27 LOWER LIMB FUNCTION

Pam Thomason, Jill Rodda, Kate Willoughby, and H Kerr Graham

Introduction anteversion (FNA) increases from GMFCS level I to level III The 2006 definition of cerebral palsy (CP) has been widely and then plateaus at a mean of 40 degrees in GMFCS levels accepted and is recommended as the most useful operational III, IV and V. Mean neck shaft angle, increases stepwise from definition of CP (Rosenbaum et al. 2007). This definition GMFCS level I through to GMFCS level V (Robin et al. 2008). includes the term ‘secondary musculoskeletal problems’ as The incidence and severity of hip displacement is directly pre- part of the spectrum of features seen in CP. This recognizes dicted by GMFCS level. In our population-based study, chil- the impact of musculoskeletal pathologies on functioning dren in GMFCS level I showed no hip displacement and those for persons with CP. The focus of this section will be the in GMFCS level V had a 90% incidence of having a migration effects of the brain lesion on the motor function of the lower percentage in excess of 30% (Soo et al. 2006). The relationship limbs, pelvis, and spine. The authors work together in a gait between GMFCS and hip displacement has implications for laboratory and hip surveillance clinic with a commitment screening and management protocols. to provide clinical services and to improve the evidence base Knowing a child’s GMFCS level is fundamental in by clinical research. The framework for this section will be establishing gross motor prognosis and monitoring changes based on the World Health Organization International (Rosenbaum et al. 2002). Changes in GMFCS levels should be Classification of Functioning (ICF) and the Gross Motor carefully documented. The GMFCS may not be stable in the Function Classification System (GMFCS) (Palisano et al. 1997, very young child. However, the most common reason for a World Health Organization 2001). change in GMFCS is an error in interpretation of the previous or current examination. Given that the GMFCS is a categorical Classification: Gross Motor Function grading system, true changes in GMFCS level sometimes Classification System occur and these may occur in both directions, that is improve- The development of the GMFCS has for the first time given ment or deterioration. After major intervention such as selec- clinicians and parents a common language to communicate tive dorsal rhizotomy (SDR) or Single Event Multilevel Surgery about cerebral palsy (Figs 27.1 and 27.2) (Palisano et al. 1997, (SEMLS), a small number of children move up a level. This is 2008). The GMFCS is a classification system and it is essential uncommon and should not be expected in more than 5% to when discussing gross motor function in children with CP. It 10% of children (Rutz et al. 2012b). Deterioration in GMFCS should be used alongside the classification of upper limb func- level is more common. For example, lengthening of the Achilles tion (Manual Ability Classification System) and communica- tendons in children in GMFCS level II can result in progressive tion abilities (Communication Function Classification System) crouch gait, and the need for assistive devices. For these chil- to provide the essential context for considering the individual dren, their gait and function deteriorates and GMFCS level child’s prognosis, goal setting, and management (Eliasson et al. changes from II to III (Rodda, Graham, and Galea 2006). 2006, Hidecker et al. 2011). For example, a child classified in GMFCS level IV aged between 6 and 12 years may perform Motor curves and cerebral palsy some stepping with a heavily adapted walker, under the supervi- The development of gross motor function in children with sion of a therapist or parent. However, following the pubertal CP can be described by a series of curves (Rosenbaum et al. growth spurt, useful walking is not sustained. It would be inap- 2002). Understanding the position of a child’s development propriate to offer such children invasive treatments to improve in relation to their gross motor curve provides a rational basis or prolong walking because these will not be successful in the for the understanding of management strategies, goal setting, long term. A more appropriate goal would be maintain stand- and long-term gross motor function (Hanna et al. 2008). For ing transfers. example, a 2-year-old child in GMFCS level II with signs of The GMFCS has strong predictive value in other domains. bilateral spastic CP, is treated with a physiotherapy programme, Certain musculoskeletal features and deformities are closely ankle–foot orthoses (AFOs), and injections of botulinum related to GMFCS level. The shape of the proximal femur neurotoxin-A (BoNT-A) to the gastrocsoleus and hamstring shows a strong correlation with GMFCS level. Femoral neck muscles. Within 3 months, the child is noted to have progressed I

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Fig. 27.1. Gross Motor Function Classification System (GMFCS) E & R between 6th and 12th birthday: descriptors and illustrations.

from standing with support to independent walking. Whilst forms of intervention in early childhood in children with CP the intervention programme may well have contributed to these is the mistaken attribution of improvements in gross motor gains in gross motor function, the child is at the stage of rapid function to the intervention, when natural history has such a acquisition of gross motor function with or without interven- large effect. Association is not causation. tion. This underlines the need for intervention studies in the In most children gross motor function reaches a plateau I first 6 years of life to be controlled. The popularity of many be­tween 3 and 6 years with some regression in later childhood

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Fig. 27.2. Gross Motor Function Classification System (GMFCS) E & R between 12th and 18th birthday: descriptors and illustrations.

(Fig. 27.3). One of the causes for this regression in gross motor cohort studies are less liable to misinterpretation than in the function is progressive musculoskeletal pathology (Graham birth to 6 years age group. 2004). After age 6 years, gait parameters deteriorate as contractures Sagittal gait patterns: unilateral spastic cerebral palsy and bony deformities increase. Changes in gross motor func- In 1987, Winters, Gage, and Hicks classified the sagittal gait tion and gait during the plateau/early decline phase, can be patterns in unilateral spastic CP, (hemiplegia) in a cross- more realistically attributed to intervention. Longitudinal sectional study (Winters, Gage, and Hicks 1987). Their four I

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Fig. 27.3. Gross motor curves in children with cerebral palsy and the five levels of the Gross Motor Function Classification System (GMFCS) modified from Hanna (2008).

group classification has been extensively used by clinicians as a Sagittal gait patterns: bilateral spastic cerebral palsy template to guide clinical management, the prescription of Sagittal gait patterns in bilateral spastic CP, (diplegia), can be orthoses, spasticity management by injection of BoNT-A, and classified as true equinus, jump gait, apparent equinus, and musculoskeletal surgery (Fig. 27.4). crouch gait (Rodda et al. 2004) (Fig. 27.5). There is a group of patients in whom the gait deviations in the sagittal plane TYPE I HEMIPLEGIA are minimal but who have substantial transverse plane issues, In type I hemiplegia there is a drop foot in the swing phase usually internal hip rotation. This gait pattern has been of gait which is due to loss of selective motor control described as ‘mild gait’. and/or weakness in tibialis anterior. There is no contracture of the gastrocsoleus and the second rocker in gait is relatively TRUE EQUINUS normal. True equinus is characterized by walking on tip toe with the knees and hips extended. It is commonly seen in younger TYPE II HEMIPLEGIA children with bilateral spastic CP when they first learn to walk. In type II hemiplegia, there is equinus in stance phase and foot The plantarflexion-knee-extension couple is overactive and the drop in swing phase. There is spasticity in the gastrocsoleus ground reaction force (GRF) is in front of the knee throughout which gradually becomes fixed resulting in a contracture. the stance phase of gait.

TYPE III HEMIPLEGIA JUMP GAIT In type III hemiplegia, there is equinus at the ankle, with Jump gait is characterized by equinus at the ankle associated reduced range of motion at the knee and co-contraction of the with incomplete extension at the knee and hip in mid-stance. hamstrings and rectus femoris. There may be excessive flexion at initial contact with rapid extension in later stance to near normal range. In other chil- TYPE IV HEMIPLEGIA dren, there is more severe knee flexion throughout stance, com- In type IV hemiplegia, there is equinus at the ankle, a flexed, bined with incomplete hip extension. This is the most common stiff knee, and involvement at the hip, with incomplete hip pattern in the pre-adolescent. extension. In the coronal plane there is excessive hip adduction and in the transverse plane, excessive internal rotation and APPARENT EQUINUS pelvic retraction. Contractures are present as seen in type III Apparent equinus is characterized by toe walking but with with the addition of hip adductor and hip flexor contracture near normal ankle range of motion. There is flexion present at I and the risk of hip dysplasia. the knees and hips. The recognition of ‘apparent equinus’ in

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Fig. 27.4. Sagittal gait patterns in spastic hemiplegia.

contradistinction to ‘true equinus’ is very important to avoid patients with a posterior pelvic tilt. When the pelvis is in the inappropriate lengthening and weakening of the gastrocsoleus. neutral range, the hamstrings are of normal length and when The child’s ‘apparent equinus’ is due to spasticity and contrac- the pelvis is anteriorly tilted, the hamstrings are excessively tures at the knee and hip levels. Instrumented gait analysis long. Without the use of IGA and the modelling of muscle (IGA) is very helpful in differentiating ‘apparent equinus’ from lengths, it is very difficult to appreciate these findings. The ‘true equinus’. The ‘apparent equinus’ pattern is often transi- popliteal angle is decreased, the hamstrings feel tight and are tional. With further growth and progression of lever arm therefore incorrectly assumed to be short (Rodda, Graham, and deformities, the majority of children will develop ‘crouch gait’ Galea 2006). (Thomason et al. 2012). Assessment/measurement (see also Chapter 18) CROUCH GAIT The World Health Organization’s ICF describes health condi- Crouch gait is characterized by excessive knee flexion in stance tions in several domains, including body structure and func- phase, incomplete extension at the hip and excessive ankle tion, activities and participation (World Health Organization dorsiflexion (calcaneus). Knee stiffness in swing is common. 2001). These domains are influenced by environmental factors The soleus is excessively long and usually weak. This is a very and personal factors. Various tools exist to measure parameters common gait pattern in adolescence and may be part of the relevant to CP in the ICF domains and new measurement tools natural history of bilateral spastic CP but is more often caused are being developed. When considering measurement of the by isolated tendo Achilles lengthening (TALs) (Rang 1990, lower limb in children with CP it is important to consider all Rodda, Graham, and Galea 2006, Stout et al. 2008, Vuillermin of the components of the ICF. There are valid and reliable tools et al. 2011). A key feature of true Crouch Gait is that the to classify and measure some aspects of gross motor function majority of muscle-tendon-units (MTU) are excessively long. in the lower limbs (Fig. 27.6). This is by definition true for all of the one-joint muscles such It is important to choose the correct measurement tool to as soleus, vasti, and gluteus maximus and often for the two- use at any given time or following an intervention. Choice of joint hamstrings. The only consistent contractures are of the measurement tool should be based on the psychometric proper- iliopsoas. In crouch gait, the hamstrings are short only in ties of the tool, the aspect of the ICF being measured as well I

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Fig. 27.5. Sagittal gait patterns in spastic diplegia.

