5 indicates that the child/young adults will be requiring wheel chair support, with basic or complex design dependent on severity [14]. A classification at the age of 6 is unlikely to change level during adolescence into adulthood. Twenty percent of scoliosis curves develop as a "S" shaped curve with balanced, symmetrical thoracic kyphotic and lumbar lordotic curves [13], normally associated with idiopathic scoliosis see **Figure 6a**, **b**.

**Figure 6.**

*(a) A typical "C" shaped curve to the left. (b) A typical "S" shaped curve with a left lumbar and right thoracic curve.*

Adolescents with GMFCS Level 4/5 are dependent on wheelchairs for mobility, which due to a neuropathic or neuromuscular disease, have a 90% increased risk of progressive spinal deformities due to the impairments in postural balance and motor control [16]. There is also an increased risk of flexed sitting patterns due to hip flexion contractures, encouraging spinal deformity due to the loss of the protective lordosis and atypical loading patterns. These can lead to pain and discomfort leading to increased decline in the patients quality of life (QOL) [17].

Ehlers Danlos Syndrome is a group of disorders that affect the connective tissue that supports the tissues and human body organs, blood vessels, bones and skin [18]. The signs include extremely loose joints, which can sublux completely, coupled with extreme pain. In recent years, this connective tissue disease has been linked with young women presenting with scoliosis [19]. The women are often in their mid-thirties, following pregnancy and present with extreme pain, often due to nerve compression.

### **3. Elastic scoliosis bracing**

Rigid and semi- rigid (foam based) spinal orthoses have been the common bracing option for adults, however the developments in dynamic elastomeric fabric orthoses (DEFO) using form fitting elasticated orthoses, have shown promise in the clinics based on the authors' clinical use. The use of "elastic" orthoses has become an additional bracing option, alongside rigid and semi-rigid scoliosis braces.

There are two variants of DEFOs that have evidence for use in scoliosis management, namely SpineCor® and Dynamic Movement Orthoses (DMO®). There are other Lycra suits, but they are mainly used in juvenile (less than 9 years of age) patients. As this chapter is concerned with adolescents (10–17 years of age) and adults (18 years of age and older) with scoliosis, they will not be discussed.

### *The Use of a Dynamic Elastomeric Fabric Orthotic Intervention in Adolescents and Adults… DOI: http://dx.doi.org/10.5772/intechopen.96391*

SpineCor® has a resemblance to an early somewhat simple, but controversial idea of using "Oblique and Spiral bandage" utilized by Richard Barwell of London in 1868 that purported to assist in scoliosis management (**Figure 7**).

An eminent surgeon of his time, Henry Bigg in 1877 [1], questioned the validity of this bandage. However, in recent years the use of diagonally activating compressive force correction now supports the early thinking behind the bandage type orthoses.

The SpineCor® brace has had some success in younger patients with idiopathic scoliosis, utilizing the compressive and de-rotative effects of strong elastic bands fixed to a bolero shaped vest and either a plastic anterior and posterior shell held in place by webbing straps, or shorts which enable fixation of the corrective straps. The original prospective observational study on the effectiveness of the SpineCor brace, used a standardised criteria proposed by the Scoliosis Research Society (SRS). The study included 170 patients (158 girls and 12 boys). Thirty-nine participants required spinal surgery, 12 withdrew and 14 participants were weaned out of the treatment as curve progression was deemed to be stable. Of the 105 remaining participants, 47 completed the 2-year brace follow up [20].

A more recent retrospective study evaluating the effects of the SpineCor brace that also followed the Scoliosis Research Society criteria for brace management of adolescent idiopathic scoliosis reported that the SpineCor® brace treatment could increase the risk of curve progression, when compared to the Boston Brace. Scoliosis curve progression occurs in the spine due to changes occurring naturally in the vertebra and intervertebral discs. If left without treatment the curve will increase exponentially [21]. The use of correctly fitted and function spinal brace will reduce the rate of curve progression and in most cases reducing the Cobb angle indicating curve regression. In the SpineCor brace study, a total of 243 patients were treated with either the Boston brace (146) or the SpineCor brace (97) with scoliosis

**Figure 7.**

*Oblique and Spiral bandage utilised by Richard Barwell of London in 1868. (Page 182 Orthopaedic Appliances Atlas, Vol.1 1952).*

curve progression recorded across the group. The average curve progression for the SpineCor cohort was 14.7° ±11.9°, compared to 9.6° ± 13.7°. The proportion of patients reaching 45° was 51% (SpineCor) and 37% (Boston Brace), however the proportion of patients referred for surgery was 39% (SpineCor) and 30% (Boston Brace). The paper concluded that the odds of reaching a ≥ 45 was 2.07 times greater when using a SpineCor brace [22].

