Cerebral Palsy

Increased lordosis in the lumbar spine is almost never a
primary deformity in cerebral palsy. It is usually secondary to
hip-flexion contractures, and it responds to appropriate correction of
those contractures by such means as stretching exercises or surgical
lengthening of the psoas tendons. Hyperlordosis may also be a
compensatory deformity below a rigid thoracic hyperkyphosis, and it
usually responds to correction of the primary problem. When surgical
spinal fusion is necessary to correct severe scoliosis, it is essential
to consider the sagittal plane spinal balance and to preserve adequate
lumbar lordosis by avoiding excessive distraction across the lumbar
spine.

Hyperkyphosis is most commonly seen in the young child
with cerebral palsy who has weak spinal extensor musculature and a
resultant long, C-shaped kyphotic posturing of the entire spine. This
is almost always flexible, correcting fully on prone lying. It is best
controlled by proper seating adaptation such as restraint straps on the
wheelchair, slight reclining of the back, or, less often, by a
thoracolumbosacral orthosis to provide sitting support. There is debate
regarding whether increasing the sitting support inhibits the
functioning of the spinal extensor muscles and weakens them further,
and whether physical therapy or muscle stimulation is helpful in
maintaining or enhancing spinal extensor muscle strength.

In rare cases, kyphosis may occur in the lumbar spine
because of overly tight hamstring muscles that cause sagittal rotation
of the pelvis. This kyphosis may disappear with proximal lengthening of
the hamstrings, but we have very little experience with this.

Children with cerebral palsy are not immune to the
spinal deformities that afflict other children, so thoracic
hyperkyphosis resulting from the Scheuermann condition

or
postural juvenile kyphosis may also occur. Indications for orthotic
treatment in these kyphotic conditions are similar to those in other
children, but spinal orthotics are not likely to be as well tolerated
in the child with cerebral palsy. If the child does not have good
voluntary trunk control then he/she will rarely tolerate an effective
orthosis.

Scoliosis is more prevalent in all types of cerebral
palsy compared with the general population, and it varies in direct
proportion to the severity of motor involvement. In patients with mild
hemiplegia, scoliosis occurs in fewer than 5%; in patients with severe
spastic quadriplegia, its occurrence is much greater, in the range of
approximately 50% to 75%; in all patients with cerebral palsy taken
together, it is approximately 25%. Specific increased risk factors for
curve progression are quadriplegia, younger age, poor sitting balance,
pelvic obliquity, and the presence of multiple curves (35,36,37).

Scoliosis in spastic quadriplegic cerebral palsy is
different from idiopathic scoliosis. It develops earlier; is more
likely to be progressive; progresses even after skeletal maturity,
especially when the curve exceeds 40 degrees; is almost always
unresponsive to orthotic control; and is more likely to require
surgical treatment.

As with idiopathic or other types of scoliosis, there
are only three appropriate options for management: observation with
documentation, orthotic treatment, or surgical stabilization.
Observation alone is indicated if the curve is of insufficient
magnitude to require treatment (<25–30 degrees) or if the patient’s
best interests may not be served by active surgical intervention. The
latter category is difficult to define. It would include the most
severely involved individuals who are unable to perceive or interact
with their environment in any meaningful fashion, because of severe and
global compromise of their cognitive and sensory perceptual abilities.
Only careful study by members of the cerebral palsy team and the
patient’s family can lead to this conclusion. In such cases, the
overall management goals are the patient’s safety and comfort.

The orthotic treatment of scoliosis in quadriplegic
cerebral palsy was based mostly on hope and empiricism until studies by
two institutions showed that it rarely succeeds in controlling a curve (38,39).
In most of the patients with quadriplegia from these centers, no
meaningful curve control was achieved by orthotic treatment. In some
cases, however, an orthosis may slow the progression of the curve,
particularly in curves of 30 to 60 degrees, allowing beneficial growth
in an immature spine before definitive surgical stabilization. At best,
no more than 15% of brace-treated curves stop progressing, and this may
simply reflect the natural history of some cases of scoliosis in
cerebral palsy (37). Ambulatory patients with
spastic diplegia may develop idiopathic-type scoliotic curves. In these
milder cases of cerebral palsy, brace control may be successful.
Orthoses and other types of external devices for trunk support may be
of value in improving sitting balance, particularly for those patients
in whom surgery is not indicated. If the patient can tolerate a
total-contact, low-profile orthosis, this is the most effective and
economical means of providing improved trunk support, even if it is a
relatively soft orthosis (40). This is often
not the case, however, and a custom-molded trunk or
total-body-supporting wheelchair insert is required. These devices are
difficult to fit properly, are often quickly outgrown, and are
expensive. They must provide adequate pelvic alignment, trunk control,
and head support. Nevertheless, the ability to sit as erectly and
comfortably as possible is essential for a totally involved patient who
can interact with the environment. Good sitting improves the patient’s
mental outlook, ability to communicate, respiratory function, ease of
feeding, gastrointestinal function, hand usage, and mobility in the
environment (41,42,43).

Surgical stabilization of progressing scoliosis is the only way to stop such a curve in most cases (44,45). The benefit of a procedure of this magnitude and expense has been questioned by some (46),
but most orthopaedic surgeons strongly believe that the surgery is
worthwhile, particularly in preventing discomfort and loss of the
ability to sit (Fig. 15.3). Postoperative
patients with reasonably balanced nonprogressive scoliosis have much
better endurance for sitting. It greatly improves their quality of life
when they can sit up comfortably for several hours, or even all day,
and not have frequent substantial back pain that requires recumbence
during most of their waking hours. According to their caregivers, they
are also much easier to feed, dress, and transport than those with
severe untreated scoliosis. It is most likely that after fusion they
will have less back discomfort, better pulmonary function, and less
decubitus skin ulceration than similarly involved patients with severe
untreated scoliosis. However, this has not been proven (47).
Subjectively, parents report significant improvements postoperatively:
one year after surgery, their children experience less pain, are
generally happier, and do not feel ill or tired as frequently as
before. However, physical functioning and comorbidities do not improve (48,49).
It is noteworthy that patients with lower extremity spasticity often
experience an increase in the magnitude of their spasticity for several
months after corrective scoliosis surgery. The increased tone gradually
resolves and may be somewhat relieved by benzodiazepines. Its cause is
not known, but is possibly related to some stretching of neurologic
structures or obligatory operative edema.

Once a curve exceeds 40 to 45 degrees it is likely to
continue progressing and surgery is usually indicated. Posterior
internal fixation with a segmental system, such as double rods with
cross-links connected to the spine with multiple hooks; sublaminar
wires; interspinous wires; pedicle screws; or combinations of these
techniques and an adequate posterior fusion mass is usually employed.
This is needed in order to achieve a balanced spine over a reasonably
level pelvis, which is the objective of such

surgery (44,50).
Whenever possible, a larger and more rigid rod is preferable so as to
provide better correction, resist deforming forces, and promote a solid
arthrodesis. It is essential to achieve spinal balance in both the
coronal and sagittal planes in order to maximize sitting balance. An
abundance of allograft bone or an effective bone graft substitute
should be available to generate the strong fusion mass (51,52).

Fusion limits are usually from the upper thoracic region
(T1–T3) to the pelvis. When the fusion does not extend to the upper
thoracic region, there is an increased risk of developing a substantial
junctional kyphosis cephalad to it. This may interfere with the ability
of the patient to see at or above the horizontal, or may require
constant and eventually painful neck hyperextension to do so. No matter
how cephalad the upper fusion level is, or whatever the type of
fixation, some patients still develop a junctional kyphosis. Applying a
bilateral, two-level, clawed-hook configuration at the cephalad end of
the rods (Fig. 15.4) with preservation of the
uppermost posterior ligaments may help prevent a junctional kyphosis.
However, this has not been consistently successful, and many surgeons
have abandoned this technique. Although the cephalad-most pair of wires
may occasionally break eventually, this will almost never require
revision.

