Tachdjian’s Pediatric Orthopaedics

From the Texas Scottish Rite Hospital for Children

John A. Herring, MDCHAPTER 31 (1419-1497)

Disorders of the Brain

Lori A. Karol

Levels of Involvement

The neuromuscular system may be affected at various levels, each of which is characterized by
changes in motor function peculiar to the site and extent of involvement. The differential features
of various levels of motor function are illustrated in Table 31.1.

Spinomuscular Level

At the spinomuscular level, motor activity is simple; impulses arising in the anterior horn cells of
the spinal cord are transmitted through peripheral nerves to myoneural junctions and then to
individual muscles. In disorders at the spinomuscular level, the loss of motor power is focal

and segmental, with complete paralysis of the muscles or muscle groups that are supplied by a
peripheral nerve or by the anterior horn cells in the spinal cord. Muscular paralysis is flaccid or
hypotonic, and degeneration, atrophy, fibrillations, and fasciculations are typical findings. The
deep tendon and superficial reflexes are diminished or absent. Pyramidal tract signs, abnormal
involuntary movements, and ataxia are absent. Trophic changes may be seen in the skin, nails, and
bone.

Spinal Level

Pathologic processes at the spinomuscular level may be further classified into various sublevels.
When the disease originates in the anterior horn cells, as in poliomyelitis, the spinal level of the
motor system is affected. Other examples of diseases at the spinal level are progressive spinal

muscular atrophy of the Werdnig-Hoffmann type, progressive bulbar palsy, syringomyelia, and
intramedullary neoplasm. Loss of function of the anterior horn cells or the motor nuclei of the
brainstem results in clinical findings of flaccid paralysis, atrophy, areflexia, degeneration, and

fasciculations.

Neural Level

At the neural level of the motor system, the peripheral nerves and nerve roots are affected, as in
obstetric brachial plexus palsy and progressive neuromuscular atrophy (Charcot-Marie-Tooth
disease). In processes affecting nerves, the sensory fibers are usually involved, with resultant
sensory changes such as anesthesia or hyperesthesia. Otherwise, the clinical findings are similar to
those of involvement of the spinal level; that is, flaccid paralysis, atrophy, degeneration, and
areflexia develop as a result of loss of conduction of motor impulses. In the absence of sensory
changes, it is difficult to distinguish between diseases of the peripheral nerves, anterior roots, and
anterior horn cells.
Myoneural Level

If the pathologic process arises at the myoneural junction, as in myasthenia gravis and familial
periodic paralysis, it is a disease at the myoneural level. In diseases of primarily muscular origin,
the motor system is involved at the muscular level. The muscular dystrophies are familiar
examples of disturbance at the muscular level in diseases with spinomuscular involvement. The
paralysis is flaccid, but reflexes persist until the late stages, when marked atrophy has already

occurred. Contractility is lost but not excitability; that is, the muscle fibers have degenerated and
have been replaced by fibroadipose tissue, but the peripheral nerves and anterior horn cells are
normal.

Extrapyramidal Level

Disorders of the motor system at the extrapyramidal level are characterized by generalized
involvement of the muscles of the limbs and trunk. Muscle tone is hypertonic. Atrophy,
asciculations, and degeneration are absent. Motion of the limbs is hyperkinetic, with loss of

associated or automatic movements. The deep tendon and superficial reflexes are normal. No
pyramidal tract responses or sensory deficits are present. Athetoid cerebral palsy (CP) is a
common example of a disease at the extrapyramidal level.

Pyramidal (Corticospinal) Level

At the pyramidal or corticospinal level, motor deficit arises from involvement of motor nuclei of
the cerebral cortex. The paresis is usually generalized and associated with hypertonicity or
spasticity of muscles. Pyramidal tract signs and pathologic reflexes are generally present. Usually
some atrophy that is not focal is present; it is caused by chronic paralysis and disuse.
Fasciculations, trophic disturbances, degeneration, and abnormal movements are absent. The
deep tendon reflexes are hyperactive, and the superficial reflexes are diminished or absent.
Spastic CP illustrates the pyramidal level of motor involvement.

Cerebellar Level

Lesions at the cerebellar level are characterized by loss of coordination and control, or ataxia. No
real loss of motor power occurs. Fasciculations, degeneration, atrophy, and trophic disturbances
are absent. The deep tendon reflexes may be diminished, but the superficial reflexes are normal.
For References, see expertconsult.com.

Cerebral Palsy

Definition CP was first described by William Little in 1862. Little correlated the findings seen in
young children with CP and associated them with difficult births. The term cerebral palsy
originated with Freud. Static encephalopathy has been used interchangeably with cerebral palsy. A
succinct and accurate definition of CP is difficult to construct because of wide variability in the
manifestations of CP. In 2008 CP was proposed as “a group of permanent disorders of the
development of movement and posture, causing activity limitation, that are attributed to
nonprogressive disturbances that occurred in the developing fetal or infant brain.” In all cases, the
following must be true:
• CP is the result of a brain lesion; therefore, the spinal cord and muscles are structurally and
biochemically normal.

• The brain lesion must be fixed and nonprogressive. Thus, all the progressive neurodegenerative
disorders are excluded from the definition.

• The abnormality of the brain results in motor impairment.

The insult to the brain may occur prenatally, perinatally, or during childhood. Although older
children with brain damage were traditionally excluded from the definition, this is not clinically
relevant from an orthopaedic standpoint. Certainly, any orthopaedist caring for a child who has
sustained an anoxic injury after nearly drowning or who is spastic after infectious meningitis would
argue that these slightly older children functionally have CP. The clinical manifestations of CP
depend on which part and how much of the brain are involved. The range of manifestations is
huge, with both a young child who is intellectually bright but walks on his toes and a
noncommunicative wheelchair-bound child with seizures meeting the afore mentioned definition
of CP.

