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Indian J Pediatr

DOI 10.1007/s12098-015-1999-5

REVIEW ARTICLE

Musculoskeletal Evaluation of Children with Cerebral Palsy


Ratna Johari 1 & Shalin Maheshwari 1 & Pam Thomason 2,3 & Abhay Khot 1,2

Received: 22 July 2015 / Accepted: 16 December 2015


# Dr. K C Chaudhuri Foundation 2016

Abstract Cerebral Palsy (CP) is the most common


chronic disability of childhood. The problems involved
are complex; not only do these children have problems
of mobility, but a plethora of associated problems [1]. A
recent definition of CP includes secondary musculoskeletal problems [2]. The inclusion of this in the definition
recognises the significance of musculoskeletal problems
and the impact these problems have on the lives of
children with CP and their families. Orthopedic management of the child with CP aims to reduce the impact of
these musculoskeletal problems to help the child with
CP to reach his maximum potential [3]. To accurately
assess children and prepare management plans, a combination of medical history, physical examination, functional assessment, medical imaging, observational and
instrumented gait analysis, and assessment of patient
and family goals must be interpreted [4]. A detailed
annual orthopedic assessment for all children with CP
is recommended [5]. For an ambulant child, more frequent assessments are required during periods of rapid
growth, observed deterioration in physical examination
measures, and after interventions, including gait
Electronic supplementary material The online version of this article
(doi:10.1007/s12098-015-1999-5) contains supplementary material,
which is available to authorized users.
* Abhay Khot
abhay.khot@me.com
1

Department of Orthopedics, Royal Childrens Hospital,


Melbourne, Australia

Hugh Williamson Gait Analysis Laboratory, Royal Childrens


Hospital, Melbourne, Australia

Murdoch Childrens Research Institute, Parkville, Australia

correction surgery. For a non-ambulant child, more frequent assessments are indicated according to hip surveillance guidelines [6, 7], during periods of observed
deterioration, and following interventions such as hip or
spine surgery. A systematic and practiced routine is conducive to efficiency and accuracy [5]. This paper discusses the Physical Examination Protocol used by the
Hugh Williamson Gait Analysis Laboratory, in Melbourne, Australia.
Keywords Cerebral palsy . Disability . Orthopedic . Physical
examination

Definition
Cerebral palsy (CP) is a very common childhood condition,
with a consistent prevalence rate estimated at 2 per 1000 children in developed countries [1, 8].
Cerebral palsy describes a group of permanent disorders of the development of movement and posture, causing activity limitation that are attributed to non- progressive disturbances that occurred in the developing fetal or
infant brain. The motor disorders of cerebral palsy are
often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy, and by secondary musculoskeletal problems. [2]
This definition of CP is the first to recognise the
significance of musculoskeletal problems and the impact
these problems have on the lives of children with CP
and their families [2].
Orthopedic management of the child with CP aims to
reduce the impact of these musculoskeletal problems to

Indian J Pediatr

help the child with CP to reach their maximum potential


[3]. The main orthopedic challenges involve those of
spasticity, delay in developmental milestones, posture
and movement problems, developing soft tissue contractures and bony deformity. As these problems change
over time and as the child grows, accurate assessment
of these problems is required to ensure appropriate and
timely orthopedic management.
To accurately assess children and prepare management plans, a combination of medical history, physical
examination, functional assessment, medical imaging,
observational or instrumented gait analysis, and assessment of patient and family goals must be undertaken
and interpreted [4]. Physical examination of the child
with CP is not easy. It is a three-way relationship between the child, the clinician and the family. The examination cannot succeed unless the clinician gains the
confidence and trust of both the child and the parent/
caregiver.
This article discusses the musculoskeletal evaluation
protocol used by the multidisciplinary team of The
Hugh Williamson Gait Analysis Laboratory, Royal Childrens Hospital, in Melbourne, Australia.

