The Fundamental Principles of Seating and Positioning in Children and Young

People with Physical Disabilities

Laura Neville BSc (Hons) Occupational Therapy Student

University of Ulster

Supervised by:

Mrs Jackie Quigg (School of Health Sciences UU)

Dr Alison Porter Armstrong (Health and Rehabilitation Sciences Research Institute UU)

Commissioned by James Leckey Design Limited

Summer 2005

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Project Background James Leckey Design Limited (Northern Ireland) is a company specialising in equipment design, manufacture and provision for children with physical disabilities. As part of a 2 ½ year project, involving the University of Ulster (with Mrs Jackie Quigg and Dr Alison Porter Armstrong) to research the clinical effectiveness of one of their product ranges, the company commissioned three BSc (Hons) Occupational Therapy students (Laura Neville, Linda McNamara and Glenda Alexander) to conduct critical reviews of the literature in three designated areas: 1) Fundamental Principles of Seating and Positioning in Children and Young People with Physical Disabilities. (Laura Neville) 2) Postural Management: Components of Specialised Seating Equipment. (Linda McNamara) 3) Early Intervention and the Effects of Adaptive Seating on Function. (Glenda Alexander) The critical reviews undertaken were completed between June and August 2005 and conducted as part of a steering group comprising of: Mr James Leckey (James Leckey Design Limited); Mr Noel McQuaid (Technical Director, James Leckey Design Limited), Mrs Clare Wright (Research Occupational Therapist, James Leckey Design Limited), Mrs Jackie Quigg (UU) and Dr Alison Porter Armstrong (UU), with formal supervision provided on a weekly basis. This is the first of three critical reviews commissioned by James Leckey Design reviewing and critically appraising the literature regarding the fundamental principles of seating and positioning in children and young people with physical disabilities. Acknowledgements

I would like to thank Mrs Jackie Quigg and Dr Alison Porter Armstrong for their continued support, direction and guidance in regarding completion of this review. Thanks also to James, Noel and Clare for providing the opportunity to engage in this project and thank you to Linda and Glenda for your constant support and encouragement and making the completion of this review possible.

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Index of Contents Page Introduction 3 Methods 4 The relationship between posture, movement, stability and function 4 Normal posture 6 The action of sitting 6 Neutral sitting posture 7 The 90-90-90 position – how functional? 8 Functional Sitting Position 9 Factors affecting positioning 13 Primitive reflex activity 13 Structural asymmetries 15 Abnormal muscle tone 18 Childhood conditions 20 Cerebral Palsy 20 Congenital hip deformity/Developmental Dysplasia 23 Rett Syndrome 24 Duchenne Muscular Dystrophy 25 Spina Bifida 26 Conclusion 27 References 28 Appendices 35

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Principles of Seating and Positioning

Introduction

The prescription of appropriate seating equipment for children and young people with physical disabilities is important, in order to provide an optimal seated position from which they may engage in functional activities. Research has evidenced the benefits of adaptive seating to include improved postural alignment (Miedaner 1990; Myhr and von Wendt 1991), development of motor skills (Green and Nelham 1991), helping the prevention of fixed deformity (Pountney et al 2002) and facilitation of upper extremity function (Myhr and von Wendt 1991; Myhr et al 1995, van der Heide 2003). It is imperative that health professionals prescribing and engineers designing seating equipment are well informed regarding the fundamental seating principles that dictate the sitting postures of children and young people and the impact they have on long term health and function.

Traditional emphasis regarding positioning is placed on achieving an upright

symmetrical posture utilising the 90-90-90 flexion at the hips, knees and ankles position (Green and Nelham 1991; Ham et al 1998; Erhardt and Merril 1998, Hong 2002). Although an important posture to achieve, this upright position is suggested as non-functional (Engström 2002) and difficult to maintain over time (Howe and Oldham 2001), resulting in adoption of compensatory postures which may lead to long term deformity and further deterioration when appropriate external support is unavailable. Seating solutions may require reaching a balance between an upright anatomical symmetrical posture and ability to function (Ham et al 1998; Pope 2002).

The focus of this research was to review and critically appraise the literature

regarding the fundamental principles of seating and positioning used with children and young people with physical disabilities. The report uses evidence from published studies, and expert opinion to identify seating and positioning principles used with children and young people with disabilities, the benefits of optimal positioning and problems which may incur as a result of incorrect positioning. An overview of normal and abnormal postures is discussed from biomechanical and neurophysiological viewpoints. Common childhood neuromotor and neuromuscular conditions are considered with respect to clinical manifestations and resultant positioning problems which health professionals and engineers must consider when prescribing / designing seating interventions to promote long term health and functional independence.

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Methods

A literature search was conducted using the following electronic data bases; AMED (Allied and Complimentary Medicine), ASSIA (Applied Social Sciences Index and Abstracts), BIDS, British Nursing Index, CINHAL (Cumulative Index of Nursing and Allied Health Literature), Embase, ISI Web of Science, MEDLINE, OTDbase, Pubmed, Proquest, Psychinfo and Zetoc. Criteria for considering relevant literature for review required all articles to be in the English language, and published between January 1990 up to and including May 2005. On occasions, earlier dated key papers were included for relevance. Tables 1-7 (appendix 1) identify the key words utilised in the search strategy with respect to the data bases searched. Electronic database searching was supplemented with hand searches, citation searches of reference lists, conference proceedings and retrieving relevant literature from published textbooks. The McMaster critical review forms (Law et al 2002) were used as a guideline for critiquing relevant studies attained.

The relationship between posture, movement, stability and function

Posture may be defined as, “ the position of one or many body segments in relation to one another and their orientation in space” (Ham et al, p26). Body ‘segments’ are referred to as the head, thorax, pelvis, lower limbs and feet, whilst the body ‘linkages’ are considered as the spinal joints, hips, knees, ankle and shoulder joints (Pope 2002). When considering posture, one should not consider it as static, but as an active and dynamic process which underpins movement and function (Hong 2005). Normally, our postures continuously shift and change position to facilitate movement to engage in functional activities. Pope (2002) identifies that posture is a prerequisite for movement. Howe and Oldham (2001) also highlight that posture and movement are inextricably linked, referring to posture as a temporary arrested movement, which is in a constant state of change. From a neurodevelopmental perspective Nichols (2001) suggests that the development of postural control and acquisition of motor milestones are intrinsically linked. Ham et al (1998) support this assumption highlighting that there is constant neuromotor activity being used to maintain body balance and posture. Engström (2002) further suggests that biological and physiological influences affect body position and posture. This is also in addition to the somatosensory, vestibular and musculoskeletal systems (Nichols 2001).

For functional movement to occur in sitting, literature suggests that stability of proximal body parts (pelvis, spine and shoulders) is a prerequisite for distal control (Green and Nelham 1991; Herman and Lange 1999). For example, pelvic stability is required for the spine so that the neck is free to move; shoulder girdle stability is required to stabilise the arm for fine motor and hand control. Washington et al (2002) however suggests that there is limited published research to support this hypothesis

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suggesting that the relationship between proximal stability and distal control is not necessarily one of cause and effect. This is supported by Case-Smith et al (1989) who identified weak correlations between proximal control and hand function in typically developing children as assessed by the Posture and Fine Motor Assessment of Infants (Case-Smith 1987). However evidence suggests that clinicians and therapeutic seating do utilise the principle of achieving pelvic stabilisation to maximise distal control for function in children with neuromotor dysfunction. This is illustrated in the literature by the use of anterior pelvic stabilization devices in seating interventions (Reid and Rigby 1996; Reid 1996, Rigby et al 2001) and by promoting anterior pelvic tilt via the use of the functional sitting position in children with cerebral palsy (Myhr and von Wendt 1990, 1991; Myhr et al 1995. Children with neuromotor and neuromuscular dysfunction will require external support from seating systems to accommodate for compromised postural control and postural deficits. Based on clinical and empirical evidence, it is accepted that the general goals of seating and positioning include the following, and will be considered in the context of this review: 1) Normalise tone or decrease abnormal influence on the body. 2) Maintain skeletal alignment. 3) Prevent, accommodate or correct skeletal deformity. 4) Provide stable base of support to promote function. 5) Promote increased tolerance of desired position 6) Promote comfort and relaxation. 7) Facilitate normal movement patterns or control abnormal movement patterns. 8) Manage pressure or prevent the development of pressure sores. 9) Decrease fatigue 10) Enhance autonomic nervous system function (cardiac, digestive and respiratory function) 11) Facilitate maximum function with minimum pathology. (Jones and Gray 2005).

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Seating and Positioning: Principles and Practice Normal Posture Following a review of the literature, it is difficult to define what constititutes ‘normal’ posture. This is because each person is unique regarding their physiological profile and continually engages in a number of postural variations which may be attributed to fatigue and emotional state (Howe and Oldham 2001). From a biomechanical viewpoint, good posture is dependent on the balance of the skeleton and symmetrical alignment of body segments. Engström (2002) states that those who balance their body in accordance with mechanical rules for human body systems (laws of physics) tend to be more erect. From a neurophysiological and developmental perspective, normal posture is also dependant on the development of normal postural control which is described as the control of the body’s position in space in order to obtain stability and orientation (Brogren et al 1998) and is influenced by the neuromotor, somatosensory, vestibular and musculoskeletal systems (Nichols 2001). Postural control requires achieving normal developmental milestones and includes the development of postural reactions (righting, protective and equilibrium reactions), developmental integration of primitive reflexes (asymmetrical tonic neck reflex, symmetrical tonic neck reflex, tonic labyrinthe reflex), normal muscle tone, normal postural tone and intentional voluntary movements (Wandel 2000).

The action of sitting It is also useful to understand the interface between the standing and seated posture. Turner (2001) suggests the ‘action of sitting’ results in flexion of the thoracic spine, flexion of the lower extremities, and backwards rotation of the pelvis towards the rear of the seat. Pelvic rotation in turn dictates the compensatory curves of the spine, which in turn dictates the position of the remaining body segments (Harms 1990). The pelvis is then placed on the seat against the backrest. The trunk extends, moving towards the backrest. The upper part of the pelvis is in contact with the lower part of the backrest, thus achieving pelvic stability, with the person now sitting in an upright neutral position.

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Neutral sitting posture Sitting skills emerge in a normally developing child approximately between 7

and 9 months and requires the child to maintain postural control of the head, trunk and extremities against the pull of gravitational forces (Wandel 2000). The majority of the literature refers to the 90-90-90 position as the normal upright neutral seated posture and considers the head, trunk and extremity positions in relation to each other. Assuming that one is sitting on a flat, right angled chair in a static or neutral position, the upright symmetrical position is characterised by extension of the trunk, the pelvis in anterior tilt, thighs slightly abducted, parallel and horizontal and the iliac crests aligned and level in the lateral plane (Ham et al 1998). The hips, knees and ankles are placed at ninety degrees of flexion, the feet are in plantar grade or 0° degrees flexion (Green and Nelham 1991) and the head is positioned in midline and maintained in the vertical plane (Erhardt and Merril 1998). The head position in relation to the spine is important as it has a direct effect on posture. Loss of head control can therefore affect body position (Ham et al 1998). When the pelvis is in anterior tilt, the centre of gravity falls anterior to the ischial tuberosities (Ham et al 1998) hence the base of support is provided through the ischial tuberosities and the upper thighs (Howe and Oldham 2001). Depending on the chair design, weight will be transferred through the back rest, and the arm rests to the floor through the feet (Pynt et al 2001). The goal of upright positioning is therefore to promote symmetry and alignment of the body segments and linkages (Ham et al 1998). The sitting position is more relaxing than the standing posture, provides a greater support surface and allows relaxation of the muscles of the lower extremities (Howe and Oldham 2001). However, there is greater potential for pelvic instability in sitting compared to standing due to the hip joint position, the anatomical shape of the ischial tuberosities (Reid and Rigby 1996) and the tendency for the pelvis to rotate backwards (Engström 2002).

In the seated posture, it is desirable that as much contact is made with the chair’s support surface in order to provide maximum stability to facilitate function (Green and Nelham 1991), with the goal of seating being able to achieve a stable base of support surface to allow function (Jones and Gray 2005). However in right angled seating, it is difficult to achieve and often results in a person acquiring a ‘slumped’ posture to compensate for fatigue and discomfort. Combined with the effects of constant activation of the erector spinae muscles, a person will gain relief from excessive muscle activity by sacral sitting, resulting in posterior rotation of the pelvis, accentuation of thoracic kyphosis and cervical lordosis, loss of lumbar lordosis of the spine (Pynt et al 2001) and increased risk of tissue trauma in the sacral area (Han et al 1998). A further goal of seating is to prevent or decrease the occurrence of pressure sore development (Jones and Gray 2005). Conflicting opinion exists regarding the causation of posterior pelvic tilt. Some authors postulate that the tendency for the pelvis to rotate posteriorly may be due to tension in the hamstrings and gluteal muscles which promotes flexion of the lumbar spine hence inducing posterior rotation of the pelvis. (Mayall and Desharnais 1995; Trefler and Taylor 1991; Pynt et al 2001; Effgen 2005). Contrary to opinion Engström (2003) attributes the tendency of thoracic spine collapse (flexion) as influencing the backward rotation of the pelvis and suggests that seating interventions should aim to improve thoracic extension to block

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flexion of the spine. Engström (2002) suggests that backward inclination induces flexion of the thoracic spine whereas a forward inclined seated position promotes thoracic extension. A study conducted by Miedaner (1990) investigating the effects of sitting positions on trunk extension for children with motor impairment supports this assumption. Miedaner concluded that anterior sitting (20° and 30° inclined) compared to level bench or floor sitting increased trunk extension, as measured by the modified Schoeber Measurement of Spinal Extension (AAOS 1975). Using a randomized complete block design a significance level of p=0.001 demonstrated a true mean difference between the different sitting positions, however small sample size limits the generalisability of this finding. The 90-90-90 Position – How Useful?

The 90-90-90 position may be regarded as an ideal seated position from an

ergonomic perspective (Engström 2001). From an anatomical view point the goal is to achieve maximum orthopaedic symmetry between left and right sides of the body via a neutral pelvis to avoid obliquity, rotation and posterior pelvic tilt (Lange 2001). Advantages of this position depicted in the literature include minimisation of orthopaedic deformity (Ham et al 1998) and promotion of proximal stability which in turn promotes distal control (Lange 2001). One of the goals of seating is to promoted relaxation and comfort (Jones and Gray 2005). Kangas (2002) suggests the 90-90-90 position can passively and temporarily reduce tone when considered as a resting position. Two studies identified support for this assumption.

