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 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. 1 Index of Contents Introduction Methods The relationship between posture, movement, stability and function Normal posture The action of sitting Neutral sitting posture The 90-90-90 position – how functional? Functional Sitting Position Factors affecting positioning Primitive reflex activity Structural asymmetries Abnormal muscle tone Childhood conditions Cerebral Palsy Congenital hip deformity/Developmental Dysplasia Rett Syndrome Duchenne Muscular Dystrophy Spina Bifida Conclusion References Appendices Page 3 4 4 6 6 7 8 9 13 13 15 18 20 20 23 24 25 26 27 28 35 2 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. 3 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 4 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). 5 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. 6 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 7 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 8 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 9 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 saddlebenched 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. 10 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 (semirecline 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 11 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. 12 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 Ø Ø Ø Ø Asymmetrical Tonic Neck Reflex Symmetrical Tonic Neck Reflex Tonic Labyrinthine Reflex Positive Supporting Reaction 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 13 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 14 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 15 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. 16 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 (‘Cshaped’) 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 17 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 18 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. 19 CHILDHOOD CONDITIONS Ø Ø Ø Ø Ø Cerebral Palsy Congenital Hip Deformity / Developmental Dysplasia Rett Syndrome Duchenne Muscular Dystrophy Spina Bifida Cerebral Palsy According to Rodgers et al (2001), Cerebral palsy may defined as a nonprogressive 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. 20 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). 21 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. 22 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. 23 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. 24 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 25 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 26 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). 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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 35 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 36 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 37 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 exp duchenne muscular dystrophy/ AND exp child/ Proquest 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 38 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 39 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 40 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 41 Findings from a multidisciplinary clinical case series of females with rett syndrome. Cass H, Reilly, Owen L, Wisbeach A, Weekes L, Wigram T & Charman T (2003). To gather systematic data from a multidisciplinary clinical assessment case load of females with rett syndrome, to allow clinical manifestations of the disorder to be statistically validated in order to discuss implications regarding management of the condition and directions for future research. To investigate the relationship between proximal and distal motor control using the Postural Fine Motor Assessment Scale for Infants (PFMAI) Case-Smith J, Fisher AG & Bauer D (1989). An Analysis of the Relationship between Proximal and Distal Motor Control. Purpose Author / Date / Title Case-Series design Cas-series design Study Design 87 participants ( 2yeras 1 month – 44 years 10 months) with a confirmed diagnosis of rett syndrome seen between 1993 and 2000. n= 76 (classic rett syndrome) n=11 (atypical presentation of disorder) 60 normal infants (2-6 months: mean 4.4 months) from day care facility tested as measured by the PFMAI. Participants All participants seen in multidisciplinary tertiary health clinic (paediatrician, PT, SLT. OT and music therapist). Assessment areas included: oralmotor skills, feeding problems, growth, breathing problems , pos tural abnormalities and joint deformities, epilepsy, stereotypies and hand movements, self-care, and cognitive and communication skills. Areas assessed by parental/carer reports, direct observation, multidisciplinary examination, clinical reports, video taped assessment sessions and assessment tools e.g. Schedule for Oral Motor Assessment. Posture and fine motor control were measured by the PFMAI, with testing conducted by J Case-Smith and 4 undergraduate students trained in PFMAI