The orthopaedic surgeon is consulted by a pediatrician or family for management of the

musculoskeletal problems that follow from the underlying brain lesion. It is of utmost importance
for the orthopaedist to evaluate the child thoroughly to ascertain why the child has CP. If the child
was born full-term, if no perinatal medical problems were noted, and especially if the child began
to develop normally and then regressed, prompt neurologic consultation must be sought. The
neurologist will differentiate CP from such dangerous entities as brain and spinal cord tumors,
metabolic encephalopathies, and progressive neurodegenerative diseases, some of which are
treatable. Epidemiology The incidence of CP is increasing slightly. In recent reports the incidence
has been estimated to be between 2.4 and 2.7 per 1000 live births. The prevalence of CP appears
to be increasing secondary to an increase in the number of infants with very low birth weight
being born and the increased survival of these tiny neonates, whereas the rate of CP in infants of a
given birth weight has remained stable. This increase in incidence is of concern because the
economic impact of CP is considerable, with the cost per case estimated at $503,000 in 1992. The
risk for CP in a child born full-term is approximately 1 in 1000. The incidence of CP has been
correlated with both gestational age and birth weight. Approximately 50% of children with CP
have low birth weight, and 28% weigh less than 1500 g at birth. The incidence of CP is increased
with multiple births. In more recent studies of multiple births the incidence of CP is 9 to 12 per
1000 in twins, 31 to 45 per 1000 in triplets, and 111 per 1000 in quadruplets. The predisposition to
CP in twin pregnancies is present even when controlling for birth weight and gestational age. The
risk for CP is high for a surviving twin when the other twin dies in utero.

Etiology

Prenatal

The brain of the fetus is susceptible to damage from maternal infections and toxins. The TORCHES
group of infections (toxoplasmosis, rubella, cytomegalovirus, herpes, and syphilis) is known to
cause significant damage to the developing brain of the fetus, and such damage leads to very
neurologically involved infants with mental retardation, microcephaly, and seizures. Orthopaedic
deformities are noted in 82% of these children.
Fetal exposure to drugs and alcohol through maternal use can also result in injury to the
developing brain. Cocaine, heroin, and marijuana can all cross the placental barrier and cause
damage to the central nervous system of the fetus. Congenital malformations of the brain that
occur during early pregnancy often result in severe CP. It has been stated that approximately 10%
of patients with CP have congenital brain malformations that are apparent on neuroimaging.
Rhesus blood group incompatibility resulting in kernicterus as a cause of CP is decreasing in
incidence with improvements in prenatal care. RhoGAM treatment of Rhnegative mothers has led
to a decline in kernicterus, which often resulted in the development of such movement disorders
as athetoid CP.

Maternal health problems, such as renal failure or infections, can lead to problems with brain
development in the fetus. Prenatal chorioamnionitis and maternal infection have been associated
with an increased risk for premature onset of labor and CP in the infant. Placental abnormalities
have been linked with a higher frequency of CP.

Perinatal

Anoxia as a result of perinatal complications may lead to the development of CP. A tight nuchal
cord or placental abruption can lead to anoxia and thus result in CP. Fetal hypoxia may be
detected by fetal heart rate monitoring, but changes consistent with hypoxia, such as late
deceleration of the heart rate with uterine contractions, are common and not specific. The
frequency of CP associated with birth asphyxia is estimated to be 1 in 3700 full-term live births.
Fetal distress during delivery has been documented in some children with CP. The mode of
delivery—vaginal or cesarean—has not been found to influence the incidence of CP.

Sepsis in the neonatal period can predispose to the development of CP in a low–birth weight
infant. Bronchopulmonary dysplasia and prolonged ventilation in preterm infants may result in
hypoxia, which predisposes the infant to CP. Extracorporeal membrane oxygenation (ECMO) has
been used to sustain babies with severe cardiorespiratory failure. CP has been diagnosed in up to
20% of surviving children who were treated with ECMO.

Cardiac surgery for the treatment of severe congenital heart disease has been linked with an
increased incidence of CP. Heart surgery before the age of 1 month resulted in CP in 25% of
infants. Clearly, these children are quite ill, with an increased risk for hypoxia, sepsis, and
prolonged ventilation.

Postnatal

Infections such as meningitis in early childhood can lead to CP. Any episode of hypoxia, such as
cardiopulmonary arrest, near-drowning, and suffocation, can also produce brain damage leading
to CP. Trauma, such as motor vehicle accidents producing head injury, severe falls, and child
abuse, may result in CP as well.

Classification Physiologic

The first classification is physiologic and describes the type of movement disorder present. The
most common movement abnormality is spasticity. Spasticity results from damage to the
pyramidal system, particularly the motor cortex in the brain. Disinhibition of pathologic reflex arcs
leads to increased tone in the extremities. The tone is dependent on velocity, which means that if
a muscle is stretched rapidly, tone increases more than if the same muscle group were stretched
gradually and gently.

Hypotonia is, as its name implies, abnormally decreased tone. Infants with CP are often described
as floppy or hypotonic. Hypotonia is usually a phase and most frequently leads to spasticity as the
infant matures.

Dystonia is described as increased tone, which is not dependent on velocity. Whereas tone in
spasticity is described as “clasped knife,” tone in dystonic CP is described as “lead pipe,” which
means that tone does not decrease with gentle prolonged stretching.

Athetosis is characterized by abnormal writhing movements that the patient cannot control. The
movements become more exaggerated as the patient tries to complete a purposeful motion.
Athetosis results from damage to the basal ganglia. Speech is often garbled and difficult to
understand, yet affected patients may be intelligent. Athetosis has frequently been the result of
neonatal kernicterus.

Cerebellar lesions lead to ataxic CP. The disturbed balance of these children results in a wide-
based and clumsy gait. Pure ataxic CP is rare.

Patients with CP frequently have a mixed form of movement disorder. It is important to correctly
classify the movement disorder of a patient with CP because the results of surgical treatment are
unpredictable for all but purely spastic patients.

Geographic

The second classification system is geographic and describes what part of the body is affected by
CP. Hemiplegia is present when only one side of the body is involved, with the upper extremity
usually more involved than the lower extremity (Fig. 31.1). Patients with spastic hemiplegia can be
further divided by their degree of gait impairment. Winters and colleagues subdivided patients
with spastic hemiplegia into four groups: (1) loss of swing-phase ankle dorsiflexion (i.e., footdrop)
but stance-phase dorsiflexion present; (2) loss of stanceand swing-phase ankle dorsiflexion
(equinus) and possible knee hyperextension in stance phase; (3) ankle involvement plus increased
stance-phase knee flexion with limited range of knee motion; and (4) ankle, knee, and hip
involvement with increased stance-phase hip flexion and limited range of hip motion.