Nwaobi et al (1983) conducted a study using electromyography (EMG) to

investigate the activity of extensor muscles of the lumbar spine in children with cerebral palsy in different seating positions. Seat surface inclinations of 0° and 15° combined with backrest inclinations of 75°, 90°, 105° and 120° provided the testing conditions. Results concluded that electrical activity was least when seat surface elevation was 0° and backrest inclination was 90°. Caution is advised in generalising results as testing positions were based on EMG activity recording 60 seconds of sitting in each position and on a small sample of eleven subjects. A subsequent study conducted by Nwaobi (1986) regarding the effects of body awareness in space on tonic muscle activity of patients with cerebral palsy concluded that muscle activity was lower in the upright position (90-90-90) than the reclined position (30° from vertical plane), with statistically significant results obtained for back extensors and hip adductors (p=0.05). One of the goals of seating is to promote increased tolerance in the desired position (Jones and Gray 2005). Findings from this study suggest that extensor tone is increased in the reclined position.

Literature also identifies that the 90-90-90 position is difficult to maintain over

time (Ham et al 1998; Howe and Oldham 2001) and may impede function (Engström 2002). One study however concluded that the upright position was more functional in comparison with anterior and posterior sitting positions. Nwaobi (1987) investigated seat orientation of upper extremity function in thirteen children with cerebral palsy (spastic and athetoid) and concluded that arm movements were significantly faster

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when positioned in 90-90-90 compared to anterior (15°) and posterior (15° and 30°) orientations. The authors attribute this outcome to either improved control of or decreased abnormal neuromuscular activity in the upright position. The authors also consider loss of horizontal eye contact and greater effort required to counteract gravitational effects in the posterior orientation as impeding upper extremity function (Nwaobi 1987). Other studies identified compare the upright neutral position to alternative sitting positions e.g. inclined/reclined seat base, inclined/reclined back rest, and their relationship to function. Findings from studies examining the effects of seat inclination on upper extremity function are mixed. Some authors (Mhyr and von Wendt 1990, 1991, Myhr et al 1995) concluded that anterior sitting in the ‘functional sitting position’ improved upper extremity function in children with neuromotor dysfunction. Other research studies report no effects on upper extremity function with regard to seat inclination (McClenaghan et al 1992) and no effects regarding anterior tipped seating on respiratory function in children (Reid and Sochaniwskyj 1991). These studies will be reviewed in the next section.

Several authors advocate the idea of bypassing the 90-90-90 position. Kangas

(2002) argues that for functional performance, movement and tone are required, but the 90-90-90 position prevents functional performance as it is essentially a resting position and too restrictive. Minkel (2001) postulates that the goal of adapted seating should extend beyond achieving perfect symmetry, but should focus on providing external support, at the angles needed by an individual to achieve an upright, stable and functional position. It is recognised that ideology based on expert opinion and experience provides important information which may be added to a growing evidence base however caution must be exemplified when using anecdotal evidence to justify practice. Shimizu et al (1994) acknowledges that deviation from the optimal upright position is often required to accommodate for fixed deformities and abnormal postural tone, yet basic positioning principles should be maintained. This is to provide equal distribution of weight, for support, stability and comfort. It may be derived that the 90°/90°/90° position is a useful baseline position to achieve to promote symmetry with further seating adaptations / components being utilised to facilitate function.

Functional Sitting Position A major goal in seating is to provide and stable base of support to promote function and to enhance autonomic nervous system function (Jones and Gray 2005). Pain et al (2003) suggests that alternative sitting positions to 90-90-90 are being proposed and include forward inclination to permit engagement in functional tasks and backwards recline for relaxation. Findings from studies regarding the effects of seat inclination on function and/or postural control are conflicting. Studies identified for review, focus on the cerebral palsy population and/or typically developing children. Several authors suggest that forward inclination promotes improved upper

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extremity function (Myhr and von Wendt 1991, Myhr et al 1995; Reid 1996); trunk extension (Miedaner 1990) and improved postural efficiency (van der Heide 2003, Myhr and von Wendt 1990, 1991). Other research studies report no effects of anterior tipped seating on respiratory function (Nwaobi and Smith 1986, Reid and Sochaniwskyj 1991, Redstone 2004); upper extremity function (McPherson et al 1991; McClenaghan et al 1992) and postural stability (McClenaghan et al 1992). Myhr and von Wendt (1991) conducted a study to find a functional sitting position for children with cerebral palsy. Twenty-three children were photographed and video filmed in six different sitting positions, including the functional sitting position (FSP). The FSP required the pelvis to be anteriorly tilted, with the upper body anterior to the fulcrum at the ischial tuberosities to allow an upright posture. Symmetrical weight bearing through the ischial tuberosities was achieved via fixation of hip belt at 45° angle and use of abduction orthoses. Results suggested that the functional sitting position in comparison to the children’s original sitting position minimised pathological reflexes, improved postural control and improved upper extremity function when children sat in forwards inclination (mean 8°, range 0° to 15°), with a firm back rest supporting the pelvis and supported by a cut out level table to eliminate lateral sway imbalance. Results support previous preliminary findings investigating the FSP and its impact on reduction of spasticity and enhancement of postural control (Myhr and von Wendt 1990), although seat inclination alone showed no identifiable effects, it is subjectively suggested that anterior tilting may stretch the hamstring muscles when the pelvis is rotated forward hence reducing spasticity. This is assuming the feet are secured and fixed and the lower extremities are not permitted to flex under the thighs, however the authors do not provide a specific description regarding the exact positioning of the feet. A five year follow up study investigating ten children with cerebral palsy (Myhr et al 1995) concluded that the FSP contributed to significant improvement (p> 0.05) in head, trunk and foot control and upper extremity function in eight of the ten children as assessed by the Sitting Assessment Scale (Myhr and von Wendt 1991). A study conducted by Reid (1996) utilising a repeated measures experimental cross over design compared the effects of level or flat benched seating versus saddle-benched seating (15° anterior tilt) on postural control and reaching motions of six children with cerebral palsy assessed by the Sitting Assessment for Children with Neuromotor Dysfunction (Reid 1995). Small sample size rendered insufficient power analysis to detect statistically significance differences, however group results and single subject data analysis suggest that saddle sitting may potentially improve postural and reaching movements. An investigation regarding the development of postural adjustments during reaching in twenty-nine typically developing children, and ten adults (van der Heide et al 2003) suggested that forward tilted seating (15° seat surface inclined) was a more efficient position for postural efficiency compared to horizontal (0°) and backwards tilted (15° seat surface reclined) sitting positions. Findings were contrary to the original hypothesis postulated by the authors, who hypothesised that backward sitting would have been the most efficient position as it would passively counterbalance the forward body sway induced by reaching movements. Sitting positions were studied via surface Electromyograms (EMG) and kinematics, therefore quantifying results.

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Miedaner (1990) investigated the effects of sitting positions on trunk extension in fifteen children with motor impairment (cerebral palsy) using the modified Schober Measurement for Spinal Extension. As previously discussed, results suggested that the anterior sitting (20° and 30° inclined) tend to increase trunk extension compared to level bench or floor sitting. Subjective observations suggest that upper extremity function is not compromised despite increased muscle activity required to maintain trunk extension when the seat is tilted forward. Further EMG analysis of spinal muscle activity regarding trunk extension would objectify results. Contrary to these findings other authors conclude that seat inclination has no discernable effects. McClenaghan et al (1992) investigated the effects of seat surface inclination on postural stability and function of the upper extremities of children with cerebral palsy. Twenty children (ten non-impaired; ten mild cerebral palsy) were included in the study, with seat surface inclinations depicted as 0° horizontal, 5° anterior tilt, and 5° posterior tilt, with seat to back inclination maintained at 90°. Previous authors (Myhr and von Wendt 1990, 1991; Miedaner 1990; Myhr et al 1995; Reid 1996; van der Heide 2003) used seat surface inclinations of greater than 5° (range 8°- 30°); however McClenaghan justifies 5° inclination by stating that greater tilting is difficult to tolerate for an extended period with this assumption based on pilot investigations. Previous research has shown that lumbar spine muscle activity increases when the seat is tilted forward (Nwaobi 1987).

McClenaghan et al (1992) concluded that significant between-group differences were observed on most dependent measures (p>0.05) however, suggested that anterior tilt seating in cerebral palsied children may actually disturb postural stability, without improving performance of the upper extremities and suggested posterior tilt as a more efficient position during periods of upper extremity function. Although only applicable to the adult cerebral palsy population, McPherson et al (1990) also concluded that no significant differences could be attributed to seat inclinations regarding upper extremity movement. Hadders et al (1999) when conducting a study to investigate the development of postural adjustments in reaching in infants in children with cerebral palsy also favoured the reclined position (semi-recline at 45°) as opposed to lying in supine, long leg sitting and upright sitting). McClenaghan (1992) also argues that the use of a table in Myhr and von Wendt (1991) study may result in weight bearing on the support surface, which in turn actually impedes the use of the upper extremities for function. This would hinder the carrying out of bilateral activities. Similar findings have been reported regarding the effects of seat inclination on respiratory function. Redstone (2004) investigated the respiratory patterns in upright and semi-reclined seating positions in ten preschool children with cerebral palsy and ten typically developing children. No significant differences could be attributed to seat position alone. Reid and Sochaniwskyj (1991) conducted a study to investigate the effects of anterior tipped seating on respiratory function of normal children and children with cerebral palsy. Twelve subjects (six normal; six cerebral palsy) were compared in level (0°) and anterior tipped (10° seat surface inclined) seating, with respiratory function measured by respitrace transducers. No significant differences in respiratory function could be attributed to seat inclination alone either in the normal or cerebral palsy groups, however it is recognised that a larger sample size may have

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yielded greater power analysis to detect statistical significance. Nwaobi and Smith (1986) in an earlier study investigated the effect of adaptive seating on pulmonary function of eight children with spastic cerebral palsy, and concluded that pulmonary function was more efficient when children were positioned in adaptive seating in the upright (90-90-90) position. Continued disparity in the literature exists regarding the direction of seat inclination for improved function and postural control. Studies reviewed used small sample sizes, lacked power analysis, and used various seat inclination values and different assessment times in seated positions and short periods of data analysis therefore making comparisons difficult. Additionally some studies do not refer to cerebral palsy as a heterogeneous group. Selection bias was also evident in some studies therefore questioning the reliability of the findings. Future research should utilise larger sample sizes and to aid power estimates so that results may be generalisable to the sample population.

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FACTORS AFFECTING POSITIONING Children with neuromotor / neuromuscular dysfunction will have compromised postural control. This section discusses postural problems and how they can affect positioning and possible seating solutions that accommodate or prevent further deviation from normal posture; however consideration regarding the effectiveness of seating components and their functional use are beyond the scope of this report. The problems depicted in the literature include a) retention of primitive reflexes; b) presence of structural asymmetries c) abnormalities of muscle tone. a) Primitive Reflex Activity Ø Asymmetrical Tonic Neck Reflex Ø Symmetrical Tonic Neck Reflex Ø Tonic Labyrinthine Reflex Ø Positive Supporting Reaction Ø Moro Reflex

Asymmetrical Tonic Neck Reflex (ATNR) According to Ham et al (1998) ATNR is elicited when the head is turned to one side. The reflex is characterised by increased extensor tone on the side to which the head is turned and increased flexor tone on the opposite side. This reflex is usually integrated between 4-6 months (Lowman 2000). According to Hong (2005) the continued presence of ATNR after 4-5 months is considered abnormal and interferes with rolling, bilateral integration, reaching and grasping activities. Due to the tendency of children to use one hand, continued repetitive movements may lead to the development of scoliosis (Ham et al 1998). Extensor postures (Levitt 2004), soft tissue balance and asymmetries in muscle tone may also lead to secondary deformities including subluxed hips and, contractures mainly affecting the hip flexors and adductors (Hong 2005). Symmetrical Tonic Neck Reflex (STNR) STNR is elicited when the head is flexed or extended (Lowman 2000). According to Ham et al (1998) when the head is flexed, the upper extremities flex and the lower extremities extend. When the head extends, the upper extremities extend and the lower extremities are pulled into flexion. This reflex is normally integrated

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between 4-6 months. Retention of STNR interferes with reciprocal creeping, walking (Lowman 2000) and upper limb function (Ham et al 1998.) Tonic Labyrinthine Reflex (TLR) TLR is elicited when in supine or being moved into flexion or extension (Lowman 2000). In supine or with the head extended, there is increased extension throughout the body. In prone or when the head is forward of the upright position, there is an increase in flexion throughout the body (Ham et al 1998). This reflex is usually integrated between 4-6 months. Retention of TLR interferes with side turning, rolling, and lying to sitting ability and creeping (Lowman 2000). Positive Supporting Reaction The positive supporting reaction is elicited when infants are supported under the shoulders and held upright with feet flat on the floor (Ham et al 1998) Proprioceptive stimuli via the feet induce extension of the lower extremities and plantar flexion of the feet (Lowman 2000). This response is usually integrated between 1-2 months and disappears when the child learns to weight bear through feet and stand independently (Ham et al 1998). Retention of this response will interfere with walking patterns and may lead to walking on toes (Lowman 2000). In addition, Ham et al (1998) suggest that proprioceptive stimuli applied to the intrinsic muscles of the feet when pushing down on foot rests can also elicit an extensor thrust, which can lead to sacral sitting and the development of extension contractures of the hip. Moro Reflex Ham et al (1998) suggest the Moro reflex is elicited when the head extends backwards, which results in an extension pattern of the body, with the arms extended, abducted and externally rotated followed by a flexion posture. This reflex is normally integrated between 4-6 months and if retained, interferes with head control, sitting equilibrium and protective reactions (Lowman 2000), all of which are needed for postural control. Seating Interventions

Goals of seating include normalising tone or decreasing abnormal influences on the body and to facilitate normal movement or pathological activity (Jones and Gray 2005). Although Wright-Ott and Egilson (2001) suggest that tilt in space may provide a child with hypotonia (low muscle tone) with greater tolerance for upright sitting, other authors suggest that tilt in space and/or reclined sitting may