administration. Posture observed by the infants ability to independently sustain movement against gravity when in prone and supine. (postural reactions encouraged by attracting infants attention to developmentally appropriate toys. Fine motor skills assessed by eliciting various grasping and hand positions by presenting children with 3 objects. Infants position ed in semi recline. Four minutes of testing allocate d for reach and manipulation of each object Methodology Previously reported trends in presentation confirmed: ↑ poor growth, fixed deformity and scoliosis in adulthood. ↑ mobility in adolescence, follwed by decline in adulthood. High dependency level. Limited cognition/communication skills. Slight improvement in ANS function in adulthood. Feeding difficulties ↑ into middle childhood, reachinf a plateau. Findings confirm that Rett Syndrome is not degenerative and suggests that intervention and support to maintain and increase motor skills, daily living skills and cognitive and communication is appropriate. Results suggest in normal children, there is a significant but weak correlation between proximal control and the development of distal skill, suggesting that [proximal-distal principle may not be an appropriate postulate on which to base treatment / intervention. Significant relationship to support a functional relationship between proximal and distal motor functions. Results and Conclusions 1 Problems of seating and mobility encountered by children with developmental dysplasia of the hip. Cox SL & Mollan RAB (1995) To confirm the clinical suspicion that significant problems are encountered by children and their care -givers in coping the treatment of splinting resulting from late diagnoses developmental dysplasia of the hip (DDH). 2) To compare children ’s and parents’ paediatric pain ratings to determine whether or not concordance exists between the two. 1) To investigate the nature and prevalence of pain in children and adolescents with spina bifida. Clancy CA, McGrath PJ & Oddson BE (2005). Pain in adolescents with spina bifida. Purpose Author / Date / Title Questionnaire focuses on seating and mobility problems. Questions generated from analysis of the literature and information obtained from detailed semi-structured interviews with surgeons and staff involved in treatment. 3 questionnaires sent to patient s in Northern Ireland; 2 in the republic of Ireland; and 6 in Engalnd. Questionnaire (pilot study) (semi structured and open) Prospective study. Study Design Parents of 11 children (2 boys; 9 girls) who were either undergoing or had undergone treatment for late diagnosed DDH, were surveyed by questionnaire. Parents (mean age 43 years 4 months) For comparison purposes sample divided into 2 groups n= 68 children with spina bifida (30 males, 38 females) Mean age 12 years 8 months (range 8-19 years). n=59 (myelomeningocele) n= 6 lipomyelomeningocele n= 3 lipomeningocele. n= 49 shunted hydrocephalus n=26 ambulated full time Children able to communicate in English and had no significant cognitive limitations. Recruited from regional children’s centre. Participants Questionnaires either distributed by post or through parental support groups and were completed by parents. 24 questionnaires distributed, of which 11 were returned (48.8% response rate). Measures administrated by one of two trained Masters level volunteer in separate area from clinic to ensure independent rating. Data collected via parental report questionnaires (The Pediatric Pain Questionnaire) and child report questionnaire (Visual Analogue Scale) and, from t medical records to investigate ain intensity, location, frequency and duration. Methodology This pilot study confirmed need for future focused research to provide products suggested. Results suggest there is a deficiency in equipment available to cope with and manage children in plaster, including products to help in seating. Special emphais is placed on mobility. 56% reported experiencing pain once a week or more often; Pain occurred more frequently in the head, neck, back, abdomen, shoulders, legs and hands. 43∕49 with hydrocephalus reported headaches. 15∕19 without hydrocehalus reported headaches.. Children reporting intense pain, also report ↑ frequency and ↑ pain locations. Parenta most reliable st reporting locality of children’s pain. Findings conclude children with soina bifida report clinically significant, under recognised & untreated pain. Results and Conclusions 2 Development of postural adjustments during reac hing in infants with cerebral palsy. Hadders-Algra M, van der Fits IBM, Stremmelaar EF & Touwen BCC (1999). To analyse the early development of postural adjustments accompanying reaching movements in children with cerebral palsy. Physical, emotional and social problems are the focus of the report. To identify problems related to the treatment (splintage) of DDH in order of priority in families of hildren with DDH. Cox SL & Kernohan WG (1998 ) . They cannot sit properly or move around: seating and mobility during treatment for developmental dysplasia of the hip in children. Purpose Author / Date / Title Longitudinal Questionnaire (semi structured and open) Study Design 7 children (age range 4-18 months). 