Further comparison studies showed that the SPoRT (Symmetric, Patient oriented, Rigid, Three-dimensional, active braces) was more effective than SpineCor® [23, 24]. The SPoRT concept provides evidence of effect and is shared in three types of rigid braces. The Sforzesco brace joins two previous brace designs (Sibilla & Lapadula), to provide a new spinal concept which avoided the need for casting the worst scoliosis presentations. Brace compliance was a key driver for the new treatment journey and included mechanical efficacy, the active brace principle, versatility, and adaptability coupled with teamwork and patient compliance. Scoliosis correction is provided through the shape of the adjustable rigid body envelope, mechanical" pushes" encouraged the spine to move into a predetermined void, that adapted and remodeled body shape to unwind the scoliosis curve presentation (**Figure 8**).

A randomised controlled trial compared the SPoRT rigid concept with the SpineCor dynamic brace to identify the treatment effect over time. Using a retrospective controlled study, two groups were reviewed using clinical and X-ray evaluations. Data was recorded at the beginning and end of the study to record the scoliosis curve angles for SPoRT braces (20 patients) and SpineCor braces (41 patients). Both brace interventions proved to be effective in improving the aesthetics of the deformities, however the SPoRT brace was found to be more effective than SpineCor in avoiding scoliosis curve progression [23].

There is evidence that the SpineCor® brace relieves pain by improving posture following the reduction of mechanical strain on the neuromuscular system in adults. The brace enables spinal joint off-loading by reducing the misalignment of the spinal segments. Pain perception data from Marcotte [25] which reviewed the effect of SpineCor spinal braces used for between 18–28 months from 30 adults (26 females/4 males), which showed 77% overall improvement in pain recorded. Half of the patients reported total pain relief, although there was no significant reduction in spinal curvature in adults. This outcome may have been similar to the reasoning in the original designs in the 18th century.

**Figure 8.** *The Sforzesco brace- the most recent addition to the SPoRT scoliosis management concept.*

### *The Use of a Dynamic Elastomeric Fabric Orthotic Intervention in Adolescents and Adults… DOI: http://dx.doi.org/10.5772/intechopen.96391*

Dynamic Elastomeric fabric orthoses (DEFO) were first reported in 1960. The authors described the use of crepe bandages wrapped around a child with athetoid cerebral palsy. This caused reduction in the muscle tone in his arms and legs which then enabled more stability and less fatigue [26].

In 1995, an Australian paper describing the use of custom "UPsuit" Lycra® suits on children and young adolescents, with semi-rigid reinforcement to provide stability to the spine and to improve sitting balance [27]. Management of the scoliosis was provided by metal spiral boning stitched in fabric pockets to provide rigidity and apply resistance to stretch - providing areas of higher resistance to stretch [28]. However, there is limited scoliosis management using Second Skin suits (**Figure 9**).

In 2007, a study from the United States introduced a basic base Lycra® short and pants with shoulder straps called the "Stabilizing Pressure Input Orthosis" (SPIO) [29]. The study stated that significant functional improvements to balance, improved postural control and muscle readiness were found, however as an observational and discussion report, the only evidence provided was photographic pre and in-intervention images. It was postulated that there were deep sensory changes which could account for the reduction in athetoid movement and the improved stability of the spine in children presenting with low tone [30].

In 1999, Rennie et al., in a repeated measures study of 8 children (7 children had cerebral palsy and 1 with Duchennes muscular dystrophy), used laboratory gait analysis to report on the outcomes of a long arm, long legged suit made by Camp Ltd., in the United Kingdom (**Figure 10**). Five participants showed a reduced root mean square error (RMSE) scores indicating improved postural stability, confirming the findings of Blair [28] and Hylton [30]. However, the improved postural stability only positively affected the distal stability in three participants, which could have been due to the short 6-week duration of the trial.