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increase pathological reflexes (Nwaobi et al 1983; Nwaobi 1986; Myhr and von Wendt 1990, 1991, Mhyr 1994; Engström 2002). As previously discussed, Nwaobi (1986) concluded that children with cerebral palsy have better postural control in the upright rather than the reclined or tilted position. In testing conditions Nwaobi (1986) found that increased extensor tone resulting from TLR was elicited by the position of the head in the reclined position, adding that loss of horizontal eye contact resulted in a greater effort to overcome gravity and consequently increased tone. This study also highlighted that asymmetry of muscle activity in back extensors and prolonged seating in the reclined position may contribute to development of a scoliosis. Ham et al (1998) also suggest that tilting of a seat and reclining the backrest can affect eye contact, as well as upper extremity function and spinal posture. Tilting may therefore reduce functional ability to participate or interact with others and the environment. Similarly Myhr and von Wendt (1990) in their pilot study concluded that the backward tilted position was the most inefficient position to reduce spasticity and enhance postural control. Pathological reflex activity increased considerably in the tilted position. Twenty-two ATNR movements were recorded in the tilted position compared to one ATNR when in the functional sitting position. Myhr and von Wendt (1991) support these findings when investigating the functional sitting position in children with cerebral palsy. Findings suggest that pathological movements were significantly reduced (p=0.001) in the functional sitting position compared to backward tilting. Herman and Lange (1999) suggest that the head should be kept close to mid line to avoid active neck rotation which may elicit an ATNR. Levitt (2004) suggests that therapists prevent extensor patterns and subsequently chair sliding by using tilt in space with hips flexed between 95° and 110°, although adds that it is not a suitable position for those displaying the Moro response, head and trunk thrust in semi lying or increased athetosis. Engström (2002) postulates that a constant tilt in space position may contribute to development of extensor patterns in the cerebral palsy population and suggests that the trunk’s position in space may change the extensor pattern. Engström suggests that an individual contoured seating unit which is forward tipped may be beneficial and highlights that dynamic seating systems may be beneficial as they enable a change of position between upright and rest. b) Structural Asymmetries Ø Spinal deformity (lordosis, scoliosis, kyphosis) Ø Pelvic Tilt Ø Windswept deformity / Hip dislocation

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Spinal deformity: Lordosis Lordosis may be defined as an anteroposterior curve of the lumbar spine in which the concavity is directed posteriorly (Rodgers et al 2001). In normal posture the lumbar spine should be slightly hollow or lordosed, however muscle imbalance may result in excessive lengthening and weakening of the abdominal and gluteal muscles and tightening of the iliopsoas and spinal erector muscles which results in the pelvis being tilted anteriorly and further increasing the curvature of the lumbar spine. This results in a lordotic posture (Howe and Oldham 2001). Lordosis may be secondary to other spinal deformities, anterior pelvic tilt, hip flexion contractures and is also associated with muscular dystrophy. Treatment usually centres on managing underlying problems and includes stretching tight hip flexors, strengthening abdominal muscles and in severe cases bracing may be required (Rodgers et al 2001). Scoliosis Scoliosis is a lateral curvature of the lumbar and/or thoracic spine often accompanied by axial rotation of the vertical bodies (Howe and Oldham 2001). The Scoliosis Research Society defines scoliosis as a lateral curvature exceeding 10° using the Cobb method (Scoliosis Research Society 2002). According to Rodgers et al (2001) curves of less than 20° are mild, curves over 40° result in permanent deformity and curves of 65° and over may result in compromised cardiopulmonary function. The development of scoliosis has been associated with asymmetrical muscle tone (Young et al 1998), retention of primitive reflexes (Ham et al 1998), poor postural tone, hip contractures (Rodgers et al 2001) and compensatory postures resulting from leg length discrepancy or abnormal pelvic tilt (lateral tilt, obliquity and rotation) (Howe and Oldham 2001), and increased interface pressure when sitting (Shoham et al 2004). Scoliosis occurring in combination with pelvic obliquity and hip deformity is usually convex to the side opposite the dislocated hip and pelvic obliquity (Gudjonsdottir and Stemmons Mercer 1997). It is recognised that scoliosis is generally progressive and can contribute to a number of secondary health problems such as positional pain, respiratory compromise, pressure sores and loss of function (Holmes et al 2003). Scoliosis may be described as fixed or structural meaning that there is permanent deformity which cannot be altered by posture, with the vertebral bodies rotating towards the convexity of the curve and the spinal processes towards the concavity of the curve (Howe and Olham 2001). In extreme cases, surgical intervention may be considered. A flexible scoliosis may be passively or actively corrected via non-surgical intervention including spinal jackets and specialised seating (Holmes et al 2003).

The literature suggests that management of scoliosis is achieved via a three point force system to the sides of the body. Ham et al (1998) suggests the use of lateral supports, which involves forces acting from anterior to posterior at the pelvis, anterior to posterior at the shoulders and posterior to anterior at the apex of the kyphotic (flexible) spine. A study conducted by Holmes et al (2003) investigating the effects of special seating on lateral spinal curvature in the non-ambulant spastic cerebral palsy population supports this assumption. Holmes et al (2003) concluded that significant static correction of the scoliotic spine can be achieved by a three point force arrangement of lateral pads to the sides of the body.

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Shoham et al (2004) investigated the influence of seat adjustment and a

thoraco-lumbar-sacral orthosis (TLSO) on the distribution of body seat pressure in fifteen children with scoliosis and pelvic obliquity. Seat adjustments included either the use of either elevation of the lower side of the pelvis or a wedge insertion beneath the raised pelvis. Results concluded that the TLSO significantly reduced the spinal curvature and interface sitting pressure (p<0.05), however seat adjustment had no significant effect on pressure distribution. Kyphosis

Kyphosis is characterised by an increased posterior curvature (‘C-shaped’) of the thoracic spine (Howe and Oldham 2001). The kyphosed posture is apparent during sacral sitting, with the pelvis posteriorly tilted and the thoracic spine in flexion (Ham et al 1998). Ham et al (1998) further add that a sling back seat and back support can accentuate kyphosis. The development of kyphosis may be associated with poor posture, weak erector spinae muscles (required for trunk extension), compensation to hip deformity (Howe and Oldham 2001), and also occurs in children with cerebral palsy who demonstrate extensor spasticity in the lower extremities due to tight hamstrings (Ham et al 1998). Head position is then affected, resulting in forward flexion or head drop. In order to compensate for head dropping, the individual will hyperextend their neck in order to visually interact with the environment, however this will compromise respiratory and swallowing function (Herman and Lange 1999). Engström (2002) suggests additional problems include neck pain, flexion contractures of the trunk muscles, restricted arm movement and increased abdominal pressure.

Several authors (Trefler and Taylor 1991; Ham et al 1998; Engström 2002;

Levitt 2004) suggest tilt in space or opening the seat to backrest angle to accommodate or support a kyphosis. Engström (2002) suggests the seating unit should be contoured for equal pressure distribution and the backrest is reclined, although does not an angle at which recline should occur. Trefler and Taylor (1991) also suggest the use of a custom contoured back support with a flexible anterior harness, adding that individuals with a 30° or greater scoliosis may require reclined seating to accommodate or correct the curve. Tilt in space may also be considered as it may reduce the effects of gravity acting on the upper body, with the weight of the body being taken through the backrest (Ham et al 1998, Pope 2002). However, the advantages of tilting must be considered against the loss of function. Pelvic Tilt Posterior pelvic tilt is a major problem in seating as it compromises pelvic stability (Engström 2002). Problems associated with posterior pelvic tilt include flattening of lumbar curve, accentuation of thoracic kyphosis (Pynt et al 2001) sacral sitting, and increased risk of pressure sore development at sacral / coccygeal area (Ham et al 1998). Causation of posterior of pelvic tilt is attributed to the tension in

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the hamstrings (Mayall and Desharnais 1995; Trefler and Taylor 1991; Ham et al 1998; Pynt et al 2001) or by flexion of the thoracic spine (Engström 2002). Effgen (2005) suggests when a child has tight hamstring muscles, footrests must angle under the seat to accommodate for tightness and allow the pelvis to remain stabilised. Seating solutions utilize anterior pelvic stabilisation devices to maintain the pelvis in anterior tilt to prevent backward rotation (Reid and Rigby 1996). Approaches used include ramped cushions (15° inclined) in conjunction with a pelvic belt, sacral pad, knee blocks and foot support (Green and Nelham 1991). Straddle seating (Reid 1996), firm back rests and use of an anterior superior iliac spine padded bar acting on the ischial tuberosities (Ham et al 1998), and anterior tipped seating combined with use of hip belt and abduction orthosis (Myhr and von Wendt 1990, 1991, Mhyr et al 1995) are other methods used to achieve pelvic stabilisation. Windswept Deformity Windswept hip deformity is an abduction contracture of one hip resulting in an adduction contracture of the contralateral hip and may be associated with pelvic obliquity and secondary scoliosis (Young et al 1998). Pelvic obliquity results in pelvic rotation in the transverse plane and pelvic tilt in the saggital plane (Gujonsdottir and Mercer 1997). Aetiology of windswept deformity is unknown; however contributing factors include acetabular dysplasia, femoral anteversion, spasticity, retention of ATNR (Reese et al 1990), muscle imbalance and hip contractures (Young et al 1998). Seating interventions may utilise a knee block system to help correct windswept hips (Levitt 2004). According to Ham et al (1998), the knee blocks should be adjusted so that a force is applied anterior to posterior, via the abducted femur, to the pelvis on the side that is rotated anteriorly. This is in addition to the use of lateral supports and a sacral pad to help de-rotate the pelvis. Trefler and Taylor (1991) suggest positioning in abduction may discourage the tendencies of adduction of both hips via the use of a pommel. Structural deformity of the hip joint is a major problem in seating. Developmental Dysplasia or Congenital Hip Dislocation will be explored in greater depth in the childhood diseases section of the report. c) Abnormal Muscle Tone Normal muscle tone refers to the ability of muscles to maintain the correct amount of tension and elasticity during movements (Wandal 2000) and may be defined as resistance to passive elongation or stretch (Harris 1991). Abnormalities of muscle include hypotonicity, hypertonicity or fluctuating tone (Hong 2002) and have been associated with deficits in postural control (Nichols 2001). Hypotonia is

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characterised by decreased muscle tone and results in muscles appearing lax and floppy (Kohlmeyer 1998) with functional movement and muscle endurance compromised (Reed 2001). Decreased muscle tone can contribute to the development of kyphosis or lordosis with increased hip flexion, lower limb contractures (Ham et al 1998), joint immobility, instability and subluxation due to large range of movement (Hong 2002), weakness, hyperextended knees and valgus or flat feet (Levitt 2004). Hypertonicity or spasticity is characterised by increased tension or contraction in the muscles (Ham et al 1998). Increased muscle tone may also contribute to scoliosis, muscle contractures, extensor/flexion synergies (Ham et al 1998), clonus, hyperreflexia (exaggerated stretch reflex) patterns (Reed 2001) and persistent primitive reflexes (Erhardt and Merill 1998). Hong (2002) highlights that hypotonicity and hypertoncity often present simultaneously. This is illustrated in the cerebral palsy population whereby the child may exhibit a hypotonus trunk, rendering trunk extension difficult, yet present with hypertonicity of the extremities (Westcott and Goulet 2005). Research has highlighted that spasticity is decreased when the hips are flexed (Nwaobi et al 1983). Nwaobi et al (1983) also found that in addition to the influence of hip flexion, orientation of the body contributed to controlling extensor muscle tone. As previously discussed, this study concluded that spasticity in the lumbar area was lower in the upright position (90°/90°/90°), compared to a backward tilted position. Nwaobi (1986) also concluded that tonic muscle activity of the back extensor and hip adductor muscles were significantly lower (p=0.05) in the upright position compared to recline. Myhr and von Wendt (1990) suggest that stretching spastic hamstring muscles can only be achieved by rotating the pelvis anteriorly, with a straight back. Research has evidenced enhanced postural control via the use of the functional sitting position which puts the pelvis into anterior tilt (Myhr and von Wendt 1990, 1991; Myhr et al 1995, van der Heide 2003).

Based on expert opinion, Herman and Lange (1999) suggest that knee flexion past 90° and ankles in dorsiflexion with slight eversion may reduce extensor spasticity. Empirical evidence suggests that dynamic seating components may also accommodate and reduce tone and enhance function (Cooper et al 2001). This evidence suggests that accommodating abnormal movement and gently returning the limb to normal alignment is more beneficial than blocking the movement. Some authors suggest that tilt in space may be required if the child has hypotonus in order to counteract the effects of gravity (Wright-Ott and Egilson 2001; Ham et al 1998; Pope 2002) yet highlight that advantages of tilt in space must be considered against loss of function.

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CHILDHOOD CONDITIONS Ø Cerebral Palsy Ø Congenital Hip Deformity / Developmental Dysplasia Ø Rett Syndrome Ø Duchenne Muscular Dystrophy Ø Spina Bifida

Cerebral Palsy According to Rodgers et al (2001), Cerebral palsy may defined as a non-progressive abnormality of the developing brain that results in neurological, motor and postural deficits in the developing child. Perceptual, cognitive, sensory and psychosocial dysfunction may also co-exist with this disorder (Ham et al 1998). Classification of cerebral palsy may be according to topographical distribution (monoplegia; diplegia; hemiplegia; paraplegia; tetraplgia or quadriplegia), quality of tone (hypotonia or spasticity; hypertonia; athetosis; ataxia), degree of involvement (mild; moderate or severe) and locality of the brain lesion (Westcott and Goulet 2005). It is reported that the incidence rate of cerebral palsy is approximately 2:1000 live births (ref) and may be attributable to pre-natal, perinatal and post-natal factors (Erdhardt and Merril 1998). Reed (2001) describes four main groups of cerebral palsy syndromes: Spastic This type displays increased muscle tone resulting from an upper motor neuron lesion, ranges from mild to severe and is categorised according to the part of the body affected. Erdhardt and Merril (1998) state that spasticity is also accompanied with persistent primitive reflexes, clonus and hyperreflexia and results in difficulty with gross and fine motor control. Athetoid / Dyskinetic This results from basal ganglia dysfunction and is characterised by slow, jerky, writhing involuntary movements which may affect the extremities (athetosis) or proximal parts of the trunk and limbs (dyskinesis). Athetosis or fluctuating tone results in tone rapidly shifting from normal or hypertonic to hypotonic or low tone (Wandall 2000). It is suggested that athetoid movements are exasberated by emotional disturbance (Ham et al 1998; Reid 2001; Westcott and Goulet 2005) and decreased by prone lying, fatigue or increased concentration (Ham et al 1998). Ataxic This results from cerebellar dysfunction and is characterised by weakness, incoordination, intention tremor, unsteady wide based gait, difficulty coordinating fine motor skills (Reed 2001) and difficulty maintaining stable alignment of the head, trunk, shoulders and pelvis Wandall (2000). According to Ham et al (1998) a child with ataxic cerebral palsy is at greater risk of developing scoliosis than those with spastic diplegia or hemiplegic cerebral palsy.

