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KEY POINTS
Children with cerebral palsy are prone to development of musculoskeletal deformities.
The underlying neurlogic insult may results in a loss of selective motor control, an increase in
underlying muscle tone, and muscle imbalance, which lead to abnormal deforming forces acting
on the immature skeleton.
The severely involved child is one who is at increased risk for developing progressive musculoskeletal deformities.
Close surveillance and evaluation are key to addressing the underlying deformity and improving and
maintaining overall function.
orthopedic.theclinics.com
INTRODUCTION
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abnormalities in gait and ambulation. The compensations that children undertake to overcome
or adapt to these secondary manifestations are
termed, tertiary manifestations.1 It is in addressing
the secondary and tertiary manifestations where
orthopedists take a lead role, with the goal of correcting lever arm dysfunction, preventing progression of deformity, and optimizing overall function.
In a sense, the role of orthopedic surgeons is to
maintain, improve, or optimize a childs function
and alter the natural history of the condition.
CLASSIFICATION
Classification systems help define and quantify
the underlying pathology. They help determine
and guide clinicians toward the most appropriate
treatment and aid in communication between clinicians. Several classification systems have been
proposed, dating as far back as the 1800s. The
most comprehensive of these is the classification
proposed by Minear in 1956,2 which takes into
consideration every aspect of a child, including
physiologic, topographic, etiologic, traumatic,
neuroanatomic, functional, and therapeutic involvement. Bax and colleagues3 proposed a
simpler classification system based on motor
abnormalities, associated impairments, anatomic
and radiographic findings, and causation and
timing. Functional classification schemes are
also commonly used and these are based on a
childs overall functional capability. The Gross
Motor Functional Classification System (GMFCS)
is the most widely used functional scheme. It divides children into 5 groups based on the overall
functional capability (Table 1).4 The Functional
Mobility Scale (FMS) describes motor function
into 6 levels in 3 domains based on typical
walking distance of 5, 50, or 500 m. This system
is used to monitor change in motor function over
time.5
Table 1
Gross motor function classification system
GMFCS
Level
I
II
III
IV
V
Description
No functional impairment
Functional limitation, may need
assistive device
Assistive device needed for
ambulation
Limited self-mobility, wheelchair
often required
Wheelchair bound
ASSESSMENT
Assessment of children with cerebral palsy centers on a complete history and physical evaluation.
The history must include birth history and associated underlying medical conditions. The clinical
evaluation should consist of a clinical evaluation
of gait, both barefoot and with the use of orthotics,
if any. Rotational profile should be checked to
evaluate underlying torsional malalignment. Range
of motion should be checked for the presence of
any contractures. The specific underlying muscle
tone is evaluated and recorded. A more detailed
evaluation may include strength testing and an
evaluation for selective motor control. Orthotics,
assistive devices, and wheelchairs are evaluated
to ensure that they fit properly.
Radiographs should be taken on the first orthopedic visit to establish a baseline; this is particularly true of the hips and pelvis. The severity of
involvement and amount of deformity present
dictate the frequency and need for sequential imaging studies on follow-up visits. Other advanced
imaging techniques may be required prior to planning of reconstructive procedures.
A comprehensive gait analysis can be performed to obtain an objective assessment of the
gait pattern that could be measured and quantified. This information can be further assessed to
help in preoperative planning. It also provides a
permanent record to compare the outcome of
surgery. Studies have shown that gait analysis
may aid and improve in surgical decision making
in children with cerebral palsy.
HIPS
The hips in children with cerebral palsy are normal
at birth. The deformity occurs from loss of selective motor control and abnormalities in muscle
tone and balance. Such deformities include
coxa valga, femoral anteversion, and acetabular
dysplasia. The muscular imbalance is typically
due to strong hip flexors and adductors overpowering the hip extensors and abductors. The rate of
hip subluxation in cerebral palsy has been reported as high as 75%.615 It is related to a childs
level of function. Lonstein and Beck7 found the
rate of hip subluxation 11% in ambulators and
57% in nonambulators. Root8 reported the incidence of dislocation to be 8% and subluxation
38%. Soo and colleagues16 found no displacement in children who were GMFCS 1% and
found 90% displacement in children who were
GMFCS V. Increased femoral anteversion and
coxa valga were also noted to be related to childrens GMFCS level; Robin and colleagues17
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Fig. 2. (A) A 7-year-old child with quadriplegic cerebral palsy, exhibiting progressive subluxation of both hips
with significant coxa valga and acetabular dysplasia; a decision was made to undergo surgical intervention. (B)
Patient underwent correction of both hips. Soft tissue releases were done followed by bilateral varus derotation
osteotomies and bilateral pelvic osteotomies to correct the acetabular component of the deformity. Postoperative radiographs taken show excellent correction of deformity.