Diplegia implies involvement of both sides of the body, with both lower extremities being involved
(though not always symmetrically) and lesser involvement of the upper extremities (Fig. 31.2). A
word of caution is needed. If the patient has abnormal tone in both lower extremities but the
upper extremities are completely normal, the examiner should beware. Patients with diplegia will
have some abnormality in the upper extremities, such as decreased fine motor control, spasticity,
or increased reflexes. If the upper extremities are normal, it is imperative to evaluate the spinal
cord. Spinal cord pathology, including tumor, may masquerade as CP.

Involvement of both lower extremities and one upper extremity is termed triplegia. Quadriplegia,
or total body involvement, is present when all four extremities are severely involved, with poor
trunk control as well (Fig. 31.3)
Familial spastic paraparesis, a genetic neurologic disease, may resemble CP in that both lower
extremities are spastic, yet the upper extremities are normal. Various forms of the disease exist,
and a history of other affected family members is helpful.

Functional

Current emphasis is on classifying patients with CP by functional level (Fig. 31.4). The Gross Motor
and Functional Classification System (GMFCS) is most commonly used to describe the patient’s
level of function before and after an intervention. The GMFCS scale has five levels. GMFCS 1
describes a patient who ambulates without aids on all surfaces and keeps up with peers. In GMFCS
2, the patient is fully ambulatory, may use lower extremity orthoses, and does not keep up fully
with peers. At GMFCS 3, the patient uses ambulatory aids such as a walker or crutches and may
use a wheelchair for longer distances. GMFCS 4 describes nonambulatory patients who are able to
propel their own wheelchair, whereas GMFCS 5 indicates an inability to transfer, propel a
wheelchair, or support the trunk. A comprehensive review of nine CP registries throughout the
world revealed the following proportion of GMFCS levels: level 1, 34.2%; level 2, 25.6%; level 3,
11.5%; level 4, 13.7%, and level 5, 15.6%. Because levels 1 and 2 are considered lesser
involvement, most patients are mildly involved, although more severely involved children are
more apparent in a pediatric orthopaedic practice.

Evaluation

History

The first step in the evaluation of a child with CP is to obtain a complete history, especially the
birth history. Birth weight, gestational age, complications, and whether the child required
ventilator assistance or hospitalization in the neonatal intensive care unit are important data. If
the birth history is normal, neurologic consultation should be considered. Evaluation of motor
milestones will reveal delayed development. Head control should be present at 3 to 6 months,
sitting by 6 to 9 months, crawling by 9 months, standing and cruising by 10 to 12 months, and
walking between 12 and 18 months. Adjustments for prematurity should be made; a premature
child may not walk by 15 months of age. Preferential use of one hand or leg and early handedness,
particularly left-handedness in small infants, are often clues that spastic hemiparesis may be
present. Likewise, dragging one leg when crawling or scooting also may be an indication of
hemiparesis. Ascertaining whether the child has other problems, such as strabismus, difficulty
swallowing, frequent choking, delayed speech development, poor eyesight, and seizures, is
important. Some 20% to 40% of children with CP have seizures, most common in hemiplegic and
quadriplegic patients. These observations may all be clues leading to the diagnosis of CP.

Physical Examination

Muscle Tone

Physical examination of a child with CP should include muscle tone in the extremities. With the
patient relaxed (even sitting on the lap of a parent), the extremities are brought through a full
range of motion. Spasticity feels like tightness in the muscles, which become tighter the quicker
the limbs are passively moved. Greater range of motion can be gained by slowly and gently
stretching the joints in question. The Tardieu test is a measure of spasticity. For example, if the
examiner is assessing hamstring spasticity, the angle at which a “grab” of resistance occurs when
quickly extending the knee with the hip in flexion is compared with the amount of extension
possible when the knee is stretched slowly.

Fine motor activities should be assessed. Passing the child a toy or a pen often reveals spastic
hemiplegia in one extremity. Having the child clap the hands or wiggle the fingers may reveal
difficulties in fine motor control.

Reflexes

Deep tendon reflexes are increased in patients with CP. Repetitive tapping of the deep tendons or
quick passive dorsiflexion of the ankle may produce clonus, which establishes the presence of an
upper motor neuron neurologic abnormality. In hemiparesis, reflexes will be asymmetric.

Infantile reflexes disappear in normal children by 3 to 6 months of age as the motor cortex
matures; however, they are retained in children with CP. Bleck’s textbook on CP outlines these
reflexes in clear detail. The startle reflex, or the Moro reflex, which should disappear by 4 months
of age, is elicited by letting the infant’s head drop back into extension with the infant supine but
slightly elevated. This causes the legs and arms to extend abruptly. A sudden loud noise can
likewise cause an older child to extend and lurch from a wheelchair. The parachute reflex is tested
by holding the child in the air and then lowering him quickly headfirst toward the examining table.
Children older than 5 months will reach out with both arms to protect themselves. Children with
CP cannot do so, and those with hemiplegia will reach out with only one arm.

The tonic neck reflex is elicited by turning the supine infant’s head to one side. The ipsilateral arm
and leg will extend while the contralateral arm and leg flex. This reflex should disappear in infancy;
persistence should raise suspicion for CP.

Balance, Sitting, and Gait

Balance, sitting, and gait are assessed by noting whether the child can sit unsupported without use
of hands or get into a sitting position without assistance or whether balance is easily disturbed in
the sitting position or as the child walks.

Clinical assessment of gait requires that the child’s joints be readily seen, so the child should be
barefoot and in shorts or a short gown. The evaluation should be conducted with the examiner
seated on a stool at the level of the child. Enough room should be available for the child to walk
naturally. Heel-to-toe walking, hopping on either foot, and running are observed. A patient with
mild hemiplegia may walk nearly normally but exhibit abnormal movement patterns while
running; the affected upper extremity will draw upward and not have a normal arm swing.