Classification
CP can be classified in several ways; by topography, by
movement disorder and by function. Classification can
assist with regions of the body that should be evaluated
(topography), help identify risks of contracture (movement disorder) and deformity (motor function), assist
with prognosis (motor function) and outcome of interventions (movement disorder, motor function) [5]. An
example of this is the relationship: risk of hip displacement to motor function [9, 10].
Each classification is discussed briefly below.

body involvement is used interchangeably with


quadriplegia.
The European system of classification classifies spastic cerebral palsy as unilateral if limbs on one side of
the body are involved and bilateral if limbs on both
sides are involved [13].
Movement Disorder
The movement disorder can be classified as spastic, hypotonic, hyperkinetic, ataxic or mixed.
Spasticity is defined as hypertonia in which one
or both of the following signs are present: 1) resistance to externally imposed movement increases
with increasing speed of stretch and varies with
the direction of joint movement, and/or 2) resistance to externally imposed movement rises rapidly above a threshold speed or joint angle [14].
Hyperkinetic movements are unwanted or excess
movements that may be seen in children with CP. Hyperkinetic disorders seen in CP include dystonia, chorea,
and athetosis. Dystonia is a movement disorder in
which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures, or both. Chorea is an ongoing
random-appearing sequence of one or more discrete involuntary movements or movement fragments. Athetosis
is a slow, continuous, involuntary writhing movement
that prevents maintenance of a stable posture [15].
Ataxia is defined as an inability to generate a normal or
expected voluntary movement trajectory that cannot be
attributed to weakness or involuntary muscle activity
about the affected joints [16].

Topography

Functional Classification

CP may be classified topographically [11] as monoplegia,


hemiplegia, diplegia, triplegia, or quadriplegia. Sometimes
paresis is substituted for the plegia portion of the term,
without any change in meaning. Monoplegia usually affecting
one lower limb is uncommon and may be the presentation of a
very mild hemiplegia. Hemiplegia refers to one side of the
body being involved. Children with diplegia have much
greater involvement of the lower than upper limbs, but there
may still be some fine motor deficiencies in the upper
limbs. Whereas in quadriplegia, all four limbs and the
trunk are involved with the upper limbs equally or more
involved than the lower limbs [12]. Sometimes total

There are three functional classifications, one for gross


motor function, and one for upper limb function and
one for communication.
The Gross Motor Functional Classification System
The Gross Motor Functional Classification System (GMFCS)
[1719] was developed to create a systematic way to describe
the functional abilities and limitations in motor function of
children with CP. The purpose is to classify a childs
present gross motor function. There are descriptors for
describing motor function for different age ranges.

Indian J Pediatr

The Manual Ability Classification System

based on the childrens self-initiated ability to handle


objects and their need for assistance or adaptation to
perform manual activities in every-day life. The five
levels are:

The Manual Ability Classification System (MACS) [20] describes how children with CP use their hands to handle objects
in daily activities. MACS describes five levels. The levels are

I Handles objects easily and successfully.


II Handles most objects but with somewhat reduced quality
and/or speed of achievement.

Figures 1 and 2 describe the descriptors for ages 6 to


12 y and 13 to 18 y.

Fig. 1 GMFCS E&R between


6th and 12th birthday: descriptors
and illustrations

GMFCS E & R between 6th and 12th birthday:


Descriptors and illustrations
GMFCS Level I
Children walk at home, school, outdoors and in the
community. They can climb stairs without the use
of a railing. Children perform gross motor skills such
as running and jumping, but speed, balance and
coordination are limited

GMFCS Level II
Children walk in most settings and climb stairs holding
onto a railing. They may experience difficulty walking
long distances and balancing on uneven terrain,
inclines, in crowded areas or confined spaces.
Children may walk with physical assistance, a handheld mobility device or used wheeled mobility over
long distances. Children have only minimal ability to
perform gross motor skills such as running and jumping.