3 boys; 4 girls 38 questionnaires returned (3 4% response rate. 113 affected families surveyed Participants Reaching movements were assessed via simultaneous recording of video data and surface EMG of arm, neck, trunk and leg muscles during reaching in various positions (lying supine, semi -recline (45°), upright and long leg sitting). 10 questionnaires distributed in England; 3 obtained via interview with parents in childrens ward in hpspital in N. Ireland: 100 distributed to those undergoing or had undergone treatment at the hospital. Questionnaire developed based on the activitied of daily iving that were established for families with children between 3 months and 3 years old. Methodology Results suggest basic orientation of postural adjustments of childr en developing spastic CP was intact, with main problems being ↓ ability to modulate postural adjustment to task specific constraints. The child with spastic athetosis showed distinct abnormalities in basic organisation of postural adjustments. Reaching movements were favoured in semi recline as opposed to upright, supine and long leg sitting 5 developed spastic hemiplegia 1developed spastic tetraplegia 1 developed spastic tetraplegia with athetosis. Such problems may be alleviated by provision of specialised devices that would permit mobility by car, in a pushchair and provide seating in the home. Reslults suggested that parents idenfied social, emotional and physical problems. Problems included size and shape of splint, transporting ans seating problems and disruption of family routines. Results and Conclusions 3 The relationship between tight hamstrings and lumbar hypolordosis in children with cerebral palsy. McCarthy JJ & Betz RR (2000) To assess the influence of tight hamstrings on the saggital alignment of the thoracic and lumbar spine in children with cerebral palsy. To investigate the effects of special seating on lateral spinal curvature in the non ambulant spastic cerebral palsy population with scoliosis. Holmes KJ, Michhael SM & Thorpe (2003). Management of scoliosis with special seating for the non -ambulant spastic cerebral palsy population – a biomechanical study. Purpose Author / Date / Year Retrospective clinical and radiographic review. Prospective study: matched pairs design Study Design 21 participants Mean age: 9.4 years old Inclusion criteria reuired subjects to be non-ambulant, have a scoliosis and require special seating within their wheelchair,. 16 subjects with spastic cerebral palsy 9 boys: 7 girls Man age: 14.7 years (range 6.5 20.8). Participants The popliteal angle was measured to assess hamstring tightness. The Cobb method was used to measure lumbar and thoracic kyphosis. Standing and sitiing lateral spine films were obtained. Shape of spine measured (spinous process angle in an assessment chair (CAPS II). Exerted forcs on chair measured by electrical transducers attached to lateral support pads and seat base. Measurements were taken in 3 alternative arrangements: 1) unsupported upper body 2) two lateral pads at the same height 3) body supported by 3-point force system. Methodology Study concludes that there is a correlation between tight hamstrings and decreasing lumbar lordosis, esp ecially in the seated postion in children with cerebral palsy. A statistical significant correlation was obtained (p<0.01) between the sitting lumbar curve and the popliteal angle. This correlation was less when standing. Significant static correction of the spine (scoliotic) may be achieved via an arrangement of lateral pads on a seating system applying a 3-point force system to the sides of the body. Results and Conclusions 4 The relationship of cumulative motor asymmetries to scoliosis in rett syndrome. McClure MK, Battaglia C & McClure RJ (1997). To investigate the interrelationships between rett syndrome scoliosis and symmetric, asymmetric motor pull, ambulation and advancement of age in order to provide a treatment rationale for slowing the progression of a scoliosis. To investigate the inter relationship of seat -surface inclination on postural stability and functional use of the upper extremities in children with cerebral palsy. McClenagahan BA, Thombs L & Milner M (1992). Effects of seat -surface inclination on postural stability and function of the upper extremities of children with cerebral palsy. Purpose Author / Date / Title Questionnaire Quasi-experimental design Study Design Findings based on 57% response rate. 262 questionnaires completed by International Rett Syndrome Association families. Able to comprehend simple instructions. Screened for visual problems. 20 children (10 non -impaired: 10 mild-moderate spastic cerebral palsy. Age range: 4-15 years. CP children able to sit independently and ambulate with or without mobility aids. Participants Responses were scored independently by two investigators.. 400 questionnaires were distributed via post, 262 were completed and returned (66%). 