Fusions should include the pelvis if pelvic obliquity
exceeds 10 degrees from the intercrestal iliac line to the top of L5 or
L4 as measured on an anteroposterior radiographic view, with the
patient seated. Otherwise, pelvic obliquity may continue to progress
and make sitting more difficult. Regardless of the condition of the
hips in patients with pelvic tilt, the spinopelvic obliquity can be
corrected only by spine surgery and not by hip or pelvic surgery (53).
Various techniques are available for pelvic fixation, including hooks,
rods, and screws anchored to the iliac bones or sacrum, but none has
withstood the test of time better than the Galveston technique (54).

Anterior spinal surgery is not routinely necessary in
cerebral palsy. The most common indication for anterior spinal surgery
in this condition is to improve curve correctability. Increased
correction may be needed in order to

In such cases a release of the deforming structures is
accomplished by dividing the anterior longitudinal ligament, excising
the annulus fibrosis, removing all of the disc material and endplate
cartilage back to the posterior annulus and posterior longitudinal
ligament, and packing bone graft into the disc spaces. This may be done
either through an open approach or by an endoscopic technique. Anterior
internal fixation is rarely necessary when strong, rigid posterior
fixation is performed. The anterior and the posterior operations are
accomplished at the same surgical

setting rather than being staged over several days or weeks. This decreases the hospital stay and complications (55,56,57).

There are several other important considerations when
managing a spinal deformity by surgical means in a patient with
cerebral palsy. As discussed earlier, one third of the patients are
malnourished (58,59)
and may have gastroesophageal reflux. Detecting and correcting these
conditions preoperatively helps improve the patient’s nutritional
status and thereby prevent postoperative wound infection and healing
problems. Determination of the serum transferrin level, albumin level,
and total lymphocyte count are commonly used for assessing nutritional
status. An index has been developed for identifying malnourished
patients on the basis of transferrin and albumin levels (60).

Intraoperative blood loss should be calculated
carefully, expressed in terms of percentage of blood volume, and
appropriately replaced in order to avoid hypovolemia or dangerous
coagulopathies, especially when blood loss nears 50% of the blood
volume. In this method, preoperative blood volume should be accurately
estimated, and the loss detected by suctioned blood plus blood in
weighed sponges should be carefully measured. Another means of
monitoring blood loss and replacement requirements is by calculating
erythrocyte mass and considering hematocrit measurements in the lost
blood as well as in the replacement source. Many surgeons monitor the
hematocrit value, which is obtained through periodic blood gas
assessments throughout the procedure. When blood replacement is
indicated, whole blood or packed RBCs may be used. Blood loss may be
minimized by techniques such as hypotensive anesthesia (usually to a
mean arterial pressure of approximately 60 mm Hg), preoperative
hemodilution, the administration of aprotinin, and the use of a
cell-saver.

Postoperative pulmonary problems, such as
hypoventilation, atelectasis, aspiration pneumonia, and the adult
respiratory distress syndrome may occur, and every preventive effort,
including rapid mobilization of the patient, must be enlisted.

Although spondylolysis and spondylolisthesis do not
occur in nonambulatory patients, they have been reported in ambulatory
patients with cerebral palsy with an incidence similar to that in the
general population. No increased severity of symptoms or relation to
hip-flexion contractures has been noted (61). The treatment is similar to that recommended for children who do not have cerebral palsy.

The hip at risk has increased valgus and ante-version
along with a shallow acetabulum, but no subluxation. Tightness and
contractures are usually present in the adductor and flexor muscles.
Without treatment, hips at risk often progress to subluxation or
dislocation, particularly if there is less than 30 degrees of abduction
in flexion or extension, and/or if there are hip flexion contractures
of greater than 20 degrees. Such progression may be very slow (months
to years) or occur much more rapidly. Because the literature lacks
valid data regarding the likelihood of such hips worsening, it is not
possible to predict the natural history of every hip at risk in
cerebral palsy. That leaves the surgeon with the options of intervening
or closely following hips at risk. Unless the patient is so physically
compromised as to preclude surgical treatment, most surgeons will
intervene, realizing that at least in some cases the hip pathology
would not have been progressive. The use of stretching exercises alone
as treatment for hips at risk is rarely, if ever, successful. In very
young children with hips at risk and mild or absent muscle tightness,
night splinting, in an attempt to improve acetabular depth, is an
option that some surgeons might choose. However, it has not been
confirmed by any well-controlled study that night

splinting can reliably increase acetabular depth, and we do not use the method.

Surgical treatment of the hip at risk consists of lengthening and weakening the tight adductors and flexors (68,69,70).
The adductor longus may be all that needs releasing, especially in
those who walk, but the gracilis and, occasionally, part of the
adductor brevis muscle also may require release in order to gain
abduction to at least 45 degrees for each extended hip and 60 degrees
for each hip in flexion. The issue of whether to release or transfer
the adductors is discussed in the section on spastic diplegia. Tenotomy
or elongation of the psoas tendon, sparing the iliacus fibers, should
be performed. Psoas tenotomy in a nonambulatory patient with spastic
quadriplegia may be performed either at the pelvic brim or at the
lesser trochanter. Tenotomy at the more distal site may produce more
hip flexor weakness, a situation of no consequence to a nonwalker but
one that may be detrimental to an ambulatory patient who needs adequate
hip flexor power to lift the limb for step climbing (2). Psoas tenotomy at the pelvic brim is performed in the manner described by Sutherland et al. (71). On rare occasions the psoas may not be tendinous at that level, and the tenotomy must be done more distally.

Postoperatively, some surgeons use abduction splinting
for several months. This is applied at night and always within the
child’s range of comfortable abduction. The value of such splinting is
questionable. Postoperatively, physical therapy is begun as early as
the second postoperative day (72). In the past,
anterior branch or even complete obturator neurectomy used to be
performed to denervate the adductor muscles in addition to lengthening
them. Now this is rarely done because most surgeons believe this
procedure is unnecessary when an adequate adductor release has been
performed. If the child has an athetoid component in addition to
spasticity, obturator neurectomy should never be done. It can result in
a severe, disabling abduction contracture.

Hip subluxation is defined as uncovering of more than
one third of the femoral head and a break in the Shenton line, but with
the femoral head maintaining at least some contact with the acetabulum.
Surgical treatment of a subluxation can prevent subsequent dislocation (73,74,75).

Soft tissue releases and prolonged splinting are rarely
successful, even in very young children. When only soft tissue surgery
is deemed necessary to treat very mild unilateral subluxation and the
patient is younger than 9 years, bilateral releases may be best because
the contralateral hip is usually somewhat abnormal or likely to become
so if only unilateral surgery is done (76).
Soft tissue surgery alone (adductor and psoas releases) does not arrest
subluxation in most children regardless of their age. In a recent
study, subluxation or dislocation persisted in 58% of the children
after such surgery (77).

The subluxated hip often has increased femoral valgus,
anteversion, or both, and requires corrective proximal femoral
osteotomy (78,79,80).
To stabilize the subluxated hip, the neck-shaft angle of the hip is
usually surgically reduced to approximately 115 degrees in an
ambulatory child, or even less in a nonambulator. In addition to
appropriate tendon releases, derotation of the excessive femoral
anteversion to approximately 10 to 20 degrees of anteversion (or 30 to
45 degrees of passive internal hip rotation) is performed to prevent
posterior subluxation (81,82).
The younger the patient, the more likely are the valgus and anteversion
to recur postoperatively, especially in children younger than 4 years (83).
On the other hand, subluxated hips are more likely to remain located
after surgery in younger children than in older children, particularly
those older than 10 years (84). A recent large
study reported that 84% of patients had a stable pain-free hip at an
average of 5 years after femoral varus osteotomy (85). If the magnitude of the subluxation exceeds 50%, an open reduction and capsulorrhaphy will improve the result (86).

Children who are ambulatory and have proximal femoral
osteotomies require approximately 7 to 10 months to regain their
preoperative function, but longer times—up to 30 months—are possible (87).
If bony surgery (a varus rotational osteotomy with or without a pelvic
osteotomy) is required, prophylactic surgery on a well-covered
contralateral hip with adequate abduction is not necessary regardless
of the age or ambulatory status of the patient (88).