GMFCS Level III


Children walk using a hand-held mobility device in
most indoor settings. They may climb stairs holding
onto a railing with supervision or assistance. Children
use wheeled mobility when traveling long distances
and may self-propel for shorter distances.

GMFCS Level IV
Children use methods of mobility that require physical
assistance or powered mobility in most settings. They
may walk for short distances at home with physical
assistance or use powered mobility or a body support
walker when positioned. At school, outdoors and in
the community children are transported in a manual
wheelchair or use powered mobility.

GMFCS Level V
Children are transported in a manual wheelchair
in all settings. Children are limited in their ability
to maintain antigravity head and trunk postures and
control leg and arm movements.

Indian J Pediatr
Fig. 2 GMFCS E&R between
12th and 18th birthday:
descriptors and illustrations

GMFCS E & R between 12th and 18th birthday:


Descriptors and illustrations
GMFCS Level I
Youth walk at home, school, outdoors and in the
community. Youth are able to climb curbs and stairs
without physical assistance or a railing. They perform
gross motor skills such as running and jumping but
speed, balance and coordination are limited.

GMFCS Level II
Youth walk in most settings but environmental
factors and personal choice influence mobility choices.
At school or work they may require a hand held mobility
device for safety and climb stairs holding onto a
railing. Outdoors and in the community youth may
use wheeled mobility when traveling long distances.

GMFCS Level III


Youth are capable of walking using a hand-held mobility
device. Youth may climb stairs holding onto a railing
with supervision or assistance. At school they may selfpropel a manual wheelchair or use powered mobility.
Outdoors and in the community youth are transported
in a wheelchair or use powered mobility.

GMFCS Level IV
Youth use wheeled mobility in most settings. Physical
assistance of 12 people is required for transfers.
Indoors, youth may walk short distances with physical
assistance, use wheeled mobility or a body support
walker when positioned. They may operate a powered
chair, otherwise are transported in a manual
wheelchair.

GMFCS Level V
Youth are transported in a manual wheelchair in all
settings. Youth are limited in their ability to maintain
antigravity head and trunk postures and control leg and
arm movements. Self-mobility is severely limited, even
with the use of assistive technology.

III Handles objects with difficulty; needs help to prepare


and/or modify activities.
IV Handles a limited selection of easily managed objects in
adapted situations. Performs parts of activities with effort
and with limited success.
V Does not handle objects and has severely limited ability to
perform even simple actions.

The Communication Function Classification System


Communication is the exchange of information between people,
and the responsibility for communication is shared between conversational partners. The Communication Function Classification System (CFCS) [21] classifies patterns of an individuals
communication performance in one of five levels of everyday

Indian J Pediatr

communication effectiveness with a partner. The CFCS requires


a rater to know how the individual with CP communicates with
both unfamiliar and familiar communication partners in different
environments in order to establish shared understandings. The
CFCS levels are:

Hospital, in Melbourne, Australia [Appendix 1 (Supplementary information)] is discussed.

History is a key component in evaluating the child. It provides


valuable information for diagnosis. In children with a definite
diagnosis, the timing of achievement of developmental milestones and the presence of associated impairments help to
decide a functional prognosis. Risk factors associated with
CP are grouped into prenatal, perinatal, and postnatal factors.
Prematurity and low birth weight are the two most important
risk factors in developed countries with high standards of obstetrical care. Postnatal risk factors like central nervous system
infections and seizures additionally play a major role in other
countries [3, 8].
The clinician may gain insight into outcomes from previous interventions as well as an understanding of concomitant
interventions.
Identification of psychosocial issues and the goals and aspirations of the child and family are also important to identify
from the subjective examination.

Effective Sender and Receiver with unfamiliar and


familiar partners.
II Effective but slower paced Sender and/or Receiver with
unfamiliar and/or familiar partners.
III Effective Sender and Receiver with familiar partners.
IV Inconsistent Sender and/or Receiver with familiar
partners.
V Seldom Effective Sender and Receiver even with
familiar partners.