228 were suitable for statistical analysis (57%). Seat surface inclinations of 0°, 5° (anterior tilt), 5° posterior tilt) with seat to backrest angle at 90° were compared within and between groups, with leg rest position at 90° from the seat surface for all experimental conditions. Methodology A significant relationship was found between the prevalence of rett syndrome scoliosis and orthopaedic risk factors. The findings suggest a treatme nt approach focusing on balancing bilateral muscle pull. Significant between group differences were observed on most dependent measures. No significant difference a could be attributed to seat inclination, however authors suggest anterior tilt may disturb postural stability, without improving upper extremity function.. Results and Conclusions 5 To compare arm movements of persons with and without cerebral palsy and to determine if the alteration of the seat angle of a chair affect quality of movements. To identify a reliable, objective and clinically useful measure for assessing changes in trunk alignment and to evaluate which of five different seated positions was the most effective in encouraging trunk extension. McPherson JJ, Schild R, Spaulding SJ, Barasamian P, Transon C & White SC (1991). Miedaner JA (1990). The effects of sitting positions on trunk extension for children with motor impairment. Analysis of upper extremity movement in four sitting positions: a comparison of persons with and without cerebral palsy. Purpose Author / Date / Title Quasi-experimental Study Design 15 children Age range (2-6 years) Diagnosis of developmental delay and/or severe hypotonic or hypertonic cerebral palsy (diplegia or quadriplegia). Age range; 18-21 years Right hand dominant Able to follow instructions 12 subjects 3 men: 3 women with mild moderate spastic cerebral palsy; 3 men and 3 women with no known pathological conditi on. Participants Tested in 5 random positions during 30 min session (floor sitting in tailor like fashion, level sitting with hips and knees at 90°, bench sitting with bench tilted forward (20°and 30°) and sitting in a commercial chair. Trunk extension measures by the modified Schober Measurement of Spinal Extension (1975). Each child rated on a scale of 1 4 to document trunk control, rated by an experienced paediatric physiotherapist. Arm movements were compared between groups and within groups in four different positions (neutral, wheelchair, posterior tillt (15°) and anterior tilt (15°). Four conditions presented in counterbalanced order. EMG activity recoreded. Methodology Significant differences observed among the five conditions. Study suggests a anterior sitting posture is the preferred position to facilitate increased trunk extension. Quantifiable and qualitative between group differences in reaching. No significant differences could be attributed to the seating positions. Results and Conclusions 6 Improvement of functional sitting position for children with cerebral palsy. Myhr U & von Wendt (1991). To find a functional sitting position for children with cerebral palsy and to compare this position with the children’s original sitting positions with various experimental positions. To create a functional sitting position (FSP) by identifying the essential factors required to reduce spasticity as well as enhancing postural control in children with cerebral palsy. Myhr U & von Wendt (1990). Reducing spasticity and enhancing postural control for the creation of a functional sitting position in children with cerebral palsy: a pilot study. Purpose Author / Date / Title Quasi-experimental Pilot study (single case design) Study Design 23 children (8 female, 15 male) Age range (2-16 years) Child A: 7 years old, spastic diplegia Child B: 15 years old , spastic tetraplegia. 2 children with severe cerebral palsy. Participants Head control, pathological movements, postural control were measured via observation and Sitting Assessment Scale. Children were filmed and photographed in 6 positions (adapted chair; adapted chair and cut out level table in font of child,; FSP without abduction orthosis; adapted chair and abduction orthosis,;FSP without table; FSP with table and abduction orthosis). In FSP seat was forward inclined. Both children filmed and photographed individually in six positions on the same occasion, including sitting in their own adapted chairs and in the proposed FSP. Testing conditions were altered by changing the seat inclination, use/non-use of abduction orthosis and with.without a table in front of them. Total time for postural control was recorded and pathological movements were counted. Methodolgy Results suggest pathological movements are minimised and postural control and upper extremity function are more 3efficient in a forward tipped seat, with a firm backrest supporting pelvis, arms supported against a table and feet permitted to move backward. Greatest reduction of spasticity gained and postural control enhanced when three factors were combined: symmetrical fixation of belt under seat, use of an abduction orthosis and placement in the FSP. This is in addition to seat