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Musculoskeletal aspects of cerebral palsy

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 Review article
MUSCULOSKELETAL ASPECTS OF CEREBRAL PALSY
H. Kerr Graham, P. Selber
From the Royal Children’s Hospital, Melbourne, Australia
Cerebral palsy is the most common cause of physical disa-
bility affecting children in developed countries, with an
incidence of 2.0 to 2.5 per 1000 live births.1 It is not a single
entity but a heterogeneous collection of clinical syndromes,
characterised by abnormal motor patterns and postures.
Although in most parts of the world the orthopaedic burden
secondary to poliomyelitis and myelomeningocele is declin-
ing rapidly, the prevalence of cerebral palsy is static or
increasing. It is the most common diagnosis after trauma in
most paediatric orthopaedic units and is therefore of enor-
mous strategic importance in terms of allocation of
resources, planning and service delivery.
The definitions of cerebral palsy have undergone a
number of refinements by developmental paediatricians and
neurologists. They stress two features. First, cerebral palsy
is the result of a lesion in the immature brain, which is non-
progressive; it is a static encephalopathy.2 It is clearly
important to differentiate cerebral palsy from progressive
neurological conditions from the standpoint of both taxon-
omy and clinical management. Secondly, cerebral palsy
results in a disorder of posture and movement which is per-
manent but not unchanging.3 To this we would add a third
feature, that it results in progressive musculoskeletal pathol-
ogy in most affected children.4 It is inappropriate to empha-
sise that the cerebral lesion is static without clearly stating
that the musculoskeletal pathology will be progressive. In
Little’s original description5 of spastic diplegia, prominence
was given to the description of the musculoskeletal deform-
ities. The newborn child with cerebral palsy usually has no
deformities or musculoskeletal abnormalities at birth. Scol-
iosis, dislocation of the hip and fixed contractures develop
during the rapid growth of childhood. Cerebral palsy is a
H. K. Graham, MD, FRCS Ed, FRACS, Professor of Orthopaedic Surgery,
University of Melbourne
P. Selber, MD, Clinical Fellow
Department of Orthopaedics, Royal Children’s Hospital, Flemington Road,
Parkville, Victoria, Australia 3052.
Correspondence should be sent to Professor Graham.
©2003 British Editorial Society of Bone and Joint Surgery
doi.10.1302/0301-620X.85B2.14066 $2.00
J Bone Joint Surg [Br] 2003;85-B:157-66.
VOL. 85-B, No. 2, MARCH 2003
useful term which describes a large group of children with
motor impairment from many causes and expressed as a
wide variety of clinical syndromes. The preferred term is
therefore ‘the cerebral palsies’.6
It was formerly considered that most cases of cerebral
palsy were the result of obstetric misadventure. Careful epi-
demiological and brain-imaging studies suggest that it fre-
quently has antenatal antecedents and is often
multifactorial. Recent studies also point to an increasing
number of specific aetiological factors including intra-
uterine infections and inherited malformations.1 These
investigations will in time lead to both primary prevention
and secondary minimisation of cerebral injury. The increase
in the incidence of cerebral palsy in preterm infants is
because of neonatal intensive care and a rise in multiple
births. The rates of cerebral palsy in babies born at term are
steady, despite strategies to reduce birth asphyxia. The most
important issue in cerebral palsy is the elucidation of the
causal pathways from population-based epidemiological
studies. This will lead to primary prevention, which in a
chronic, incurable condition is clearly the most humane and
cost-effective strategy.
Cerebral palsy is subdivided according to the movement
disorder and its topographical distribution. Spastic and
mixed motor disorders account for more than 85% of chil-
dren on current registers; dyskinetic cerebral palsy is much
less common.1,6 The most common topographical syn-
dromes are spastic hemiplegia, spastic diplegia and spastic
quadriplegia which is also known as ‘whole-body involve-
ment’.1,3,6
Measuring function and natural history
In arthrogryposis multiplex congenita, Staheli (personal
communication) stated that in the first decade the most
important priority is function, in the second appearance and
in the third and subsequent decades the avoidance of pain.
This is equally true in the cerebral palsies. Most functional
gains are made in the first decade. Gross motor function in
the cerebral palsies is related to the degree of involvement,
which in turn is a manifestation of the site and severity of
the cerebral lesion. All children with spastic hemiplegia
walk independently; most of those with spastic diplegia will
walk but many require assistive devices. Those with spastic
157
158 H. KERR GRAHAM, P. SELBER