as the age and GMFCS level of the child. A detailed discussion and recording of gait from multiple viewpoints, can be replayed of measurement tools is beyond the scope of this chapter. Some in slow motion and can be reviewed repeatedly. In an effort to of the tools that we use commonly are discussed below. quantify and objectify the outcome of observational gait analy- sis, a number of gait scores have been developed of varying Body structure and function degrees of complexity, sophistication, and reliability. These INSTRUMENTED GAIT ANALYSIS include the Physician Rating Scale, the Observational Gait Evaluation of gait and functioning in children with CP can be Scale and the Edinburgh Visual Gait Score (Koman, Mooney, considered in the format of a diagnostic matrix (Davids et al. and Smith 1993, Mackey et al. 2003, Read et al. 2003, Wren 2003). The role of IGA is crucial to the evaluation of gait et al. 2005). dysfunction, especially in relation to planning and assessing the outcome of major interventions such as SDR and SEMLS. As LONGITUDINAL ASSESSMENTS WITH RADIOLOGY: IGA becomes more reliable, cheaper, and more accessible, it is HIP SURVEILLANCE anticipated that assessment quality for children with CP and Mercer Rang suggested many years ago that all children with the outcomes based on such assessments, will continue to CP should have regular hip examinations and radiographs improve (Thomason et al. 2012). (Rang 1990). The goal was to prevent dislocation by early detection and early preventive (adductor release) surgery. VIDEO GAIT ANALYSIS Several centres in Europe and Australia have developed these Video gait analysis (VGA) is a central part of the diagnostic concepts into formal ‘hip surveillance programmes’ (Dobson matrix. A visual record of a child’s gait and functioning on et al. 2002, Hagglund et al. 2005, Kentish et al. 2011). Children digital video can be of much greater value than observational with a confirmed diagnosis of CP are offered regular clinical gait analysis and a written report. Digital video can be archived and radiographic examination of their hips and access to both in a permanent fashion, is objective and can be shared by mul- preventive and reconstructive surgery. In both Victoria, Australia I tiple observers over a long period of time. It allows observation and southern Sweden, the prevalence of late dislocation has

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Fig. 27.6. Outcome measures per International Classification of Functioning domain used in the lower limb in cerebral palsy.

decreased and the need for salvage surgery has been reduced and to monitor hip displacement both before and after opera- (Dobson et al. 2002, Hagglund et al. 2005). tive intervention. In children with CP, the migration percentage In children with CP, the hip is normal at birth and then increases by 2% to 10% per year, depending on GMFCS level, displaces because of limitations in activity, accompanied by soft until the migration percentage reaches about 50%. At this point tissue contractures and bony deformities. Factors which may the displacement increases rapidly and is associated with pro- contribute to hip displacement include increased muscle forces gressive deformities of the femoral head and acetabulum, fol- across the hip, which have been modelled to be increased six- lowed by loss of articular cartilage and degenerative arthritis fold (Miller et al. 1999). The shape of the proximal femur is (Graham 2004). The incidence of hip displacement (migration also important in terms of torsion (femoral neck anteversion, percentage >30%) is 35% of children with CP in population- (FNA) and neck shaft angle (NSA)) (Robin et al. 2008). based studies and is directly related to the child’s GMFCS level The most useful radiographic index for measuring hip (Soo et al. 2006, Hagglund, Lauge-Pedersen, and Wagner displacement in children with CP is the migration percentage 2007, Connelly et al. 2009). Early hip displacement is silent of Reimers (1980). This measures the percentage of the femoral and formal screening by radiographs of the hips, with careful head which lies outside the acetabulum. Migration percentage positioning is advised. The frequency of such radiographs can be reliably measured from hip radiographs, taken in supine should be directly related to the risk of hip displacement, which with good positioning and a standardized technique (Parrott et is in turn related to the child’s GMFCS level. In GMFCS level al. 2002). The measurement error should be less than 10% and I the risk of hip displacement is very low and radiographs are serial measurement of migration percentage is the most useful only required if there are findings on clinical examination means to study hip displacement in children with CP. It is the raising concerns. More regular radiographs are required in key index for making decisions regarding surgical management GMFCS levels II and III. Radiographs every 6 to 12 months I

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may be required in GMFCS levels IV and V to monitor pro- (PEDI) (Haley 1997) are measures frequently used to assess gressive hip displacement. Evidenced-based recommendations motor function. The PEDI may be a more appropriate tool for on hip surveillance in children with CP have been published use with children in GMFCS levels IV and V. and can be downloaded from www.ausacpdm.org.au (Wynter et al. 2011). Management CP is the most common cause of the upper motor neurone Activity syndrome (UMNS) in childhood, with positive features such FUNCTIONAL MOBILITY SCALE (SEE APPENDIX 5) as spasticity, hyper-reflexia, and co-contraction and negative The Functional Mobility Scale (FMS) describes the level of features such as weakness, loss of selective motor control, assistance a child requires to mobilize over three different sensory deficits and poor balance (Burke 1988). The negative distances, in three different environments: the home (5m), features have a greater influence on locomotor prognosis school (50m), and the community (500m) (Graham et al. (Hutchinson and Graham 2008). Weakness and loss of selective 2004). Three numbers are assigned depending on the level of motor control determine when or if a child will walk. Weakness assist­ance required in each of these settings. For example, a child and balance deficits may dictate long-term dependence ona in GMFCS level III is frequently capable of independent walking aid. The CP brain lesion is a ‘static encephalopathy’. walking in a sheltered familiar environment such as the home. This is in contrast to musculoskeletal pathology in the limbs, The same child may require the use of elbow crutches to move which is progressive and constantly changing during growth around in the school environment but these may be too slow and development (Graham 2004). to keep up with the rest of the family during trips to the shop- In the absence of biological therapies to correct the brain ping mall, when a wheelchair may be preferred. The FMS lesion, management of children with CP is directed to reducing grading for such a child is 5,3,1. The FMS was designed as an the effect of the positive features of the UMNS such as spastic- outcome measure and is sensitive to change (Harvey et al. 2007, ity and to assist the child to compensate for the negative effects Harvey et al. 2009). For example, children in GMFCS level III such as weakness. Treatment is also aimed at reducing the often require a posterior walker to ambulate, prior to multilevel effects of the progressive musculoskeletal deformities which surgery. After optimum biomechanical realignment and correc- result from growth and maturation. tion of spastic contractures, these children can often progress to lesser levels of support. Some will be able to walk increasing PROGRESSIVE MUSCULOSKELETAL PATHOLOGY distances independently; others will require crutches or sticks In children with CP the musculoskeletal pathology is pro­ when previously they were dependent on a posterior walker. gressive. Chronic neurological impairment affects the growth These important changes can be monitored and reported using and development of muscles and bones. The key feature of the the FMS. musculoskeletal pathology in CP is failure of longitudinal growth of skeletal muscle, ‘short muscle disease’ (Graham and FUNCTIONAL ASSESSMENT QUESTIONNAIRE Selber 2003, Graham 2004). The conditions for normal muscle The Functional Assessment Questionnaire (FAQ) is a 10-level, growth are regular stretching of relaxed muscle, under physio- parent report walking scale, which describes a range of walking logical loading conditions and normal levels of activity. In abilities across the entire spectrum of CP, from non-ambulatory children with CP, skeletal muscle does not relax during activity to independent ambulation at a high level (Novacheck, Stout, because of spasticity and the children have greatly reduced levels and Tervo 2000). In addition to the 10-level walking scale, of activity because of weakness and poor balance. The newborn there is an additional list of 22 items describing a variety of child with CP does not have contractures or lower limb deform- higher level functional activities requiring varying degrees of ities and most do not show signs of spasticity. With time spas- walking ability, balance, strength, and coordination. The FAQ ticity develops, activity levels are reduced, the growth of is a valid and reliable scale and has been shown to be sensitive muscle-tendon-unit (MTU) lags behind bone growth and con- to change. It is a simple scale, which can be quickly completed tractures develop. Although the MTU is often short in CP, by parents or care-givers and provides an excellent longitudinal muscle fibres may not be short. Paradoxically in teenagers with view of the child’s gross motor and walking abilities. crouch gait, the majority of lower limb the muscle-tendon-units The FMS and FAQ are complementary scales and are both are excessively long. Only the iliopsoas and occasionally the gaining increasingly widespread acceptance and use in assessing hamstrings are short. The soleus, vasti, and hip extensors are children with CP as baseline measures, longitudinal measures excessively long indicating that disordered biomechanics play a and as outcome measures after intervention. profound role in the relationship between MTU length and bone length (Graham and Selber 2003). GROSS MOTOR FUNCTION Torsional deformities develop in the long bones and The Gross Motor Function Measure (GMFM) (Russell et al. instability of joints including the hip and subtalar joint may I 1989) and the Pediatric Evaluation of Disability Inventory develop, secondary to growth abnormalities and disordered

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Fig. 27.7. Musculoskeletal management algorithm for children and adolescents with cerebral palsy.

biomechanics. Eventually premature degenerative arthritis may spastic CP, which confirm that the musculoskeletal pathology develop. Torsional deformities in long bones, hip subluxation and the attendant gait disorder are progressive during child- and midfoot breaching are referred to as lever arm deformities. hood (Johnson, Damiano, and Abel 1997, Bell et al. 2002, Muscles act on bony levers to produce a moment which, if Gough et al. 2004). These studies provide an important insight great enough, will induce movement about the joint. If the lever into natural history and a framework to interpret the results of is mal-directed (tibial torsion) or the fulcrum unstable (hip management strategies in the lower limbs. subluxation) the moment generating ability of the muscle is reduced. Lever arm deformities may then result in lever arm PHYSIOTHERAPY IN MULTIDISCIPLINARY MANAGEMENT disease. In the lower limbs, external tibial torsion combined OF THE LOWER LIMB IN CEREBRAL PALSY with pes valgus may impair the plantarflexion moment of the Management of children with CP is multidisciplinary with gastrocsoleus. This may contribute to defective plantarflexion- physiotherapists, occupational therapists, speech pathologists, knee-extension coupling and crouch gait (Graham 2010). psychologists, and teachers, working together with physicians A management algorithm is represented in Fig. 27.7. An and surgeons to optimize the potential of the individual child important therapeutic window exists for spasticity manage­ (Rang 1990). Physiotherapy is the most popular and widely ment before the development of fixed contractures (Hutchinson used management strategy for the lower limb in children with and Graham 2008). A second therapeutic window exists for CP and is highly valued by the parents. It involves the use of the correction of fixed musculoskeletal deformities, before a variety of interventions. The physiotherapy management of the onset of decompensation (Rang 1990). There are three children with CP has been based on a variety of theoretical important longitudinal studies of gait in children with bilateral perspectives. Physiotherapists use biomechanical approaches to I