The most common complications after proximal femoral
osteotomy surgery are femoral fractures and decubitus ulcers. Children
with tracheostomies and gastrostomies are at the greatest risk (89).

If acetabular dysplasia is present (acetabular angle >25 degrees) (90), it should almost always be corrected surgically (Fig. 15.5).
An exception might be a child younger than 4 or 5 years with very mild
dysplasia and recently fully corrected abnormal valgus and anteversion,
in whom acetabular remodeling may occur from the stimulation of
redirecting the force vector across the hip joint from the lateral
acetabular rim to the center of the acetabulum. We do not favor this
and recommend correction of any acetabular dysplasia. Older children
have much less potential for biologic remodeling to normalize the
acetabulum after femoral varization and rotation osteotomies.

The dysplastic acetabulum in cerebral palsy is shallow.
In nonambulators the acetabular deficit is superior and posterior to,
and usually more severe than, the deficit in ambulatory patients. It is
almost always associated with an increase in the femoral neck-shaft
angle and femoral anteversion. The femoral anteversion is greater in
ambulators (63).

Acetabular dysplasia is corrected surgically by choosing
the appropriate pelvic osteotomy and being certain that its
prerequisites are met. Anteroposterior and 30 degree false profile
radiographs are helpful in assessing the

pathoanatomy
of the acetabulum. Although not usually necessary, arthrographic
evaluation or three-dimensional reformatted CT scanning images can be
helpful in the decision-making process to define the pathoanatomy (90). Clinical assessment of the acetabulum when it is exposed at surgery confirms the choice of procedure.

The Salter, Steel, Sutherland, Pemberton, pericapsular,
Albee shelf, Dega, and Chiari osteotomies, and shelf augmentation of
the acetabular rim, have all been successful in patients with cerebral
palsy when appropriate indications are met (91,92,93,94,95,96,97,98,99).
If the acetabulum is found to be deficient superiorly, or superiorly
and anteriorly, an anterolateral rotational osteotomy, such as a
Salter, Steel, or Sutherland procedure, or a Pemberton osteotomy, is
appropriate. Often there is superior and posterior deficiency, in which
case restoration of lateral and posterior coverage by a
shelf-augmentation procedure, an Albee shelf, a Dega procedure, or a
pericapsular osteotomy (100) may be more
appropriate. The Chiari osteotomy can be performed if the superior
acetabular rim has not been so proximally eroded that the cut will
enter the sacroiliac joint. Long-term follow-up of pelvic osteotomies
in cerebral palsy indicate that gradual deterioration from early
postoperative results often occurs, and consideration of wider release
of soft tissues, more radical varus derotation, and greater shortening
of the femur may be beneficial (101).

Hip dislocation may be addressed by relocation procedures (102) (Fig. 15.6), by accepting the dislocation, by proximal femoral resection (103) with or without prosthetic capping, or, less commonly, by hip arthrodesis or total hip replacement arthroplasty (104,105).
If the dislocation occurred within a year of presentation, and/or if
the anatomy does not appear excessively distorted, most surgeons
customarily elect to perform anterior open reduction and
capsulorrhaphy, combined with appropriate soft tissue releases (usually
adductors and psoas tendon) and a proximal femoral shortening,
varization, and rotation osteotomy. Often, a degree of acetabular
dysplasia is

associated
with the dislocation, and it is wise to correct this at the same time.
The pelvic procedure should be tailored to the situation, as described
in the previous discussion of the subluxated hip. There is a lack of
convincing outcome studies of the benefit of such late reductions, and
this treatment is based only on positive anecdotal experience.

If the hip has been dislocated for longer than 1 year,
achieving a painless, mobile, stable hip from open reduction and other
surgery is less likely because of joint incongruity and eroded
articular cartilage on the femoral head. When such a hip is painless,
no treatment is required. When the hip is painful, proximal femoral
resection with muscle interposition, as originally described by Castle
and Schneider, has a high success rate (106,107).
This resection is performed at the subtrochanteric level; the thickened
capsule and gluteus medius muscle are sewn over the acetabular inlet,
and a muscle cuff of vastus lateralis is sewn over the beveled femoral
stump (Fig. 15.7). It is critical to carefully
save as much vastus lateralis as possible during the initial
dissection, and to provide a good, thick muscle covering over the
femoral stump. Postoperative management should consist of a bilateral
pantaloon or a one-and-a-half-hip spica cast for approximately 3 to 4
weeks, then comfortable but effective exercises to assure maintenance
of the desired range of hip motion. This range is a minimal flexion
contracture, at least 100 degrees of flexion, neutral rotation, and
abduction to at least 20 degrees. Postoperative traction or external
fixators are rarely necessary because the casting appears to be more
comfortable for the patient and easier to manage. The result is usually
good motion and good pain relief, but definite thigh shortening, which
must be accommodated in the seat of the wheelchair. Following proximal
femoral resection it is very common to have spasm and discomfort for
several days, weeks, or even months (107). This
usually will eventually resolve, but analgesics and antispasmodic
medications may be necessary for a prolonged time. A successful trial
of an intrathecal baclofen injection may indicate the efficacy of using
a baclofen pump during this period. Recently the addition of prosthetic
interposition to proximal femoral resection, often using a noncemented
proximal humeral prosthesis in the small femoral canal, has been
reported to give good pain relief for this problem in the short term (108).
Heterotopic ossification about the hip is extremely common after
proximal femoral resection, and preoperative or immediate postoperative
single-dose radiation therapy should be considered in order to minimize
its magnitude.

Femoral head resection, when combined with
subtrochanteric valgus pelvic support osteotomy, has been successful in
relieving the pain of chronic hip dislocation (109,110).
Specific indications and advantages of this treatment versus proximal
femoral resection are not available in the literature, and the
selection of one or the other is therefore according to the surgeon’s
preference. Our preference is for resection. The Girdlestone
intertrochanteric resection has been tried for the painful dislocated
hip. The likelihood of continuing postoperative pain from femoral-iliac
impingement has caused most surgeons to abandon this procedure.

Arthrodesis is another option, but it may be difficult
to accomplish in nonambulatory patients with severe spasticity. When
enough flexion is provided to make sitting comfortable, it may limit
the ability to comfortably lie

supine.
It may also preclude appropriate positioning of the patient undergoing
spine surgery. Arthrodesis, therefore, is not often performed in
nonambulatory patients. In ambulatory patients with unilateral hip
disease, arthrodesis is a reasonable option with high success in bone
union, pain relief, hip stability, and postural improvement (111,112,113).

The reported experience is small with respect to replacement arthroplasty for painful dislocated hips (112).
This procedure is indicated most often for the ambulatory adult with
mild to moderate spasticity and severe degenerative arthritis of the
hip. Nonwalking adults with painful untreated hip dislocations respond
better to proximal femoral resection (111).

The located osteoarthritic hip in cerebral palsy need
not be treated surgically unless it is painful, and nonsurgical methods
have been tried without success. In such patients, both arthrodesis and
replacement arthroplasty have been successful, the latter being
preferred because of less postoperative morbidity and easier management
(112). Arthrodesis is usually performed in a
position appropriate for walking, sitting, and lying: 30 degrees of
flexion, 5 to 10 degrees abduction, and neutral rotation for an
ambulatory patient, and 45 degrees of flexion for a nonambulator.
Leg-length equalization should be considered in walking patients
because it may defer the development of back pain (112).

Extension contracture of the hip occasionally occurs in
the severely spastic child with quadriplegia. The probable causes are
contracted hip extensors and tightly contracted hamstrings, which are
hip extensors as well as knee flexors. This condition is often
accompanied by extensor thrust, a rigid and sustained hip extension
that can seriously interfere with comfortable sitting because the
extension contracture does not allow adequate hip flexion. When severe,
it can literally push and slide the child out of the wheelchair. The
treatment options for mild extension contracture are proximal
lengthening of the hamstrings or injecting neuromotor blocking agents
such as botulinum-A toxin or alcohol into the hamstrings. If the
extension contractures are more than just mild, lengthening of the
proximal hamstrings is indicated (114). If this
is not adequate, posterior capsulotomy of the hip and release of the
hip extensors and external rotator muscles may also be necessary.
Unfortunately, recurrence of extension contractures is common and often
rapid, following proximal hamstring lengthening.