CNS pathology

PVL

Loss of Loss of connections to LMN

inhibition LMN (and other pathways)

Positive features Negative features Fig. 1

of UMN syndrome of UMN syndrome
Diagram showing the neuromusculoskeletal pathol-
ogy in cerebral palsy. The pathology of the central
nervous system in cerebral palsy is defined as a static
encephalopathy. Given the infinite variability of the
location and severity of the lesions the clinical syn-
dromes are in turn extremely variable. In motor
• Spasticity • Weakness terms, cerebral palsy results in an upper motor neu-
• Hyper-reflexia • Fatiguability rone lesion which in this diagram is considered to
have a series of positive and negative features that in-
• Clonus • Poor balance teract to produce the familiar musculoskeletal pathol-
• Co-contraction • Sensory deficits ogy. Historically, clinicians have concentrated on the
positive features and the negative features have been
relatively ignored. It is probable that the optimum
management of children with cerebral palsy will re-
Neural Mechanical quire integrated management of both the positive fea-
tures and the negative features.

Musculoskeletal pathology

Muscle shortening

Bony torsion

Joint instability

Degenerative arthritis

quadriplegia rarely have functional walking. Motor function
can be reliably measured using the gross motor function
measure and classified using the gross motor functional
classification system.7,8 In a recent study Rosenbaum et al9
described motor development in the cerebral palsies as a
series of curves of motor development. These aid our under-
standing of gross motor development in children with cere-
bral palsy of all degrees of severity. They are an excellent
guide to prognosis and have significant implications in the
understanding of the potential and limitations of manage-
ment strategies.
Neuromusculoskeletal pathology in the cerebral
palsies
The progressive nature and effects on the neuromusculo-
skeletal pathology have been understood with the help of a
number of animal models as well as clinical studies. The
key feature of the musculoskeletal pathology is a failure of
longitudinal growth of skeletal muscle (Fig. 1). An apt,
orthopaedic synonym for cerebral palsy is ‘short muscle dis-
ease’.3 The conditions for normal muscle growth are regular
stretching of relaxed muscle under conditions of physiologi-
cal loading. In children with cerebral palsy, the skeletal
muscle does not relax during activity because of spasticity
and these children have greatly reduced levels of activity
because of weakness and poor balance. Ziv et al10 demon-
strated the imbalance between the growth of long bones and
muscle-tendon units in an animal model, the hereditary
spastic mouse. Affected juveniles develop contractures of
the gastrocnemius and equinus deformity because of a fail-
ure of longitudinal muscle growth. Cosgrove and Graham11
confirmed the role of spasticity in the genesis of contrac-
tures in the hereditary spastic mouse by showing that
muscle growth can be enhanced by the injection of botuli-
num toxin A (BTX-A) soon after birth.
The musculoskeletal pathology is much more complex
and damaging than simply the development of contractures.
THE JOURNAL OF BONE AND JOINT SURGERY
 MUSCULOSKELETAL ASPECTS OF CEREBRAL PALSY
Torsion of long bones, joint instability and premature
degenerative changes in weight-bearing joints are common
and debilitating3,12,13, and young adults with cerebral palsy
commonly experience pain from early degenerative joint
disease.14
The primary aim in the management of spasticity is to
prevent the development of fixed contractures. If they occur,
correction of fixed musculoskeletal deformities is required
before the onset of decompensation. Once complex decom-
pensated joint pathology has developed, the surgical options
are limited, the rate of complications escalates and the out-
come of salvage surgery is frequently indifferent. There are
two important longitudinal studies of gait in children with
spastic diplegia which confirm that the musculoskeletal
pathology and the attendant gait disorder are progressive
during childhood.15,16 They provide an important insight
into the natural history of the process and act as a frame-
work for the interpretation of the results of surgical inter-
vention.
The upper motor neurone syndrome: weakness
and spasticity
Cerebral palsy is the most common cause of the upper
motor neurone syndrome (UMN) in childhood (Fig. 1)
which is characterised by positive features such as spastic-
ity, hyper-reflexia and co-contraction, and negative features
including weakness, loss of selective motor control, sensory
deficits and poor balance.17 Clinicians have traditionally
focused more on the positive features because it is possible
to treat spasticity, but it is the negative features which deter-
mine the locomotor prognosis. Weakness and loss of selec-
tive motor control determine when or if a child will walk.
Deficits in balance will dictate dependence on a walking
aid.
The management of spasticity in the cerebral
palsies
The management of spasticity has become increasingly