Gait should be observed from the front of the child and then from the side, and the hips, knees,
and ankles should be systematically assessed from each perspective. A crouched gait consisting of
increased flexion at the hip and knee, toewalking with genu recurvatum, or a footdrop in the
swing phase of gait may all be indicative of CP. Disturbance in clearance of the swing-phase limb
may be caused by footdrop or an inability to flex the knee.

Other Assessments
Rarely are imaging studies ordered by orthopaedic surgeons when establishing the diagnosis of CP.
If questions persist regarding a correct diagnosis, referral to a pediatric neurologist is indicated. At
the neurologist’s discretion, imaging studies such as cranial ultrasonography, brain magnetic
resonance imaging (MRI), and computed tomography (CT) may be pursued. Similarly, laboratory
studies may be necessary to look for evidence of metabolic diseases associated with delays in
development and CP-like symptoms, such as congenital hypothyroidism or dopa-responsive
dystonia. However, a detailed discussion of these metabolic conditions is beyond the scope of this
chapter.

Histopathologic and Imaging Findings

Two findings frequently described on histopathologic examination or imaging studies of the brain
in children with CP are periventricular leukomalacia and intraventricular and periventricular
hemorrhage. Periventricular leukomalacia is defined as patchy areas of necrosis in the
periventricular white matter adjacent to the lateral ventricles. It results from an ischemic insult to
the arterial watershed area close to the ventricular walls. Pyramidal tract fibers mapping to the
lower extremities pass through this area and are therefore more susceptible to injury than fibers
responsible for the upper extremities and face.

The areas of the brain immediately adjacent to the ventricles are also most susceptible to
hemorrhage. Hemorrhage may be seen on ultrasound, MRI, or CT. Mild hemorrhages involve the
germinal matrix adjacent to the ventricles, whereas more severe hemorrhages extend into the
ventricles themselves and into the parenchyma of the brain. Hypoxia is known to predispose to
periventricular and intraventricular hemorrhage. Approximately one-half of preterm infants with
CP are found to have abnormalities on neuroimaging, such as echolucency in the periventricular
white matter or ventricular enlargement on cranial ultrasound. In children with CP born at or near
term, approximately two-thirds have abnormalities on neuroimaging, including focal infarction,
malformations, and periventricular leukomalacia.

Gait Analysis

Gait analysis has become popular in the assessment of movement disorders in children with CP.
Accurate documentation of dynamic range of motion may help in planning surgical treatment and
assessing the results of orthopaedic operations, and complements but does not replace a good
clinical examination. When gait analysis graphs are scrutinized together with information from the
clinical examination and slow-motion videotape, better understanding of a patient’s gait can be
gained. A detailed discussion of gait analysis can be found in Chapter 5. The Functional Mobility
Scale is a questionnaire that assesses the child’s ambulatory ability at 5 m (within the home), 50 m
(short distances), and 500 m (community ambulation) and should be considered in conjunction
with gait analysis in surgical decision making.

Cadence. Cadence parameters include walking speed, step length, number of steps per minute,
and the proportion of time spent in the stance and swing phases. Patients with CP usually have
disturbances in cadence parameters. In good walkers with spastic diplegia, walking velocity is
maintained despite decreased step length by increasing cadence (quick, short steps). Good
walkers with CP increase their speed by increasing cadence, but spasticity interferes with
increasing step length. In those with more severe diplegia and quadriplegia, walking speed is
diminished, with decreased cadence and step length. The proportion of time spent in stance
phase, particularly double-limb stance, increases because the child has greater difficulty with
balance and advancing the limb. Children with hemiplegia show asymmetry in step length and in
single- and double-support time.

Kinematics.

Certain kinematic patterns are seen in patients with CP. In the hip, scissoring, which is caused by
tightnessin the adductor musculature and, in part, by weakness of the hip abductors, leads to a
narrow base of gait and difficulty advancing the swing-phase limb past the stance-phase limb (Fig.
31.5). In the sagittal plane, increased hip flexion and anterior pelvic tilt may be part of crouch gait
as a result of increased tone in the iliopsoas (Fig. 31.6). Increased femoral anteversion may be
documented by gait analysis as increased internal rotation of the hips. Asymmetric pelvic rotation
may be present, and gait analysis is particularly helpful when the examiner is trying to ascertain
whether the abnormal rotation is originating from the pelvis, hips, or tibiae (Fig. 31.7).

At the knee, sagittal-plane motion is usually abnormal. In patients with tight hamstrings, the knee
remains flexed at initial contact and is unable to extend normally during stance phase. In swing
phase, spasticity of the rectus femoris may inhibit the patient’s ability to flex the knee and clear
the floor (Fig. 31.8). Genu recurvatum during stance phase may be present in response to a tight
Achilles tendon, which causes difficulty advancing the tibia forward over the foot.

Ankle kinematics often shows disturbances in plantar flexion, dorsiflexion, and push-off. Plantar
flexion on weight acceptance is generally abnormal in patients with equinus contractures.
Dorsiflexion during midstance is diminished in the presence of a tight Achilles tendon, and push-off
is reduced if the ankle is already plantar-flexed from the equinus. Swing-phase dorsiflexion may be
absent as a result of weakness or inactivity of the tibialis anterior and lead to footdrop (Fig. 31.9).

Gait analysis is particularly useful in assessing the cause of toe-walking. It is tempting to attribute
all toe-walking to tight Achilles tendons; however, some children walk on their toes in response to
crouch above the ankle and have neutral ankle dorsiflexion but increased flexion at the knee and
hip. Lengthening of the gastrocnemius or Achilles tendon in these children would result in a
calcaneus gait, with persistent crouch at the hip and knee but excessive dorsiflexion at the ankle
leading to inefficient push-off.

Electromyography.