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maintain range of motion and muscle length. Techniques used include passive and active ranging of joints, stretching of muscles, splinting, and casting. Serial casting can be combined with BoNT-A injections for dynamic contractures (Hutchinson and Graham 2008). The introduction of a suitable AFO is often a critical step in providing an improved base of support and achieving progress in a child’s ability to stand and walk. Physiotherapists use approaches such as neurodevelopmen- tal treatment based on the work of Dr and Mrs Bobath, motor learning and conductive education (Hari and Akos 1988, Valvano and Long 1991, Catanese et al. 1995, Shepherd 1995). Physiotherapists have also begun to move away from passive interventions towards activity-focused interventions which reflect recent views of motor learning theories. The importance of activity-based interventions is increasing in focus (Damiano 2006). More recently, Progressive Resistance Strength Training Fig. 27.8. The spasticity compass for children with cerebral palsy. (PRST) has been used to increase muscle strength and endur- ance (Damiano, Dodd, and Taylor 2002, Dodd, Taylor, and needs to be carefully planned with a team approach involving Graham 2003, Verschuren et al. 2011). Treadmill training the child’s community therapist. Additional therapy may be is becoming an increasingly popular intervention to improve provided within the tertiary hospital, rehabilitation centre or, walking speed and endurance (Damiano and DeJong 2009, where possible, the child’s home. Mutlu, Krosschell, and Gaebler-Spira 2009, Willoughby et al. 2010). These modalities are rarely utilized in isolation. Spasticity MOVEMENT DISORDER MANAGEMENT AND management with BoNT-A, intrathecal baclofen (ITB), and THE SPASTICITY COMPASS SDR may also improve outcomes (McLaughlin et al. 2002, The ‘spasticity compass’ can be used to classify and compare Albright 2008, Ward 2009). It is increasingly difficult to deter- interventions for spasticity management as focal or generalized mine which combinations are best for children with CP. The in their effect and as temporary or permanent (Fig. 27.8). Each approaches used by physiotherapists will be influenced by many intervention can then be located in the appropriate quadrant. factors and must be evidenced based. Intervention requires For example, oral medications are general (all nerves or all sensitivity to the child’s age, cognitive, sensory, and perceptual muscles in all body areas) but temporary in effect (Ward 2009). factors as well as type and severity of CP. The treatment setting, Selective dorsal rhizotomy (SDR) is a neurosurgical procedure whether based in a hospital, community centre, at school or in which 30% to 50% of the dorsal rootlets between L1 and home, and the focus of these services are also important factors. S1 are transected for the permanent relief of spasticity in a The child’s environment, including family, cultural, and con- highly selected group of children with bilateral spastic CP textual factors must be considered. Interventions will be less (McLaughlin et al. 2002, Albright 2008). The principal effects effective if they are not carried over into the child’s everyday are on the lower limbs although there may be minor effects on environment at home. Interventions need to be planned using the upper limbs. The position on the grid is therefore perma- a family-centred, problem-based approach, clinical reasoning, nent and half way between general and focal. and best available evidence. Management plans should be nego- ITB is the most effective current method available for tiated with the child and their family/carers and, most impor- the management of severe spasticity, dystonia and mixed move- tantly, outcomes of the intervention should be evaluated. ment disorders in CP and has been reviewed extensively in Physiotherapy programmes also provide education and several recent publications (Butler and Campbell 2000, Albright emotional support to parents coping with the diagnosis of CP 2008). and starting the journey through the uncharted waters of Chemodenervation is invaluable in the management of raising a child with a life-long physical disability (Rang 1990). focal spasticity and dystonia. Phenol neurolysis was much Coordination of access to other services including physical more widely utilized before the introduction of BoNT-A. The medicine and rehabilitation, orthotics, and orthopaedic surgery principal limitation on its use is pain at the site of injection are also integral to the role of the physiotherapist. Parents will and post injection dysesthesia. This is because phenol is not often seek the view of the child’s therapist on recommendations selective and has the same effect on sensory nerve fibers as for spasticity management, the type of orthosis and the timing motor fibres. Hence its use in mixed nerves in children with and type of surgical intervention. The need for clear commu- CP is very limited. However, in the lower limb in CP the nication and teamwork within the multidisciplinary team is principal application is neurolysis of the obturator nerve for I obvious. When major interventions are scheduled, therapy adductor spasticity as these nerves have such limited sensory

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distribution and precautions for use of Phenol do not apply effects including temporary incontinence and dysphagia have (Khot et al. 2008). been reported. Dysphagia, aspiration, and chest infection are the most serious complications after injection of BoNT-A and BOTULINUM NEUROTOXIN-A IN THE MANAGEMENT if unrecognized or inadequately treated could lead to death OF THE LOWER LIMB IN CEREBRAL PALSY from asphyxia (Naidu et al. 2010). Injection of skeletal muscle with BoNT-A results in a dose- Recent concerns from studies in animal models and from dependent, reversible chemodenervation, by blocking presyn- clinical studies in children have raised concerns about denerva- aptic release of acetylcholine at the neuromuscular junctions. tion atrophy (Fortuna et al. 2011). The mechanism of action Because of the neurotoxin’s rapid and high affinity binding to of BoNT-A dictates that a clinically successful injection will be receptors at the neuromuscular junctions of the target muscle, followed by a period of muscle weakness and muscle atrophy. little systemic spread of neurotoxin occurs. Neurotransmission What is not yet understood is the duration of atrophy and the is restored initially by sprouting of new nerve endings, but these long-term implications for muscle growth and function. In are eliminated at about 3 months when the original nerve adolescents, the management of weakness is a much greater endings regain their ability to release acetylcholine (de Paiva et challenge than the management of spasticity, in GMFCS levels al. 1999). BoNT-A may be useful in children with CP to II and III (Gough, Fairhurst, and Shortland 2005). Until the manage dynamic gait problems and to delay the need for ortho- long-term implications of denervation atrophy are more paedic surgery until the child is older (Koman, Mooney, and clearly understood, it would be prudent to limit the use of Smith 1993). BoNT-A in the antigravity muscles of ambulant children with The most common and most important indication for CP. Both the dose and frequency of injections should be BoNT-A therapy in ambulatory children with CP is the injec- reduced to the absolute minimum required to achieve the tion of the gastrocsoleus for spastic equinus (Graham et al. desired functional goals. 2000). Before widespread use of BoNT-A for spastic equinus, the majority of children with CP who walked on their toes had BOTULINUM NEUROTOXIN-A AS AN ANALGESIC AGENT a lengthening of their Achilles tendons by age 4 to 6 years. This IN CEREBRAL PALSY resulted in progressive crouch gait which was much more disa- BoNT-A can be used to treat muscle spasm following oper- bling than the original equinus gait (Vuillermin et al. 2011). A ative procedures, such as adductor release surgery. Injection of non-operative programme of care utilizing BoNT-A should be BoNT-A can be useful for short-term relief of pain associated viewed as complementary to orthopaedic surgical reconstruc- with hip displacement. Target muscles include the hip tion and not as an alternative. Information about dosing, dilu- adductors, medial hamstrings, and hip flexors. Pain relief is tion, muscle targeting, and safety have been published elsewhere associated with a decrease in spastic adduction and scissoring (Cosgrove, Corry, and Graham 1994, Graham et al. 2000, postures (Barwood et al. 2000). Some children with neglected Naidu et al. 2010). hip displacement have limited life expectancy and may not Injection of BoNT-A for spastic equinus increases the survive salvage surgery. BoNT-A may provide useful palliation dynamic length of the gastrocsoleus with improvements in ankle in such circumstances. Better still is to prevent painful hip dorsiflexion during gait, as determined by the Physician Rating displacement. Scale (Cosgrove, Corry, and Graham 1994, Koman et al. 1994, 2000, Boyd and Graham 1997). Improvements have been ORTHOPAEDIC SURGICAL MANAGEMENT OF THE LOWER LIMB reported in studies using instrumented gait analysis, including IN CEREBRAL PALSY kinematics and electromyography (Bjornson et al. 2007). This The ambulant child may lead to small but important gains in gross motor function. Surgery for children with bilateral spastic CP used to start at The evidence base supporting the use of BoNT-A in CP is quite the ankles with TALs for equinus gait and then move up to the good as confirmed in several randomized controlled trials and knee and then to the hip. Mercer Rang caricatured this approach systematic reviews. However, the size of the treatment effect is as the ‘Birthday Syndrome’ (Fig. 27.9). Children spent most small and often short-lived (Desloovere et al. 2001, Bjornson of their birthdays in hospital, in casts or in rehabilitation. et al. 2007, Simpson et al. 2008). The current concept for the management of musculoskeletal In non-ambulant children with CP, BoNT-A may help deformities is Single Event Multilevel Surgery (SEMLS). reduce adductor spasticity but does not prevent hip displace- In a recent systematic review of SEMLS, evidence was ment (Graham et al. 2008, Willoughby et al. 2012). found for large improvements in gait dysfunction, moderate improvements in health related quality of life and only small ADVERSE EVENTS AND BOTULINUM NEUROTOXIN THERAPY changes in gross motor function (McGinley et al. 2012). In the IN CEREBRAL PALSY first randomized clinical trial of SEMLS a 50% improvement BoNT-A is generally safe in children with CP. Most adverse in gait function (Gillette Gait Index, GGI) and a 4.9% improve- events are localized, minor and self-limiting. Systemic side ment in gross motor function (GMFM-66) was reported I

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Fig. 27.9. Mercer Rang’s ‘Birthday Syndrome’ in cerebral palsy.