sophisticated and effective in recent years. Treatment can be
classified as temporary or permanent and as focal or gener-
alised.17,18 Intramuscular injections are temporary and
focal. The most commonly used agents are phenol and
BTX-A. Phenol has a small but useful role as a neurolytic
agent but can only be used for motor nerves such as the
obturator nerve in the presence of adductor spasticity and
the musculocutaneous nerve for spasticity of the elbow flex-
ors.17,19 BTX-A is a potent neurotoxin produced by the bac-
terium Clostridium botulinum under anaerobic conditions. It
binds to cholinergic nerve endings and inhibits release of
the neurotransmitter acetylcholine by blocking the binding
of acetylcholine vesicles to the plasma membrane of the
motor endplate. Neurotransmission is then restored by the
sprouting of new nerve endings. It takes about three months
for the original nerve endings to be restored during which
VOL. 85-B, No. 2, MARCH 2003
159
time the newly sprouted nerve endings are removed. Thus,
the effect of BTX-A is pharmacologically completely
reversible.20 When injected into skeletal muscle BTX-A
causes a dose-dependent, reversible chemodenervation of
muscle. The treatment converts paresis with muscular
hyperactivity to paresis with muscular hypoactivity. The
efficacy and safety of BTX-A in the management of spastic-
ity associated with cerebral palsy have been established in a
number of open-label studies, controlled studies and pla-
cebo-controlled, randomised clinical trials.21-23 Applica-
tions in cerebral palsy include the management of toe-
walking (dynamic equinus), scissoring (adductor spasticity)
and a crouching gait (hamstring spasticity).24-26 Additional
applications include the management of spasticity of the
upper limb and hemiplegia, and perioperative relief from
pain.27-29 In general, BTX-A should be considered to be a
focal or regional intervention. Some groups, however, have
developed an integrated multilevel approach, which in clini-
cal effect is equivalent to a medical rhizotomy.30
The benefits of the drug Baclofen are limited by side-
effects when administered orally. The poor lipid solubility
means that the drug reaches the target tissue in very low
concentrations. Administration of Baclofen directly into the
subarachnoid space can circumvent this by an implanted,
battery-driven, microprocessor-controlled pump. Intrathecal
Baclofen (ITB) is emerging as a powerful and useful
method for the management of severe generalised spastic-
ity.31,32 The principal indications are in children with severe
spastic quadriplegia with whole-body involvement, in
whom consistent reduction in muscle tone and improve-
ments in comfort and ease of care have been reported.33 ITB
is used in walking patients in some centres but the effects of
gait and function have not yet been fully studied. Some chil-
dren with spasticity associated with hereditary spastic para-
paresis respond particularly well. ITB is expensive, invasive
and associated with a high incidence of complications some
of which are life-threatening. There are reports of rapidly
progressive scoliosis. Spinal deformities should be docu-
mented before ITB therapy and monitored carefully both
clinically and radiologically. Mechanisms for safer and
more effective delivery of medication for spasticity are
clearly required.
Another neurosurgical approach for the management of
generalised spasticity of the lower limbs is selective dorsal
rhizotomy (SDR), described by Fasano more than 20 years
ago and popularised by Peacock, Arens and Berman34 and
von Koch et al35 from South Africa. Current practice
involves approaching the lumbar spine via an en-bloc lam-
inoplasty from L1 to S1 and section of 20% to 40% of the
dorsal rootlets which make up the L1 to S1 posterior nerve
roots. The replacement of the laminae and the preservation
of the facet joints reduces the risk of subsequent spinal
deformity. SDR results in an immediate and marked reduc-
tion in spasticity accompanied by weakness of the lower
limb. Intensive physiotherapy is required to regain strength
and function. The reduction in spasticity is usually followed
160 H. KERR GRAHAM, P. SELBER
by a marked improvement in the range of movement of
joints and in dynamic gait function.36 Improvements docu-
mented by instrumented gait analysis include increased
walking speed, increased stride length, a reduction in
dynamic equinus, increased range of movement at the hip,
knee and ankle and a reduction in the energy cost of walk-
ing. SDR reduces spasticity but has no effect on selective
motor control, weakness, poor balance or fixed deformi-
ties.19,36 Some studies suggest that orthopaedic deformities
including pes planovalgus, subluxation of the hip and spinal
deformities, may increase after SDR.37,38 Uncontrolled
studies of SDR reported substantial functional gains, which
were not confirmed by controlled trials.39-41 Spasticity is
only one component of the upper motor neurone syndrome
and not the most important in determining prognosis.17,42
Weakness and loss of selective motor control are more