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Clinical Manifestations In addition to motor impairment, a multitude of clinical problems co-exist with cerebral palsy and include cognitive and learning disabilities (Ham et al 1998); sensory deficits including hyperresponsivity or hyporesponsivity (Erhardt and Merril 1998) proprioceptive, visual and vestibular dysfunction (Westcott and Goulet 2005); epilepsy (Hare et al 1998); hydrocephalus (McDonald et al 2004); behavioural disturbances (Ham et al 1998); oral motor dysfunction due to retention of primitive reflex activity affecting eating and swallowing ability (Erdhardt and Merril 1998); gastro-oesophageal reflux and speech and language difficulties (McDonald et al 2004). This report focuses on the musculoskeletal impairments of body segments associated with cerebral palsy. SPINE

Literature suggests that children with cerebral palsy are likely to develop spinal deformity (scoliosis, thoracic kyphosis and lordosis) with the highest incidence occurring in individuals with spastic quadriplegia (Gudjonsdottir and Stemmons Mercer 1997). Research has evidenced that there is a correlation between tight hamstrings and hypolordosis in children with cerebral palsy (McCarthy and Betz 2000). Additional contributing factors to spinal deformity include decreased stability and asymmetrical posture (Westcott and Goulet 2005), primitive reflex activity (Ham et al 1998), atypical muscle imbalance, tone and weight bearing (Gudjonsdottir and Stemmons Mercer 1997), leg length discrepancy and pelvic obliquity (Howe and Oldham 2001). Consequences of spinal deformity include decreased range of movement, positional pain, and functional limitations. PELVIS According to Lowes and Orlin (2005) pelvic abnormalities in cerebral palsy include obliquity, posterior and anterior rotation. The relationship between tight hamstrings and hypolordosis may also contribute to the posterior rotation of the pelvis. HIP Hip displacement (dislocation or subluxation) is a major disability in cerebral palsy and can cause difficulties in sitting, positioning (Hankinson and Morton 2002), ambulation and perineal hygiene (Scrutton et al 2001). According to McDonald et al 2004) hip displacement is measured by the migration of the head of femur away from the acetabulum, with hip subluxation exceeding 33% migration and hip dislocation exceeding 80% migration. Aetiology is unknown however; contributing factors include persistence of ATNR, acetabular dysplasia, hypertonicity, hip contractures, decreased ambulation and muscle imbalance. Research evidence suggests that children with tonal asymmetry and severe spasticity appear to be at increased risk of dislocation, with a windswept deformity on the opposite side (Young et al 1998).

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Pountney et al (2001) support this assumption by stating that imbalance in muscle strength and length around the hip leads to dysplasia and subsequent hip subluxation. Gudjonsdottir and Stemmons (1997) suggest that an important predictor in hip stability is the age at which a child is able to pull to stand. Cornell (1995) reported that less than 2% of children who are able to pull to stand before the age of three years have hip subluxation or dislocation. Hip dislocation, pelvic obliquity and scoliosis are related problems in cerebral palsy (Gudjonsdottir and Stemmons Mercer 1997), with Letts et al (1984) reporting that dislocation occurs first, followed by obliquity, then scoliosis. Research has evidenced that postural management interventions have an important role in preventing dysplasia of the hip in children with cerebral palsy (Pountney et al 2001). A retrospective study of 59 children with bilateral cerebral palsy concluded that children using all Chailey Adjustable Postural Support Systems (CAPS) maintained significantly more hip integrity (p<0.05) compared with other groups. FOOT / ANKLE Lowes and Orlin (2005) suggest that impairments of the foot and ankle include reduced dorsiflexion resulting from shortened gastrocnemius, plantar flexion during weight bearing resulting from hypertonicity and ankle instability and the acquisition of a flat foot position due to breakdown of the arch of the foot resulting from decreased weight bearing ability. Seating and Postural Implications

Children with cerebral palsy will have difficulty with stationary postures, transitionary movements and functional mobility (Westcott and Goulet 2005). Literature suggests that appropriate seating should aim to normalise tone, inhibit reflex activity, prevent deformity, promote optimal function, maintain postural alignment, maintain tissue integrity and maximise stability (Mhyr and von Wendt 1990; Healey et al 1997; Ham et al 1998) with McDonald et al (2004) suggesting that the provision of adaptive equipment to children with cerebral palsy should be individualised based on functional and contextual factors. Disparity in the literature exists regarding the optimal sitting position for a child with cerebral palsy. Some authors advocate the upright posture (Nwaobi et al 1983; Nwaobi 1986, 1987; Green and Nelham 1991). Others are in favour of straddled (Reid 1996) and forward inclined seating (Myhr and von Wendt 1990, 1991; Miedaner 1990; Myhr et al 1995; van der Heide 2003) and there are those that advocate a reclined posture (McClenaghan et al 1992; Hadders et al 1999) to enhance postural control. It is recognised that some of these studies do not refer to cerebral palsy as a heterogeneous group. Please refer to previous sections reviewing these studies.

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Congenital Hip Deformity / Developmental Dysplasia Developmental dysplasia is a condition of pathological hip instability characterised by dislocation or subluxation of the femoral head from the acetabulum (Lowes and Orlin 2005) with incidence being reported at 2:1000 live births (Cox and Kernohan 1998). Rodgers et al (1998) attributes the cause of developmental dysplasia to both environmental (birth complications) and genetic factors. Ham et al (1998) support this assumption stating that developmental dysplasia may be due to hormonal joint laxity, genetically determined joint laxity and delivery in the breech position. Limited hip abduction and asymmetry are manifestations of this condition, with typical neonates displaying 75° and 90° abduction in each hip (Lowes and Orlin 2005). This condition is also characterised by poor hip socket development, poor weight bearing surface and leg length discrepancy if the femur is subluxed from the acetabulum (Lowes and Orlin 2005). Early diagnosis is imperative as this condition is treatable in the early stage, however long term permanent damage will incur if left untreated of if late diagnosis occurs (Cox and Kernohan 1998, Rodgers et al 1998, Lowes and Orlin 2005). Treatment usually comprises of orthopaedic surgery and splinting (Cox 1995). Positioning problems include reduced abduction and flexion at the hip joint, leg length discrepancy, (Lowes and Orin 2005) and if not corrected early, delayed walking and abnormal gait patterns will be evident (Ham et al 1998). Two studies identified discuss the seating and mobility issues encountered by children with developmental dysplasia. Cox (1995) used a survey method with parents of 11 children who either had undergone or were undergoing treatment for late diagnosed developmental dysplasia. Results highlighted that there was insufficient equipment able to accommodate children in plaster and splints and there was a need to develop seating products, with emphasis placed on mobility. Small sample size and a response rate of 48% limit the generalisability of these findings. Cox et al (1998) further researched seating and mobility in a subsequent study, again utilising a survey method. To identify problems, a survey of 113 affected families in England and Northern Ireland was conducted. Results identified problems in areas of mobility, which creates emotional and social difficulties in family routines. Regarding mobility, it was found that due to the child’s loss of mobility, parents resorted to lifting and carrying the child more frequently, which became problematic as the child increased in age and size. Seating equipment in the home often had to be adapted and improvised to accommodate the size of the splint, therefore compromising safety. Lack of mobility and seating problems were found to restrict the child’s movement and restricted parental activity. The authors (Cox et al 1998) suggest problems could be improved in this population by provision of special devices that would allow mobility in the car, in a pushchair and provide seating in the home environment. Caution must be applied when generalising results as finding are based on a 38% response rate.

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Rett Syndrome Rett Syndrome is a rare neurodevelopmental disorder which predominantly affects females (Cass et al 2003). It is characterised by progressive loss of intellectual functioning, loss of fine and gross motor skills, loss of purposeful hand movements and development of stereotypical hand movements such as hand wringing, washing and clapping (Parker 2000; Reed 2001), difficulty or inability ambulating (Parker 2000) and marked changes in emotional development and behaviour (Ham et al 1998). Research has evidenced the prevalence of fractures as 20.9% amongst this population (McDonald et al 2002). Rett syndrome is also classified as a pervasive developmental disorder as it also characterised by severe and complex impaired social interaction, communication and behaviour (Rodgers et al 1998) According to Parker (2000) normal development occurs between the first 6-18 months of life, after which regression appears to occur. Clinical manifestations also include muscle atrophy, increased spasticity and seizures (Ham et al 1998), hypotonia, ataxia, and trunk rocking (Effgen 2005). Scoliosis is the primary orthopaedic complication of Rett syndrome with onset associated with stereotypical arm and hand movements, slowing down of righting and equilibrium reactions, age (McClure et al 1998) alterations in muscle tone, spasticity, and muscle incoordination (Harrison and Webb 1990). Research has evidenced that there is a significant relationship between the prevalence of Rett syndrome scoliosis and orthopaedic risk factors. McClure et al (1998) concluded that rett scoliosis may be due orthopaedic asymmetries rather than a neurological form of scoliosis, with age, abnormal upper body positioning, and non-ambulation as significant predictors of scoliosis. Cass et al (2003) also suggest that early asymmetry of the pelvis as well as shoulder protraction and elevation may be a precursor to fixed deformity. Clinical implications therefore would be to promote bilateral symmetrical muscular balance through proper sitting and lying positions (McClure et al 1998). Ham et al (1998) suggest that in the early stages, weight bearing should be encouraged to help minimise and delay the onset of deformity, as well as the use of spinal jackets. Ham et al (1998) also suggest that soft moulded seats with supports at the backrest are recommended.

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Duchenne Muscular Dystrophy Duchenne Muscular Dystrophy (DMD) is a genetic disorder characterised by progressive proximal muscle weakness (Reed 2001). This disorder only affects boys, with few surviving beyond 20-30 years old and mortality as a consequence of cardiopulmonary compromise (Ham et al 1998). In DMD, muscles break down and are replaced with fat and scar tissue, (pseudo hypertrophy) resulting in the muscles appearing bulky, with the calf muscles looking unusually large (Parker 2000). Impairment of muscle is affected proximally to distally ( Lowes and Orlin 2005) with Rodgers at al (1998) suggesting involvement begins in the proximal musculature of the pelvis, proceeding to the shoulder girdle and subsequently to the distal muscle groups. Thompson et al (1998) add that muscle involvement is bilateral and symmetrical. To compensate for muscle weakness, the child may resort to using the upper extremities to assist knee extension by using his hands to ‘walk up’ from floor to standing (Gower’s sign) (Ham et al 1998; Lowes and Orlin 2005). In addition hyperextension or lordosis of the lumbar spine may be apparent as a compensatory posture in order to maintain an upright position and head in midline (Ham et al 1998). Early signs of DMD are evident when the child displays increased plantar flexion by walking on their toes at approximately 1 year old (Parker 2000). Brown (2002) suggests that loss of ambulation may occur between 8-11 years, with Lord et al (1990) reporting wheelchair dependence at 6-15 years. As muscle weakness progresses, flexion contractures (Reed 2001) and scoliosis (Lowes and Orlin 2005) will occur. Research highlights that pain is related to spinal deformity (Lui et al 2003). Brown (2002) highlights that pelvic obliquity will coincide with scoliosis resulting in difficulty in sitting due to unequal weight distribution over the ischial tuberosities. Bakker et al (2000) suggest that correct positioning and stretching may delay the development of contractures and spinal deformity. Parker (2000) also highlights good body alignment is imperative especially with older children for efficient respiratory function as many may be reliant on ventilators to assist breathing. Due to the progression of DMD, the child’s postural needs will continually change; hence seating interventions must be able to accommodate change. Intervention may also be complex as proximal stabilisation is one of the first functions to diminish in DMD, therefore external stabilisation with adaptive seating is required (Clark et al 2004). Intervention involves the use of knee-ankle-foot orthoses (Ham et al 1998; Thompson et al 1998; Rodger et al 1998; Lowes and Orlin 2005) however a recent systematic review suggested that although the use of knee ankle foot othoses can prolong assisted walking and standing, it is uncertain whether they can prolong functional walking (Bakker et al 2000). Ham et al (1998) also highlight that the success of seating systems is variable as it cannot prevent the onset of scoliosis in this population. Initially a light-weight self-propelling chair may be required, progressing onto a powered wheelchair. Clark et al (2004) suggest common clinical practice regarding seating is to level the pelvis, with a firm seat base, align the trunk with lateral supports to facilitate head alignment and support the elbows and forearms on a tray or with arm supports. Ham et al (1998) highlight that reclining or tilting the seating system may reduce the load on the spine, however may be contraindicated by the child adopting an exaggerated lordosed position of the lumbar spine to maintain upright balance and the head in midline. According to Clark et al (2004) limited research exists regarding

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the effects of postural support in seating on health and function of young people with neuromuscular disorders. Clark et al (2004) conducted a prospective two-period randomised crossover study to measure the effects of postural support in seating on posture, respiration and upper limb function for young people with neuromuscular disorders. Nineteen participants aged 6-22 years old, with a diagnosis of DMD (n=15) or Freidreich’s Ataxia (n=4) were assessed in wheelchair seating and in adaptive seating via a standard protocol. Sitting posture, respiration and upper limb function was compared when sitting in a standard wheelchair and in adaptive seating. Results concluded that there were no significant differences in respiratory function and no overall improvement in upper limb function when compared in the two seating systems, however suggested that adaptive seating can improve the posture of this client group by changing body alignment of young people in the chair. Small sample size and difficulty with accurate postural measurements in the clinical setting limit the generalisability of these findings. The proposed protocol used in the study has not yet been tested for reliability and validity. Spina Bifida Spina bifida, is described as a congenital defect of the vertebral arches in the spinal column (Rodgers et al 1998) whereby the neural tube fails to unite therefore exposing a gap over which the skin is defective (Ham et al 1998). Parker (2000) highlights three spina bifida classifications and includes spina bifida occulta (minor defect not obvious at the skin surface), meningocele (protruding sac containing meninges) and myelomeningocele (protruding sac containing meninges and spinal cord). It is suggested that the cause of spina bifida results from genetic and environmental factors (Reed 2001; Rodgers et al 1998). Numerous clinical manifestations are apparent with spina bifida and include the following: fine motor and hand skill delay (Reed 2001), hydrocephalus (Pountney and McCarthy 1998), impaired or loss of sensation, paralysis, vasomotor dysfunction (Westcott and Goulet 2005), perceptual dysfunction (visual, auditory, propriceptive, tactile , kinaesthetic and hypo- or hyperresponsitivity), learning disability (Reed 2001), pressure sores (Vaisbuch 2000) and psychosocial problems (Pountney and McCarthy 1998). Evidence also suggests that children with spina bifida frequently report clinically significant yet under recognised and untreated pain (Clancy et al 2005). Neurological dysfunction will also contribute to the onset of orthopaedic problems posing problems for seating and positioning, with the level of the lesion determining functional ability. Spinal deformities associated with spina bifida include scoliosis, kyphosis or kyphoscoliosis (Reed 2001), with deformity being present at birth or occurring as the child develops (Pountney and McCarthy 1998). Retention of primitive reflexes, abnormal muscle tone, limited range of movement in the extremities, poor postural control of trunk, poor coordination and presence of hip

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dislocation / subluxation (Reed 2001) and flexion contractures of knees and ankle (Pountney and McCarthy 1998) are factors which must be considered regarding seating intervention. Ham et al (1998) suggest that seating objectives with this population are to provide a stable base of support, maintain alignment of the spine, relieve discomfort over pressure areas, encourage cardiopulmonary function and improve independence.