Extension plus abduction contracture of the hip is not
common. It most often occurs after injudicious release of the flexors
and adductors in patients who have athetosis, and it is also seen after
aggressive flexor and adductor releases in patients who have severe
spasticity or rigidity and previously undetected cospasticity in the
hip extensor and abductor muscles. Cospasticity can be difficult to
detect even by careful physical examination, but both flexion and
extension of the joint are substantially limited, as are abduction and
adduction. Gait analysis may detect cospasticity in ambulatory patients.

Because of their writhing movements, patients who have
athetosis rarely develop contractures that require surgical releasing.
A problem exists with the mixed spastic-athetoid patient with
tightness, in whom the surgeon cannot determine precisely just how much
to weaken the spastic muscle. It is definitely better to err on the
side of underrelease.

The treatment of mild cases of extension plus abduction
contracture of the hip consists of stretching exercises and proper
seating in the wheelchair. Data regarding the use of tone-reducing
measures in this situation are currently lacking, but a trial of
intrathecal baclofen may be useful. In patients with more severe
involvement, surgical treatment is necessary. This involves release of
the proximal hamstrings, the femoral and iliotibial band insertions of
the gluteus maximus, the external rotator muscles, and even posterior
capsulotomy of the hip joint, if necessary. In long-standing severe
contractures, femoral shortening may be necessary to prevent
overstretching the sciatic nerve (115).

Ideally, an orally administered medication would
appropriately temper spasticity and allow improved voluntary muscle
control to occur over the long term. Unfortunately, such an agent does
not exist. Many types of muscle relaxants, antispasmodics, and
neuroinhibitory medications have been tried to little or no avail,
mostly because therapeutic doses are so large that the substantial
accompanying drowsiness is unacceptable (125,126).
Other undesirable side effects include drooling, ataxia, and behavioral
problems. Oral pharmacotherapies that are generally accepted as
effective for problems in cerebral palsy have been limited to
anticonvulsants for the treatment of seizures and benzodiazepines (such
as diazepam) for superimposed postoperative myospasms. The latter drug
increases presynaptic and postsynaptic inhibition, and perhaps has a
tranquilizing effect that mutes the perception of pain. Children with
dystonia may benefit from trihexyphenidyl (Artane), which may improve
hand function and speech to some extent. This drug may cause dry mouth,
constipation, and urinary retention.

Baclofen (Lioresal) is an agonist of the neuroinhibitor
γ-aminobutyric acid (GABA) that inhibits reflex activity. Its poor
blood-brain barrier permeability, short half-life, and slow onset of
action require that frequent and high doses be given; it produces
somnolence plus other side effects that limit its usefulness as an oral
medication. Rapid withdrawal of the drug may increase seizure activity.

Dantrolene sodium acts on muscle calcium ion release and
not on the nervous system. Because it produces weakness with little
effect on spasticity and has numerous potential side effects, it is
rarely useful in cerebral palsy (127).

The purposes of injecting various substances into muscles or around nerves are

The most common lower-extremity muscle that is injected
is the gastrocnemius; this is done in order to reduce equinus
deformity. Other common sites are the hamstrings and hip adductors.
Repeated injections may be necessary to achieve the desired effect, and
injecting some substances can be painful unless general anesthesia is
used.

The shortest-acting drug is a local anesthetic agent,
injected in the immediate vicinity of a specific nerve to block its
fibers and allow the physician to observe the effect. If the effect is
beneficial, then repeating the injection with another longer-acting
agent should reproduce the effect. If the goal is to make the effect
even more long lasting or permanent, phenol is used for destroying the
nerve fibers.

Another means of weakening the muscle is to inject 45%
alcohol into its fibers in a more regional distribution in order to
inhibit nerve transmission and, thereby, muscle contraction. Alcohol
nonselectively denatures proteins, and thereby affects axons, myoneural
junctions, and muscle fibers. Its use is painful and requires general
anesthesia. When successful, the effect of alcohol injection lasts for
a variable period, but usually approximately 6 weeks (129,130).

A new agent for intramuscular injection is botulinum-A
toxin (BTX, Botox), a neurotoxin produced by clostridia bacteria. When
delivered into or near sites of nerve arborization, it diffuses 2 to 3
cm from the delivery point, and blocks the release of acetylcholine
from presynaptic vesicles at the myoneural junction. Recovery of tone
results from the sprouting of new nerve terminals, and this process
peaks at approximately 60 days (131,132).
The agent is injected using a 23- or 25-gauge needle, usually without
local or general anesthesia. Topical anesthesia does not prevent the
muscle discomfort, but this discomfort rarely lasts more than 5
minutes. In general, when more than one muscle group is to be addressed
in a young child, this is done under conscious sedation or general
anesthesia. The muscles are located by palpation. To reach muscles that
are deep or difficult to localize, ultrasonography can guide placement
of a needle that is used for both stimulation of the target muscle and
injection of the botulinum-A toxin (133,134).

BTX is expensive, and optimal dosage regimens have not
been agreed upon. It has been shown to be a safe technique, whose
effects on muscles begin to be seen in 12 to 72 hours, and usually last
for 3 to 6 months or sometimes longer (135,136,137).
Injections may be repeated after 2 or more weeks, and up to six
injections may be given at a site, until the desired response in muscle
tone reduction has been achieved.

Frequent injections increase the antigen load, and the
resultant increase in antibodies may limit future clinical response.
This treatment is contraindicated in the presence of fixed-joint
contractures. It is most useful in very young patients (usually younger
than 6 years) when a limited number of muscles are involved (137),
and the surgeon wishes to defer surgical intervention until the child
is older, has a stabilized gait pattern, or other components of the
child’s problem can be more precisely identified and addressed.

A double-blind trial in 12 patients with equinovarus or
equinovalgus foot deformities showed improvement in 5 of 6 treated
patients, and improvement in 2 of 6 placebo-injected patients (138).
A study of 14 children who had BTX treatment for equinus reported that
there was marked improvement (mean 6.7 months) in six of the children,
slight improvement in three, and no improvement in five (138).
Among the larger studies, one with 114 children has shown that, when
treated with BTX, equinus foot positions improved in 50% to 60% of
patients who had bilateral gastrocnemius injections (139),
and a randomized, double-blind, placebo-controlled study of 125
children who were given injections showed an average increase of
knee-extended ankle dorsiflexion of 19 degrees in the children treated
with BTX (140). Other studies have found this
treatment to be long lasting with few side effects, and as effective if
not more effective than serial casting in improving equinus gait (136,137). A combination of the two methods may be beneficial (141,142).

BTX has shown beneficial effects when used in the upper
extremity, particularly in relieving tight elbow flexors and thumb
flexors. This has resulted in improvements in hygiene and cosmesis, but
not in fine motor function (143). Apart from
its effect on motor function, BTX is usually effective in relieving
pain caused by muscle spasms. Unanswered but essential questions are

Baclofen (Lioresal) is an agonist of the neuroinhibitor GABA, which interferes with release of

excitatory transmitters that cause spasticity (144).
When injected intrathecally, it acts on the synaptic reflexes of the
spinal cord to broadly decrease lower-extremity spasticity for
approximately 8 hours (2). In the upper
extremity there is less reduction of tone, about equal to that achieved
by selective posterior rhizotomy, with no change in the range of motion
of joints (144). Baclofen has poor lipid
solubility and does not cross the blood-brain barrier very effectively;
so, if given orally, large doses are required, and excessive lethargy
usually occurs. Dizziness and/or drooling may also result. Beneficial
long-term effects are realized when administered intrathecally with a
refillable, subcutaneously implanted pump. This provides a ten times
greater concentration in the spinal fluid than does comparable oral
dosing. In fact, a mere 1/30th of the oral dose given intrathecally
produces a similar or greater response (145).
Problems and complications from using the drug and pump are decreasing
as experience increases; they include headache, somnolence, seizures,
hypotension, infection, urinary retention, catheter breakage, spinal
fluid fistula, equipment malfunction, and the need for frequent
refilling with the medication. There is another critical risk factor
that one should be aware of. Interruption of the flow, whether because
of pump, dose, or tubing failure, can have serious and even fatal
consequences from severe withdrawal reactions.