important than spasticity and are more difficult to manage.
Uncontrolled studies are subject to bias and controlled clini-
cal trials are required to investigate efficacy. The manage-
ment of spasticity in the cerebral palsies requires a
multidisciplinary approach which should have representa-
tion from developmental paediatrics, neurology, physiother-
apy, occupational therapy, neurosurgery and orthopaedic
surgery.17
Managing weakness in the cerebral palsies
In comparison to the management of spasticity and the cor-
rection of fixed deformities, the management of weakness
has been neglected until relatively recently. The traditional
views were that muscle strengthening in children with cere-
bral palsy was neither possible nor desirable because it
might increase spasticity. Recent research has shown that
muscle strength can be reliably measured in children with
cerebral palsy and that those who participate in strengthen-
ing programmes demonstrate increases in muscle power and
improvements in function.43-45 Much work remains to be
done to devise appropriate strength-training programmes for
children with cerebral palsy and to assess their impact over
longer periods. The management of spasticity and surgery
for correction of deformity may need to be combined with
strength training for optimum benefits. Gait patterns in spas-
tic diplegia are characterised by more hip and knee flexion
than in spastic hemiplegia.46,47 Bilateral weakness of the
lower limbs may be more responsible than spasticity for the
final gait pattern. It would seem logical to intervene for both
the positive and negative features of the UMN syndrome
simultaneously (Fig. 1).
Orthopaedic surgery for fixed deformities
Most children who have management of their spasticity by
BTX-A, ITB and SDR will still require orthopaedic surgery
for the correction of fixed deformities.3,13,17-19,23 There is
evidence that the optimum management of spasticity will
delay the onset of such problems and, in doing so, postpone
the need for orthopaedic surgery.18,21,23,24 Contractures
seem to be less prevalent and less severe in the child who
has access to good management of spasticity but bony tor-
sional deformities and pes valgus remain common.18,37
Orthopaedic surgery has a major role in the management of
children with spastic cerebral palsy by correction of fixed
deformity, which in certain circumstances may improve
function and quality of life.3,13,18 As outlined above, in the
second decade, appearance and integration are of central
importance to the child with physical disabilities. Surgery to
correct deformity will improve appearance and preserve
function.
Orthopaedic surgery: spastic hemiplegia
In spastic hemiplegia, orthopaedic surgery has a central role
in the management of the common deformities which affect
the upper and lower limbs. In the upper limb these include
elbow flexion, forearm pronation, wrist flexion and ulnar
deviation and thumb-in-palm and swan-neck deformities in
the digits.3,13 In the spastic stage, injection of multiple
target muscles with BTX-A can improve appearance and
function. Corry et al27 reported the results of the first pla-
cebo-controlled trial of injections of BTX-A in the upper
limb in hemiplegia and found significant reduction in
muscle stiffness and improvements in the range of move-
ment of the joints with less robust evidence for functional
improvements. Fehlings et al28 found significant functional
gains in a single-blind study, which utilised objective out-
come measures. The development of validated functional
outcome tools in recent years has been a crucial develop-
ment. The Melbourne upper-limb assessment and the qual-
ity of upper extremity skills test (QUEST) provide
orthopaedic surgeons with valid methods of measuring the
functional outcome of the management of spasticity and
orthopaedic surgery for deformities of the upper limbs in
children with spastic hemiplegia.48,49
Fixed deformities of the upper limb are usually corrected
in a one-stage multilevel approach by a combination of
management of spasticity, lengthening of fixed muscle-
tendon contractures and correction of dynamic imbalance
by tendon transfers. The goals of surgery are both cosmetic
and functional.3,13
Gait patterns in spastic hemiplegia have been classified
by Winters, Gage and Hicks46 and this provides a template
for surgical management47 (Fig. 2). Groups I and II include
children with distal involvement expressed as equinus con-
tracture and drop foot. They can be managed effectively by
lengthening of the gastrocsoleus and provision of an appro-
priate ankle-foot orthosis. However, children with proximal
involvement, groups III and IV, may benefit from instru-
mented gait analysis and more extensive, multilevel sur-
gery.50 Equinovarus deformity can be successfully managed
by a combination of tendon-lengthening and tendon transfer
but studies to date have either been uncontrolled or lacking
in objective measures of outcome. Controversy remains as
THE JOURNAL OF BONE AND JOINT SURGERY
 MUSCULOSKELETAL ASPECTS OF CEREBRAL PALSY 161