One study identified investigated the effect on interface pressure distribution in a group of children with complete paraplegia due to myelomeningocele and a group of aged matched controls in different sitting positions (Vaisbuch et al 2000). This study concluded that the lean forward position (hips flexed to 45°) produced the largest reduction in interface pressure, however the authors acknowledge the children felt apprehensive in this posture. The tilt position also reduced interface pressure, with the authors suggesting that tilting is used to relieve pressure during periods of non functional activity. No other studies were identified regarding seating principles with this population.

Conclusion Following a review of the literature, it is concluded that positioning principles are based on empirical and evidence and expert opinion regarding children and young people with neuromotor and neuromuscular disabilities. The majority of research conducted reflects the impact of seating and positioning with the cerebral palsy population. Continued disparity in the literature, small sample sizes and short periods of data collection limit the generalisability of the findings, although are important in terms of clinical significance. Limited research exists regarding children with neuromuscular conditions. It is recognised that appropriate positioning in children with physical disabilities is important to facilitate engagement in functional activity and enable participation with the environment (Jones and Gray 2005). Research has evidenced that proper positioning can improve upper extremity function (Mhyr and von Wendt 1991, Mhyr et al 1995), postural alignment (Washington et al 2002), and prevent the development of deformity (Pountney et al 2002). Children and young people who lack postural control and are unable to maintain appropriate postures therefore will require external support from seating systems. The goal of intervention hence is to provide adaptive seating to create a functional seated position to maintain health and function as part of a postural management approach.

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References Bakker JPJ, de Groot IJM, Beckerman H, de Jong BA & Lankhorst GJ (2000) The effects of knee-ankle-foot orthoses in the treatment of duchenne muscular dystrophy: review of the literature. Clinical Rehabilitation, 14, 343-359. Brogren E, Hadders-Algra M & Foressberg H (1998) Postural control in sitting children with cerebral palsy. Neuroscience and Behavioural Reviews, 22, 591-596. Brown G (2002) Muscular dystrophy. In A Turner, M Foster & E Johnson E (Ed). Occupational Therapy and Physical Dysfunction, Principles, Skills and Practice. London: Churchill Livingstone. Case-Smith J, Fisher AG & Bauer D (1989) An analysis of the relationship between proximal and distal control. American Journal of Occupational Therapy, 43, 657-662. Cass H, Reilly S, Owen L, Wisbeach A, Weekes L, Slonims V, Wigram T & Charman (2003) Findings from a multidisciplinary clinical case series of females with rett syndrome. Developmental Medicine and Child Neurology, 45, 325-337. Clancy CA, McGrath PJ, Oddson BE (2005) Pain in children and adolescents with spina bifida. Developmental Medicine and Child Neurology, 47, 27-34. Clark J, Michael S & Morrow M (2004) Wheelchair postural support for young people with progressive neuromuscular disorders. International Journal of Therapy and Rehabilitation, 11, 365-371. Cooper D, Dilabio RT, Broughton G & Brown D (2001) Dynamic seating components for the reduction of spastic activity and enhancement of function. Seventeenth International Seating Symposium, February 22-24. Cornell MS (1995) The hip in cerebral palsy. Developmental Medicine and Child Neurology, 37, 3-18. Cox SL & Kernohan WG (1998) They cannot sit properly or move around: seating and mobility during the treatment for developmental dysplasia of the hip in children. Pediatric Rehabilitation, 2, 129-134. Cox SL (1995) Problems of seating and mobility encountered by children with developmental dysplasia of the hip. Clinical Rehabilitation, 9, 190-197. Engström B (2002) Ergonomic Seating A True Challenge Wheelchair Seating and Mobility Principles. Sweden: Posturalis Books. Erdhardt RP, Merril SC (1998) Neurological dysfunction in children. In ME Neistadt, EB Crepeau (Ed) Willard and Spackman’s Occupational Therapy. London: Lippincott Williams & Wilkins.

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33

Reid DT (1995) Development and preliminary validation of an instrument to assess quality of sitting of children with neuromotor dysfunction. Physical and Occupational Therapy in Pediatrics, 15, 53-81. Reid DT & Sochaniwsky A (1991) Effects of anterior-tipped seating on respiratory function of normal children and children with cerebral palsy. International Journal of Rehabilitation Research, 14, 203-212. Rigby P, Reid D, Schoger S, & Ryan S (2001) Effects of a wheelchair mounted rigid pelvic stabiliser on care giver assistance for children with cerebral palsy. Assistive Technology, 13, 2-11. Rodgers SL, Gordon CT, Schazenbacher KE & Case-Smith (2001) Common diagnosis in pediatric occupational therapy practice. In J Case-Smith (Ed) Occupational Therapy for Children. London: Mosby. Scoliosis Research Society (2002) Retrieved from: http://www.srs [accessed 5th July 2005]. Scrutton D, Baird G, & Smeeton N (2001) Hip dysplasia in bilateral cerebral palsy and natural history in children aged 18 months to 5 years. Developmental Medicine and Child Neurology, 43, 586-600. Shimizu ME, Tanaka S, Takamagari H, Honda K, Shimizu H & Nakamura S (1994) Optimal positioning for an adult athetoid cerebral palsy patient in a wheelchair. Hiroshoma Journal of Medical Science, 43, 69-72. Shoham Y, Meyer S, Katz-Leurer M & Weiss PLT (2004) The influence of seat adjustment and a thoraco-lmbar-sacral orthosis on the distribution of body seat pressure in children with scoliosis and pelvic obliquity. Disability and Rehabilitation, 26, 21-26. Thompson N, Fahal I & Edwards RHT (1998 ) Muscle disorders in childhood. In M Stokes (Ed) Neurological Physiotherapy. London: Mosby. Trefler E & Taylor SJ (1991) Prescription and positioning: evaluating the physically disabled individual for wheelchair seating. Prosthetics and Orthotics International, 15, 217-224. Turner C (2001) Posture and seating for wheelchair users: an introduction. British Journal of Therapy and Rehabilitation, 8, 24-28. Tyldesley B & Grieve J (2002) Muscles, Nerves & Movement in Human Occupation. Oxford: Blackwell Science. Van der Heide JC, Otten B, van Eykern LA, Hadders-Algra M (2003) Development of postural adjustments during reaching in sitting children. Exp Brain Research, 151, 32-45.

34

Vaisbuch N, Meyer S, Weiss PL (2000) Effect of seated posture on interface pressure in children who are able bodied and who have myelomeningocele. Disability and Rehabilitation, 22, 749-755. Washington K, Deitz JC, White OR & Scwartz IS (2002) The effects of a contoured foam seat on postural alignment and upper-extremity function in infants with neuromotor impairment. Physical Therapy, 82, 1064-1076. Wandel JA (2000) Positioning and handling. In JW Solomon (Ed) Pediatric Skills for Occupational Therapy Assistants. London: Mosby. Wandel JA (2000) Cerebral palsy. In JW Solomon (Ed) Pediatric Skills for Occupational Therapy Assistants. London: Mosby. Westcott SL & Goulet CG (2005) Neuromuscular system: structures, functions, diagnosis, and evaluation. In SK Effgen (Ed) Meeting the Physical Therapy Needs of Children. Philadelphia: FA Davis Company. Wright-Ott C, Egilson S (2001) In J Case-Smith (Ed) Occupational Therapy for Children. London: Mosby. Yang TC, Chan RC, Wong TT, Bair WN, Kao CC, Chuang TY & Hsu TC (1996) Quantitative measurement of improvement in sitting balance in children with spastic cerebral palsy after selective rhizotomy. American Journal of Physical Medicine and Rehabilitation, 75, 348-352. Young NL, Wright JG, Lam TP, Rajaratnam K, Stephens D & Wedge JH (1998) Windswept hip deformity in spastic quadriplegic cerebral palsy. Pediatric Physical Therapy, 10, 94-100.

35

APPENDIX 1 The following tables indicate data bases searched and key words utilized regarding specific themes. TABLE 1: Seating Data Base Key Words CINAHL exp seating/ AND Child exp equipment design AND child exp seating/ AND child dynamic seating BIDS seating Pubmed seating seating principles seating and positioning Proquest adaptive seating seating seat$ AND children AND position Embase exp seat/ AND child dynamic seating AMED exp seating/ AND child exp equipment design/ AND exp seating

AND child dynamic seating Medline (Ovid) dynamic seating seating and positioning OTDbase seating ASSIA positioning and seating Psychinfo seating British Nursing Index seating Seat$ Zetoc seating pe*diatric seating

36

ISI Web of Science seating TABLE 2: Positioning / Posture Data Base Key Words CINAHL patient positioning BIDS posture positioning Pubmed seating and posture Proquest seating and positioning Embase positioning AMED exp positioning/ patient positioning AND exp pelvis/ Medline (Ovid) seating and positioning OTDbase positioning ASSIA seating and positioning Psychinfo positioning British Nursing Index patient positioning Zetoc seating and positioning ISI Web of Science seating and positioning seating and posture positioning and posture

37

TABLE 3: Cerebral Palsy Data Base Key Words CINAHL exp cerebral palsy/ AND posture/ AND

child BIDS cerebral palsy Pubmed cerebral palsy and seating Proquest cerebral palsy and positioning cerebral palsy and seating spastic cerebral palsy and posture spastic cerebral palsy and position Embase exp cerebral palsy/ AND child exp cerebral palsy/ AND posture/ AND

child AMED exp cerebral palsy/ AND seating Medline (Ovid) cerebral palsy and seating OTDbase cerebral palsy ASSIA cerebral palsy and positioning cerebral palsy and posture Psychinfo cerebral palsy and posture British Nursing Index Exp cerebral palsy/ AND seating Zetoc cerebral palsy and seating ISI Web of Science Cerebral palsy cerebral palsy and positioning cerebral palsy and posture

38

TABLE 4: Duchenne Muscular Dystrophy Data Base Key Words CINAHL exp duchenne muscular dystrophy/ AND

child BIDS muscular dystrophy Pubmed duchenne muscular dystrophy and seating duchenne muscular dystrophy and

posture Proquest exp duchenne muscular dystrophy/ AND

exp child/ Embase duchenne muscular dystrophy/ AND exp

child/ AMED duchenne muscular dystrophy Medline (Ovid) duchenne muscular dystrophy and seating OTDbase duchenne muscular dystrophy ASSIA duchenne muscular dystrophy duchenne muscular dystrophy AND

posture Psychinfo duchenne muscular dystrophy British Nursing Index duchenne muscular dystrophy Zetoc duchenne muscular dystrophy and seating duchenne muscular dystrophy and child duchenne muscular dystrophy and

posture ISI Web of Science Duchenne muscular dystrophy

39

TABLE 5: Congenital Hip Deformity / Developmental Dysplasia Data Base Key Words CINAHL exp hip deformity congenital/ AND child BIDS congenital hip deformity developmental dysplasia Pubmed congenital hip deformity and seating congenital hip deformity and posture Proquest developmental dysplasia Embase exp hip dislocation congenital/ Medline (Ovid) Congenital hip deformity AND child

AND hip dislocation AMED exp hip dislocation congenital/ OTDbase developmental dysplasia ASSIA developmental dysplasia Psychinfo developmental dysplasia British Nursing Index exp congenital abnormality/ AND exp

developmental dysplasia/ Zetoc congenital hip developmental dysplasia developmental dysplasia and seating developmental dysplasia and child ISI Web of Science developmental dysplasia

40

TABLE 6: Rett Syndrome Data Base Key Words CINAHL rett syndrome BIDS rett syndrome Pubmed rett syndrome and posture Proquest rett syndrome and posture Embase exp rett syndrome/ AND exp child AMED exp rett syndrome/ AND exp child Medline (Ovid) rett syndrome and child rett syndrome and posture rett syndrome and posture rett syndrome and child development OTDbase rett syndrome ASSIA rett syndrome Psychinfo rett syndrome British Nursing Index rett syndrome Zetoc rett syndrome ISI Web of Science rett syndrome

41

TABLE 7: Spina Bifida Data Base Key Words CINHAL spina bifida and seating spina bifida and positioning BIDS spina bifida Pubmed spina bifida and seating spina bifida and posture Proquest spina bifida and seating AMED exp child/ AND exp abnormalities/ AND

exp spina bifida/ exp child/ AND exp meningomyelocele Medline (Ovid) spina bifida and posture spina bifida and children OTDbase spina bifida ASSIA spina bifida and posture and seating Psychinfo spina bifida and children British Nursing Index spina bifida and seating Zetoc spina bifida and seating spina bifida and posture spina bifida ISI Web of Science spina bifida and child spina bifida and posture spina bifida and seating

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ulde

rs,

legs

and

han

ds.

43∕4

9 w

ith h

ydro

ceph

alus

re

porte

d he

adac

hes.

15

∕19

with

out h

ydro

ceha

lus

repo

rted

head

ache

s..

Chi

ldre

n re

porti

ng in

tens

e pa

in, a

lso

repo

rt ↑

freq

uenc

y an

d ↑

pain

loca

tions

. Pa

rent

a m

ost r

elia

ble

st

repo

rting

loca

lity

of

child

ren’

s pai

n.

Find

ings

con

clud

e ch

ildre

n w

ith so

ina

bifid

a re

port

clin

ical

ly si

gnifi

cant

, un

der

reco

gnis

ed &

unt

reat

ed p

ain.

Res

ults

sugg

est t

here

is a

de

ficie

ncy

in e

quip

men

t av

aila

ble

to c

ope

with

and

m

anag

e ch

ildre

n in

pla

ster

, in

clud

ing

prod

ucts

to h

elp

in

seat

ing.

Spe

cial

em

phai

s is

plac

ed o

n m

obili

ty.

This

pilo

t stu

dy c

onfir

med

ne

ed fo

r fut

ure

focu

sed

rese

arch

to p

rovi

de p

rodu

cts

sugg

este

d.

Met

hodo

logy

Dat

a co

llect

ed v

ia p

aren

tal

repo

rt qu

estio

nnai

res (

The

Pedi

atric

Pai

n Q

uest

ionn

aire

) an

d ch

ild re

port

ques

tionn

aire

(V

isua

l Ana

logu

e Sc

ale)

and

, fr

om t

med

ical

reco

rds

to

inve

stig

ate

ain

inte

nsity

, lo

catio

n, fr

eque

ncy

and

dura

tion.

M

easu

res a

dmin

istra

ted

by o

ne

of tw

o tra

ined

Mas

ters

leve

l vo

lunt

eer i

n se

para

te a

rea

from

cl

inic

to e

nsur

e in

depe

nden

t ra

ting.

24 q

uest

ionn

aire

s dis

tribu

ted,

of

whi

ch 1

1 w

ere

retu

rned

(48.