Musculoskeletal Examination
Musculoskeletal Pathology
Children with CP have no musculoskeletal deformities
at birth; these develop as the child grows [22]. The
most common feature of the musculoskeletal pathology
is contracture of muscle-tendon units. Muscles have reduced cross-sectional area and alterations in fascicle
length and pennation angle [23]. Muscles are also weaker and stiffer than in typically developing children.
Muscles that cross two joints are more difficult to
stretch during functional activities, and contractures result in more functional impairments than contractures of
one-joint muscles [23].
Bony torsion deformity and joint contractures may also
develop over time and will affect function, particularly gait.
Development of hip displacement and dysplasia, if undetected, may lead to pain and hip dislocation and may have a
significant effect on quality of life [10].
Since the musculoskeletal pathology is progressive,
standardised techniques for assessment of muscle length, contractures, bony torsion and joint contractures is helpful for
general assessment, surgical planning and early detection of
hip displacement and spine deformity [5].
The physical examination measures are interpreted in
conjunction with information from multiple sources including the clinical history, functional scales, observational and instrumented gait analysis (in ambulant children), and medical imaging. The synthesis of these
seemingly disparate areas of information into a diagnostic matrix is vital for a global and comprehensive orthopedic assessment [4].
The physical examination protocol as used by the Hugh
Williamson Gait Analysis Laboratory, Royal Childrens

History

Physical Examination
Muscle Length and Joint Contracture
Muscle length measurement in the child with CP is by proxy
by measuring the range of motion of the joint which the spastic muscle crosses. Particular care and standardised positioning are required especially for muscles that cross two joints.
There are assumptions which must be considered when measuring joint range for muscle length if measurement is to be
considered accurate. The joint must not have restricted motion
and the range of motion depends on the applied force or torque
[5].
The method for assessing passive joint range of motion is
to standardise the conditions and starting position for assessment. Apply a slow stretch to the muscle while eliciting as
little muscle activation as possible. Once the end of range is
reached the angle of the joint is measured to the nearest degree, using a goniometer aligned to standardised points or
bony prominences [5].
There is use of two examiners, one to hold and maintain the
limb position while the other examiner measures the angle
assists with reliability and repeatability of the measurement.
Reliability studies suggest that the standard error of measurement [24, 25] of passive joint range is of the order of 5.
It is helpful to note that measuring contractures is more
accurate under anesthesia when stretch reflexes and spasticity
are abolished and anxiety or compliance are not an issue.
Measurement under anesthesia is important to confirm

Indian J Pediatr

presence and magnitude of contractures prior to undertaking


surgical procedures.
Interpretations of these measurements with respect to gait
and function can be complicated by spasticity, muscle weakness and altered selective motor control. The position in which
the child is examined, that is non weight bearing vs. weight
bearing as in standing and walking can influence interpretation and agreement between physical examination and gait
analysis measurement [5].
Video Assessment of Physical Examination
Video recordings are useful to demonstrate walking ability
and gait pattern. Video or still camera pictures of physical
examination measures may be useful to document range and
demonstrate the childs progress to their parents or carers.
Recently, the introduction of video analysis software has been
suggested to improve reliability of observational gait assessment [26]. Watching the video repeatedly and in slow motion
may help to better understand the gait. Measurements of range
of motion using these programs should be used with strict
protocols in place and caution with acceptance of measurement accuracy as the location of camera and position of child
in relation to camera (parallax) may be an issue [5, 26].
Skeletal Alignment- Bone and Joint Deformity
Femoral neck anteversion is the angle between the femoral
neck axis and the knee joint axis when both are projected onto
the transverse plane of the femur. The patient is placed prone
and the pelvis is stabilised. The knee is flexed to 90 degrees
and the trochanter is palpated. The hip is rotated until the
trochanter is most prominent. The angle as measured compared to the vertical is the femoral neck anteversion [3, 5].
Tibial torsion is defined as the angle between the knee and
ankle joint axes when both are projected onto the transverse
plane of the tibia. The patient is placed prone and the pelvis is
stabilised. The angle between the bimalleolar axis and the line
perpendicular to the line down the centre of the thigh is a
measure of the external tibial torsion [3, 5].
Measures of the alignment of structures of the foot are also
important. The standing alignment of the foot must be documented. This should specify the overall posture of the foot and
ankle. The hind foot position should be noted as neutral, varus
or valgus. Specific deformities such as hallux valgus or dorsal
bunion may be present. The ankle and subtalar alignment
should be assessed in the prone position, and the orientation
of the midfoot and the forefoot to the hind foot must be documented. If deformity is noted, its corrigibility must be
assessed and the contribution of muscle contractures and spasticity to the physical appearance must be noted [3, 5].
Bony torsion and joint deformities are structural and do not
vary with positioning or muscle activity. It is still important