(Thomason et al. 2011). The 5-year results of this clinical trial against their will or without their full consent is likely to be show that these improvements were largely maintained at 5 resentful, may develop depression and struggle with rehabilita- years post-SEMLS (Thomason, Selber, and Graham 2013). tion. Careful preoperative discussions about setting realistic In the SEMLS approach, the gait pattern is identified and goals, helps to ensure that patient’s, parents’, and surgeon’s goals evaluated by IGA as part of the diagnostic matrix. SEMLS in are consistent and achievable (Thomason and Graham 2013). bilateral spastic CP can be considered to be an exercise in cor- A comprehensive plan is then developed for the correction recting anatomical deformities based on the clinical findings of all muscle tendon contractures, torsional malalignments, from the diagnostic matrix assessments. It is necessary to con- and joint instabilities in one operative session. Rehabilitation sider all components of the matrix so that surgical planning is requires at least 1 year and improvements continue into the optimized for the individual child (Davids et al. 2003). For second year, postoperatively (Thomason and Graham 2013, example, muscle weakness is an impairment which is easily Thomason, Selber, and Graham 2013). overlooked and which may have a greater impact on energy cost The principal components of a successful SEMLS pro- of walking and function in the community than multiple mus- gramme are as follows: culoskeletal deformities. 1. Planning based on the diagnostic matrix, including instru- It is essential to also consider the child in the context of mented gait analysis. participation, psychological and environmental factors, which 2. Preparation and education of the child and family. may make successful surgical outcomes less predictable. It is 3. Optimal perioperative care, including epidural analgesia. important to have a frank discussion around the family and 4. Carefully planned and supervised rehabilitation. child’s goals and aspirations. This requires a multidisciplinary 5. Appropriate orthotic prescription. approach with the involvement of the family and child, the 6. Close monitoring of functional recovery. rehabilitation team as well as community therapists. For a child 7. Follow-up gait analysis at 12 to 24 months after the SEMLS. in GMFCS level III, independent ambulation is rarely achiev- 8. Removal of fixation plates and other implants. able and it is essential to have these discussions ahead of the 9. Follow-up until skeletal maturity, for new or recurrent surgery and rehabilitation. A detailed examination of the child’s deformities (Thomason and Graham 2013). level of activity and participation, using measures such as the The surgical team should consist of two experienced sur- Canadian Occupational Performance Measure, Children’s geons and two experienced assistants. Expert anaesthesia and Assessment of Participation and Enjoyment, or the Activity pain management is essential. Postoperative nursing care must Scale for Kids (Law et al. 1990, Young et al. 2000, King et al. be vigilant. The use of epidural analgesia carries risks of masking 2004) may be useful. the signs of compartment syndromes and nerve stretch palsies. It is crucial to distinguish between the needs of children The surgery is a series of steps which correct deformity (Fig. and adolescents. The decision to proceed with SEMLS for 27.10). However, for 6 to 9 months after surgery, children are younger children is largely made by their parents. However, more dependent and less functional than they were prior to adolescents must be given the freedom to make their own surgery. A child who walks into hospital with a typical CP gait informed decisions regarding surgery and rehabilitation. An pattern leaves hospital in a wheelchair with straighter legs, but I adolescent who feels that they have been forced into SEMLS may be unable to walk independently for weeks or even months.

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restored and the plantar-flexion-knee-extension couple is com- petent. Functional recovery and orthotic prescription can be monitored by a gait laboratory visit every 3 months for the first year after surgery and yearly thereafter. Our approach to SEMLS rehabilitation has been described in more detail else- where (Thomason and Graham 2013).

The non-ambulant child As children spend less time weight bearing and walking, and more time in seated postures, flexion deformities at the hip, knee may develop. Managing ankle and foot deformities is important to allow bracing, enable the child to wear typical footwear and to have their feet rest comfortably on the footplate of a wheelchair. Correction of severe external tibial torsion by supramalleolar osteotomy of the tibia may be necessary (Selber et al. 2004). Where children are able to participate actively in standing transfers, achieving stable and pain-free feet and ankles can be important. Stabilization of the foot for pes valgus is more reliably achieved by a subtalar fusion than by os calcis lengthening (Shore et al. 2013). Correction of hallux valgus and dorsal bunion by soft tissue balancing and fusion of the first metatarsophalangeal joint is effective for deformity correction, pain relief, and comfortable shoe wear (Davids et al. 2001).

Surgery for hip displacement. The risk of developing progres- sive hip displacement is very high for non-ambulant children. Hip displacement may be managed by preventive or reconstruc- tive surgery or, for many children, a combination of both Fig. 27.10. The principal soft tissue and bony procedures which when combined constitute ‘Single Event Multilevel Surgery’ (SEMLS). (Fig. 27.11) (Miller, Dabney, and Rang 1995). The aim of hip surgery is to achieve and maintain a pain-free, enlocated hip to maintain comfortable sitting, not to improve gross motor function. Only a carefully tailored and carefully monitored rehabilitation programme can ensure that the child will reach a higher level Preventive hip surgery. Preventive surgery (Fig. 27.12) aims to of function. facilitate proximal femoral and acetabular development by cor- The child may commence full weight bearing once they recting adduction and flexion contractures. Normalizing the are comfortable a day or two after soft tissue surgery. Weight range of hip abduction is crucial to normal hip development. bearing can commence after 2 to 3 days if the child has had a Preventive surgery involves lengthening of the hip adductors femoral osteotomy with stable internal fixation, or after a and is typically advised when the migration percentage of one maximum of 1 to 2 weeks if there has been more extensive or both hips exceeds 30% to 40% and hip abduction is less reconstructive surgery at the foot–ankle level. Casts are only than 40 degrees on one or both sides (Presedo et al. 2005). required after foot and ankle surgery. Removable extension Phenol neurolysis of the anterior branch of the obturator nerve splints may be used at the knee level after hamstring-rectus is helpful in addressing both adductor spasticity and pain from surgery. The goal is to achieve full extension of the knee, com- muscle spasm post-surgery (Khot et al. 2008). Painful spasms bined with regaining full flexion, so that the transferred rectus in the lengthened muscles can be very distressing to both the femoris does not become scarred and adherent in its new site. child and their carers. Postoperatively, analgesia and muscle New AFOs must be prepared for immediate fitting after cast relaxants can assist in managing pain. A ‘broomstick’ or ‘A’ removal, usually 6 weeks after surgery. The initial postoperative frame cast is often used to maintain the child’s legs in abduction brace is usually a solid AFO. The orthotic prescription must be and can complement pain management strategies. Children carefully monitored throughout the first year after surgery. A can, and should, be encouraged to continue with many daily less supportive AFO, such as a hinged or posterior leaf spring, activities while wearing the plaster, including prone lying, long- may be introduced when the sagittal plane balance has been sitting, and supported standing. I

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Fig. 27.11. The progression of hip displacement in children with cerebral palsy from (a) a normal hip, (b) progressive displacement to (c) dislocation with structural changes both in the proximal femur and acetabulum.

with migration percentage >40% (Miller, Dabney, and Rang 1995). Reconstructive surgery consists of three main components: adductor release, femoral osteotomy and pelvic osteotomy. Firstly, abduction range is restored by a revision adductor release. Correction of the shape of the femur is achieved by varus derotation osteotomy of the proximal femur. Shortening of the proximal femur is also an effective way to reduce soft tissue tension and restore range of motion and symmetry about the hips. In windswept deformities, symmetry can be achieved by a combination of asymmetrical soft tissue releases and adjusting the amount of rotation in each osteotomy (Fig. 27.13). Stable internal fixation is achieved by using a fixed angle blade plate, avoiding the need for a hip spica in most children. Significant acetabular dysplasia should be corrected by aSan Diego pelvic osteotomy and older children and teenagers may benefit from a periacetabular osteotomy (Clohisy et al. 2006). Fig. 27.12. Neurolytic injections do not prevent hip displacement and After reconstructive surgery, there are no restrictions to adductor surgery has limited benefits, particularly in non-ambulant chil- weight bearing or movements of the hips, apart from considera- dren in Gross Motor Function Classification System levels IV and V. tion of the child’s level of comfort. Correction of the neck shaft angle can produce a ‘widening’ at the child’s thighs and therapists supporting the family may need to consider modifi- It is important to note that most studies of adductor cations to wheelchair seating to accommodate the increase in releases report a high failure rate in children who are non- hip width. Blade plates are usually removed about 12 months ambulant and who are followed up for more than 3 to 5 years after surgery. (Miller et al. 1997a, Shore et al. 2012). The majority of these One stage correction of hip displacement by bony recon- children will need bony reconstructive surgery, 2 to 10 years struction is a very effective and reliable method of stabilizing after preventive surgery. the severely subluxated or dislocated hip in CP (Miller et al. 1997b, McNerney, Mubarak, and Wenger 2000). The radio- Reconstructive hip surgery. The most common indication for logical status of most hips remains satisfactory in the short and reconstructive surgery is a persistently high migration percent- longer term. Pain is prevented or relieved and sitting tolerance age after adductor surgery. If the migration percentage remains is usually dramatically improved. Radiological monitoring of >40%, at >12 months after adequate adductor releases and the hip development should continue at least until skeletal matu- child is >4 years with little or no degenerative changes, recon- rity. The single biggest threat to hip status after successful hip structive surgery is indicated (Miller et al. 1997b, McNerney, reconstruction is progression of scoliosis and pelvic obliquity. Mubarak, and Wenger 2000). Reconstructive surgery is also It is very difficult to maintain hip stability on the high side of I advised as the index surgery in children >8 years presenting an oblique pelvis.

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Fig. 27.13. Reconstructive hip surgery for children with cerebral palsy Fig. 27.14. There are multiple options for salvage surgery of the hip in involves a combination of adductor lengthening, varus derotation short- children with cerebral palsy but none of them are predictable or reliable ening osteotomy of the proximal femur (VDRO) and pelvic osteotomy in relieving pain and improving quality of life. (San Diego).

Salvage surgery for the hip. Pain following neglected hip dis- Referral to a pain management service is important as a placement is reported to occur in between 10% and 90% of number of adolescents can be managed non-operatively, in the adolescents and young adults. Fixed deformity, especially the short term. ITB may also be considered for optimizing tone windswept deformity, is also a major impediment to comfort- management prior to salvage surgery. able sitting and care (Barakat et al. 2007). The need for surgery to ‘salvage’ the neglected hip (Fig. 27.14) is best avoided by SPINAL DEFORMITY AND SCOLIOSIS SURGERY early hip surveillance and appropriately timed preventive and Natural history of scoliosis in cerebral palsy reconstructive surgery. GMFCS level is the single strongest predictor of severe scoliosis Excision of the proximal femur is widely used but has (Cobb angle >40 degrees) in children with CP (Persson-Bunke unreliable outcomes and a high complication rate (Castle and et al. 2012). Scoliosis may present as early as age 4 to 6 years, Schneider 1978, Abu-Rajab and Bennet 2007). More limited and occasionally even younger. The rate of progression acceler- excision of the head of the femur combined with a valgus oste- ates when the curve reaches 40 to 50 degrees and as the child otomy may be slightly better (McHale, Bagg, and Nason 1990). enters the pubertal growth spurt (Fig. 27.15). This acceleration Total hip arthroplasty is very effective but is only indicated in in the rate of progression may catch parents, pediatricians and a very small subset of ambulant children with well-controlled unaware. In the space of 1 to 2 years it is possible for a curve movement disorders (Root, Goss, and Mendes 1986). Metal on to progress from a moderate flexible curve, easily correctible in metal, resurfacing arthroplasty combined with proximal femoral single stage posterior surgery with moderate risks, to a severe derotation and shortening may reduce the risk of dislocation rigid curve requiring anterior and posterior surgery, which has and provide good pain relief and function, in more involved increased risks of morbidity and mortality (Miller 2005). A adolescents (Prosser, Shears, and O’Hara 2012). ‘point of no return’ may be reached where the magnitude of the I

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Parents and caregivers need instructions regarding protection (a) (b) of the fusion when the child is transferring to and from their wheelchair, and by limiting hip flexion in the short term. Review of wheelchair seating and postural supports is often required as ‘seat to shoulder’ height typically increases with correction of the scoliosis. Curve progression after successful fusion is usually <10 degrees although this may be greater in immature individ­ uals with growth potential. Spinal fusion in children with CP improves sitting position and appears to improve upper limb function, eating and respiratory function (Tsirikos et al. 2004, Miller 2007). It may also prolong life and improve the quality of life (Narayanan et al. 2006, Mercado, Alman, and Wright 2007).