Group I Group II Group III Group IV

Drop foot True equinus Equinus/jump knee Equinus/jump knee/
hip flexion

Fig. 2

Diagram showing sagittal gait patterns in spastic

hemiplegia which have been classified by Winters et
al46 using quantitative kinematic data. These patterns
are easily recognised and provide a useful template
for surgical and orthotic management.

α >90º α >90º α >90º α >90º

— Gastrocsoleus Gastrocsoleus Gastrocsoleus
— — Hamstrings/RF Hamstrings/RF
— — — Psoas
Hinged AFO Hinged AFO Hinged AFO Solid AFO/GRAFO

Group I Group II Group III Group IV

True equinus Jump knee Apparent equinus Crouch gait

Fig. 3

Diagram of sagittal gait patterns in spastic diplegia

according to Rodda and Graham47 which provide a
template for understanding the disorder of gait, the
musculoskeletal pathology and surgical and orthot-
ic management.

α >90º α >90º α >90º α >90º

Gastroc Gastroc (Gastroc) —
— Hamstrings/RF Hamstrings/RF Hamstrings/RF
— (Psoas) Psoas Psoas
Hinged AFO Hinged AFO Solid AFO GRAFO

to the best surgical combination for this important and
common deformity. Injection of the tibialis posterior and
gastrocsoleus with BTX-A is effective in the younger child
with spastic deformities. In most children the effects are
temporary but in a few the varus deformity does not recur.21
Split transfer of the tibialis posterior seems to be ideal for
the younger child with a very flexible equinovarus deform-
ity.51 Split transfer of the tibialis anterior, combined with
intramuscular lengthening of the tibialis posterior and gas-
trocsoleus, is a better option for the older child with a stiffer
deformity.52,53 Split tendon transfers are clearly more satis-
factory and predictable than complete transfers. With early
muscle balancing progressive bony deformities are uncom-
mon and bony surgery should rarely be required.
VOL. 85-B, No. 2, MARCH 2003
Orthopaedic surgery: spastic diplegia
Despite new and effective means of managing spasticity,
most children with spastic diplegic cerebral palsy will
develop progressive musculoskeletal deformities as they
grow. These include fixed contractures of the two joint mus-
cles and a range of bony deformities, collectively referred to
as ‘lever arm disease’.54 These secondary deformities result
in progressive loss of function in most children and many
will benefit from correction by orthopaedic surgery. Tradi-
tionally, the child with spastic diplegia who presented with
toe-walking was managed by lengthening of tendo Achillis.
This was effective for the correction of the tip-toe gait but
resulted in a progressive crouching gait in many children as
162 H. KERR GRAHAM, P. SELBER
Fig. 4
Photograph showing that gait deviations in walking children with
spastic diplegia can be characterised using three-dimensional kine-
matics and kinetics and dynamic electromyography. Biomechanical
analysis is the foundation for understanding the individual patient’s
gait deviations from which a prescription for integrated management
may be derived, including management of spasticity, correction of
deformity and orthotic prescription.
the contractures at the knee and hip progressed. A clear con-
sensus has emerged that orthopaedic surgery to correct gait
problems should address all deformities simultane-
ously.3,13,18,54,55 Single-event multilevel surgery refers to
the correction of all orthopaedic deformities in one session,
requiring only one hospital admission and one period of
rehabilitation.
Sagittal gait patterns in spastic diplegia have recently
been classified by Rodda and Graham47 who provided a
management algorithm, based on the patterns (Fig. 3). The
precise operative prescription is based on a full biomechani-
cal assessment in the motion analysis laboratory. The out-
come of the surgery and rehabilitation can be determined by
a follow-up analysis 12 to 24 months after surgery.54 Sur-
gery for gait correction is practised in some centres without
preoperative gait analysis but it is difficult to plan and
impossible to evaluate the outcome without objective and
repeatable outcome measures. However, the availability of
motion analysis remains restricted to specialist centres in
most countries. Randomised clinical trials are required to
evaluate the role of instrumented gait analysis for the satis-
faction of both the orthopaedic surgeon and the funding
bodies.
Fig. 5
Photograph showing that physiological measure-
ments in the gait laboratory are important in provid-
ing functional outcome measures after intervention.
This child is wearing a face mask connected to a port-
able metabolic cart from which oxygen consumption
and production of carbon dioxide can be measured on
a breath-by-breath basis.
Instrumented analysis in modern gait laboratories