Large long-term outcome studies are not yet available.
In a study of 21 children treated for an average of 53 months with
baclofen, the Ashworth scale showed a substantial decrease in
spasticity. No functional change was detected by the GMFM or the
Pediatric Evaluation of Disability Inventory. Caregiver satisfaction
was high. One hundred and fifty-three treatment-associated adverse
events occurred (145). It appears that this
treatment may be most helpful for patients with spasticity or
spasticity plus dystonia who have hygiene problems or difficulty with
sitting (146,147,148),
or those who are ambulatory but have underlying weakness. This second
group cannot tolerate the additional weakness imposed by selective
dorsal rhizotomy. Although there is insufficient data on which to base
a recommendation for its use in specific patients, it appears that
intrathecal baclofen is of definite benefit in about one half of the
patients in studies thus far reported (2). It
may also be of some aid in assessing the potential benefit of selective
posterior rhizotomy. In the doses that are used for treating
spasticity, baclofen has no effect on athetosis (149).

This procedure has been employed for several decades (150), but only in the past 20 to 25 years has it been widely used in North America (150,151,152).
Unlike earlier attempts to beneficially alter the central nervous
system by surgical or electrical means (e.g., cerebellar stimulation) (153),
this procedure has met with growing acceptance. The principle of
selective posterior rhizotomy is to reduce spasticity and balance
muscle tone by altering the control exhibited by the anterior horn
cells in the spinal cord. The normal inhibitory influences on the γ
efferent system, produced by higher centers in the brain and carried to
the anterior horn cell by long intraspinal tracts, are deficient in
cerebral palsy, as is the ability to coordinate movement as mediated
through the extrafusal fibers from the α motor neurons. Stimulatory
inputs from the muscle spindles in the lower limbs arrive through the
afferent fibers in the dorsal roots. Selective posterior rhizotomy
attempts to limit these inputs. This is done by sectioning only those
dorsal rootlets whose impulses exert excessive faciliatory influence on
the anterior horn cell, thereby arriving at a balance.

The operation is performed under general anesthesia
without relaxants. The lumbar laminae and the intervening ligaments are
removed as an en bloc laminoplasty from L1
to S1, preserving the facet joints. Then, individual posterior rootlets
from the L1 to S1 posterior roots are carefully dissected out and
electrically stimulated. The electromyographic and physical responses
are noted, and those rootlets that supply the target muscles are
surgically divided. Rootlets are spared if they show decremental and
squared-type electromyographic responses. They are divided if the
responses are incremental, clonic, multiphasic, sustained, or
contralateral, although this determination is not always easy. In most
cases, up to 70 rootlets per root are stimulated, and 25% to 50% are
sectioned. The laminae and ligament complex are then replaced. The
theoretical advantage of laminoplasty compared to laminectomy at each
level is that it is quicker and may result in less scarring in the
spinal canal. Also, replacement of the intact laminae may prevent the
occurrence of lumbar instability or deformity in some patients.
Laminoplasty will not prevent kyphosis in the thoracic spine.

The patient best served by selective posterior rhizotomy is the young child (age 3 to 8 years) with

Children who were born full-term are more likely to have
rigidity plus spasticity, and therefore may not respond as favorably to
selective posterior rhizotomy (2,155,156).
Availability of and coordination with postoperative physical therapy
are prerequisites for the procedure, because the patient may require
physical therapy three or four times per week for as long as a year to
regain strength. The procedure is not indicated in children with
athetosis, ataxia, rigidity, dystonia, antigravity muscle and truncal
weakness or hypotonia, overlengthened tendons, or severe fixed

contractures. Information regarding any benefits in those with spastic quadriplegia is limited (2). The results of unilateral rhizotomy in spastic hemiplegia are not available, but at least one source does not recommend it (157).

In the early postoperative period, the patient is weaker
than before surgery and requires intensive physical therapy, but once
the acute rehabilitation phase is complete, improvement in the lower
extremities may be dramatic and perhaps lasting. Sometimes improvements
even in upper-extremity function, seizure control, bladder function,
swallowing, speech, and personality are seen after an overall reduction
in muscle tone (156). Results thus far show
lasting reduction in spasticity; increased range of motion of hip,
knee, and ankle; often a plantigrade foot in stance; increased stride
length and walking speed; and no increase in sensory deficits (157,158,159,160,161,162,163,164). Some randomized trials comparing rhizotomy with physical therapy have now been published (165,166,167,168). These indicate that rhizotomy is more successful in reducing muscle tone, and often in improving motor function as well.

Although this procedure reduces spasticity and tone, it
does not affect contractures of joints or the overly contracted
musculotendinous unit. These must be treated by orthopaedic surgical
procedures after rehabilitation following the rhizotomy. It is
estimated that up to 65% of patients require additional procedures,
with those older than 4 years at the time of rhizotomy requiring more
orthopaedic procedures (169,170).

Selective posterior rhizotomy can be beneficial, but it
is not a miracle procedure, nor does it cure cerebral palsy. The
patient will still have poor motor control and balance, muscle
weakness, contractures, sensory involvement, and/or persisting
primitive reflexes if he or she did so preoperatively. A 1-year
follow-up study comparing selective posterior rhizotomy plus intensive
physical therapy with just intensive physical therapy alone reported
that ankle dorsiflexion, foot progression angle, and hip and knee
extension in stance were better in the rhizotomy group, but these
improvements were not associated with significant improvements in
functional gait (163).

As with virtually everything else in medicine, there are
some caveats. An occasional patient develops rapid and severe hip
subluxation or dysplasia after this type of rhizotomy (171).
Heterotopic ossification around the hip has been reported to occur
after varus derotation osteotomy of the proximal femur, in patients
with spastic quadriplegia who have previously had selective posterior
rhizotomy (172). Patients with preexisting
lumbar lordosis of more than 60 degrees on a lateral radiograph taken
in the seated position are at risk for the development of postoperative
lumbar hyperlordosis (173,174). Spondylolysis and spondylolisthesis have also been reported (175).
In a recent study of patients who had wide laminectomies with their
rhizotomies, 36% developed spinal deformities postoperatively with
scoliosis, lumbar hyperlordosis, thoracic hyperkyphosis, and
spondylolisthesis being the most common (176).
This may be contrasted with another report of 79 patients who had
rhizotomy with laminoplasty. Sixteen percent developed scoliosis and
12% had spondylolisthesis at mean follow-up of almost 6 years (177).
Another study noted that after rhizotomy, the only consistent negative
effect was some increase in sagittal plane anterior pelvic tilt in
independent ambulators (178). Perhaps the most
common problem after rhizotomy, other than weakness, is the development
of planovalgus foot deformities, which are reported in up to 50% of the
cases (178,179). The deformity is often severe enough to require surgical correction.

Long-term results with many patients followed over
several decades are not yet available, but thus far an increased risk
of developing scoliosis or kyphosis has not been reported. Another
question remaining to be answered is whether late neuropathic
arthropathy will occur, but this seems unlikely.

Selective posterior rhizotomy is a demanding procedure
for the patient, the family, and the professional staff. Although there
is little risk involved, careful patient selection and surgical
technique are mandatory, the procedure is expensive, and long-term
results are not known. It may be of benefit to some children. On the
basis of experience in several centers, however, its use is decreasing
in the United States.