Compression at the pelvis in DEFO shorts [31, 32] can reduce the pain experienced by adults with intellectual disabilities, who experience high rates of falls and have gait and balance issues. The reason for this may be because the DEFO suits and shorts reduce the amplitude and range of movement in the spine and pelvis,

**Figure 10.** *The original basic Lycra® long legged and armed suit used in the Rennie study.*

therefore providing pelvic stability [33] which consequently enhance the quality of life in both children and adults.

Scoliosis suits were first reported by Matthews and Crawford in 2004 [34] in a single case study using a DEFO to treat a child with a tumour on the spine. The tumour had previously been excised at the age of 7 yrs. The tumor extraction operation caused a 33-degree Cobb angle scoliosis to appear at the tumour site T9 (**Figure 10**). The child, did not want a rigid brace due to the rigid brace appearance [34]. The orthotist prescribed a non-invasive vest top suit to control the curve, based on experience, with children presenting with hypotonic cerebral palsy with scoliosis.

The suit applies a tight fit at the hips and thighs to prevents the suit from riding up and therefore a resistant force is applied to the shoulders. The suit uses the principle of pressure applied to the shoulders to facilitate an improved sitting posture, as seen when neurodevelopmental physiotherapists place their hands on the shoulders of children with low spinal muscle tone and retract the shoulders coupled with downward pressure to facilitate an extension of the spine in sitting. The compression of the suit also enables the patient to become more aware of their bodies position in space through heightened proprioception.

Elastomeric fabric sideways translation reinforcement panels, which were designed to reduce the scoliosis Cobb angle were added, using the patient preintervention x-rays and blue printing process (**Figure 11a** and **b**), as described in the Boston Brace clinical manual. The manual describes how to use the patient X-rays to identify the vertebra levels, position of the spinal curve and rotation [35] to guide prescription. The addition of, overlying a "V" shaped translation panel (see yellow arrows on **Figure 11b**) to the convex side of the curve ensured a lateral force was applied below the apical vertebra as close to midline as possible – this provided a long low pressure on the ribs which encourage the patient to move away from the pressure point into the comparative void of no reinforcement on the right side. A further shoulder compression panel (see red arrows on **Figure 11b**), extends upward posteriorly over the left superior border of the scapula and over the anterior shoulder, before continuing downwards until clear of the axilla and then continues diagonally anteriorly across the anterior ribs to finish at the right greater trochanter. The left shoulder compression, spinal derotation panels were so efficient, that the suit required the addition of touch and close fastening over the left shoulder to allow for the left shoulder to rise to accommodate the right compression, so assisting curve reduction.

*The Use of a Dynamic Elastomeric Fabric Orthotic Intervention in Adolescents and Adults… DOI: http://dx.doi.org/10.5772/intechopen.96391*

### **Figure 11.**

*(a) Before Suit X-ray shows 32.7° thoracic curve convex to the left. (b) The blueprinting which includes 3 point force (yellow) arrows, red full and dotted shoulder compression and de-rotation effect, and the upward shoulder movement (orange arrow) The two downward pink arrows indicate the counter force on the legs and hips that applies the pressure to the shoulders. (c) The scoliosis suit shows the lateral translation curve "V" shaped pane. The shoulder de-rotation and compression panel on the right of the photograph coupled with the touch and close over the shoulder pressure relief fastening. (d) In-suit X-ray shows 15° curve a reduction of 17.7 degrees, which was held for 2½ years. Note dual zips of suit.*

The patient was provided with a 18 mm heel raise to accommodate the pelvic tilt, but this had no effect on the scoliosis. Throughout the next 2.5 years the patient was provided with three suits to accommodate natural growth. The suit was worn 23 hours a day. The in-suit x-rays (**Figure 11d**) showed that the curve was held at 15° Cobb angle, a reduction of 45%. The tumour re-occurred after 2 ½ year and was again removed, with additional surgery to staple the vertebrae above and below the tumor site to prevent a re-occurrence of the scoliosis.

Matthews & Bridges [36] reported a single case study of a 5-year-old child, presenting with myotonic dystrophy (a genetic disorder affecting muscle function), coupled with pectus carinatum (a chest wall deformity in which the breastbone pushes outward instead of being flat), and a left 70° Cobb angle thoracic curve, apex T8 with a vertebral rib angle difference greater than 20° and therefore very likely to worsen (**Figure 13a** and **b**). The x-ray provided the blueprint for a vest top scoliosis suit to be prescribed and supplied. One year later, routine x-rays showed that the curve had reduced to 35°, a 50% Cobb angle reduction in suit and the vertebral rib angle difference had reduced to under 20° and meaning that the curve was unlikely to get worse (**Figure 12**).