8%

resp

onse

rate

). Q

uest

ionn

aire

s eith

er d

istri

bute

d by

pos

t or t

hrou

gh p

aren

tal

supp

ort g

roup

s and

wer

e co

mpl

eted

by

pare

nts.

Parti

cipa

nts

n= 6

8 ch

ildre

n w

ith sp

ina

bifid

a

(30

mal

es, 3

8 fe

mal

es)

Mea

n ag

e 12

yea

rs 8

mon

ths

(ran

ge 8

-19

year

s).

n=59

(mye

lom

enin

goce

le)

n= 6

lipo

mye

lom

enin

goce

le

n= 3

lipo

men

ingo

cele

. n=

49

shun

ted

hydr

ocep

halu

s n=

26 a

mbu

late

d fu

ll tim

e C

hild

ren

able

to c

omm

unic

ate

in

Engl

ish

and

had

no si

gnifi

cant

co

gniti

ve li

mita

tions

. R

ecru

ited

from

regi

onal

ch

ildre

n’s c

entre

. Pa

rent

s (m

ean

age

43 y

ears

4

mon

ths)

Fo

r com

paris

on p

urpo

ses

sam

ple

divi

ded

into

2 g

roup

s

Pare

nts

of 1

1 ch

ildre

n (2

boy

s;

9 gi

rls)

who

wer

e ei

ther

un

derg

oing

or h

ad u

nder

gone

tre

atm

ent f

or la

te d

iagn

osed

D

DH

, wer

e su

rvey

ed b

y qu

estio

nnai

re.

Stud

y D

esig

n

Pros

pect

ive

stud

y.

Que

stio

nnai

re (p

ilot s

tudy

) (s

emi s

truct

ured

and

ope

n)

3 qu

estio

nnai

res s

ent t

o pa

tient

s in

Nor

ther

n Ir

elan

d; 2

in th

e re

publ

ic o

f Ire

land

; and

6 in

En

galn

d.

Que

stio

ns g

ener

ated

from

an

alys

is o

f the

lite

ratu

re a

nd

info

rmat

ion

obta

ined

from

de

taile

d se

mi-

stru

ctur

ed

inte

rvie

ws w

ith su

rgeo

ns a

nd

staf

f inv

olve

d in

trea

tmen

t.

Que

stio

nnai

re fo

cuse

s on

seat

ing

and

mob

ility

pro

blem

s.

Purp

ose

1) T

o in

vest

igat

e th

e na

ture

an

d pr

eval

ence

of p

ain

in

child

ren

and

adol

esce

nts w

ith

spin

a bi

fida.

2)

To

com

pare

chi

ldre

n’s a

nd

pare

nts’

pae

diat

ric p

ain

ratin

gs to

det

erm

ine

whe

ther

or

not

con

cord

ance

exi

sts

betw

een

the

two.

To

con

firm

the

clin

ical

su

spic

ion

that

sign

ifica

nt

prob

lem

s are

enc

ount

ered

by

child

ren

and

thei

r car

e-gi

vers

in

cop

ing

the

treat

men

t of

splin

ting

resu

lting

from

late

di

agno

ses d

evel

opm

enta

l dy

spla

sia

of th

e hi

p (D

DH

).

Aut

hor /

Dat

e / T

itle

Cla

ncy

CA

, McG

rath

PJ &

Odd

son

BE

(200

5).

Pain

in a

dole

scen

ts w

ith sp

ina

bifid

a.

Cox

SL

& M

olla

n R

AB

(199

5)

Prob

lem

s of s

eatin

g an

d m

obili

ty

enco

unte

red

by c

hild

ren

with

de

velo

pmen

tal d

yspl

asia

of t

he h

ip.

2

Res

ults

and

Con

clus

ions

Res

lults

sugg

este

d th

at

pare

nts i

denf

ied

soci

al,

emot

iona

l and

phy

sica

l pr

oble

ms.

Pro

blem

s inc

lude

d si

ze a

nd sh

ape

of sp

lint,

trans

porti

ng a

ns se

atin

g pr

oble

ms a

nd d

isru

ptio

n of

fa

mily

rout

ines

. Su

ch p

robl

ems m

ay b

e al

levi

ated

by

prov

isio

n of

sp

ecia

lised

dev

ices

that

w

ould

per

mit

mob

ility

by

car,

in a

pus

hcha

ir an

d pr

ovid

e se

atin

g in

the

hom

e.

5 de

velo

ped

spas

tic

hem

iple

gia

1dev

elop

ed sp

astic

te

trapl

egia

1

deve

lope

d sp

astic

te

trapl

egia

with

ath

etos

is.

Res

ults

sugg

est b

asic

or

ient

atio

n of

pos

tura

l ad

just

men

ts o

f chi

ldre

n de

velo

ping

spas

tic C

P w

as

inta

ct, w

ith m

ain

prob

lem

s be

ing

↓ ab

ility

to m

odul

ate

post

ural

adj

ustm

ent t

o ta

sk

spec

ific

cons

train

ts.

The

child

with

spas

tic a

thet

osis

sh

owed

dis

tinct

abn

orm

aliti

es

in b

asic

org

anis

atio

n of

po

stur

al a

djus

tmen

ts.

Rea

chin

g m

ovem

ents

wer

e fa

vour

ed in

sem

i rec

line

as

oppo

sed

to u

prig

ht, s

upin

e an

d lo

ng le

g si

tting

Met

hodo

logy

Que

stio

nnai

re d

evel

oped

bas

ed

on th

e ac

tiviti

ed o

f dai

ly iv

ing

that

wer

e es

tabl

ishe

d fo

r fa

mili

es w

ith c

hild

ren

betw

een

3 m

onth

s and

3 y

ears

old

.

10 q

uest

ionn

aire

s dis

tribu

ted

in

Engl

and;

3 o

btai

ned

via

inte

rvie

w w

ith p

aren

ts in

ch

ildre

ns w

ard

in h

pspi

tal i

n N

. Ir

elan

d: 1

00 d

istri

bute

d to

thos

e un

derg

oing

or h

ad u

nder

gone

tre

atm

ent a

t the

hos

pita

l.

Rea

chin

g m

ovem

ents

wer

e as

sess

ed v

ia si

mul

tane

ous

reco

rdin

g of

vid

eo d

ata

and

surf

ace

EMG

of a

rm, n

eck,

tru

nk a

nd le

g m

uscl

es d

urin

g re

achi

ng in

var

ious

pos

ition

s (ly

ing

supi

ne, s

emi-

recl

ine

(45°

), up

right

and

long

leg

sitti

ng).

Parti

cipa

nts

113

affe

cted

fam

ilies

sur

veye

d 38

que

stio

nnai

res r

etur

ned

(34%

re

spon

se ra

te.

7 ch

ildre

n (a

ge ra

nge

4-18

m

onth

s).

3 bo

ys; 4

girl

s

Stud

y D

esig

n

Que

stio

nnai

re (s

emi s

truct

ured

an

d op

en)

Long

itudi

nal

Purp

ose

To id

entif

y pr

oble

ms

rela

ted

to th

e tre

atm

ent (

splin

tage

) of

DD

H in

ord

er o

f prio

rity

in

fam

ilies

of h

ildre

n w

ith

DD

H.

Phys

ical

, em

otio

nal a

nd

soci

al p

robl

ems a

re th

e fo

cus

of th

e re

port.

To a

naly

se th

e ea

rly

deve

lopm

ent o

f pos

tura

l ad

just

men

ts a

ccom

pany

ing

reac

hing

mov

emen

ts in

ch

ildre

n w

ith c

ereb

ral p

alsy

.

Aut

hor /

Dat

e / T

itle

Cox

SL

& K

erno

han

WG

(199

8) .

They

can

not s

it pr

oper

ly o

r mov

e ar

ound

: sea

ting

and

mob

ility

dur

ing

treat

men

t for

dev

elop

men

tal

dysp

lasi

a of

the

hip

in c

hild

ren.

Had

ders

-Alg

ra M

, van

der

Fits

IB

M, S

trem

mel

aar E

F &

Tou

wen

B

CC

(199

9).

Dev

elop

men

t of p

ostu

ral

adju

stm

ents

dur

ing

reac

hing

in

infa

nts w

ith c

ereb

ral p

alsy

.

3

Res

ults

and

Con

clus

ions

Sign

ifica

nt st

atic

cor

rect

ion

of th

e sp

ine

(sco

liotic

) may

be

ach

ieve

d vi

a an

ar

rang

emen

t of l

ater

al p

ads

on a

seat

ing

syst

em a

pply

ing

a 3-

poin

t for

ce sy

stem

to th

e si

des o

f the

bod

y.

A st

atis

tical

sign

ifica

nt

corr

elat

ion

was

obt

aine

d (p

<0.0

1) b

etw

een

the

sitti

ng

lum

bar c

urve

and

the

popl

iteal

ang

le.

This

co

rrel

atio

n w

as le

ss w

hen

stan

ding

. St

udy

conc

lude

s tha

t the

re is

a

corr

elat

ion

betw

een

tight

ha

mst

rings

and

dec

reas

ing

lum

bar l

ordo

sis,

espe

cial

ly in

th

e se

ated

pos

tion

in c

hild

ren

with

cer

ebra

l pal

sy.

Met

hodo

logy

Shap

e of

spin

e m

easu

red

(spi

nous

pro

cess

ang

le in

an

asse

ssm

ent c

hair

(CA

PS II

).

Exer

ted

forc

s on

chai

r mea

sure

d by

ele

ctric

al tr

ansd

ucer

s at

tach

ed to

late

ral s

uppo

rt pa

ds

and

seat

bas

e. M

easu

rem

ents

w

ere

take

n in

3 a

ltern

ativ

e ar

rang

emen

ts: 1

) uns

uppo

rted

uppe

r bod

y 2)

two

late

ral p

ads a

t th

e sa

me

heig

ht 3

) bod

y su

ppor

ted

by 3

-poi

nt fo

rce

syst

em.

The

Cob

b m

etho

d w

as u

sed

to

mea

sure

lum

bar a

nd th

orac

ic

kyph

osis

. St

andi

ng a

nd s

itiin

g la

tera

l spi

ne fi

lms w

ere

obta

ined

. Th

e po

plite

al a

ngle

was

m

easu

red

to a

sses

s ham

strin

g tig

htne

ss.

Parti

cipa

nts

16 su

bjec

ts w

ith sp

astic

cer

ebra

l pa

lsy

9

boys

: 7 g

irls

Man

age

: 14.

7 ye

ars (

rang

e 6.

5-

20.8

). In

clus

ion

crite

ria re

uire

d su

bjec

ts to

be

non-

ambu

lant

, ha

ve a

scol

iosi

s and

requ

ire

spec

ial s

eatin

g w

ithin

thei

r w

heel

chai

r,.

21 p

artic

ipan

ts

Mea

n ag

e: 9

.4 y

ears

old

Stud

y D

esig

n

Pros

pect

ive

stud

y: m

atch

ed

pairs

des

ign

Ret

rosp

ectiv

e cl

inic

al a

nd

radi

ogra

phic

revi

ew.

Purp

ose

To

inve

stig

ate

the

effe

cts o

f sp

ecia

l sea

ting

on la

tera

l sp

inal

cur

vatu

re in

the

non-

ambu

lant

spas

tic c

ereb

ral

pals

y po

pula

tion

with

sc

olio

sis.

To

ass

ess t

he in

fluen

ce o

f tig

ht h

amst

rings

on

the

sagg

ital a

lignm

ent o

f the

th

orac

ic a

nd lu

mba

r spi

ne in

ch

ildre

n w

ith c

ereb

ral p

alsy

.

Aut

hor /

Dat

e / Y

ear

Hol

mes

KJ,

Mic

hhae

l SM

& T

horp

e (2

003)

. M

anag

emen

t of s

colio

sis w

ith

spec

ial s

eatin

g fo

r the

non

-am

bula

nt

spas

tic c

ereb

ral p

alsy

pop

ulat

ion

– a

biom

echa

nica

l stu

dy.

McC

arth

y JJ

& B

etz

RR

(200

0)

The

rela

tions

hip

betw

een

tight

ha

mst

rings

and

lum

bar h

ypol

ordo

sis

in c

hild

ren

with

cer

ebra

l pal

sy.

4

Res

ults

and

Con

clus

ions

Sign

ifica

nt b

etw

een

grou

p di

ffer

ence

s wer

e ob

serv

ed o

n m

ost d

epen

dent

mea

sure

s.

No

sign

ifica

nt d

iffer

ence

a

coul

d be

attr

ibut

ed to

seat

in

clin

atio

n, h

owev

er a

utho

rs

sugg

est a

nter

ior t

ilt m

ay

dist

urb

post

ural

stab

ility

, w

ithou

t im

prov

ing

uppe

r ex

trem

ity fu

nctio

n..

A si

gnifi

cant

rela

tions

hip

was

fo

und

betw

een

the

prev

alen

ce

of re

tt sy

ndro

me

scol

iosi

s and

or

thop

aedi

c ris

k fa

ctor

s. T

he

findi

ngs s

ugge

st a

trea

tmen

t ap

proa

ch fo

cusi

ng o

n ba

lanc

ing

bila

tera

l mus

cle

pull.

Met

hodo

logy

Seat

surf

ace

incl

inat

ions

of

0°,

5° (a

nter

ior t

ilt),

5° p

oste

rior

tilt)

with

seat

to b

ackr

est a

ngle

at

90°

wer

e co

mpa

red

with

in

and

betw

een

grou

ps, w

ith le

g re

st p

ositi

on a

t 90°

from

the

seat

su

rfac

e fo

r all

expe

rimen

tal

cond

ition

s.

400

ques

tionn

aire

s wer

e di

strib

uted

via

pos

t, 26

2 w

ere

com

plet

ed a

nd re

turn

ed (6

6%).

22

8 w

ere

suita

ble

for s

tatis

tical

an

alys

is (

57%

).

Res

pons

es w

ere

scor

ed

inde

pend

ently

by

two

inve

stig

ator

s..

Parti

cipa

nts

20 c

hild

ren

(10

non-

impa

ired:

10

mild

-mod

erat

e sp

astic

ce

rebr

al p

alsy

. A

ge ra

nge:

4-1

5 ye

ars.

C

P ch

ildre

n ab

le to

sit

inde

pend

ently

and

am

bula

te

with

or w

ithou

t mob

ility

aid

s.

Abl

e to

com

preh

end

sim

ple

inst

ruct

ions

. Sc

reen

ed fo

r vis

ual p

robl

ems.

262

ques

tionn

aire

s com

plet

ed

by In

tern

atio

nal R

ett S

yndr

ome

Ass

ocia

tion

fam

ilies

. Fi

ndin

gs b

ased

on

57%

resp

onse

ra

te.