Spasticity leads to electrical overactivity on electromyography (EMG) during gait, and it seems that
the more spastic the child, the greater the EMG signal and the less phasic the muscles in their
contraction (Fig. 31.10). Dynamic EMG data collected during gait analysis can be correlated with
kinematic and kinetic graphs to gain a fuller understanding of the biomechanics of the child’s gait.
For example, normally at initial contact, the ankle plantar-flexes while the anterior tibialis fires on
the EMG. Kinetic plots show absorption of power as weight acceptance occurs. In stance phase a
child with CP may exhibit early heel rise, seen as plantar flexion on kinematic plots, which
correlates with gastrocsoleus overactivity on EMG. We find data from EMG most useful when
evaluating a child with a stiff knee in the swing phase for rectus femoris transfer and when
assessinga child with an equinovarus foot for tendon transfer (either anterior tibialis or posterior
tibialis split transfer).
Kinetics. Kinetics are the forces exerted across joints during gait. Each joint has well-described
kinetic patterns, and the reader is referred to work by Gage for descriptions. Two particular forces
are clinically interesting: hip pull-off power and ankle push-off power. Hip pull-off power, the force
exerted by the iliopsoas and other hip flexors to lift the stance-phase limb off the ground and into
the swing phase, is often diminished in patients with CP and leads to decreased energy efficiency
(Fig. 31.11). Ankle push-off power, the force exerted by the gastrocsoleus at terminal stance to
push the stance-phase limb off the ground, is diminished in patients with equinus or calcaneus gait
(Fig. 31.12).

Oxygen Consumption. The goal of orthopaedic intervention is to improve the quality and efficiency
of walking. By comparing oxygen cost and consumption with normal values through collection of
preoperative and postoperative data, the efficiency of the child’s gait can be measured objectively.
Heart rate is an indirect measure of oxygen consumption and can easily be measured in the clinic.

Children with CP have been found to have six times higher heart rates when walking than able-
bodied peers do, with the highest heart rates seen in children with crouch gait.

Controversies in Gait Analysis. Critics of gait analysis in patients with CP have pointed out that
patients fitted with markers and electrodes and placed in front of video cameras do not walk as
they do at home or school. Considerable variability in how patients walk from clinic visit to clinic
visit has been noted. Basing treatment plans on kinematic and kinetic data from a few strides
across a gait laboratory may not always be in the patient’s best interest. Gait analysis can be an
adjunct to clinical examination, but the data should be scrutinized and the videotape reviewed to
judge whether the data are representative of the gait seen in the clinic or described by the parents
in the home.

Very active gait analysis laboratories note that their studies change the surgical plan in patients
with CP in up to 52% to 70% of instances. In many cases, gait analysis reduces the number of
individual procedures needed in a single-event multilevel surgery (SEMLS) approach. The decision-
making process with the use of gait analysis data is only as good as the astuteness and familiarity
of the orthopaedic surgeon reading the graphs.

The Gillette Gait Index (GGI) is a numerical calculation that represents how different the kinematic
data are from age-matched normal values. Improvement in the GGI following SEMLS has been
documented in studies.

Summary. Gait analysis in our center is used to

1. Clearly document the three-dimensional movement of the lower extremity during gait
preoperatively

2. Document changes in gait over time as the patient grows, because gait may deteriorate in
children with CP

3. Allow preoperative and postoperative comparisons of results after tendon and/or bone surgery
and to gather research data

4. Analyze the rotational profile of the patient before surgery to help the surgeon select the
correct site and amount of rotational change desired
5. Confirm a surgical plan when needed.

Gait analysis does not tell surgeons whether they should operate, but it may help them fine-tune
the operative plan when questions exist. Additionally, gait analysis provides an objective
assessment of changes seen after SEMLS, including alterations in joint position, dynamic range of
motion, and time and distance parameters.

Muscle Strength

It has long been known that muscles in children with CP are typically spastic and that motor
control of the muscles (i.e., the child’s ability to volitionally contract and relax the muscle) is
impaired. Reports have documented that muscle weakness is also a problem in children with CP.
Documented muscle weakness is more pronounced as the CP GMFCS level worsens.

Prognosis for Ambulation

Many authors have proposed criteria for predicting the ultimate ability of a child with CP to walk.
Inability to sit by 2 years of age, persistence of two or more infantile reflexes beyond 12 to 15
months, and lack of head control by 20 months imply a poor prognosis for ambulation. The
presence or absence of mental retardation does not influence the ability to walk.

The geographic type of CP that a child has influences whether the child will walk. All children with
spastic hemiplegia develop the ability to ambulate. Eighty-six percent to 91% of children with
spastic diplegia become ambulatory and 0% to 72% of patients with spastic quadriplegia learn to
walk.

The discrepant figures are due to variation in differentiation between spastic diplegia and
quadriplegia among studies.

The age at which children with CP begin to walk varies with the severity of their neurologic
disease. Patients with spastic hemiplegia generally walk between the ages of 18 and 21 months.
Children with spastic diplegia who walk usually do so by 4 years of age. Many agree that the ability
to walk plateaus by 7 years of age, thus implying that if a child is nonambulatory at 7 years, the
child will probably never become ambulatory.

Treatment

Once the diagnosis of CP has been made, the physician must select a course of treatment.
Treatment choices are now more numerous than ever and include observation, physical therapy,
botulinum toxin, intrathecal administration of baclofen, rhizotomy, and orthopaedic surgery. With
the increasing popularity of nonorthopaedic management of spasticity, some centers have noted
an overall decrease in orthopaedic surgical procedures in this patient group. Treatment, whether
surgical or nonsurgical, must be goal oriented. The goals of treatment of children with CP that
have been linked to productive lives as adults are communication, education, mobility, and
ambulation.

Note that walking ranks below mobility. Although ambulation remains a desirable goal, it should
not be pursued so fervently that attention to overall development of the child is ignored.
Bleck quoted Rang as advising orthopaedists to remember that “the child with cerebral palsy
becomes the adult with cerebral palsy.” Childhood is the optimal time for intervention to
maximize the function of a patient with CP. It is the orthopaedists’ duty to ensure that the
musculoskeletal treatment of the child prevents future problems with pain and deformity as an
adult. Patients with CP do not usually have severely shortened life spans. In a study of all children
with CP born between 1966 and 1984, the 20-year survival rate was 89%. If the patients were
ambulatory, had manual dexterity, and were not mentally retarded, the 20-year survival rate was
higher than 99%. Survival is clearly linked with the patient’s GMFCS level. In a study from the
Swedish database, all GMFCS level 1 and 2 children survived to 19 years of age, whereas only 60%
of GMFCS level 5 children were still alive. Patients with gastrostomy tubes were most likely to
succumb to early death, which is indicative of their medical fragility. However, survival of these
frail patients has increased over the time period from 1983 to 2010. The overall 30-year survival
rate is estimated to be 87%.