Kyphosis and lordosis in cerebral palsy Kyphosis in the dorsal spine is very common in younger chil- dren with weak paraspinal muscles and is best managed by (c) (d) having the wheelchair seat slightly reclined, the use of chest straps and occasionally a Lycra ‘upsuit’ or thoraco-lumbar-sacral orthoses (TLSO). With advancing age, the thoracic kyphosis and the secondary cervical lordosis may become more fixed. Cervical pain in adults with CP is common. Kyphosis in the lumbar spine may be related to severe contractures of the hamstrings. Proximal hamstring recession at the time of adductor releases may be beneficial. Lordosis in the lumbar spine is much more common and is frequently related to hip flexion contractures. Lengthening of the hip flexors before the lordosis becomes excessively severe or fixed may be appropriate (Miller 2005).

Management of the lower limb in cerebral palsy by sagittal gait pattern It is helpful to consider the sagittal gait pattern in both unilat- eral and bilateral spastic CP. The classifications are useful as they provide insight into which levels of the lower limbs are affected, Fig. 27.15. Scoliosis may progress rapidly in children with cerebral palsy the extent of involvement and the biomechanical principles who are non-ambulant, particularly at the time of the pubertal growth which need to be considered when planning management. The spurt (a, b). The only effective method for the correction of severe sco- classifications do not provide recipes for management but high- liosis in children with cerebral palsy is a long instrumented fusion from light important aspects which are useful in the decision making T2 to the pelvis (c, d). process (Figs 27.4 and 27.5).

surgery required for correction, outstrips the child’s physiologi- Sagittal gait patterns: unilateral spastic cerebral palsy cal reserves of cardiorespiratory function (Lipton et al. 1999). TYPE I HEMIPLEGIA The functional goal of scoliosis surgery in CP is to improve The prominent impairment is a drop foot in the swing phase sitting balance and ease the burden of care. The biomechanical of gait. There is no contracture of the gastrocsoleus. Neither goal is to achieve a stiff, well-balanced spine over a level pelvis spasticity management nor musculoskeletal surgery is necessary. with flexible enlocated hips. In the majority of children, the Gait and function can be improved by the use of an AFO, surgical goal is a long posterior spinal fusion with the fusion usually a leaf spring AFO or a hinged AFO. limits from high in the dorsal spine to the pelvis (Dias et al. 1996, Miller 2005). TYPE II HEMIPLEGIA Children with known respiratory disease require evaluation In type II hemiplegia, there is spasticity in the gastrocsoleus I and preoperative chest management including physiotherapy. which gradually becomes fixed resulting in a contracture

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and equinus gait. Management requires correction of fixed inappropriate lengthening and weakening of the gastrocsoleus contracture in the gastrocsoleus (to correct second rocker) and with further deterioration in gait and functioning. These chil- provision of an AFO (to provide heel strike, swing phase dren may require SEMLS at proximal levels and IGA is essential clearance, and appropriate pre-positioning of the foot during for planning the appropriate surgical interventions. pre-swing). CROUCH GAIT TYPE III HEMIPLEGIA Sagittal plane: excessive ankle dorsiflexion and knee flexion, In type III hemiplegia there is a contracture of the gastrocsoleus incomplete hip extension at the ankle plus knee involvement with co-contraction of the In crouch gait, the iliopsoas is usually contracted. The ham- hamstrings and rectus femoris. Children in this transitional strings are short only in patients with a posterior pelvic tilt. group may benefit from lengthening of the medial hamstrings When the pelvis is in the neutral range, the hamstrings are of and rectus femoris transfer, in addition to gastrocsoleus length- normal length and when the pelvis is anteriorly tilted, the ening and a hinged AFO. hamstrings are excessively long. Without the use of motion analysis and the plotting of muscle lengths, it is very difficult TYPE IV HEMIPLEGIA to appreciate these findings (Thomason et al. 2012). Con­ In type IV hemiplegia, all levels of lower limb are involved and sequently the majority of children with crouch gait are managed there is involvement in the sagittal, coronal, and transverse by excessive hamstring lengthening to improve knee extension planes at the hip. Hip dysplasia is common and often presents when in fact the hamstrings are of normal length or excessively late. In addition to the surgery outlined for type III hemiplegia, long. Such surgery results in increased anterior pelvic tilt which correction of type IV hemiplegia includes lengthening of the may lead to recurrent crouch, back pain and spinal instability adductor longus and the psoas over the brim of the pelvis as (spondylolysis and spondylolisthesis) (Rodda, Graham, and well as a proximal femoral derotation osteotomy (Winters, Galea 2006). Gage, and Hicks 1987, Dobson et al. 2005). Severe crouch gait can be defined as knee flexion >30 degrees throughout stance, with excessive ankle dorsiflexion Sagittal gait patterns in bilateral spastic cerebral palsy and incomplete hip extension (Rodda et al. 2004). It can be TRUE EQUINUS part of the natural history of gait in bilateral spastic CP but the Sagittal plane: ankle equinus, knees and hips extended majority of affected individuals have had lengthening of the True equinus can be managed in the younger child by injections Achilles tendons (Stout et al. 2008, Vuillermin et al. 2011). of BoNT-A to the gastrocsoleus and the provision of hinged There is often a delay between lengthening of the gastrocsoleus AFOs. By the time children develop fixed contractures and and the development of crouch gait. The Achilles tendons are require surgery, true equinus is rare. When it persists, there are often lengthened in children with bilateral spastic CP between usually occult contractures of the hamstrings and iliopsoas. the age of 3 and 6 years. It may take another 3 to 6 years before Single level surgery (gastrocsoleus lengthening) is almost never crouch gait becomes a significant functional problem and it is the correct strategy in bilateral spastic CP, no matter how often not until the adolescent growth spurt when the maximum tempting it may appear on observational gait analysis. deterioration in gait and functioning occurs. Instead of ‘growing up’ the adolescent with progressive crouch gait ‘sinks down’, JUMP GAIT with an inability to maintain an extension posture at the hip Sagittal Plane: ankle equinus, knees and hips incomplete and knee during the stance phase of gait (Vuillermin et al. extension in mid-stance 2011). Contributing factors seem to be a progressive mismatch Many children require SEMLS for appropriate manage­ between the strength of the one-joint muscles contributing to ment. This is discussed in detail under orthopaedic surgical the body support moment (gluteals, vasti, and soleus) and management. increased demand because of rapid increases in height and weight at the pubertal growth spurt. This typically occurs in APPARENT EQUINUS conjunction with progressive bony deformities known as lever Sagittal plane: ankle plantargrade, knees and hips flexed arm disease. Around the time of the pubertal growth spurt, Many children with bilateral spastic CP who walk on their toes, increasing patella alta (sometimes with fractures of the patella never achieving heel contact, have an ankle range of motion or avulsions of the inferior pole), increasing external tibial close to the normal range. Such children are at risk of inap- torsion and breakdown of the midfoot with severe pes valgus, propriate management with injections of BoNT-A to the gas- all contribute to increasing crouch, fatigue and decreasing trocsoleus or even worse, lengthening of the gastrocsoleus. The ability to walk. Understanding the biomechanics of severe important contractures are more proximal, at the level of the crouch gait has led to improved surgical management in recent knee and hip. The recognition of ‘apparent equinus’ in contra- years with the development of more effective techniques to distinction to ‘true equinus’ is very important to avoid achieve lasting correction (Ma et al. 2006, Stout et al. 2008). I

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Management should be based on the findings of IGA and gait training. The movement disorder is mild and easily the hamstrings should only be lengthened when found to be managed by injections of BoNT-A, most often into the gastroc- short. Surgery may include iliopsoas lengthening, semitendino- nemius muscles (Graham et al. 2000). Neither selective dorsal sus transfer, distal femoral extension osteotomy, patellar tendon rhizotomy nor ITB are appropriate choices because the spastic- shortening, and correction of pes valgus and external tibial ity is mild and focal. torsion. Before skeletal maturity, growth plate surgery is an Older child: children with type I hemiplegia rarely need ortho- option to correct small, residual knee flexion deformities (Young paedic surgery. Equinus contracture may develop in children et al. 2010). with type II hemiplegia and lengthening of the gastrocsoleus is very helpful. Surgery can usually be deferred until age 4 to 6 Management of the lower limb and spine in cerebral years by the use of an AFO combined with injections of palsy by Gross Motor Function Classification BoNT-A and a programme of physiotherapy. The choice of System level lengthening procedure is based on a careful Silfverskiold test to Management of the child or adolescent with CP should be determine the amount of contracture in the gastrocnemius and considered within the context of their GMFCS level, functional soleus respectively (Graham 2010). mobility level, and the biomechanics of the gait pattern or In bilateral spastic CP, some children develop a mild con- movement pattern. Management may include non-surgical and tracture, usually involving only the gastrocnemius. Careful surgical options at different times and in different combina- assessment by instrumented gait analysis is essential to identify tions. Gait correction surgery is an option for many children proximal involvement which would require correction. Isolated, with CP in GMFCS levels I to III but with important differ- single level surgery for equinus in bilateral spastic CP is rarely ences at each level. indicated and is associated with a 40% risk of severe crouch gait, in long-term follow up (Borton et al. 2001). GMFCS level I Children at this level have the ability to par- Adolescent: maintaining high level sporting activities may ticipate in recreational physical activities with no or minimal become more difficult. Maintaining muscle length and strength adaptations. through PRST gym-based programmes can be very helpful. Function: FMS 6,6,6 or 6,6,5; FAQ 9 to 10 Sagittal gait pattern: GMFCS level II Children manage walking on level surfaces Unilateral spastic cerebral palsy: type I or II hemiplegia but uneven terrain, steps/stairs, long distances present difficul- Bilateral spastic cerebral palsy: true equinus, mild jump gait ties. An assistive device or wheelchair may be required for Musculoskeletal: community distances particularly when older. Lower limb: there is usually more distal than proximal involve- Function: FMS 6,5,5 to 5,5,1; FAQ 8 to 9 ment with mild spasticity and occasionally dystonia. Gait dis- Sagittal gait pattern: turbances such as equinus in stance and foot drop in swing are Unilateral spastic cerebral palsy: type II to IV hemiplegia the most likely concerns for the child and family. These may Bilateral spastic cerebral palsy: jump gait, apparent equinus, indicate a combination of impaired selective motor control, crouch gait weakness in tibialis anterior, and either dynamic tightness or Musculoskeletal: mild contracture in the gastrocsoleus. There may be mild Lower limb: involvement at the knee and hip level as the child becomes Type IV hemiplegia: there is involvement of the entire lower older, particularly in children with bilateral spastic CP. limb in type IV hemiplegia. The usual pattern is equinus at the Hips: Mean femoral neck anteversion: 30° ankle, a stiff flexed knee, a hip that is internally rotated, Mean neck shaft angle: 136° adducted and flexed and a pelvis which is retracted, anteriorly Hip disease is rare and is usually a mild developmental tilted and oblique in the coronal plane. The foot may be equi- dysplasia. novarus or equinovalgus (Michlitsch et al. 2006, Graham Spine: scoliosis is seen in <20% of adolescents and the curve 2010). Evaluation should include IGA including dynamic is small (10–25°) and does not progress or require surgery. If a EMG, pedobarography, and standardized radiographs in the spinal curvature develops, it will be an adolescent idiopathic weight bearing position (Davids, Gibson, and Pugh 2005). type curve. Incidence is similar to children who are typically Malalignment is present when there is internal rotation of the developing. femur combined with external tibial torsion. Long-term reli- ance on the ‘sound’ leg for push off may result in excessive Management: external tibial torsion. It is difficult to evaluate the external Younger child: physiotherapy will be the mainstay of manage- tibial torsion on the ‘sound side’ without gait analysis but it ment with emphasis on a family and task/context focused rarely requires correction. Limb length discrepancy is often approach. A leaf spring AFO or hinged AFO is useful for significant and may require correction by growth plate surgery, I dynamic equinus and can improve the base of support and aid in selected children (contralateral epiphysiodesis). Dystonia