Stud

y D

esig

n

Qua

si-e

xper

imen

tal d

esig

n

Que

stio

nnai

re

Purp

ose

To in

vest

igat

e th

e in

ter-

rela

tions

hip

of se

at-s

urfa

ce

incl

inat

ion

on p

ostu

ral

stab

ility

and

func

tiona

l use

of

the

uppe

r ext

rem

ities

in

child

ren

with

cer

ebra

l pal

sy.

To in

vest

igat

e th

e in

terr

elat

ions

hips

bet

wee

n re

tt sy

ndro

me

scol

iosi

s and

sy

mm

etric

, asy

mm

etric

m

otor

pul

l, am

bula

tion

and

adva

ncem

ent o

f age

in

orde

r to

pro

vide

a tr

eatm

ent

ratio

nale

for s

low

ing

the

prog

ress

ion

of a

scol

iosi

s.

Aut

hor /

Dat

e / T

itle

McC

lena

gaha

n B

A,

Thom

bs L

&

Miln

er M

(199

2).

Effe

cts o

f sea

t-su

rfac

e in

clin

atio

n on

pos

tura

l sta

bilit

y an

d fu

nctio

n of

th

e up

per e

xtre

miti

es o

f chi

ldre

n w

ith c

ereb

ral p

alsy

.

McC

lure

MK

, Bat

tagl

ia C

&

McC

lure

RJ (

1997

).

The

rela

tions

hip

of c

umul

ativ

e m

otor

asy

mm

etrie

s to

scol

iosi

s in

rett

synd

rom

e.

5

Res

ults

and

Con

clus

ions

Qua

ntifi

able

and

qua

litat

ive

betw

een

grou

p di

ffer

ence

s in

reac

hing

. N

o si

gnifi

cant

di

ffer

ence

s cou

ld b

e at

tribu

ted

to th

e se

atin

g po

sitio

ns.

Sign

ifica

nt d

iffer

ence

s ob

serv

ed a

mon

g th

e fiv

e co

nditi

ons.

Stu

dy su

gges

ts a

an

terio

r sitt

ing

post

ure

is th

e pr

efer

red

posi

tion

to fa

cilit

ate

incr

ease

d tru

nk e

xten

sion

.

Met

hodo

logy

Arm

mov

emen

ts w

ere

com

pare

d be

twee

n gr

oups

and

with

in

grou

ps in

four

diff

eren

t po

sitio

ns (n

eutra

l, w

heel

chai

r, po

ster

ior t

illt (

15°)

and

ant

erio

r til

t (15

°).

Four

con

ditio

ns

pres

ente

d in

cou

nter

bala

nced

or

der.

EM

G a

ctiv

ity re

core

ded.

Each

chi

ld ra

ted

on a

scal

e of

1-

4 to

doc

umen

t tru

nk c

ontro

l, ra

ted

by a

n ex

perie

nced

pa

edia

tric

phys

ioth

erap

ist.

Trun

k ex

tens

ion

mea

sure

s by

the

mod

ified

Sch

ober

M

easu

rem

ent o

f Spi

nal

Exte

nsio

n (1

975)

. Te

sted

in 5

rand

om p

ositi

ons

durin

g 30

min

sess

ion

(flo

or

sitti

ng in

tailo

r lik

e fa

shio

n,

leve

l sitt

ing

with

hip

s and

kne

es

at 9

0°, b

ench

sitti

ng w

ith b

ench

til

ted

forw

ard

(20°

and

30°)

and

si

tting

in

a co

mm

erci

al c

hair.

Parti

cipa

nts

12 su

bjec

ts

3 m

en: 3

wom

en w

ith m

ild-

mod

erat

e sp

astic

cer

ebra

l pal

sy;

3 m

en a

nd 3

wom

en w

ith n

o kn

own

path

olog

ical

con

diti

on.

Age

rang

e; 1

8-21

yea

rs

Rig

ht h

and

dom

inan

t A

ble

to fo

llow

inst

ruct

ions

15 c

hild

ren

Age

rang

e (2

-6 y

ears

) D

iagn

osis

of d

evel

opm

enta

l de

lay

and/

or se

vere

hyp

oton

ic o

r hy

perto

nic

cere

bral

pal

sy

(dip

legi

a or

qua

drip

legi

a).

Stud

y D

esig

n

Qua

si-e

xper

imen

tal

Purp

ose

To c

ompa

re a

rm m

ovem

ents

of

per

sons

with

and

with

out

cere

bral

pal

sy a

nd to

de

term

ine

if th

e al

tera

tion

of

the

seat

ang

le o

f a c

hair

affe

ct

qual

ity o

f mov

emen

ts.

To id

entif

y a

relia

ble,

ob

ject

ive

and

clin

ical

ly

usef

ul m

easu

re fo

r ass

essi

ng

chan

ges i

n tru

nk a

lignm

ent

and

to e

valu

ate

whi

ch o

f fiv

e di

ffer

ent s

eate

d po

sitio

ns w

as

the

mos

t eff

ectiv

e in

en

cour

agin

g tru

nk e

xten

sion

.

Aut

hor /

Dat

e / T

itle

McP

hers

on JJ

, Sch

ild R

, Spa

uldi

ng

SJ, B

aras

amia

n P,

Tra

nson

C &

W

hite

SC

(199

1).

Ana

lysi

s of u

pper

ext

rem

ity

mov

emen

t in

four

sitti

ng p

ositi

ons:

a

com

paris

on o

f per

sons

with

and

w

ithou

t cer

ebra

l pal

sy.

Mie

dane

r JA

(199

0).

The

effe

cts o

f sitt

ing

posi

tions

on

trunk

ext

ensi

on fo

r chi

ldre

n w

ith

mot

or im

pairm

ent.

6

Res

ults

and

Con

clus

ion

Gre

ates

t red

uctio

n of

sp

astic

ity g

aine

d an

d po

stur

al

cont

rol e

nhan

ced

whe

n th

ree

fact

ors w

ere

com

bine

d:

sym

met

rical

fixa

tion

of b

elt

unde

r sea

t, us

e of

an

abdu

ctio

n or

thos

is a

nd

plac

emen

t in

the

FSP.

Thi

s is

in a

dditi

on to

seat

incl

ined

fo

rwar

ds a

nd a

rms s

uppo

rted

on a

tabl

e.

No

disc

erna

ble

effe

cts w

ere

iden

tifie

d by

seat

in

clin

atio

n al

one.

Res

ults

sugg

est p

atho

logi

cal

mov

emen

ts a

re m

inim

ised

an

d po

stur

al c

ontro

l and

up

per e

xtre

mity

func

tion

are

mor

e 3e

ffic

ient

in a

forw

ard

tippe

d se

at, w

ith a

firm

ba

ckre

st su

ppor

ting

pelv

is,

arm

s sup

porte

d ag

ains

t a

tabl

e an

d fe

et p

erm

itted

to

mov

e ba

ckw

ard.

Met

hodo

lgy

Bot

h ch

ildre

n fil

med

and

ph

otog

raph

ed in

divi

dual

ly in

six

posi

tions

on

the

sam

e oc

casi

on,

incl

udin

g si

tting

in th

eir o

wn

adap

ted

chai

rs a

nd in

the

prop

osed

FSP

. Te

stin

g co

nditi

ons w

ere

alte

red

by

chan

ging

the

seat

incl

inat

ion,

us

e/no

n-us

e of

abd

uctio

n or

thos

is a

nd w

ith.w

ithou

t a ta

ble

in fr

ont o

f the

m.

Tot

al ti

me

for

post

ural

con

trol w

as re

cord

ed

and

path

olog

ical

mov

emen

ts

wer

e co

unte

d.

Chi

ldre

n w

ere

film

ed a

nd

phot

ogra

phed

in 6

pos

ition

s (a

dapt

ed c

hair;

ada

pted

cha

ir an

d cu

t out

leve

l tab

le in

font

of

child

,; FS

P w

ithou

t abd

uctio

n or

thos

is; a

dapt

ed c

hair

and

abdu

ctio

n or

thos

is,;F

SP w

ithou

t ta

ble;

FSP

with

tabl

e an

d ab

duct

ion

orth

osis

). In

FSP

seat

w

as fo

rwar

d in

clin

ed.

Hea

d co

ntro

l, pa

thol

ogic

al

mov

emen

ts, p

ostu

ral c

ontro

l w

ere

mea

sure

d vi

a ob

serv

atio

n an

d Si

tting

Ass

essm

ent S

cale

.

Parti

cipa

nts

2 ch

ildre

n w

ith se

vere

cer

ebra

l pa

lsy.

C

hild

A: 7

yea

rs o

ld, s

past

ic

dipl

egia

C

hild

B: 1

5 ye

ars o

ld ,

spas

tic

tetra

pleg

ia.

23 c

hild

ren

(8 fe

mal

e, 1

5 m

ale)

A

ge ra

nge

(2-1

6 ye

ars)

Stud

y D

esig

n

Pilo

t stu

dy (s

ingl

e ca

se d

esig

n)

Qua

si-e

xper

imen

tal

Purp

ose

To c

reat

e a

func

tiona

l sitt

ing

posi

tion

(FSP

) by

iden

tifyi

ng

the

esse

ntia

l fac

tors

requ

ired

to re

duce

spas

ticity

as w

ell

as e

nhan

cing

pos

tura

l con

trol

in c

hild

ren

with

cer

ebra

l pa

lsy.

To fi

nd a

func

tiona

l sitt

ing

posi

tion

for c

hild

ren

with

ce

rebr

al p

alsy

and

to c

ompa

re

this

pos

ition

with

the

child

ren’

s orig

inal

sitti

ng

posi

tions

with

var

ious

ex

perim

enta

l pos

ition

s.

Aut

hor /

Dat

e / T

itle

Myh

r U &

von

Wen

dt (1

990)

. R

educ

ing

spas

ticity

and

enh

anci

ng

post

ural

con

trol f

or th

e cr

eatio

n of

a

func

tiona

l sitt

ing

posi

tion

in

child

ren

with

cer

ebra

l pal

sy: a

pilo

t st

udy.

Myh

r U &

von

Wen

dt (1

991)

. Im

prov

emen

t of f

unct

iona

l sitt

ing

posi

tion

for c

hild

ren

with

cer

ebra

l pa

lsy.

7

Res

ults

and

Con

clus

ions

Res

ults

reve

aled

that

in

posi

tions

with

the

back

rest

ve

rtica

l and

with

the

use

of a

hi

p be

lt, a

ll ch

ildre

n he

ld

thei

r fee

t pos

terio

r to

the

knee

join

t axi

s reg

ardl

ess o

f se

at in

clin

atio

n.

8 ou

t of t

he 1

0 ch

ildre

n as

sess

ed, w

ho u

sed

the

FSP

over

the

five

year

per

iod,

sh

owed

slig

ht b

ut si

gnifi

cant

im

prov

emen

t, th

e re

mai

ning

tw

o ch

ildre

n ha

d de

terio

rate

d.

Stud

y co

nclu

des t

hat t

he F

SP

cont

ribut

es to

impr

oved

ab

ility

to u

se th

e up

per

extre

miti

es (h

and

and

arm

fu

nctio

n).

Met

hodo

logy

The

child

ren

wer

e fil

med

and

ph

otog

raph

ed w

hils

t per

form

ing

stan

dard

ised

task

s in

5 di

ffer

ent

sitti

ng p

ositi

ons (

back

rest

ve

rtica

l and

sea

t sur

face

in

clin

atio

n va

ryin

g be

twee

n 0°

10

° for

war

d in

clin

ed, 1

0°

back

war

d re

clin

ed, a

nd a

lso

in

recl

ined

pos

ition

s in

with

seat

an

d ba

ckre

st l

ean

back

war

d fr

om th

e ho

rizon

tal p

lane

and

ve

rtica

l pla

ne, r

espe

ctiv

ely

(1

5 °)

.

Ten

child

ren

wer

e fil

med

and

ph

otog

raph

ed a

fter t

he

intro

duct

ion

of th

e FS

P an

d su

bseq

uent

ly fi

ve y

ears

late

r. H

ead,

trun

k, fo

ot c

ontro

l, ar

m

and

hand

func

tion

wer

e as

sess

ed

by th

e Si

tting

Ass

essm

ent S

cale

Parti

cipa

nts

10 n

on-d

isab

led

child

ren

(7 g

irls;

3 b

oys)

M

ean

age

6.7

year

s (ra

nge:

4-9

ye

ars)

.

10 c

hild

ren

with

CP

Stud

y D

esig

n

Cas

e St

udy

Ret

rosp

ectiv

e

Purp

ose

To d

eter

min

e th

e sp

onta

neou

s pos

ition

ing

of

the

low

er e

xtre

miti

es re

lativ

e to

the

mov

emen

t axi

s at t

he

knee

join

t, an

d to

det

erm

ine

thre

ext

ent o

f spo

ntan

eous

us

e of

the

arm

s for

supp

ort i

n di

ffer

ent s

ittin

g po

sitio

ns, i

n

a gr

oup

of n

on-d

isab

led

child

ren.

To

obt

ain

a ba

selin

e co

mpa

rison

with

m

easu

rem

ents

of c

hild

ren

with

cer

ebra

l pal

sy.

To re

-ass

ess c

hild

ren

who

w

ere

prev

ious

ly in

trodu

ced

to

and

test

ed in

the

FSP

five

year

s pre

viou

sly.

To

re-e

xam

ine

the

relia

bilit

y of

the

Sitti

ng A

sses

smen

t Sc

ale.

Aut

hor /

Dat

e / T

itle

Myh

r U (1

994)

. In

fluen

ce o

f diff

eren

t sea

t and

ba

ckre

st in

clin

atio

ns o

n th

e sp

onta

neou

s pos

ition

ing

of th

e ex

trem

ities

of n

on-d

isab

led

child

ren.

Myh

r U, v

on W

endt

L, N

orrli

n S

&

Rad

ell U

(199

5).

Five

yea

r fol

low

-up

of fu

nctio

nal

sitti

ng p

ositi

on in

chi

ldre

n w

ith

cere

bral

pal

sy.

8

Res

ults

and

Con

clus

ions

Res

ults

of t

he st

udy

sugg

est

that

pul

mon

ary

func

tion

was

hi

gher

in a

dapt

ive

seat

ing

com

pare

d to

non

-ada

ptiv

e se

atin

g.

Res

ults

sugg

est i

mpl

icat

ions

fo

r spe

ech,

sitti

ng fo

r pr

olon

ged

perio

ds a

nd th

e pr

even

tion

of p

ulm

onar

y hy

perte

nsio

n.

Res

ults

hig

hlig

hted

that

ex

tens

or a

ctiv

ity w

as lo

wes

t w

hen

back

rest

incl

inat

ion

rem

aine

d at

90°

and

the

seat

su

rfac

e at

0° (

uprig

ht si

tting

). Pr

elim

inar

y fin

ding

from

st

udy

sugg

ests

that

the

orie

ntat

ion

of th

e he

ad

/nec

k/bo

dy in

rela

tion

to

grav

ity m

ay p

lay

an

impo

rtant

role

in c

ontro

lling

ex

tens

or a

ctiv

ity.