Nonsurgical Treatment

Physical Therapy

Frequently, the first treatment rendered to a child with CP is physical therapy. Yet no controlled
studies have confirmed that regular physical therapy improves the outcome of a child with CP.
One of the first well-designed studies investigating the effect of physical therapy was performed
by Wright and Nicholson in 1973. They found no difference in motor skills or reflexes after 12
months in children who had neurodevelopmental training or physical therapy and those who did
not. Other studies followed and again showed no discernible improvement after different forms of
therapy. In defense of physical therapy, these studies are difficult to carry out because they
involve different age groups and children with varying severity of neurologic impairment and
usually encompass just a brief period of therapy. However, as Bleck pointed out, “The burden of
proof is on the proponents of the treatment. Critics need not prove inefectiveness but can insist
on positive data.” The efficacy of physical therapy can be proved or disproved only with a properly
designed, collaborative, multicenter, randomized, controlled trial.

Physical therapy, ranging from the medical model, with the aims of attaining ambulation, range of
motion, or transfers, to neurodevelopmental training, sensory integration, and even craniosacral
therapy, has been proposed. Electrical stimulation of the muscles has also been used in these
patients. Families like physical therapy and attribute gains in their young child’s ability to
interaction with the therapist. However, some of these gains are simply the result of neurologic
maturation of the child.

Our approach to physical therapy is to establish therapy for monitoring the developmental
milestones of very young children, around the age of 2 or 3 years. Therapy is continued if gains are
being made in attaining ambulation. School-based programs are used in elementary school and
often include adaptive physical education. Postoperative intensive physical therapy is essential to
reestablish range of motion and strength after surgical intervention. Strength training of weak
muscles has been successful in improving motor function. We also draw on the expertise of
physical therapists in assessing orthotic needs and wheelchair seating when appropriate. No
evidence supports the continued use of physical therapy for range of motion, particularly in a
nonambulatory child. Physical therapists play an important consultative role in making treatment
decisions for patients whom they treat on a regular basis and whom we examine on a relatively
infrequent basis.

It is common for parents of children with CP to resist discontinuing physical therapy. We believe
that goals must be set for therapy and, if progress toward these goals is not being made, either the
goals need to be reassessed or therapy should be stopped because it is not useful. Setting
measurable functional goals has led to greater success after physical therapy.

In an older child, transitioning from physical therapy to therapeutic recreation is desirable and
generally met with enthusiasm by the patient. Adaptive sports or swimming allows the child to
participate with peers and affords greater enjoyment than exercises in the therapy gym do. Time
in school should be spent on education at this age and not on physical therapy.

Casting

Inhibitive casting has waxed and waned in popularity as a mode of treatment of spasticity in
children with CP. It is based on the presence of areas on the feet that are target centers for
increased tone at the ankle and, some believe, throughout the lower extremities in certain
patients. Usually, short-leg casts are applied with extended toe plates, careful molding of the heel,
and metatarsal head control. This has been used by physical therapists and by some physicians.
The time spent in casts varies but is geneally a minimum of 6 weeks and is followed by the use of

orthoses.

In our experience, casting has a limited role in patients with CP. We have used casting in rare cases
of very young children (<4 years) with equinus contractures to delay surgical heel cord
lengthening. Because equinus recurs after casting, permanent resolution of the equinus is not a
goal. Some studies show short-lived improvements in passive and dynamic ankle range of motion
following casting, whereas others have reported longer resolution of equinus contractures in
children younger than 6 years.

Orthoses

Orthoses can be helpful in improving gait in ambulatory patients with CP. Bracing is best
prescribed when specific treatment goals for gait improvement are identified. Ankle-foot orthoses
(AFOs) are helpful in positioning the ankle and foot during gait. In young children, dynamic equinus
can be improved, with a plantigrade foot position obtained and the tendency for genu recurvatum
decreased.

AFOs come in various designs (Fig. 31.13). Solid ankle AFOs are used when minimal dorsiflexion of
the ankle is possible. Of the various types, they afford the easiest fit. Solid ankle AFOs also can be
used in patients with excessive dorsiflexion and knee flexion; by stabilizing the ankle, extension of
the knee is encouraged. Ground reaction AFOs have an anterior shell of plastic across the proximal
end of the tibia and are rear entry. The goal of a ground reaction AFO is to provide push-back on
the knee during stance phase and help the knee extend. Patients who walk in mild crouch with
knee flexion contractures of less than 10 degrees may benefit from this design. Hinged AFOs
permit a more natural gait by allowing some dorsiflexion during stance but blocking plantar flexion
and therefore eliminating equinus and footdrop.
Greater power generation in preswing is seen with hinged AFOs than with solid ankle designs484;
however, hinged AFOs are bulkier and break more frequently, and they offer no benefit if the
patient does not have passive dorsiflexion of the ankle. If crouch gait is present, a hinged AFO will
worsen knee flexion, thereby leading to ankle calcaneus and a less efficient gait. Posterior leaf
spring AFOs are fabricated from more pliable polypropylene and have more severe posterior trim
lines at the ankle. They are touted as providing a little push-off at terminal stance. They tend to
break and may not provide enough hindfoot control in most patients with CP. Gait analysis has
shown that posterior leaf spring AFOs function similar to hinged AFOs; that is, they allow some
dorsiflexion in stance phase through deformation of the brace, and they control equinus in the
swing phase but do not return energy at push-off.

Tone-reducing (or dynamic) AFOs incorporate a molded footplate, termed an inhibitive footplate,
and have higher trim lines that extend across the dorsum of the foot. The goal of these orthoses is
to apply pressure similar to that of an inhibitive cast and therefore reduce tone throughout the
lower extremities. Little scientific evidence supports this design inasmuch as no significant
functional changes in gait have been noted with the use of tone-reducing as opposed to standard
AFOs. Nonetheless, they remain popular with many families.