inclined forwards and arms supported on a table. No discernable effects were identified by seat inclination alone. Results and Conclusion 7 Five year follow-up of functional sitting position in children with cerebral palsy. Myhr U, von Wendt L, Norrlin S & Radell U (1995). To re-examine the reliability of the Sitting Assessment Scale. To re-assess children who were previously introduced to and tested in the FSP five years previously. To obtain a baseline comparison with measurements of children with cerebral palsy. To determine the spontaneous positioning of the lower extremities relative to the movement axis at the knee joint, and to determine thre extent of spontaneous use of the arms for support in different sitting positions, in a group of non-disabled children. Myhr U (1994). Influence of different seat and backrest inclinations on the spontaneous positioning of the extremities of non -disabled children. Purpose Author / Date / Title Retrospective Case Study Study Design 10 children with CP 10 non-disabled children (7 girls; 3 boys) Mean age 6.7 years (range: 4 -9 years). Participants Head, trunk, foot control, arm and hand function were assessed by the Sitting Assessment Scale Ten children were filmed and photographed after the introduction of the FSP and subsequently five years later. The children were filmed and photographed whilst performing standardised tasks in 5 different sitting positions (backrest vertical and seat surface inclination varying between 0° 10° forward inclined, 10° backward reclined, and also in reclined positions in with seat and backrest lean backward from the horizontal plane and vertical plane, respectively (15 °). Methodology Study concludes that the FSP contributes to improved ability to use the upper extremities (hand and arm function). 8 out of the 10 children assessed, who used the FSP over the five year period, showed slight but significant improvement, the remaining two children had deteriorated. Results revealed that in positions with the backrest vertical and with the use of a hip belt, all children held their feet posterior to the knee joint axis regardless of seat inclination. Results and Conclusions 8 Electromyographic investigation of extensor activity in cerebral -palsied children in different seating positions. Nwaobi OM, Brubaker CE, Cusick B & Sussman (1983). To determine if the myoelectric activity of the extensor muscles of the lumbar spine is affected by positions of the seat surface and seat back, or by their positions relative to one another. To compare the effects of adaptive and non adaptive seating on pulmonary function. Nwaobi OM & Smith (1986). Effect of adaptive seating on pulmonary function of children with cerebral palsy. Purpose Author / Date / Tilte Experimental Quasi-Experimental Study Design 11 children (7 boys: 4 girls) with spastic CP. Age range: 4-8 years 8 children with spastic CP Age range: 5-12. Non-ambulant No apparent evidence of intrinsic lung disease. Participants Using surface electrodes EMG activity was recorded for the lumbar extensor spinae muscles in seven different testing conditions. Seat surface inclinations of 0° and 15° combined with backrest inclinations of 75°, 90°, 105°, and 120°.. Children were positioned in 90-90-90 in both seating units. Vital capacity, forced expiratory volume in one second, and expiratory time as measure by a spirometer , of children with CP were measured in a typical sling back wheelchair and in a wheelchair with modular inserts. Methodology Results highlighted that extensor activity was lowest when backrest inclination remained at 90° and the seat surface at 0° (upright sitting). Preliminary finding from study suggests that the orientation of the head /neck/body in relation to gravity may play an important role in controlling extensor activity. Results suggest implications for speech, sitting for prolonged periods and the prevention of pulmonary hypertension. Results of the study suggest that pulmonary function was higher in adaptive seating compared to non -adaptive seating. Results and Conclusions 9 Seating orientations and upper extremity function in children with cerebral palsy. Nwaobi OM (1987). To measure the performance time of a prescribed upper extremity activity in four different seating orientations relative to the ve rtical plane to determine the effect of body orientation on voluntary motor function. To determine if tonic activity of these muscles change in response to body orientation, and which body orientation provides the lowest level of muscle activity. To measure the tonic myoelectric activity of the low back extensors, hip adductors and ankle plantar flexors in two body orientations. Nwaobi UM (1986). Effects of body orientation in space on tonic muscle activity of patients with cerebral palsy. Purpose Author / Date / Title Quasi-experimental Experimental Study Design 13 children with CP 3 athetoid CP 10 spastic CP Age range: 8-16 years Unable to ambulate independently Require adaptive seating for upright positioning Fair-poor gross upper extremity control. Fair head and trunk con trol Poor fine motor skills. 