Met

hodo

logy

Vita

l cap

acity

, for

ced

expi

rato

ry

volu

me

in o

ne se

cond

, and

ex

pira

tory

tim

e as

mea

sure

by

a sp

irom

eter

, of

chi

ldre

n w

ith C

P w

ere

mea

sure

d in

a ty

pica

l slin

g ba

ck w

heel

chai

r and

in a

w

heel

chai

r with

mod

ular

inse

rts.

Chi

ldre

n w

ere

posi

tione

d in

90

-90-

90 in

bot

h se

atin

g un

its.

Usi

ng su

rfac

e el

ectro

des E

MG

ac

tivity

was

reco

rded

for t

he

lum

bar e

xten

sor s

pina

e m

uscl

es

in se

ven

diff

eren

t tes

ting

cond

ition

s. S

eat s

urfa

ce

incl

inat

ions

of 0

° and

15°

co

mbi

ned

with

bac

kres

t in

clin

atio

ns o

f 75°

, 90°

, 105

°, an

d 12

0°..

Parti

cipa

nts

8 ch

ildre

n w

ith sp

astic

CP

A

ge ra

nge:

5-1

2.

Non

-am

bula

nt

No

appa

rent

evi

denc

e of

in

trins

ic lu

ng d

isea

se.

11 c

hild

ren

(7 b

oys:

4 g

irls)

w

ith sp

astic

CP.

A

ge ra

nge:

4-8

yea

rs

Stud

y D

esig

n

Qua

si-E

xper

imen

tal

Expe

rimen

tal

Purp

ose

To c

ompa

re th

e ef

fect

s of

adap

tive

and

non

adap

tive

seat

ing

on p

ulm

onar

y fu

nctio

n.

To d

eter

min

e if

the

myo

elec

tric

activ

ity o

f the

ex

tens

or m

uscl

es o

f the

lu

mba

r spi

ne is

aff

ecte

d by

po

sitio

ns o

f the

seat

surf

ace

and

seat

bac

k, o

r by

thei

r po

sitio

ns re

lativ

e to

one

an

othe

r.

Aut

hor /

Dat

e / T

ilte

Nw

aobi

OM

& S

mith

(198

6).

Effe

ct o

f ada

ptiv

e se

atin

g on

pu

lmon

ary

func

tion

of c

hild

ren

with

ce

rebr

al p

alsy

.

Nw

aobi

OM

, Bru

bake

r CE,

Cus

ick

B &

Sus

sman

(198

3).

Elec

trom

yogr

aphi

c in

vest

igat

ion

of

exte

nsor

act

ivity

in c

ereb

ral-

pals

ied

child

ren

in d

iffer

ent s

eatin

g po

sitio

ns.

9

Res

ults

and

Con

clus

ions

Res

ults

dem

onst

rate

d th

at

mus

cle

activ

ity w

as a

ffec

ted

by b

ody

orie

ntat

ion,

with

to

nic

mus

cle

activ

ity lo

wer

in

the

uprig

ht p

ositi

on w

ith

stat

istic

ally

sign

ifica

nt

diff

eren

ces f

or th

e hi

p ad

duct

ors a

nd b

ack

exte

nsor

s.

Exte

nsor

tone

may

incr

ease

in

the

recl

ined

pos

ition

.

Res

ults

con

clud

e or

ient

atio

n of

the

body

in sp

ace

affe

cts

uppe

r ext

rem

ity fu

nctio

n.

The

leve

l of u

pper

ext

rem

ity

perf

orm

ance

was

hig

hest

in

the

uprig

ht p

ositi

on.

Met

hodo

logy

Surf

ace

elec

trode

s wer

e us

ed to

m

easu

re m

yoel

ectri

c ac

tivity

if

the

low

bac

k ex

tens

ors,

hip

addu

ctor

s and

ank

le p

lant

ar

flexo

r mus

cles

in tw

o se

atin

g po

sitio

ns.

Th

e se

atin

g po

sitio

ns w

ere

0° in

ve

rtica

l pla

ne a

nd 3

0° fr

om th

e ve

rtica

l pla

ne.

Each

par

ticip

ant

was

pos

ition

ed

in 9

0-90

-90

with

th

e us

e of

a p

omm

el p

rior t

o te

stin

g.

The

child

ren

wer

e pl

aced

ra

ndom

ly in

dif

fere

nt se

atin

g or

ient

atio

ns (3

0°, 1

5°, a

nd 0

° po

ster

ior i

nclin

atio

n an

d 15

°ant

erio

r inc

linat

ion)

.

Parti

cipa

nts

12 c

hild

ren

(8 b

oys:

4 g

irls)

A

ge ra

nge:

6-1

8 ye

ars

Dia

gnos

is o

f mild

-mod

erat

e sp

astic

dip

legi

a.

Fair

head

and

trun

k co

ntro

l Fa

ir to

poo

r fin

e m

otor

skill

s N

o fix

ed d

efor

mity

. 13

chi

ldre

n w

ith C

P

3 at

heto

id C

P 10

spas

tic C

P A

ge ra

nge:

8-1

6 ye

ars

Una

ble

to a

mbu

late

in

depe

nden

tly

Req

uire

ada

ptiv

e se

atin

g fo

r up

right

pos

ition

ing

Fair-

poor

gro

ss u

pper

ext

rem

ity

cont

rol.

Fair

head

and

trun

k co

ntro

l Po

or fi

ne m

otor

skill

s.

Stud

y D

esig

n

Expe

rimen

tal

Qua

si-e

xper

imen

tal

Purp

ose

To m

easu

re th

e to

nic

myo

elec

tric

activ

ity o

f the

lo

w b

ack

exte

nsor

s, hi

p ad

duct

ors a

nd a

nkle

pla

ntar

fle

xors

in tw

o bo

dy

orie

ntat

ions

. To

det

erm

ine

if to

nic

activ

ity

of th

ese

mus

cles

cha

nge

in

resp

onse

to b

ody

orie

ntat

ion,

an

d w

hich

bod

y or

ient

atio

n pr

ovid

es th

e lo

wes

t lev

el o

f m

uscl

e ac

tivity

.

To m

easu

re th

e pe

rfor

man

ce

time

of a

pre

scrib

ed u

pper

ex

trem

ity a

ctiv

ity in

four

di

ffer

ent s

eatin

g or

ient

atio

ns

rela

tive

to th

e ve

rtica

l pla

ne

to d

eter

min

e th

e ef

fect

of

body

orie

ntat

ion

on v

olun

tary

m

otor

func

tion.

Aut

hor /

Dat

e / T

itle

Nw

aobi

UM

(198

6).

Effe

cts o

f bod

y or

ient

atio

n in

spac

e on

toni

c m

uscl

e ac

tivity

of p

atie

nts

w

ith c

ereb

ral p

alsy

.

Nw

aobi

OM

(198

7).

Seat

ing

orie

ntat

ions

and

upp

er

extre

mity

func

tion

in c

hild

ren

with

ce

rebr

al p

alsy

.

10

Res

ults

and

Con

clus

ion

The

sadd

le se

at h

as th

e po

tent

ial t

o m

odify

the

qual

ity o

f sea

ting

post

ure

and

reac

hing

mov

emen

ts in

ch

ildre

n w

ith c

ereb

ral p

alsy

. Sa

ddle

ben

ch a

llow

ed

impr

oved

pos

tura

l con

trol a

s m

easu

red

by th

e Si

tting

A

sses

smen

t for

Chi

ldre

n w

ith

Neu

rom

otor

Dys

func

tion.

Res

ults

con

clud

e th

at n

o si

gnifi

cant

diff

eren

ces i

n re

spira

tory

par

amet

ers w

ere

attri

bute

d to

seat

incl

inat

ion

in

eith

er th

e no

rmal

gro

up o

r th

ose

with

CP.

R

esul

ts su

gges

t how

ever

that

in

crea

sed

tidal

vol

ume

and

min

ute

vent

ilatio

n m

ay

incr

ease

d in

ant

erio

r tip

ped

seat

ing,

alth

ough

are

not

st

atis

tical

ly si

gnifi

cant

.

Met

hodo

logy

The

two

expe

rimen

tal c

ondi

tions

w

ere

the

sadd

le se

at (1

5°

forw

ards

incl

inat

ion)

and

a fl

at

woo

den

benc

h.

Clin

ical

ass

essm

ent o

f sea

ted

post

ural

con

trol w

as m

easu

red

by th

e Si

tting

Ass

essm

ent f

or

Chi

ldre

n w

ith N

euro

mot

or

Dys

func

tion.

Res

pira

tion

para

met

ers o

f tid

al

volu

me,

resp

iratio

n ra

te a

nd

min

ute

vent

ilatio

n a

re

com

pare

d in

resp

onse

to tw

o se

ated

pos

ition

s 1)

flat

seat

ing

2) a

nter

ior s

eatin

g (1

0° fo

rwar

d tip

ped)

. R

espi

rato

ry in

duct

ance

pl

ethy

smog

raph

y us

ed to

reco

rd

resp

irato

ry fu

nctio

n.

Parti

cipa

nts

6 ch

ildre

n w

ith m

ild-m

oder

ate

spas

tic C

P.

Abl

e to

sit o

n a

flat b

ench

w

ithou

t hol

ding

on.

In

depe

nden

tly m

obile

via

use

of

wal

ker o

r man

ual w

heel

chai

r.

12 c

hild

ren

(6 n

on-im

paire

d,

mea

n ag

e 9.

7 ; 6

with

spa

stic

C

P), m

ean

age

6.0)

C

hild

ren

with

CP

able

to

ambu

late

eith

er w

ith o

r with

out

mob

ility

aid

s

Stud

y D

esig

n

Rep

eate

d-m

easu

res

expe

rimen

tal c

ross

-ove

r des

ign.

Expe

rimen

tal

Purp

ose

To e

valu

ate

post

ural

con

trol

and

uppe

r ext

rem

ity

mov

emen

t con

trol i

n ch

ildre

n w

ith c

ereb

ral p

alsy

usi

ng a

sa

ddle

seat

.

To in

vest

igat

e th

e ef

fect

s of

usin

g an

ant

erio

r inc

lined

seat

ba

se o

n tid

al v

olum

e,

resp

iratio

n ra

te a

nd m

inut

e ve

ntila

tion

func

tion

of

norm

al c

hild

ren

and

child

ren

with

cer

ebra

l pal

sy.

Aut

hor /

Dat

e / T

itle

Rei

d D

T (1

996)

Th

e ef

fect

s of t

he sa

ddle

seat

on

seat

ed p

ostu

ral c

ontro

l and

upp

er

extre

mity

mov

emen

t in

child

ren

with

cer

ebra

l pal

sy.

Rei

d D

T &

Soc

hani

wsk

j (19

91).

Ef

fect

s of a

nter

ior t

ippe

d se

atin

g on

re

spira

tory

func

tion

of n

orm

al

child

ren

and

child

ren

with

cer

ebra

l pa

lsy.

11

Res

ults

and

Con

clus

ion

Res

ults

con

clud

e de

velo

pmen

t of p

ostu

ral

adju

stm

ent d

urin

g re

achi

ng is

no

n-lin

ear a

nd n

ot fi

nish

ed

until

11

year

s old

. A

ntic

ipat

ory

post

ural

mus

cle

activ

ity ,

cons

iste

ntly

pre

sent

in

adu

lts, w

as b

asic

ally

ab

sent

bet

wee

n 2-

11 y

ears

. Fi

ndin

gs su

gges

t tha

t the

fo

rwar

d til

ted

sitti

ng p

ositi

on

is th

e m

ost e

ffic

ient

rega

rdin

g po

stur

al c

ontro

l. R

esul

ts sh

ow th

at to

nal

sym

met

ry is

rela

ted

to

win

dsw

ept d

efor

mity

alo

ne,

whe

reas

incr

ease

d ag

e, se

vere

sp

astic

ity, a

nd d

irect

ion

of

tona

l asy

mm

etry

wer

e as

soci

ated

with

win

dsw

ept

defo

rmity

and

hip

di

sloc

atio

n.

Prev

alen

ce ra

tes i

nclu

ded

52%

(win

dsw

ept h

ips)

, 25%

(to

nal a

sym

met

ry),

in h

ip

subl

uxat

ion

(63%

) and

hip

su

rger

y (6

3%).

The

side

with

th

e st

rong

est t

one

was

mor

e fr

eque

ntly

dis

loca

ted

of h

eld

in fi

xed

addu

ctio

n.

Met

hodo

logy

29 c

hild

ren

and

10 a

dults

stud

ies

via

EMG

and

kin

emat

ics d

urin

g re

achi

ng in

4 d

iffer

ent

cond

ition

s: si

tting

with

seat

su

rfac

e ho

rizon

tal,

with

and

w

ithou

t tas

k lo

ad, 1

5° fo

rwar

d

of se

at su

rfac

e an

d 15

° ba

ckw

ards

tilt

of se

at su

rfac

e

Dat

a ga

ther

ed fo

rm m

edic

al

reco

rds a

nd p

hysi

cal

exam

inat

ion

(sta

ndar

dise

d)

Parti

cipa

nts

29 h

ealth

y ch

ildre

n, a

ge ra

nge

2-11

. 10

you

ng a

dults

(mea

n ag

e 23

.6±2

yea

rs)

103

subj

ects

with

spas

tic C

P re

crui

ted

from

two

stud

y in

stitu

tions

.

Stud

y D

esig

n

Expe

rimen

tal

Cro

ss-s

ectio

nal s

tudy

Purp

ose

To

inve

stig

ate

the

deve

lopm

ent o

f pos

tura

l ad

just

men

ts a

ccom

pany

ing

reac

hing

mov

emen

ts in

si

tting

chi

ldre

n.

To d

eter

min

e th

e pr

eval

ence

of

win

dsw

ept h

ip d

efor

mity

an

d hi

p di

sloc

atio

n, a

nd th

eir

rela

tions

hip

to a

sym

met

ry o

f m

uscl

e to

ne.

Aut

hor /

Dat

e / T

itle

Van

der

Hei

de JC

, Ote

n B

, van

Ey

kern

LA

& H

adde

rs-A

lgra

(2

003)

. D

evel

opm

enta

l of p

ostu

ral

adju

stm

ents

dur

ing

reac

hing

in

sitti

ng c

hild

ren.

You

ng N

L, W

right

JG, L

am T

P,

Raj

arat

nam

K, S

teph

ens D

, &

Wed

ge JH

(199

8).

Win

dsw

ept d

efor

mit

y in

spas

tic

quad

riple

gic

cere

bral

pal

sy.