There are several possible roles for physical therapy in
cerebral palsy, some generally well accepted and some not. The brain
lesion affects motor function in four major ways, with individual
variation in the involvement of each. These are

Numerous attempts have been made over the past several
decades to modify the central nervous system, and to tackle substantial
motor dysfunction by external physical means. The goal is to produce
major improvements in voluntary control, tone, and muscle and body
balance. In general, most therapists work in a cephalocaudal sequence
to try to establish head control, then trunk balance for sitting, then
reciprocal lower-extremity mobility for crawling, pulling to stand,
and, hopefully, functional walking. The treatment methods include
neurodevelopmental therapy, sensory integration therapy, patterning,
conductive education, pressure-point stimulation, bracing, stretching,
and many recreation-based therapies (180). For
any of these techniques to succeed, the brain probably has to be
modified or reprogrammed to make new connections that function almost
as well as the original ones (2), and this may not be possible.

A different therapy approach, described in a pilot
study, is that of using low-intensity transcutaneous electric
stimulation in an attempt to strengthen weaker antagonistic
lower-extremity muscles. The treatment is applied only at night. In a
small group of patients, some motor improvement was noted, but was lost
after the stimulation was withdrawn (181). More investigation of this approach is necessary before its clinical relevance is determined.

The results and benefits of most of these methods continue to be debated (180,182,183,184,185,186,187,188,189).
There is considerable difference of opinion about which modalities
should be employed, at what age, how often (from once per week to
virtually continuous therapy for 18 hours a day), by whom, and how the
results are to be measured, documented, and assessed. Some published
studies suggest that in children with cerebral palsy, certain physical
therapy programs may improve contractures of joints, motor status, and
social motivation (5,186,187), whereas others suggest that they do not (183). Although short-term improvements are reported, long-term carryover is rare.

Significant problems with the literature include small
heterogenous samples, nonrandomized treatment, lack of controlled data,
and attrition of patients during many of the studies (183).
In many instances it is difficult or impossible to determine whether or
not the therapy was of benefit, or whether the reported improvement in
motor skills was the result of the child’s obligatory neurologic
maturation and the learning of substitution patterns and coping
methods, completely independent from any restructuring of neurocentral
control patterns. For some children, certain therapy programs may make
a difference. In this context, it is important that motor function be
documented periodically by the therapist during the child’s early
growth years. This can be done using such systems as the Movement
Analysis of Infants, the Gross Motor Performance Measure, Pediatric
Evaluation of Disability Inventory, and WeeFIM (190,191).

Much less controversial is the finding that a program
that focuses on compensation techniques during the first 3 years of a
child’s life is helpful. Such a program demonstrates to parents how
positioning can facilitate mobility and maintain the range of motion of
the joints, and educates parents about cerebral palsy in general. The
physical therapist attempts to set out benchmarks for appropriate motor
milestones within the capabilities of the particular child. The child
is assisted through the progression of milestones: rolling over,
crawling, standing, and eventually walking, while maximizing strength,
balance, and flexibility of the joints. Spasticity in the growing child
prevents the development of normal muscle growth and length, and the
therapist’s attempt is to approximate normalcy to the extent possible.

A major source of psychological stress in mothers of
handicapped children is the dependence of the child on the mother for
accomplishing the activities of daily living. A therapy program that
teaches mothers easier ways to work with their child is of great value (192).

Whenever possible, it is better that a parent perform
much of the physical therapy, and not rely totally on the physical
therapist (5). This saves professional fees,
minimizes trips to the therapist and interference with school time, and
positively involves parents and even siblings with the child. Many
parents, however, decline such involvement because of other family or
career demands, or simply because they are too tired to commit the time
and effort that are necessary.

Frequent sessions and protracted therapy programs are
expensive and time consuming, and generate hopes and expectations for
all involved. If there is no benefit, or if ineffective modalities are
employed, the therapy process can raise false hopes, increase
frustrations for the child and the family, waste large sums of money,
and sometimes unbalance interactions among the members of the family
unit (5,180).

There is general agreement that postoperative
rehabilitative physical therapy is not only helpful but also usually
essential in order to maximize the benefits of most types of
orthopaedic surgery in cerebral palsy. The physical therapist and the
patient must work together to overcome the early postoperative
weakness, stiffness, and discomfort. The goals are to maintain or
improve the range of motion of the joints, regain preoperative muscle
strength, maximize ambulation, and improve overall functioning to the
extent possible. How long should the postoperative therapy continue,
and how frequently should it be done? This varies with the scale of the
surgery. It may be necessary for as short a period as 1 or 2 weeks, or
as long as several months. The family may be able to provide the
treatments on a daily basis. If all treatments must be provided by the
therapist, two or three times per week is standard.

Another potential benefit of physical therapy is that it
prevents contractures of joints by ensuring the supervision of a daily
range-of-motion program for those who lack the motor strength and
voluntary control to maintain the range of movements by themselves.
This type of program can be done by a parent, an aide, or a caretaker,
and does not require the services of a physical therapist, other than
in developing and monitoring the program. As previously noted, however,
there are no data to validate the benefit of such a program. In some
severely involved children, it is impossible to prevent contractures,
no matter how aggressive the treatment.

An unresolved issue is the duration of maintenance
physical therapy. Although some parents want and even demand therapy
two or three times a week throughout the lifetime of their child, there
is no evidence that any type of physical therapy can have a beneficial,
lasting effect on motor function beyond early-to-middle childhood (age
4 to 8 years). Children older than this no doubt benefit more by
devoting their time (and their families’ and society’s resources) to
the development of communication, cognitive, and recreational skills,
instead of endless therapy sessions.

It is irrefutable that the physical therapist can be of
great benefit to the child and family. She or he is a resource person,
and sometimes helps out in

Of great importance is a good two-way communication
pathway between the therapist and the physician. The therapist is often
the primary person who documents the child’s neuromotor status,
monitors progress, and may recommend surgery or other treatments.

In traditional practice, orthotic devices are used for
preventing deformity, improving function by substituting for a weakened
muscle, or protecting a weakened part. In cerebral palsy, they are most
commonly used for

Progress has been made in understanding the biomechanics
of the lower extremities in cerebral palsy and in developing strong,
lightweight, comfortable new materials for orthoses. Lower-extremity
orthotics will almost never have to extend above the knee with patients
who have spastic diplegia (194). For foot
control, the University of California Biomechanics Laboratory (UCBL)
orthosis is most popular. It can maintain forefoot, hindfoot, and
subtalar alignment in a supple (not rigid) foot, but cannot control the
ankle.

The various types of ankle-foot orthoses (AFOs),
including the solid-ankle type, which controls the entire foot,
provides medial–lateral ankle stability, and maintains the ankle in a
rigid plantigrade position to preserve the plantar flexion–knee
extension couple. The solid-ankle AFO is the best choice for the
spastic foot, and can be used in almost any instance. AFOs facilitate
standing balance by counteracting the tendency toward equinus (195,196).
Modifications to the solid-ankle AFO have been made in order to improve
foot and ankle function. An example is the posterior leaf-spring AFO,
which does not provide mediolateral ankle stability, but allows some
plantar flexion and dorsiflexion from neutral while preventing foot
drop (197). This type is used when spasticity
is minimal. It stores minimal kinetic energy during dorsiflexion, and
gives back minimal energy at push-off.

Articulated AFOs have hinged ankles and plantar flexion
stops, which prevent equinus and excessive extensor thrust while
allowing free dorsiflexion during gait. They also allow more tibialis
anterior muscle function (198). Theoretically,
articulated AFOs should allow for better dorsiflexion activities, such
as bending over and stair climbing; however, they provide less ankle
support, are bulky, do not fit into shoes well, and are more likely to
cause heel irritation. They may also prevent a needed plantar
flexion–knee extension couple, and may allow triggering of the
gastrocnemius stretch reflex, causing the patient to fight the brace (190).
Most physicians are of the opinion that the hinged AFO is indicated
only for preventing recurvatum of the knee in the mid-stance phase of
gait. Tone-reducing features molded into AFOs have not been shown to be
of any benefit (199).