Fig. 3. (A, B) Photographs of a resected hip from an 15-year-old child with quadriplegic cerebral palsy. He had
presented with significant hip pain and a windswept deformity and a decision was made to perform a resection
of the hip. Resected specimen shows denudation of the superolateral portion of the hip with complete loss of
cartilage.
improvement in pain in 10 of 11 cases. Proximal femoral resection and interpositional arthroplasty were initially introduced by Castle and
Schneider.35 McCarthy and colleagues36 revised
the technique to perform the resection at the level
of the ischial tuberosity; in their series, all but 1
patient achieved improvement in seating. The concerns related to proximal femoral resection are
heterotopic ossification, increased time to pain relief, prolonged hospital stay, and migration of the
proximal femur. To address the issue of heterotopic ossification, Egermann and colleagues37
used femoral head to cap the proximal femur
and showed a decreased rate of heterotopic ossification. Another option in lieu of proximal femoral
resection is a valgus osteotomy (McHale procedure). The goal of this procedure is to aim the
femoral head away from the acetabulum while
allowing for an indirect transfer of load. Several
studies have shown an improvement in seating
and pain relief.38,39 Leet and colleagues40 compared the results of proximal femoral resection
with a valgus osteotomy with a mean follow-up
of 3.4 years. Those treated with the McHale procedure had a shorter hospital stay and decreased
proximal femoral migration. Both groups achieved
improved seating and caretaker satisfaction.
KNEES
Anterior knee pain may present as a significant
issue in children with cerebral palsy. It is often
related to the patellofemoral joint. The common
causes of anterior knee pain include patella alta,
patellar subluxation or dislocation, quadriceps
weakness, angular deformity, and rotational malalignment. Senaran and colleagues41 looked at
patients with anterior knee pain secondary to
patellafemoral symptoms; in their study, the patients were classified based on patella alta, fracture of the inferior pole of the patella, and patellar
subluxation or dislocation. The investigators advocated aggressive treatment to prevent future
deterioration.
Knee flexion contractures frequently occur in
children with cerebral palsy. It is more severe in
nonambulatory children. In younger children (age
<12 years) with less severe deformity (contractures <15 ), serial casting has shown effective at
achieving and maintain correction for at least
1 year.42 In more severe cases, hamstring release
may be required to achieve adequate range of
motion. In younger children with severe knee
flexion deformity, guided growth in the form of
an anterior hemiepiphysiodesis of the distal femur
may be attempted.43 In ambulatory children with
knee flexion contractures, treatment should be
aimed at correction of the contracture and
improvement in the gait pattern. Typically, these
patients have a crouch gait pattern. Children
with crouch gait ambulate with the hip, knee,
and ankle in flexion. This gait pattern has been
described as increased knee flexion in stance
phase in spastic diplegics.4446 The development
of crouch gait has often been attributed to the natural progression of gait in ambulatory children
with cerebral palsy.47 Muscle weakness has also
been implicated in the development of progressive crouch gait after lengthening of the triceps
surae.4850 The cause of crouch is probably multifactorial and cannot be totally attributed to any
single factor. In children with crouch gait, there
is a failure of the plantar flexionknee extension
couple; hence, the knee maintains a flexed position with the ground reaction force falling behind
the knee.
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FEET
The feet are an essential part of gait providing a
stable base of support to allow for standing and
ambulation. The foot consists of 2 columnsthe
lateral and the medial columns, and 3 partsforefoot, midfoot, and the hindfoot. It is essential that
all these segments be assessed to correct the
underlying deformity. The underlying muscular
imbalance in cerebral palsy plays a large role in
the development and progression of deformity.