includes a standardised physical examination, two-dimen-
sional video recording of gait, three-dimensional kinematics
and kinetics, dynamic electromyography, pedobarography
and measurement of the energy cost of walking.54 Bio-
mechanical analysis has added greatly to the understanding
of pathological gait patterns and provides an objective out-
come measure after surgery for correction of gait (Fig. 4).
However, physiological monitoring by the measurement of
self-selected walking speed and the energy cost of walking
(Fig. 5) complements the biomechanical measures, provid-
ing a global measure of function.54-56 It is important that
children who are taller and straighter after gait correction
are not made weaker and slower.
Correction of fixed contractures of muscle-tendon units
in spastic diplegia is achieved by muscle-tendon recession
or fractional lengthenings.54-57 The operative procedures
must be conservative, stable for early weight-bearing and
preserve strength (Figs 6 and 7). The accepted operations
are lengthening of the psoas at the pelvic brim by either the
Sutherland or Gage technique, fractional lengthening of the
medial hamstrings, transfer of rectus femoris to semitendi-
nosus for a stiff-knee gait and recession of the distal apone-
THE JOURNAL OF BONE AND JOINT SURGERY
 MUSCULOSKELETAL ASPECTS OF CEREBRAL PALSY
Fig. 6
Photograph showing that in spastic diplegia spastici-
ty and contracture affect the flexors of the hip, the
knee, and the ankle which results in a sagittal gait
pattern known as ‘jump gait’. In this gait pattern,
management of spasticity and correction of deformi-
ty needs to be applied bilaterally, at all three levels.
urosis of gastrocnemius by the Strayer technique.57-61 Open
lengthening of the adductors of the hip, tenotomy of the ilio-
psoas at the lesser trochanter and lengthening of tendo
Achillis are rarely required in spastic diplegia.54 These pro-
cedures may result in overcorrection, which is impossible to
salvage.54,62 However, there has never been a randomised
clinical trial in which operative techniques have been com-
pared. Views on the surgical management of spastic diplegia
are not supported by a high level of evidence.63
Correction of bony torsion and of pes valgus requires
rotational osteotomies and stabilisation of the foot, respec-
tively.54 Correction of in-toed gait and medial femoral tor-
sion by external rotation osteotomy of the femur is very
effective and the results are lasting.64 Proximal femoral
osteotomy is the procedure of choice for in-toed gait in the
presence of hip dysplasia or subluxation. If the hips are sta-
ble, distal femoral osteotomy is an equally effective proce-
dure, which offers some advantages over proximal femoral
osteotomy.65 In either case, precise correction, stable fixa-
tion and early mobilisation are essential to a good outcome.
Supramalleolar osteotomy of the tibia and fibula is the best
technique for correction of lateral tibial torsion. Fixation
can be achieved by crossed Kirschner wires or the ‘T’ plate
from the AO (ASIF) small fragment set.66 Correction of the
abducted valgus foot can be successfully achieved in most
VOL. 85-B, No. 2, MARCH 2003
163
Fig. 7
Photograph showing that surgical correc-
tion of fixed deformities should address
all three levels simultaneously with sin-
gle-event multilevel surgery.
children by lengthening of the os calcis, as described by
Evans67 and popularised by Mosca.68 This procedure avoids
a fusion in the hindfoot but the results may not be as durable
as subtalar fusion. Severe deformities may require an extra-
articular fusion of the subtalar joint by the Princess Marga-
ret Rose technique, incorporating screw fixation and auto-
genous bone graft from the iliac crest.69 Combined
lengthening of the os calcis and subtalar fusion is very
effective in neglected feet with severe deformities. Bony
surgery in children with spastic diplegia can be associated
with slow rehabilitation and loss of function. The rehabilita-
tion programme is as important as the surgery and must be
closely supervised.54,55
Orthopaedic surgery: spastic quadriplegia
The medical and orthopaedic management of the child
with spastic quadriplegia or whole-body involvement is
particularly challenging. Hip displacement and spinal
deformities are common, are often rapidly progressive and
occur in the context of multiple medical co-morbidities
including epilepsy, respiratory disease, nutritional deficien-
cies and osteopenia.13 The most common movement disor-
der is spastic dystonia, which may be successfully
managed in some children by ITB.13,19 Orthopaedic sur-
164 H. KERR GRAHAM, P. SELBER
gery should only be practised in the context of a multidisci-
plinary team, offering expertise in the management of