Another type of AFO is the floor-reaction type, of which
there are several varieties. It uses the plantar flexion–knee extension
couple to prevent knee-flexion crouch and gain appropriate stance-phase
knee extension during gait. This is possible provided the foot is
plantigrade, no significant knee-flexion contracture is present (<
10 degrees), hip extension is full, and no major rotational
malalignments are present in the limb. This approach has essentially
eliminated the need to ever prescribe a knee-ankle-foot orthosis (KAFO)
in an ambulatory child with cerebral palsy. The main indication for
KAFOs is to brace the lower limbs for ease of transfer in a
nonambulatory patient.

Obtaining beneficial elongation of tight or contracted
musculotendinous units or joint capsules can sometimes be accomplished
by gentle, nonpainful, repeated passive stretching, followed by
maintaining the correction with casts, splints, or adjustable orthotic
devices. This method of treatment has the potential to cause pain to
the child, and to raise false hopes for improvement when used in
patients with more-than-mild spasticity, tightness, or contractures, or
in those who are unable to accurately communicate their discomfort.

With properly selected patients, manipulation and
casting can sometimes improve, at least temporarily, contractures of
the ankle, knee, elbow, wrist, or hand (200).
The process may be repeated from every few days to once a week; various
casting materials, dropout casts, removable splints, and orthotics with
dial-lock or ratchet hinges may be used. The use of nerve or muscle
blocks as an adjunct may sometimes be worthwhile.

Another method, known as inhibition casting,
has been employed for several years. This treatment is applied to the
lower extremities by carefully molding bilateral plaster below-knee
casts in an attempt to inhibit certain normal tonic plantar reflexes,
especially grasp, and therefore reduce overall lower-extremity tone. A
probable added benefit of this treatment is stabilization of the foot
and ankle by the well-molded cast. The casts are either left in place
or changed weekly for 3 or 4 weeks, during which

time
intensive physical therapy is performed. Hinged AFOs are then
substituted for the casts. This method has been successful according to
some reports (201,202,203,204),
but because the noted improvement may not be maintained for more than a
few months, others have not considered it to be of value (205).

Surgical treatment is a rapid and dramatic means of
altering the structure and functioning of the musculoskeletal system in
children with cerebral palsy. It is almost always the only effective
means of treatment when fixed myostatic contracture exists. Lengthening
a muscle also weakens it. This may help with muscle balance around a
joint. Strengthening an antagonist muscle may still be desirable, but
difficult or impossible to achieve. In most cases, the muscles that
contract eccentrically, producing deceleration (e.g., hamstrings) and
shock absorption, are the most appropriate for lengthening, and loss of
function rarely occurs. Concentric or accelerator muscles (e.g.,
gastrocnemius) initiate, rather than modulate, movement, and may be
substantially weakened by lengthening. In these situations, as with the
iliopsoas muscle, it may be better to intramuscularly lengthen the
tendinous portion and accept some residual contracture in exchange for
preserving needed strength.

The long-standing effects of spasticity as well as the
delay in the development of normal gait patterns contribute to skeletal
abnormalities that interfere with efficient walking. Internal tibial
torsion and femoral anteversion, both normal in early development and
self-correcting with normal positioning and mobility, tend to persist
in the young spastic diplegic patient. In the older patient, long-term
difficulties with foot clearance actually lead to an exaggerated
remodeling of tibial torsion into an excessive external position.
Correction of musculotendinous length and reduction of spasticity in
the presence of persistent skeletal malrotation is insufficient.

Sequential gait analyses more than 4 years apart in
children with cerebral palsy who had no treatment show that in most
cases gait function decreases over time. This includes temporal and
stride parameters and kinematics. Children with cerebral palsy who have
orthopaedic surgery do improve their gait (206).
The subsequent text contains recommendations for the treatment of the
most common problems and deformities in an ambulatory child with
spastic diplegia.

In most cases, the patient has several abnormal elements
in the disturbed gait. The best results are obtained from surgery only
if all of the abnormalities are identified preoperatively and corrected
during the same surgical setting (207,208,209),
thereby ensuring that only one rehabilitation period is required.
Little or no benefit is derived from simply performing one procedure,
such as a triceps surae lengthening, and waiting to assess its effect
on other abnormal elements of the child’s gait before proceeding to
correct them. This strategy does not improve the final result, and
results in unnecessary discomfort, hospitalization, and rehabilitation.

The best age for surgery varies with the patient and the
problems. Ideally it would be after the gait pattern has stabilized,
which is approximately the age of 4 or 5 years in nonafflicted
children, and either the same or later in children with cerebral palsy,
but before the age of 8 years. Surgery improves walking velocity and
stride length in young children, but functional outcome measures such
as the GMFM usually show minimal change (210).
Certainly surgery can be successful in older children and adults. In
children of ages 6 to 15 years, a recent study showed that multilevel
surgery in spastic diplegia improved sagittal plane kinematics and
power generation at the hip and ankle (211).
Some components of gait, such as velocity, are not reliably improved in
older children, especially after the age of 12 years (212).
In cases in which the hips are at risk for dislocation, or when there
is progressive or substantial acetabular dysplasia, surgery should not
be delayed, regardless of age.

In the immediate postoperative period, the major
concerns relating to the orthopaedic surgical procedures are pain
management, relief of superimposed myospasms, and minimization of the
child’s anxiety. Giving the analgesic medication through the existing
intravenous access line, using patient-controlled analgesia when
possible, routinely administering diazepam for 48 to 72 hours to lessen
myospasms, providing continuous caudal analgesia through a small
catheter, and having kind, supportive personnel present are most
helpful to the child.

Overall, the postoperative management is aimed at
restoration of motion in the joints, muscle strength, and improved gait
as rapidly as possible. During this period of up to several months, it
may be beneficial to use night splints for comfort and to prevent
positioning of the joints in positions that could contribute to
recurrent contractures. Such splinting should never exceed the level
that can be comfortably tolerated by the child. In this regard, it is
sometimes necessary to alternate control of one ankle and the opposite
knee on successive nights, or use splinting only during the day, when
it can be effectively monitored. The value of night splinting is not
universally accepted, and valid well-controlled studies proving its
efficacy are lacking. It is expensive and can be burdensome and
uncomfortable for the patient because of ill-fitting splints, the
frustration of substantially restricted mobility, or maintenance of a
continuous overstretch on muscles.

When only soft tissue surgery has been done, ambulation
may begin within a few days postoperatively. If osteotomies or subtalar
arthrodeses were performed, partial weight bearing may begin as early
as 3 weeks postoperatively, assuming that the internal fixation is
adequate. Full, unrestricted weight bearing should probably be deferred
until radiographic evidence of adequate early bone healing is seen,
usually by approximately 6 weeks. In patients with spastic diplegia it
takes at least 3 months, and sometimes longer, to regain the
preoperative level of strength after most multiple lower-extremity
surgical procedures.

The normal gait cycle is broken down into stance phase
(60% of the gait cycle) and swing phase (40%). In the initial and
terminal 10% of stance both feet are in contact with the ground (double
support), whereas during the middle 40% single-limb stance occurs
(single support). Stance phase accomplishes the functions of load
acceptance, joint segment length modulation, forward progression of the
body over a stable plantigrade foot, and propulsion of the limb into
swing phase. Swing phase allows for the advancement of the limb
forward, while maintaining clearance of the foot in the face of
gravity. The phases of the gait cycle have been further divided into
loading response, midstance, terminal stance, preswing and initial
swing, midswing, and terminal swing (213). This
terminology is useful for describing abnormal gait patterns with
specific reference to the part of the gait cycle in which they occur.
It is also important for the physician to understand the sequential
firing patterns of normal muscles during gait (Fig. 15.8).