The necessity of correcting the foot in ambulatory
children relies on restoring the lever arm to allow
for appropriate push off, especially in children
with a crouch gait pattern. In nonambulatory children, correction of deformity is centered on
achieving a corrected foot that allows for shoe
wear, bracing, standing, and pain relief. If the
deformity is flexible, it may be amenable to bracing
or soft tissue procedures. Radiographs may be
used to document malalignment in various foot
segments. Gait analysis with an electromyogram
may be performed to assess and evaluate dynamic muscle imbalance. Dynamic pedobarographs may be used to assess plantar pressure
distribution.
Planovalgus and equinovarus are the common
deformities seen in cerebral palsy. In achieving
appropriate correction of the foot, the role of the
gastrocnemius cannot be stressed enough. The
tightness of the Achilles tendon exacerbates the
deformity, preventing the calcaneus from coming
down to achieve an appropriate correction; most
of this contracture tends to occur in the gastrocnemius and not the soleus. Lengthening of the
The equinovarus foot is more common in hemiplegic children. The treatment relies on balancing
or removing the deforming force and addressing
the bony deformities. In mild cases, soft tissue
correction may suffice; this may take the form of
split tendon transfers.70,71 Complete tendon transfers are not advocated. In the more severe cases,
soft tissue procedures in combination with bony
procedures, such as a sliding calcaneal osteotomy
or a closing wedge osteotomy for residual varus is
required. In the most severe cases, an arthrodesis
may be required.72 Posterior tibialis tendon surgery prior to age 8 years should be avoided
because of a high rate of overcorrection. In hemiplegia and children over age 8 years, the tibialis
posterior is the most common overactive tendon
and a split transfer to the peroneus brevis is a reliable procedure. When the varus is accentuated
during the swing phase of gait or if varus seems
mainly from the forefoot, then a split transfer of
the tibialis anterior is a good option. Dynamic electromyogram is helpful to sort out these 2 options.
Almost all varus deformities are associated with
equinus and need to have a lengthening of the
gastrocnemius or tendo-Achilles depending on
the source of contracture. An open Z-lengthening
of the tendo-Achilles is safer then blind percutaneous approaches.
Forefoot deformities also occur often in combination with other deformities. Hallux valgus can
be managed with toe straps in the brace. Surgical
correction is reserved for recalcitrant deformities.
Dorsal bunions may be secondary to an underlying
muscle imbalance or from an iatrogenic injury.
Surgery is reserved for recalcitrant cases that
interfere with shoe wear or bracing.
SPINE
The development of spinal deformity has been well
described in children with cerebral palsy. Spine
involvement, as with the development of any other
deformity in cerebral palsy, is related to the functional level of the child. Scoliosis is the most common form of spinal deformity in cerebral palsy; its
incidence has been reported as high as 77% in
some studies.7376 A majority of these studies
agree that the incidence of scoliosis increases
with the severity of involvement of a child.
Koop73 found a higher incidence of scoliosis in patients with quadriplegic involvement. Their study
showed that 30% of quadriplegic children
developed scoliosis of greater than 40 at skeletal
maturity compared with only 2% in children with
hemiplegia. In a more recent study, PerssonBunke and colleagues77 analyzed the relationship
of the development of scoliosis with childrens
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Fig. 4. (A) Initial radiographs taken of a 15-year-old boy with quadriplegic cerebral palsy. He was noted to have
an XX degree curve; continued observation was advised. (B, C) On follow-up PA and lateral radiographs taken XX
months later, he was noted to have progression of his curvature to XX degrees. (D) Traction films taken show
excellent correction of his curvature with improvement of pelvic obliquity. (E, F) Postoperative films after a
posterior spinal fusion was performed, which shows excellent correction of the curvature and pelvic obliquity.
The child is sitting comfortably with a well-balanced spine.
often required to control and correct pelvic obliquity.85 In most cases, the goals of surgery can be
achieved through a posterior only approach,
although occasionally, for a large, rigid curve, an
anterior and posterior approach may be required.
The necessity to go anterior can be determined
by both clinical methods and radiographic
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SUMMARY
The orthopedic manifestations of children with cerebral palsy are wide and varied, with the clinical
presentation directly correlating with the severity
of a childs involvement. Although the underlying
injury is static, its effects on a childs musculoskeletal system are progressive throughout the
growing years. Close clinical surveillance and
observation are needed to address these deformities as they develop and progress. The goal of
treatment should not focus on the specific deformity but on the child as a whole, in an attempt to
improve overall function.
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