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may be present particularly in the upper limb. It is important (a) (b) to note that type IV hemiplegia is associated with progressive hip displacement in some children (Rutz et al. 2012a). This is clinically silent, in the initial stages and radiological surveillance is required. Bilateral spastic cerebral palsy: the movement disorder is usually spastic, especially in those born preterm. The natural history of deformities in the lower limbs in GMFCS level II is for gradual progression during childhood with more rapid dete- rioration during the adolescent growth spurt. The natural history of gait is progressive deterioration including increasing stiffness throughout the lower limb joints and increasing ten- dency to flexed knee gait and ultimately crouch gait (Johnson, Damiano, and Abel 1997, Bell et al. 2002, Gough et al. 2004). The transition from equinus gait to crouch gait is often acceler- ated by procedures, which weaken the gastrocsoleus, especially (c) (d) lengthening of the Achilles tendons (Vuillermin et al. 2011). The principal gait dysfunctions in bilateral spastic CP are stiff- ness and excessive flexion. Musculoskeletal pathology in bilateral spastic CP, GMFCS level II, includes increased femoral neck anteversion, and con- tractures of the two joint muscles, the psoas, hamstrings and gastrocnemius (Graham 2004) (Fig. 27.16). There is usually pes valgus and in adolescents hallux valgus. There is sometimes excessive external tibial torsion resulting in lower limb mala- lignment. In asymmetric bilateral spastic CP, pelvic retraction may make clinical estimation of rotational malalignment during gait very difficult. Occasionally in bilateral spastic CP, varus may be present but is more apparent than real because of exces- sive FNA and ‘rollover varus’. If present, varus is usually mild Fig. 27.16. (a, b) Asymmetric ‘jump gait’ in a 10-year-old boy with and flexible. spastic diplegia before Single Event Multilevel Surgery (SEMLS). (c, d) Hips: Mean femoral neck anteversion: 36° At 5-year follow-up after SEMLS deformities remain corrected and gait Mean neck shaft angle: 141° and functioning showed a substantial improvement. Risk of hip displacement (migration percentage >30%): 15% In bilateral spastic CP, GMFCS level II, the mean femoral neck anteversion is increased at 36 degrees and there is a 15% risk should be completed before the child leaves primary school. of hip displacement. The hip displacement is generally mild Teenagers do not cope well with SEMLS and rehabilitation and progresses slowly. (Thomason and Graham 2013). Spine: small curves (10–25°) are seen in <20% of adolescents Type IV hemiplegia: unilateral multilevel surgery is usually but they do not progress and do not require surgery. required between the ages of 6 and 12 years. Progressive sub- Management: luxation of the hip is an indication to proceed with unilateral Younger child: early management will be similar to GMFCS multilevel surgery in which stabilization of the hip and correc- level I but with more frequent physiotherapy and BoNT-A tion of the limb deformities are combined (Dobson et al. 2005, injections as indicated by functional limitations, together with Rutz et al. 2012a). IGA is essential in type IV hemiplegia use of hinged or solid AFOs. If the spasticity is mild/moderate because the number of gait deviations is considerable. It is very and at multiple levels, it can be managed by multilevel injec- important to differentiate between primary deviations, second- tions of BoNT-A repeated at 12 month intervals. Selective ary compensations and tertiary coping mechanisms. dorsal rhizotomy is a better option when the spasticity is severe, Correction of soft tissue contractures in the sagittal plane generalized, and adversely affecting gait and function will most likely be accompanied by external rotation osteotomy (McLaughlin et al. 2002). of the femur and internal rotation osteotomy at the supramalle- Older child: this is the ideal time (6–12y) for SEMLS. SEMLS olar level of the tibia and fibula to correct malalignment (Fig. is rarely needed before age 6 years. All surgery and rehabilitation 27.10). Equinovarus deformities are variable in severity and I

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more resistant to surgical correction than in bilateral spastic CP. (a) In younger children with documented over activity in the tibia- lis posterior, both intramuscular recession and Split Posterior Tibial Tendon (SPOTT) transfer are good options (Graham 2010). Ideally this should be undertaken before deformities become fixed, thus avoiding the need for bony surgery. In children with documented over activity in both the tibialis anterior and tibialis posterior, a combination of Split Anterior Tibial Tendon (SPLATT) transfer and intramuscular recession of the tibialis posterior, gives good long-term results (Graham 2010). (b) A number of children with unilateral spastic CP benefit from contralateral epiphysiodesis to reduce limb length discrep- ancy. In these children, bone age is often well ahead of chrono- logical age. Children with type IV hemiplegia rehabilitate quickly and easily because surgery is unilateral and the sound side helps with rapid mobilization. Bilateral spastic cerebral palsy: preventive surgery for hip displacement consisting of lengthening of the hip adductors is effective (Shore et al. 2012). Hip flexion contractures are dealt with by lengthening of the psoas tendon at the brim of the Fig. 27.17. (a) Severe pes valgus results in pain, the inability to tolerate pelvis. This is a stable lengthening and does not require immo- ankle–foot orthoses (AFOs) or even to wear normal shoes. (b) After bilization. Prone lying is required postoperatively to encourage reconstruction the feet are pain free, braceable and this contributes to hip extension. Femoral anteversion is corrected to 0 to 10 much improved gait and function. Used with permission from Lippincott Williams and Wilkinson/Wolters Kluwer Health: Lovell and Winter degrees, leaving only 10 to 20 degrees of internal rotation at Pediatric Orthopaedics. © 2014 Lippincott Williams and Wilkinson. the hip. The proximal osteotomy effectively lengthens the psoas and is the preferred technique. operations on the gastrocsoleus are the most stable and safest At the knee, hamstring contracture and mild dynamic in terms of avoiding calcaneus (Shore, White, and Graham deformity may be treated by distal hamstring lengthening 2010, Firth et al. 2013). without any fixed flexion contracture at the knee. Distal ham- Equinus leads to excessive loading of the forefoot and with string lengthening is ineffective when knee flexion contracture time may cause breaching of the midfoot. A series of complex exceeds 5 to 10 degrees (Young et al. 2010). Recurvatum is segmental malalignments of the midfoot, hindfoot, and fore- sometimes seen after excessive hamstring lengthening and a foot develops referred to as ‘pes valgus’ (Graham 2010). persistent equinus contracture. When hamstrings are length- Symptoms may include pain and callosities over the collapsed ened in cases of normal hamstring function (normal muscle medial arch, particularly the head of the talus. This leads to length and velocity in swing), excessive anterior pelvic tilt and discomfort in shoes and inability to wear AFOs (Fig. 27.17). pelvic range of motion may develop (Rodda et al. 2006). Evaluation includes the usual components of the diagnostic Hamstring lengthening should either not be considered or at matrix with special emphasis on weight bearing radiographs of least should be done cautiously and include management to the feet and ankles, rather than three dimensional motion treat the anterior pelvic tilt (Young et al. 2010). analysis (Davids, Gibson, and Pugh 2005). Factors affecting The main complication of surgery for equinus is gradual the choice of operative procedure include the age of the patient failure of the plantarflexion-knee-extension couple, leading to and the clinical and radiographic severity of the deformity. calcaneus and crouch gait. This is much more disabling and The midfoot can be stabilized and deformity corrected by difficult to treat than the original equinus gait. In bilateral lengthening of the lateral column of the foot (os calcis length- spastic CP ‘a little equinus is better than calcaneus’ (Rang 1990, ening) or extra-articular fusion of the subtalar joint. The indica- Firth et al. 2013). The ‘over-lengthening’ is mediated by bio- tion for os calcis lengthening is a flexible valgus deformity mechanical changes and growth, not surgical imprecision. of the heel in association with an abductus deformity of the When the GRF falls behind the knee, the soleus responds to forefoot, in a patient who walks independently, GMFCS levels the continual stretch by adding more sarcomeres in series. In I or II (Mosca 1995). Arthrodesis of the subtalar joint is a reli- time, the soleus becomes functionally too long, biomechani- able means of correcting hindfoot valgus and with secondary cally incompetent and calcaneus-crouch progresses rapidly. correction of the midfoot. It is useful for more severe deformi- Deferring the surgery until age 6 to 8 years reduces the risks of ties in patients who require assistive devices and long-term I both recurrence and over correction. The more proximal orthotic support, GMFCS levels III and IV (Shore et al. 2012).