In normal gait, motion occurs at all levels and in three
planes of motion: coronal, sagittal, and transverse. In normal gait,
most motion occurs in the sagittal plane. In the normal child, minimal
excursions of pelvic motion occur throughout the gait cycle, because
there is successful maintenance of length by the locomotor segments
managed by hip, knee, and ankle motion during stance phase (Fig. 15.9).
This allows for the minimal expenditure of energy during normal gait. A
minimal expenditure of energy is described as one of the prerequisites
of normal gait, which are

In the child with cerebral palsy, abnormal selective
motor control of the two joint muscles causes dysfunctional rotations
of the joints, excessive pelvic and trunk excursions in the coronal,
transverse and sagittal planes, and therefore inefficient energy
conservation (Fig. 15.10) (2).

Normal and pathologic walking are best studied and
understood with the use of computerized three-dimensional gait analysis
techniques. A number of commercial systems are now in place worldwide
and are used for both clinical and research purposes. Modern gait
analysis systems include collection and computation of
three-dimensional kinematics (joint and segment motion) and kinetics
(joint moments and powers), surface and fine wire electromyography
(EMG), and foot pressure measurement (pedobarograph). Motion data
relating to joints are collected by multiple cameras which determine
the three-dimensional coordinates of reflective markers placed on
specific limb and joint landmarks (Fig. 15.11).
Kinetic data are computed from the collection of simultaneous position
(kinematic) and force plate data. Force plates, which measure the
ground reaction forces, are embedded in the walking surface.
Electromyographic data are also recorded from surface or deep muscles
during gait and standing as an adjunct to the assessment of abnormal
muscle tone. Typical protocols used in clinical gait analysis have been
well documented (214,215).
Gait data supplement a detailed physical evaluation of passive range of
motion of joints, muscle strength, balance, and spasticity. Examination
of recent pertinent radiographs (spine, hip, knee, foot, and alignment)
may also be necessary. The interpretation of gait analysis data
requires considerable training and background knowledge of the
methodologies used to collect and compute the data. Dedicated and
experienced professionals are required in order to assure high quality
gait data and interpretation.

A recent study indicated that when experienced
physicians added gait analysis data to their own clinical evaluations,
they changed their surgical recommendations in approximately 50% of the
cases, with more decreases than increases in procedures (216).
Whether this will improve surgical outcomes and reduce costs has not
been well studied. Although preoperative gait analysis is theoretically
desirable, it is neither possible nor necessary for every child who
undergoes surgery. Nevertheless, every surgeon who treats children with
cerebral palsy should have a good knowledge of normal and pathologic
human gait and understand gait analysis and pattern recognition (217). The application of his or her observational gait assessment will then be more accurate.

Observational gait analysis is done by repeatedly
watching the child walk, viewing the gait from the front, back, and
side. One component should be studied at a time (e.g., cadence, stride
length, the foot, the knee, the hip, lower-extremity rotational
alignment, the pelvis, the trunk), recognizing that often there are
differences between the sides. Gage has described observational gait
analysis in detail (2). For a given child, intraobserver observations are more accurate than interobserver observations (218).
In either situation, the opportunity to study a videotape of the gait
from the front, back, and sides, with zoom capability for the feet,
increases the accuracy of the assessment.

Pattern recognition allows the surgeon to observe the
gait cycles of the walking child, look for the priorities of gait and
deviations from normal, and correlate this information with the
findings on static physical examination. If all this information is
consistent with a recognized standard pattern (e.g., one of the types
of hemiplegia or a diplegic gait with equinovalgus feet, tight
hamstrings, tight hip flexors and adductors, and increased femoral
anteversion), there is a stronger likelihood of success after applying
the proper surgical procedures to address each problem.

It would be ideal to review a high-tech gait analysis on
every ambulatory cerebral palsy patient preoperatively if cost and
accessibility to sophisticated gait labs were not a problem. In
reality, surgeons will be quite comfortable without gait analysis when
treating those patients who have apparently straightforward patterns of
gait pathology (Fig. 15.12). Nevertheless, gait
analysis provides a more precise definition of the gait abnormality in
patients with more complex conditions, such as those with severe
diplegia and those who have had previous surgery with little success.
Gait analysis is very valuable is distinguishing children who have
dyskinesia from those with spasticity; this is especially important
because surgical results are much less predictable in dyskinesia (219).
Finally, gait analysis provides an objective documentation of the pre-
and postoperative gait when assessing the results of surgery.

Hallux valgus and bunions occur commonly in cerebral
palsy, most likely because of a combination of muscle imbalance with
adductor hallucis overactivity and externally applied forces, such as
inadequate clearance resulting from equinovalgus deformity of the foot
and ankle, forcing the phalanges of the great toe into valgus (220,221).
Equinus, and particularly valgus, of the foot must be corrected first
in order to achieve a lasting good result from hallux valgus surgery in
cerebral palsy.

Indications for surgery in this condition are pain and
difficulty with wearing proper shoes; rarely is cosmesis alone an
indication. There are myriad possible operations for the correction of
hallux valgus, and most have been used with varying degrees of success
in cerebral palsy. When spasticity is very mild, such procedures as
ostectomy, capsulorrhaphy, adductor hallucis release or transfer, or
proximal or distal first metatarsal osteotomy can be successful (222,223,224,225,226). With recurrence, or with patients who have more spasticity, the McKeever metatarsophalangeal arthrodesis (227),
with the joint in a few degrees of valgus and 10 degrees of
dorsiflexion relative to the floor, is an excellent procedure, as
confirmed by long-term follow-up studies (221,228).

Normal function of the ankle during gait is best understood by consideration of three rockers (2).
At initial contact, which is a heel strike in the normal child, the
ankle is in slight equinus. During first rocker it moves into further
plantar flexion as the foot is lowered to the walking surface under
control of an eccentrically contracting anterior tibialis. During
second rocker the tibia advances forward over the plantargrade foot as
the gastrocsoleus eccentrically contracts. The ankle dorsiflexes
approximately 10 degrees. Third rocker occurs in terminal stance and is
the push-off generated by concentric contracture of the gastrocsoleus.
In early swing the ankle is in 20 degrees of plantarflexion, and the
pretibial muscles fire concentrically to dorsiflex the foot for
clearance. These rockers are evident in normal kinematic and kinetic
plots (Fig. 15.13).

Ankle equinus, varus of the hindfoot and, often, varus and supination of the forefoot are present in this deformity (Fig. 15.16).
It is most often seen in hemiplegia, but may also occur in children
with diplegia and quadriplegia. The hindfoot varus is most likely
caused by overactivity of the tibialis posterior muscle, whereas varus
and supination of the forefoot are more likely the result of
overactivity of the tibialis anterior, which may also contribute to
hindfoot varus. Peroneal muscle weakness may also be a factor.

This is the most common condition in patients with
diplegia, followed in frequency of occurrence by equinovarus and
calcaneus, both of which have an approximately equal prevalence (270) (Fig. 15.18).
The cause is probably muscle imbalance, triceps surae overactivity,
weakness of the tibialis posterior muscle, and relative overpull of the
peroneal musculature (271). The treatment of
equinus is as described in the preceding text. Spasticity in the ankle
plantarflexors limits ankle dorsiflexion and shifts the motion to the
subtalar and midfoot joints. In young patients with diplegia,
spasticity of the peroneals may contribute, but this tends to diminish
with time.

The typical rotational alignment of the tibia at birth
is medial, with progressive movement toward the adult orientation of
lateral (foot-thigh axis 10 degrees, bimalleolar axis 20 to 25 degrees)
by the age of 6 or 7 years. This remodeling is believed to occur
through the normal torque afforded by walking.

In children with appropriate motor control, muscle
balance, and ligamentous integrity, normal ambulation usually corrects
the neonatal femoral anteversion to the normal adult range of 10 to 20
degrees. In children with cerebral palsy, the delay in ambulation in
conjunction with abnormal or spastic muscular balance may delay or
prevent correction of anteversion and may sometimes even lead to an
increase from the neonatal value. There is no “natural” correction of
femoral anteversion by growth/maturation in patients with cerebral
palsy (211). Intoeing is also caused by
increased spasticity in the internal rotator muscles of the hip (the
medial hamstrings or the anterior fibers of the gluteus medius and
tensor fascia lata).