however, to ensure that the child is positioned correctly to


allow the measurement to be made as specified. Joint deformities are included with bony deformities because it can be
difficult to distinguish between them without reference to
medical imaging [3, 5].
Muscle Strength
Muscle strength is defined as the ability to generate normal
voluntary force in a muscle or normal voluntary torque
(moment) about a joint [15]. In practice, it is the ability to
generate a moment that is assessed. True moment generating
capacity is dependent on the muscle length and contraction
velocity as well as the inherent properties of both muscle and
tendon.
The Modified Oxford scale [27] is described in Appendix 1
(Supplementary information). The muscle strength grading is
through full available joint range but strength may vary considerably throughout range. Hence, there may be some limitations in using this scale. This is sometimes more pronounced
in children with CP, for example quadriceps lag. Chronic excessive knee extensor forces of rectus femoris spasticity and
crouch gait, often cause patella alta. This may be associated
with terminal knee extensor dysfunction.
Extensor lag is measured with the patient positioned supine
(to relax hamstrings if a problem) or sitting, and the leg flexed
at the knee over the end of the examination table. The child is
asked to actively extend the knee as much as possible. The
extensor lag is recorded as the difference between the active
range and passive range of motion [5].
Clinical Measurements of Spasticity
The Modified Ashworth Scale (MAS) [28] was initially described as a measure of spasticity, but this may depend on the
speed at which the test is performed. In the original paper it
was suggested that the movement of the limb segments should
be performed through the full range of movement over a one
second time interval. This is too fast to be a test of tone but not
fast enough to be test of spasticity during walking. The Hugh
Williamson Gait Analysis Laboratory protocol uses a slower
examination speed and uses the MAS as a measure of stiffness
rather than spasticity [5].
The Modified Tardieu Test is a measure of spasticity,
which, being recorded as an angle, is convenient for correlating with gait analysis [29]. In this test, which is a simplified
version of Tardieus original protocol [30], muscle length is
assessed first using the procedures outlined above, moving the
limb segment slowly. The test is then repeated, performing the
movement as fast as possible. If spasticity is present then the
joint will catch at a specific angle and it is this angle that is
reported as the result of the test. It is common to repeat the
movement three times to get a feel for the catch and make a