Although it is not normal to expect a 50% reduction in Cobb angle, this result proved that scoliosis suits could treat higher angle curves, than rigid braces successfully. This result also highlighted that the shoulder on the convex curve side should extend down over the upper arm to prevent the shoulder protraction (shoulder moving anteriorly) around the edge of the vest segment (**Figure 13c**).

In 2016, Matthews et al. [23] reported on an audit of 180 children and adolescents with, or at risk of developing scoliosis from five centres in the south of England, UK. The study reported on 121 subjects who had been supplied with DEFO suits and scoliosis suits within three centres. The centres used local protocols to use DEFOs to treat children identified as GMFCS Level 4/5, who were primarily wheelchair ambulators, starting to develop a small lateral body shift and were at risk of developing scoliosis prophylactically. Fifteen years of scoliosis suit management experience by the clinical team had indicated that patients with GMFCS level 4/5 had reduced surgical interventions due to early suit provision. The audit also reported the noncompliance in the use of rigid braces in this sample. It is understood that rigid brace use in neurological onset scoliosis is difficult to wear due to the rigidity and lack of movement within the orthosis. There is no convincing evidence in the recent literature to suggest that rigid spinal orthotic management in neurological onset scoliosis is effective [37], which was also confirmed in the audit data.

Over the years, the children who had DEFO scoliosis suits were followed through their peak growth rates as adolescents. As the scoliosis development was identified early, the clinical team were able to observe and develop an

### **Figure 12.**

*This diagram shows the vertebral rib angle difference. If over 20° the curve is considered to get worse. https:// boneandspine.com/rib-vertebral-angle-in-scoliosis/#how-to-measure-the-rib-vertebral-angle.*

*The Use of a Dynamic Elastomeric Fabric Orthotic Intervention in Adolescents and Adults… DOI: http://dx.doi.org/10.5772/intechopen.96391*

### **Figure 13.**

*(a) Pre suit X-ray. (b) X-ray in scoliosis suit. (c) The child in suit. "V" shaped translation panel is clearly seen originating from right hip to left waist and back to under right axilla. Note: shoulder protracting around the vest segment.*

understanding of which scoliosis curves were at high risk of progressing. The clinical team understood that during growth, peak growth occurs during early adolescence and continued throughout adolescence. Scoliosis- specific DEFO suits were shown to maintain the corrections in Gross Motor Functional Classification Scale (GMFCS) level 4 and slow the curve progression for those patients in GMFCS level 5 throughout adolescence.

It is recognised that most of the research in this field is in paediatrics, however there are now papers in preparation on an adult male using the DEFO scoliosis suit management. The N = 1 single case study (currently in press) reports on the changes to an adult male with ataxia telangiectasia (a rare inherited disorder that affects the nervous and immune system resulting in difficulty with coordinating movement from early childhood) with poor head control and issues with poor control of posture and extrapyramidal movements. The design included baseline pre-intervention gait laboratory data collection of the patient sitting, carrying out a routine hand function, the quality-of-life task of taking a drink to his mouth and reach to touch function. This was repeated in a postural scoliosis design suit, which included bilateral enclosed shoulders and standard strength paneling, and then again in a stronger, higher specification paneled postural scoliosis suit with interesting outcomes [38].

Ehlers Danlos Syndrome (EDS), an inherited condition that affects connective tissue is characterised by hyper-elasticity of the joints and fragility of the skin [39]. There is anecdotal evidence of patients with scoliosis, where pain is a major problem due to the connective tissue lack of rigidity that allows joints to dislocate. Serious pain is often experienced in women with EDS caused scoliosis, often from the age of 30 years, preventing or making their activities of daily living difficult. They also have increased reliance on strong pain medication. The use of DEFO scoliosis suits for this client group has produced improved posture with similar results to the adult use of SpineCor brace. The effect on the scoliosis angle was minimal, but effective, because pain was reduced due to the improved positioning and stabilisation of joints [25]. Therefore, the improved quality of life often leads to reduced pain relief medication.