12 children (8 boys: 4 girls) Age range: 6-18 years Diagnosis of mild-moderate spastic diplegia. Fair head and trunk control Fair to poor fine motor skills No fixed deformity. Participants The children were placed randomly in different seating orientations (30°, 15°, and 0° posterior inclination and 15°anterior inclination). The seating positions were 0° in vertical plane and 30° from the vertical plane. Each participant was position ed in 90-90-90 with the use of a pommel prior to testing. Surface electrodes were used to measure myoelectric activity if the low back extensors, hip adductors and ankle planta r flexor muscles in two seating positions. Methodology Results conclude orientation of the body in space affects upper extremity function. The level of upper extremity performance was highest in the upright position. Results demonstrated that muscle activity was affected by body orientation, with tonic muscle activity lower in the upright position with statistically significant differences for the hip adductors and back extensors. Extensor tone may increase in the reclined position. Results and Conclusions 10 Effects of anterior tipped seating on respiratory function of normal children and children with cerebral palsy. Reid DT & Sochaniwskj (1991). To investigate the effects of using an anterior inclined seat base on tidal volume, respiration rate and minute ventilation function of normal children and children with cerebral palsy. To evaluate postural control and upper extremity movement control in children with cerebral palsy using a saddle seat. Reid DT (1996) The effects of the saddle seat on seated postural control and upper extremity movement in children with cerebral palsy. Purpose Author / Date / Title Experimental Repeated-measures experimental cross -over design. Study Design Children with CP able to ambulate either with or without mobility aids 12 children (6 non-impaired, mean age 9.7 ; 6 with spastic CP), mean age 6.0) Able to sit on a flat bench without holding on. Independently mobile via use of walker or manual wheelchair. 6 children with mild-moderate spastic CP. Participants Respiration parameters of tidal volume, respiration rate and minute ventilation are compared in response to two seated positions 1) flat seating 2) anterior seating (10° forward tipped). Respiratory inductance plethysmography used to record respiratory function. Clinical assessment of seated postural control was measured by the Sitting Assessment for Children with Neuromotor Dysfunction. The two experimental conditions were the saddle seat (15° forwards inclination) and a flat wooden bench. Methodology Results suggest however that increased tidal volume and minute ventilation may increased in anterior tipped seating, although are not statistically significant. Results conclude that no significant differences in respiratory parameters were attributed to seat inclination in either the normal group or those with CP. Saddle bench allowed improved postural control as measured by the Sitting Assessment for Children with Neuromotor Dysfunction. The saddle seat has the potential to modify the quality of seating posture and reaching movements in children with cerebral palsy. Results and Conclusion 11 To investigate the development of postural adjustments accompanying reaching movements in sitting children. To determine the prevalence of windswept hip deformity and hip dislocation, and their relationship to asymmetry of muscle tone. Van der Heide JC, Oten B, van Eykern LA & Hadders -Algra (2003). Young NL, Wright JG, Lam TP, Rajaratnam K, Stephens D, & Wedge JH (1998). Windswept deformit y in spastic quadriplegic cerebral palsy. Developmental of postural adjustments during reaching in sitting children. Purpose Author / Date / Title Cross-sectional study Experimental Study Design 103 subjects with spastic CP recruited from two study institutions. 10 young adults (mean age 23.6±2 years) 29 healthy children, age range 2-11. Participants Data gathered form me dical records and physical examination (standardised) 29 children and 10 adults studies via EMG and kinematics during reaching in 4 different conditions: sitting with seat surface horizontal, with and without task load, 15° forward of seat surface and 15 ° backwards tilt of seat surface Methodology Results show that tonal symmetry is related to windswept deformity alone, whereas increased age, severe spasticity, and direction of tonal asymmetry were associated with windswept deformity and hip dislocation. Prevalence rates included 52% (windswept hips), 25% (tonal asymmetry), in hip subluxation (63%) and hip surgery (63%). The side with the strongest tone was more frequently dislocated of held in fixed adduction. Results conclude development of postural adjustment during reaching is non-linear and not finished until 11 years old. Anticipatory postural muscle activity , consistently present in adults, was basically absent between 2 -11 years. Findings suggest that the forward tilted sitting position is the most efficient regarding postural control. Results and Conclusion