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Fusion of the talonavicular joint is a third option (de Coulon muscle groups which contribute to the body support moment, et al. 2011). the gastrocsoleus, quadriceps, and hip extensors are crucial. Management of equinovarus by intramuscular recession of The musculoskeletal pathology in GMFCS level III is tibialis posterior, combined with correction of femoral torsion, similar to that in GMFCS level II but the contractures of the as part of SEMLS gives good results. In children with bilateral muscle-tendon-units are usually more severe and the deformi- spastic CP, over correction to valgus is common. ties in the bony levers (femur and tibia) and joint instability External tibial torsion may occur in isolation or in conjunc- (hip and foot) are more pronounced. Severe ‘lever arm deformi- tion with medial femoral torsion. In isolation, external tibial ties’ are common in GMFCS level III with increased femoral torsion results in an external foot progression angle and ‘lever neck anteversion, marked external tibial torsion and pes valgus arm disease’ because the foot lever is effectively shortened and (Fig. 27.18). mal-directed, in relation to the line of progression. However, Severe crouch gait can be defined as knee flexion >30 when external tibial torsion and medial femoral torsion coexist degrees throughout stance, with excessive ankle dorsiflexion and the foot progression angle may be relatively normal but the incomplete hip extension (Rodda et al. 2004). crouch gait may knees face inwards and there is still very significant lever arm occur in GMFCS level I but is invariably mild because children disease (malignant malalignment syndrome). Supramalleolar in GMFCS level I have good strength and selective motor osteotomy of the tibia is an effective means of addressing this control. Severe crouch gait may occur in GMFCS level IV, but problem. External foot progression angle is usually the result of given that sustained ambulation is not feasible in adult life, combined pes valgus and external tibial torsion. The decision to correction by invasive surgery is not appropriate. Severe crouch perform os calcis lengthening, supramalleolar osteotomy (SMO) gait is a major functional issue in GMFCS levels II and III. of the distal tibia, combination requires a very careful assess- There is often a delay between lengthening of the gastroc- ment using all components of the diagnostic matrix. soleus and the development of crouch gait. Instead of ‘growing Hallux valgus is commonly associated with deformities in up’ the adolescent with progressive crouch gait ‘sinks down’, the hindfoot, midfoot, and proximal gait deviations, such as with an inability to maintain an extension posture at the hip stiff-knee gait, which causes toe scuffing. The most reliable and knee during the stance phase of gait. Most severe crouch procedure is fusion of the first metatarsophalangeal joint, either gait is iatrogenic and therefore preventable (Vuillermin et al. in conjunction with, or after correction of the proximal deform- 2011). Contributing factors seem to be a progressive mismatch ities (Davids et al. 2001). between the strength of the one-joint muscles contributing to Adolescent: the time for SEMLS and intensive rehabilitation the body support moment (gluteals, quadriceps, and gastrocso- has passed. The emphasis moves to maintenance of function leus) and increased demand because of rapid increases in height and mobility via strengthening programmes within the home, and weight at the pubertal growth spurt. This typically occurs school or gym environment. Minor surgery for recurrent in conjunction with progressive bony deformities known as deformities or minor gait deviations may be required. lever arm disease. Around the time of the pubertal growth spurt, increasing patella alta (sometimes with fractures of the GMFCS level III Children walk using an assistive device in patella or avulsions of the inferior pole), increasing external most situations but may use a wheelchair for community tibial torsion and breakdown of the midfoot with severe pes distances. valgus, all contribute to increasing crouch, fatigue, and decreas- Function: FMS 5,4,4 to 2,2,1; FAQ 6 to 8 ing ability to walk. Sagittal gait pattern: Hips: Mean femoral neck anteversion: 40° Unilateral spastic cerebral palsy: not applicable unless other Mean neck shaft angle: 149° comorbidities Risk of hip displacement (migration percentage >30%): Bilateral spastic cerebral palsy: jump gait, apparent equinus, 41% crouch gait Spine: small curves (10–35°) are seen in 20% of adolescents Musculoskeletal: but they do not progress and do not require surgery. Lower limb: the predominant movement disorder in GMFCS Management: level III is spasticity but some children have dystonia or a mixed Younger child: the focus in the early years is on the develop- movement disorder. Weakness of the major lower limb muscles, ment of gross motor function and mobility with emphasis on particularly those contributing to body support is a major prevention of contractures and developing strength. Physio­ feature. Flexed knee gait patterns predominate and weakness is therapy is combined with orthotics and the judicious use of usually the primary determinant of long-term gait and com- BoNT-A injections as an adjunct to therapy. munity function rather than spasticity. It is important to dif- Hip displacement in children with CP cannot be prevented ferentiate between those individuals who are being ‘pulled by non-operative measures including physiotherapy, abduction down’ by spasticity and those who are ‘falling down’ because of bracing and injections of BoNT-A (Graham et al. 2008, Lundy, weakness. The strength and selective motor control of the Doherty, and Fairhurst 2009). Hip surgery for children with I

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(a) (b) (c)

Fig. 27.18. Severe jump gait in Gross Motor Function Classification System level III prior to surgery. Post SEMLS. Post growth plate surgery to the knees. The goals of growth plate surgery are sagittal plane balance and the ability to tolerate ankle-foot-orthoses (AFOs) which together improve gait and functioning. Used with permission from Lippincott Williams and Wilkinson/Wolters Kluwer Health: Lovell and Winter Pediatric Orthopaedics. © 2014 Lippincott Williams and Wilkinson.

CP can be classified as preventive, reconstructive, and salvage can be included (Ma et al. 2006, Young et al. 2010). If the child (Miller, Dabney, and Rang 1995). Surgery to prevent hip dis- is at skeletal maturity and the knee flexion deformity is 10 to placement refers to soft tissue releases incorporating open 30 degrees, a distal femoral extension osteotomy with patella lengthening of the adductor longus and gracilis and lengthen- tendon shortening is most likely to assist in achieving better ing of the psoas over the brim of the pelvis to prevent or reverse knee extension and lasting correction of crouch gait (Stout early hip displacement in younger children. Because the et al. 2008). outcome of preventive surgery depends on the age of the child Adolescent: Ideally SEMLS will have been undertaken prior to (younger children have better results) and the initial migration adolescence. The ongoing management of weakness and muscle percentage (migration percentage <40%), early and regular tightness will be emphasized in a physiotherapy programme hip surveillance is recommended. The results in children in and ideally in a community setting such as a gym. Both PRST GMFCS level III, who are walking with external support are and aerobic exercise for fitness and weight control are important very good. Several studies have reported that the outcome of (Thomason and Graham 2013). preventive surgery is much better in ambulators than in non- ambulators (Miller et al. 1997a, Presedo et al. 2005). The GMFCS level IV: At this GMFCS level, children may have effectiveness of adductor surgery is predicted by GMFCS level limited walking (requiring physical assistance) within the home (Shore et al. 2012). or classroom but will use wheeled mobility at other times. Older child: SEMLS using the principles and techniques Adolescents will most likely use a manual or powered wheel- described for children with a GMFCS level II is appropriate. chair in all settings. Surgery for crouch gait has been revolutionized in recent years Function: FMS 2,1,1 to 1,1,1; FAQ 1 to 5 by improved understanding of the causes, biomechanics and by Sagittal gait pattern: the introduction of more effective surgical procedures and reha- Bilateral spastic cerebral palsy: jump gait, apparent equinus, bilitation (Stout et al. 2008). Most severe crouch gait is caused crouch gait by single level surgery for equinus gait. When this is eliminated Musculoskeletal: in a population, crouch gait becomes less common, less severe Lower limb: Flexion deformities at the hip and knee are very and more easily managed. Traditional surgery with over reliance common as are deformities at the foot and ankle, especially on hamstring lengthening is not very effective (Rodda, Graham, external tibial torsion and pes valgus. Hallux valgus and dorsal and Galea 2006, Young et al. 2010). If the knee flexion deform- bunions are also quite common. ity is between 5 and 10 degrees, semitendinosus transfer to the The majority of children in GMFCS level IV have mixed adductor tubercle is the technique of choice but if the deformity tone or ‘spastic-dystonia’. In children and adolescents with is between 10 and 25 degrees and the child is pre-pubertal, then severe, generalized hypertonia, ITB by implanted pump offers I guided growth at the anterior physis of the knee by ‘8’ plates the most reliable method of sustained tone reduction (Albright

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2008). Weakness of the trunk and paraspinal muscles results (a) in a postural kyphosis and the incidence of scoliosis is high (Persson-Bunke et al. 2012). Hips: Mean femoral neck anteversion: 40° Mean neck shaft angle: 155° Risk of hip displacement (migration percentage >30%): 69% Spine: Severe curves (>40°) are seen in 25% of adolescents. Progressive scoliosis is common in GMFCS level IV. The majority of children will require surgical correction. Postural kyphosis is also common in GMFCS level IV owing to the effects of paraspinal weakness and impairments of posture and balance. Management of spinal deformities is considered in more detail in the section on GMFCS level V. Management: (b) In GMFCS level IV, management goals are based on a realistic appraisal of long-term motor prognosis including standing and assisted walking in early childhood. In later childhood mainte- nance of comfortable sitting by detecting and treating early hip displacement is important. Later, monitoring of spinal deform- ity with appropriate treatment and provision of custom seating are the most important issues. Younger child: Younger children may enjoy supported stand- ing and assisted ambulation. It may be appropriate to employ some of the procedures described for children in GMFCS level III to assist with this. While physiotherapy and orthotic pre- scription, combined with BoNT-A or ITB, are appropriate forms of management, attainment of standing and walking will be limited and slow. Parents, carers, and therapists should rec- ognize that sustained ambulation into adolescence and adult Fig. 27.19. Windswept hips in a 10-year-old boy with cerebral palsy in life is not achievable (Palisano, Rosenbaum, and Bartlett 2008), Gross Motor Function Classification System level IV. Note the pelvic therefore extraordinary measures to maintain ambulation are obliquity and severe hip displacement on the left side. Following bilateral contraindicated. Hip surveillance is essential owing to the high VDROs and left pelvic osteotomy the hips are enlocated (in joint) and the pelvis is more level. risk of progressive hip displacement (Soo et al. 2006). Older child: Managing deformity of the foot and ankle is important to allow bracing and wearing of shoes. This can also allow the child’s feet to rest comfortably on the footplate of children in GMFCS level IV and many will go on to require their wheelchair. Correction of severe external tibial torsion by reconstructive hip surgery (Shore et al. 2012). supramalleolar osteotomy of the tibia and/or stabilization of Reconstructive hip surgery poses a major challenge to chil- the foot by a subtalar fusion may be necessary for correct foot dren in GMFCS level IV who often have complex comorbidi- positioning to allow the child to participate in assisted transfers ties. Nutrition and respiratory status should be optimized (Selber et al. 2004, Shore et al. 2013). If pain and comfortable before embarking on any major surgery, especially bilateral hip shoe wear is a problem for the child, hallux valgus and dorsal surgery (Fig. 27.19). bunions can be corrected by soft tissue surgery and fusion of Reconstructive surgery consists of a combination of three the first MTP joint (Davids et al. 2001). main components: adductor releases, femoral osteotomy and Hip displacement may be managed by preventive or recon- pelvic osteotomy. For those children who have recurrent dis- structive surgery or, for many children, a combination of both. placement after previous adductor releases, a revision adductor For children with reduced hip abduction range, preventive hip release at the time of bony reconstruction is always required. surgery is undertaken to lengthen the adductors. While the aim Correction of the shape of the abnormal femur is achieved by of preventive surgery is to slow the progression of hip displace- varus derotation osteotomy of the proximal femur. Shortening ment, an increase in abduction range can also assist carers with of the proximal femur is also an effective way to reduce soft daily tasks such as dressing and bathing. It is important to tissue tension and restore range of motion and symmetry remember that preventive surgery has a short-term effect for about the hips. Significant acetabular dysplasia is corrected by I