Indian J Pediatr

measurement of the final catch. In the absence of spasticity the


Modified Tardieu Test angle will be approximately the same
as the muscle length angle. The larger the difference between
the two angles the greater the spasticity is assumed to be [5].
Selective Motor Control
There are few systematic approaches to specifying selective
motor control at a muscle or muscle group level. The three
point scale (0/1/2) for selectivity described in the Hugh
Williamson Gait Analysis Laboratory Protocol [Appendix 1
(Supplementary information)] is one approach. The selective
motor control assessment of the lower extremity [31] may be
used to quantify general selective motor control of the lower
limbs [5].
Instrumented Gait Analysis
Instrumented gait analysis (IGA) is a useful tool to assess an
individuals gait pattern and how the gait deviates from the
typically developing gait pattern of age matched peers. IGA is
an objective, standardized, reproducible and quantifiable
method to evaluate gait pathology [3]. Components of IGA
may include:
Kinematics: Measurement of joint motion during walking.
Dynamic electromyography: Electrophysiological monitoring of muscle activity using surface or fine wire (for tibialis
posterior) electrodes placed on key muscles during walking.
Kinetics: Assessment of force vectors using force plates.
Energetics: Evaluation of oxygen consumption and energy
cost of walking using gas analysis systems.
Dynamic pedobarography: Pressure changes of the sole of
the feet during walking [3].
Interpretation of IGA data is done in conjunction with medical history, physical examination, medical imaging and patient and family concerns and goals. From this a comprehensive list of impairments that are affecting the childs gait can
be determined and a management plan can be agreed with the
child and family. A full description of IGA is beyond the
scope of this paper [3].

anterior-posterior pelvic radiograph (AP) has been shown to


be the radiographic measure to best monitor hip displacement.
Regular physical assessment and AP pelvic radiographs
should be conducted according to specific guidelines. At the
Royal Childrens Hospital, Melbourne hip displacement is
monitored following the Australian Hip Surveillance Guidelines, 2014. (These guidelines can be downloaded from the
website: www.ausacpdm.org.au.)
Radiographic evaluation of spine includes frontal and lateral views of the whole spine in standing (whenever possible),
sitting, or supported sitting. The location of scoliosis is documented as well as decompensation and shoulder balance. The
flexibility of the scoliosis is assessed by passive side bending
as well as during partial suspension.
Patella position for patella alta can be measured with a
lateral radiograph of the knee taken in full extension. The
superior pole of the patella is typically one finger width proximal to the adductor tubercle. Alta may contribute to patellafemoral instability, pain and subluxation.
Radiographic imaging of the foot in weight-bearing is another vital part of the comprehensive orthopedic assessment.
Normative values have been published [32, 33] and provide
objective information to assess the effects of surgery to stabilize the mid-foot by oscalcis lengthening or subtalar fusion
[32, 33].
Computed Tomography
This is a valid and reliable method for measuring bony torsion
of the femur and tibia. It is a useful adjunct to clinical measures of torsion [34]. The imaging team must be experienced
and appropriate protocols must be followed. The exposure to
ionising radiation remains a concern, it may be replaced by
magnetic resonance imaging or ultrasound.
EOS
Low- dose stereoradiography with three dimensional image
reconstruction using the EOS system may offer the opportunity for reliable measurements of bony torsion with much
lower doses of radiation [35, 36].

Medical Imaging
Medical imaging is an important component of the diagnostic
matrix with different emphasis according to whether the child
is ambulant or non-ambulant [4].
Radiographs
It is important to obtain baseline hip and spine radiographs. It
is necessary to monitor hip instability. Reimers migration
percentage, which is the percentage of femoral head that is
not covered by the acetabulum measured on an

Conclusions
A detailed annual orthopedic assessment for all children with
CP is recommended.
For the ambulant child, an annual assessment is a reasonable minimum in clinical practice when changes are slow or
limited. However, more frequent assessments are required
during periods of rapid growth, observed deterioration in
physical examination measures, and after interventions, including gait correction surgery.

Indian J Pediatr

For the non-ambulant child, more frequent assessments are


indicated according to hip surveillance guidelines [6, 7], during periods of observed deterioration, and following interventions such as hip or spine surgery.
A systematic and practiced routine is conducive to efficiency and accuracy.

13.

14.

15.

Contributions RJ and SM: Data collection, manuscript preparation


and submission; PT: Manuscript review and data collection; AK: Data
collection, manuscript preparation, review, submission and will act as
guarantor for the paper.

16.

Compliance with Ethical Standards

17.

Conflict of Interest None.


Source of Funding None.

18.

19.

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