medical co-morbidities, paediatric management of pain
and intensive care.17
The prevalence of hip displacement is approximately 1%
in spastic hemiplegia, 5% in spastic diplegia and 35% to
55% in spastic quadriplegia.13,70 Children with hip dis-
placement and either spastic hemiplegia or spastic diplegia,
have such a significant gait disorder that they are very
quickly referred to the orthopaedic surgeon. However, in
spastic quadriplegia the diagnosis of hip displacement is fre-
quently delayed, which limits the management options. The
reasons for delayed diagnosis are simple. Hip displacement
in the spastic child is silent in the early stages and the par-
ents, carers and paediatricians are focused on much more
obvious issues such as feeding difficulties and the manage-
ment of seizures. The goal of management of the hip should
be early detection of abnormality by systematic screening
and prevention of dislocation by simple soft-tissue sur-
gery.70,71 It is more effective to prevent dislocation by
simple soft-tissue surgery than to subject a child to the risks
of major reconstructive operations. When the latter are
required, a one-stage open reduction, combined with a varus
derotation and shortening osteotomy of the proximal femur
and a pelvic osteotomy, offers the best prospect of long-
term stability of the hip.72-74 The results of salvage surgery
are at best indifferent and unpredictable. Recent studies uti-
lising CT and three-dimensional reconstruction have added
to our knowledge of the pathoanatomy of hip displacement
in cerebral palsy.75,76 Defining the direction of the displace-
ment and the location of the acetabular deficiency have a
direct bearing in planning reconstructive surgery.
Correction of spinal deformity is a challenging and
important part of the surgical management of the children
with spastic quadriplegia. Prevention of hip dislocation,
fixed hip deformity and pelvic obliquity are important in
minimising spinal deformity.13 The standard care for severe
spinal deformity is a long, instrumented posterior fusion to
the pelvis.13 Adequate correction may require anterior sur-
gery if the deformity has been neglected. We know that
good correction of spinal deformities can be achieved with
acceptable morbidity and mortality in children with severe
spastic quadriplegia. We do not know if the surgery
improves the quality of life because we lack the appropriate
instruments to measure it.
The survival of increased numbers of children affected
by cerebral palsy inevitably means an increase in the num-
bers of young adults with varying degrees of physical disa-
bility and extensive musculoskeletal pathology. These
adults have complex needs, which few medical systems
have the planning or resources to deal with.14
Conclusions
Orthopaedic surgery has much to offer in the management
of many children with cerebral palsy, including those with
spastic hemiplegia, spastic diplegia and spastic quadriple-
gia.3,13,54 However, evidence for the efficacy of most ortho-
paedic operations is lacking. We run the risk of being
marginalised and funding being directed elsewhere. The
size of the treatment effect of injections of BTX-A in the
management of spastic equinus is much less than for
muscle-tendon surgery. However, the use of BTX-A is sup-
ported by several randomised clinical trials and there are no
comparable controlled trials of orthopaedic surgery in chil-
dren with cerebral palsy.77 Orthopaedic surgeons have
focused too much on the measurement of deformity and dis-
ability and not enough on validated functional outcome
measures. The National Centre for Medical Rehabilitation
and Research has developed a model to translate medical
findings into clinical benefits for individuals with disabling
conditions, by describing five domains of disability: patho-
physiology, impairment, functional limitation, disability and
societal limitation.78 The International Classification of
Impairments Disabilities and Handicaps of the World
Health Organisation similarly widens the perspective from a
narrow focus on disability to an emphasis on health and
function in society.79 The next generation of orthopaedic
researchers in the field of disability must embrace this wider
perspective or risk being isolated, their research being
underfunded and their findings ignored.
Orthopaedic surgery for children with cerebral palsy
undoubtedly makes them different and most orthopaedic sur-
geons are convinced that by correcting deformities we are
improving function and quality of life. We must improve the
evidence for the value of orthopaedic surgery for children
with cerebral palsy by appropriate clinical trials and by
applying comprehensive, balanced, validated outcome meas-
ures.
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