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a pelvic osteotomy at the time of bony reconstruction (Flynn older children (Miller et al. 1997b). Scoliosis and pelvic obliq- and Miller 2002). uity are so prevalent that hip and spine management should be Following surgery, radiological monitoring of hip develop- considered together. ment and scoliosis should continue until skeletal maturity, at If the opportunity for reconstructive surgery has been least. In GMFCS levels IV and V, the single biggest threat to missed and hip salvage surgery is required, the degree of femoral hip status after successful reconstruction is progression of sco- head and acetabular deformity should be carefully evaluated in liosis and pelvic obliquity. the context of the child’s health, functioning, and life expect- Progressive scoliosis is common in GMFCS level IV but ancy (Flynn and Miller 2002). Referral to an appropriate pain differs from that seen in GMFCS level V. It tends to start a management service should be considered as a number of teen- little later, is not so rapidly progressive and the outcomes of agers can be managed non-operatively in the short term. Reflex surgery are better because medical comorbidities are fewer and spasms of the hip adductors and flexors are almost always part less severe. Management of scoliosis will be considered in more of the pain problem in dislocated hips. Short-term sympto- detail in the section on GMFCS level V. matic relief can often be achieved by injecting the hip joint with Adolescent: Continued clinical and radiological screening of bupivicaine and corticosteroid and injecting the hip adductors hip and spine status are paramount. Surgery as required accord- and flexors with BoNT-A. Open releases of the contracted hip ing to the principles and techniques stated above. adductors and phenolization of the obturator nerve may be helpful. These interventions have been reported to give short- GMFCS level V term pain relief but no long-term studies have been reported Mobility is dependent on wheelchair use and usually the assist- (Lundy, Doherty, and Fairhurst 2009). ance of another person. There is immense difficulty with anti- It is also important to optimize tone management prior to gravity head and trunk movement and minimal upper and salvage surgery as the surgery is much easier to perform when lower limb control. global tone is reduced. ITB pump may be an appropriate choice Function: FMS 1,1,1; FAQ 1 at best 2 possibly. for managing tone, and if so, should be done before hip surgery. Sagittal gait pattern: not applicable as children are non In a few children a marked reduction in tone around the hips ambulant. may reduce pain and postural deformities to such a degree that Musculoskeletal: salvage surgery may not be required. Lower limb: The movement disorder can be dominated by There is no single, reliable salvage surgery for the painful spasticity or dystonia, or a combination of both, and is usually dislocated hip, therefore the best treatment is prevention. The severe and generalized. ITB can help reduce discomfort and need for salvage surgery is best avoided by early hip surveillance enhance day to day care. Physical activity is extremely limited and appropriately timed preventive and reconstructive surgery. and most of the day would be spent in a sitting position and Spinal deformity affects approximately two-thirds of chil- some time in lying. Contracture of the hip flexors, knee flexors dren in GMFCS level V but is variable in its onset, severity, and the feet will tend to occur but the most serious problems progression, and effects. Kyphosis in the dorsal spine is very are those posed by hip displacement and spinal deformity. common in younger children with weak paraspinal muscles and Hips: Mean femoral neck anteversion (FNA): 40° is best managed by having the wheelchair seat slightly reclined, Mean neck shaft angle (NSA): 163° the use of chest straps and occasionally a thoraco-lumbar-sacral Risk of hip displacement (migration percentage orthosis (TLSO). Kyphosis in the lumbar spine is less common >30%): 90% and may be related to severe contractures of the hamstrings. Spine: severe curves (>40°) are seen in 50% of adolescents. Proximal hamstring recession may be beneficial. Lordosis in the They progress rapidly and surgical correction is usually required. lumbar spine is much more common and is frequently related The long ‘C’ shaped curves in cerebral palsy, present early in to hip flexion contractures. Lengthening of the hip flexors childhood, are likely to be progressive and can progress rapidly. before the lordosis becomes excessively severe or fixed may be Progression may continue after skeletal maturity if the curve is appropriate. An ITB pump may be helpful in the management more than 40 degrees (Miller 2005). of muscle imbalances about the hip that are causing secondary Management: Management is for comfort, optimal function lumbar lordosis or kyphosis deformities. (despite being limited) and ease of care giving. Posture and A variety of options may be considered for managing sco- alignment of the body in the wheelchair is paramount for liosis. Non-operative management such as physiotherapy, injec- comfort, therefore there is emphasis on the status and manage- tions of BoNT-A and electrical stimulation have been tried and ment of the feet, hips, and spine to ensure this. are not effective for scoliosis (McCarthy et al. 2006). Customized All ages: Preventive hip surgery has a high failure rate and seating with moulded inserts may improve sitting balance and should be considered to be a temporizing measure for most comfort, but do not slow curve progression. Bracing of scoliosis children in GMFCS level V (Shore et al. 2012). Reconstructive in cerebral palsy is poorly tolerated and is also ineffective in I surgery is technically easier and probably more successful in avoiding progression in the long term. It may be appropriate

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to offer bracing or seating modifications in younger children, Castle MF, Schneider C (1978) Proximal femoral resection-interposition combined with careful clinical and radiographic monitoring. arthroplasty. J Bone Joint Surg Am 60: 1051–4. Catanese AA, Coleman GJ, King JA, Reddihough DS (1995) Evaluation of This gives parents and caregivers the opportunity to learn about an early childhood programme based on principles of conductive educa- the natural history of the curve in their child, and come to tion: the Yooralla project. J Pediatr Child Health 31: 418–22. terms with the need for major spinal surgery. In one study, Clohisy JC, Barrett SE, Gordon JE, Delgado ED, Schoenecker PL (2006) Periacetabular osteotomy in the treatment of severe acetabular dysplasia. bracing was helpful in curves <40 degrees in ambulant children J Bone Joint Surg Am 88 (Suppl. 1): 65–83. but these are not the typical curves seen in GMFCS V (Terjesen, Connelly A, Flett P, Graham HK, Oates J (2009) Hip surveillance in Lange, and Steen 2000). Bracing in children in GMFCS V may, Tasmanian children with cerebral palsy. J Pediatr Child Health 45: 437–43. at best, slow curve progression in younger children with small Cosgrove AP, Corry IS, Graham HK (1994) Botulinum neurotoxin in the curves and good compliance. Surgery may be postponed but management of the lower limb in cerebral palsy. Dev Med Child Neurol will still be required for the majority of children (McCarthy 36: 386–96. Damiano DL (2006) Activity, activity, activity: rethinking our physical et al. 2006). therapy approach to cerebral palsy. Phys Ther 86: 1534–40. The functional goal of scoliosis surgery in cerebral palsy is Damiano DL, DeJong SL (2009) A systematic review of the effectiveness to improve sitting balance and ease the burden of care (Tsirikos of treadmill training and body weight support in pediatric rehabilitation. et al. 2004). The biomechanical goal is to achieve a stiff, J Neurol Phys Ther 63: 27–44. Damiano DL, Dodd K, Taylor NF (2002) Should we be testing and training well balanced spine over a level pelvis with flexible, enlocated muscle strength in cerebral palsy? Dev Med Child Neurol 44: 68–72. hips. In the majority of children, the surgical goal is a long Davids JR, Mason TA, Danko A, Banks D, Blackhurst D (2001) Surgical posterior spinal fusion with the fusion limits from high in the management of hallux valgus deformity in children with cerebral palsy. J Pediatr Orthop 21: 89–94. dorsal spine to the pelvis. Spinal fusion in children with CP Davids JR, Ounpuu S, DeLuca PA, Davis RB (2003) Optimization of improves sitting position and appears to improve upper limb walking ability of children with cerebral palsy. J Bone Surg Am 85: function, eating and respiratory function. It may also prolong 2224–34. Davids JR, Gibson TW, Pugh LI (2005) Quantitative segmental analysis of life and improve the quality of life (Mercado, Alman, and weight-bearing radiographs of the foot and ankle for children: normal Wright 2007). alignment. J Pediatr Orthop 25: 769–76. de Coulon G, Turcot K, Canavese F, Dayer R, Kaelin A, Ceroni D (2011) Talonavicular arthrodesis for the treatment of neurological flat foot Acknowledgements deformity in pediatric patients: clinical and radiographic evaluation of 29 We acknowledge the help of medical illustrators Bill Reid and feet. J Pediatr Orthop 31: 557–63. Andrew Stanish for designing and producing the illustrations de Paiva A, Meunier FA, Molgo J, Aoki KR. Dolly JO (1999) Functional for this chapter. We also thank Mary Sheedy for typing the repair of motor endplates after botulinum neurotoxin type A poisoning: biphasic switch of synaptic activity between nerve sprouts and their drafts, compiling the illustrations, and proofreading the copy. parent terminals. Proc Natl Acad Sci U S A 96: 3200–5. Desloovere K, Molenaers G, Jonkers I, et al. (2001) A randomized study of combined botulinum neurotoxin type A and casting in the ambulant child with cerebral palsy using objective outcome measures. Eur J Neurol REFERENCES 8 (Suppl. 5): 75–87. Dias RC, Miller D, Dabney K, Lipton G, Temple T (1996) Surgical correc- Abu-Rajab RB, Bennet GC (2007) Proximal femoral resection-interposition tion of spinal deformity using a unit rod in children with cerebral palsy. arthroplasty in cerebral palsy. J Pediatr Orthop B 16: 181–4. J Pediatr Orthop 16: 734–40. Albright AL (2008) Neurosurgical options in cerebral palsy. 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