Bracing above the knee with knee-ankle-foot orthoses (KAFOs) or hip-knee-ankle-foot orthoses is
not generally done in those with CP. The weight and bulk of the braces usually interfere with
rather than improve the child’s ability to walk. Short-term use of KAFOs or knee immobilizers after
soft tissue surgery for crouch gait can be helpful in retraining children to walk.

Our indications for bracing are

1. To obtain a plantigrade foot position and reduce genu recurvatum in patients with dynamic
equinus

2. To support the foot in dorsiflexion during the swing phase when footdrop is present

3. To assist the foot postoperatively while weakness is being treated by physical therapy

4. To improve mild crouch

We do not advocate the use of AFOs in nonambulatory patients who are able to wear shoes, but e
do on occasion prescribe them when footwear is difficult and is improved with orthoses. We also
do not use AFOs in a preambulatory young child because they interfere with the child’s ability to
crawl and move about the floor.

Some patients walk worse with AFOs than without them. In patients with significant crouch gait,
flexion of the knee and hip with the foot supported in an AFO forces the child to either pull up out
of the braces or toe-walk despite the orthosis (Fig. 31.14). Toe-walking has not ever been found to
be harmful, so it is often better to allow the child to walk on toes brace free or to proceed with
surgery when appropriate. Valgus deformities of the hindfeet are often difficult to brace because
braces can produce painful calluses and blisters over the prominence of the talar heads.
Comfortable footwear without orthoses is preferable for these feet. Finally, as young children with
CP become adolescents, they often refuse to wear orthoses for cosmetic reasons. Allowing the
patient to make a personal choice in whether to wear a brace is particularly important in this age
group.

Medical Treatment of Spasticity

Oral Medication

Oral pharmacotherapy, including diazepam and baclofen, has been used in an attempt to reduce
tone in patients with CP. Side effects include somnolence. Oral tizanidine has been used to relieve
spasticity with improved results.

Patients with movement disorders such as dystonia or choreiform movements have been treated
by various medications, with varying success.

Intrathecal Baclofen. The inability to adequately reduce tone

with oral baclofen because of the side effects with large dosages

led to trials of intrathecal baclofen. When baclofen is injected

into the intrathecal space, small doses can effectively reach the

target neural tissue of the spinal cord and produce a greater

reduction in tone.408 Baclofen, a γ-aminobutyric acid agonist,

acts at the spinal cord level to impede release of the excitatory

neurotransmitters that cause spasticity.24 After proof of efficacy

with test injections of the drug, implantable pumps filled with

baclofen are surgically inserted into the anterior abdominal wall

and the dose of medication is titrated. Continuous infusion of

the medication is delivered by the pump, which requires refilling

approximately every 3 months. Studies have shown decreased

upper and lower extremity tone and improvements in range of

motion with continuous intrathecal baclofen infusion.37

Complications occur in approximately 20% of patients.

The complications are usually related to catheter dislodgment or fracture, but serious infection
occurs in 5%.24,52

Overdose of intrathecal baclofen is very rare but serious

and can produce somnolence and hypotonia, which leads to

respiratory depression requiring mechanical ventilation.538

Currently, no strict indications for the use of intrathecal baclofen have been determined, but most
pumps are

implanted in patients with severe spasticity that interferes with positioning and function of the
upper and lower

extremities. Pumps are currently being implanted in both

nonambulatory and ambulatory patients. Spasticity, as

graded by the Ashworth scale, has been shown to decrease

1 year following implantation of intrathecal baclofen pumps

regardless of preoperative severity.415 Caregiver satisfaction

in the nonambulatory group is high, with parents noting

easier positioning, transfers, and personal care, as well as

improved pain control.230,413 Pain relief and improved sleep

are typically seen within 6 months of pump implantation.487

Many of these patients still need orthopaedic surgery for hip

subluxation and should be monitored for the development

of such.580 Although progressive hip subluxation can occur,

significant deterioration in migration percentage is uncommon.334 Close monitoring for scoliosis

is also warranted

because rapid progression of deformity has been described

in some studies but refuted in others.586,653 Several studies

comparing progression of scoliosis in patients who had intrathecal baclofen pumps implanted
versus a well-matched control group of children with severe CP who were not treated

with baclofen found no statistical difference in progression of

scoliosis in the treated group. Both groups of patients experienced curve progression, which
supports the premise that

it is the severity of neurologic involvement and functional

disability that predisposes the patient to scoliosis and not

the medical management of spasticity (Fig. 31.15) Additionally, the presence of an intrathecal
baclofen pump

has not been linked with a higher complication rate following

scoliosis surgery.676
Botulinum Toxin. Botulinum toxin injections have become

a popular form of treatment in patients with CP. The toxin is

produced by the anaerobic bacterium Clostridium botulinum

and works by blocking the release of acetylcholine at the neuromuscular junction. It is injected at
known anatomic sites

of innervation,646 often guided by EMG or ultrasound. The

targeted muscle becomes weak because of lack of innervation until the neuromuscular junction
theoretically sprouts

new endings.99 Muscle histopathology studies following serial

botulinum toxin injections has shown worrisome muscle fiber

neurogenic atrophy in a recent study, bringing into question

the concept of reversability of the effects of the medication.645 Botulinum toxin is available in two
forms, Botox and

Dysport, which are not pharmacologically equivalent.517

Muscles that are considered to be candidates for injection

are those that produce dynamic deformities in the absence

of fixed contracture. For example, a child who walks in

ankle equinus because of spasticity in the gastrocsoleus but

who exhibits dorsiflexion on passive range of motion may

be a candidate for injection into this muscle.426 The drug

begins taking effect after 2 to 3 days, and its effect wears

off after approximately 3 months. The amount of botulinum toxin that can be injected at any one
setting has an

upper limit, so it works best in children in whom only one

or two muscles are to be injected. It offers less benefit to

a child with multiple muscle involvement. From a technical standpoint, superficial large muscles
are the easiest to

inject. Deep muscles, such as the iliopsoas, can be injected

under imaging for accurate localization.658,662

Most research that has been published in the orthopaedic literature on the use of botulinum toxin
for CP
has involved gastrocsoleus injection. Two randomized,

double-blind, placebo-controlled trials found improved

initial foot contact and gross motor function in botulinum toxin–treated patients.332,644 Gait
studies have found

improvements in sagittal-plane stance-phase dorsiflexion—in other words, less equinus—after

botulinum toxin

injection.192,613 Cosgrove, one of the first proponents of

this treatment, notes that patients who are younger, who

have diplegia rather than hemiplegia, and who have greater

dynamic shortening of the muscle with less contracture

have the best response. He and others proposed that botulinum toxin may allow tendon surgery
to be delayed until

a later age, when the risk for recurrence is lower.126,331

Annual injections have been found equally efficacious as

injections every 4 months.259

Botulinum toxin injections have been compared with

serial casting for the nonsurgical treatment of equinus. In

one study, the results were similar between children treated

with serial casting and those who underwent botulinum

toxin injection.123 Conflicting results were seen in another

report, which found Botox alone to be less effective than

either casting or combined Botox and casting for ankle equinus.13 Yet another study reported that
casting plus Botox

provided improved passive dorsiflexion and motor control

when compared with Botox alone.261 Finally, a meta-analysis

of all randomized controlled studies found no benefit of

Botox and casting over either casting or Botox alone.531

Botox injections into other muscles have been studied.94,126 Hamstring injections may decrease
crouch at the

knee. Injection of the adductors is reported to lessen scissoring and significantly increase hip
abduction.373 However,
adductor injections to decrease hip subluxation is ineffective. A randomized controlled study in
Australia compared

young children with CP treated with botulinum toxin injections into the adductors and hamstrings
every 6 months and

daily use of an abduction brace with a similar group of young

children who were observed without intervention. Both

groups experienced progressive subluxation, although the

treated group had a minimal decrease in the rate of progression. The authors concluded that the
program of botulinum

toxin injection and bracing did not significantly influence

the outcome of the hip.235

Frequently, parents adopt the philosophy that no harm

will be done with injection and surgery can be performed

later if necessary. One study found that only 10% of children

treated with Botox (and guided by gait analysis) underwent

surgery by 7 years of age as opposed to 52% of 7-year-olds

who did not receive injections.406 Although the use of Botox

may delay the need for operative treatment, it has not been

shown to decrease the need for tendon surgery in the long

run. The results wear off, and over time repeated injections

become undesirable.

Indications for botulinum toxin injection are

1. A child with a dynamic equinus deformity and no fixed

plantar flexion contracture

2. A child with equinus gait without multilevel crouch

3. A child younger than 4 years who cannot tolerate AFOs

because of dynamic equinus

4. Parents’ desire for injections and refusal of tendonlengthening surgery

Rarely, we recommend botulinum toxin injection before

surgery to investigate possible response to a planned surgical

intervention.

Botox has been used in a few hospitals to reduce muscle spasm after orthopaedic surgery.
Although improved

pain relief and reduced medication requirement have been

reported in a small series, widespread use of Botox in the

perioperative period has not yet been adopted.49 Similarly,

Botulinum toxin has been used in nonambulant children to

improve ease of care and comfort. A randomized controlled

study comparing injection of botulinum toxin in nonambulators to a sham procedure showed

positive results in the

treated group.121

Some reports of adverse events linked to botulinum toxin

injection have led to warnings from regulatory agencies that

have tempered the enthusiasm for Botox treatment.22,30 Sixteen deaths from dysphagia and
respiratory compromise led

to issuing the warning. A follow-up study of 334 patients

who received 596 Botox treatments found a 23% incidence

of temporary adverse events, which included generalized

weakness in one child, worsening of dysphagia, and respiratory infections; however, no deaths
occurred in this group.441

A large Australian series of 1147 children treated with botulinum toxin injections found a 1.3%
incidence of hospital

admission for respiratory compromise and linked the adverse

reactions to higher GMFCS levels.425 Because many severely

involved patients have multiple comorbid conditions as a

result of the severity of their medical condition, it is difficult

to definitively establish whether the complications described

are due to the injection of botulinum toxin or are inherent

because of the patients’ underlying fragile health.

In conclusion, the botulinum toxin injection is of use in

the treatment of lower extremity spasticity. Recommended

dose guidelines have been published.363 Complications are

rare but serious and are seen most frequently in the GMFCS

5 population.

Surgical Treatment

General Considerations

When evaluating a child with CP for surgical intervention,

a few general principles must be kept in mind. Speaking

frankly with the family about the goals of surgery and the

expected postoperative course is important. Frequently, the

family will have unrealistic expectations and believe that

surgery will “cure” the child and help him walk normally.

It must be explained that CP is a brain disease and that

orthopaedic surgery for CP will make the child walk differently, hopefully better, but will not make
the child walk

“normally.”

Timing of Surgery

Experts recommend combining multiple tendon surgeries

and osteotomies into a single surgical event (SEMLS).163

Rang advised avoiding “birthday surgery,” or hospitalizing

the child every year for yet another soft tissue surgery or

osteotomy.488 Aside from avoiding repeated hospitalizations as one joint is released at a time,
contractures present at one joint affect the position and movement of the

rest of the extremity, so correcting all concomitant contractures and bony malalignment
simultaneously during

one surgery is important to avoid recurrence or overcorrection of deformity. Because gait changes
and matures until

approximately 7 years of age, when feasible it is wise to

avoid surgery until this time. At this age, multiple levels

can be treated simultaneously to optimize the ambulatory

skills of the patient, followed by a single aggressive course

of physical therapy to maximize the benefits from surgery.

This surgical approach has been termed “single-event multilevel surgery.” The literature supports
the fact that wellexecuted surgery addressing all contractures can result in

stabilization of patients’ GMFCS level, thereby preserving

their function or in some cases resulting in an improved

GMFCS level at follow-up (i.e., improving their gait closer

toward normal).227

In some patients, surgery cannot be delayed until the

age of 7 years. Young children with hip subluxation should

undergo surgery when the problem is first recognized to

improve coverage of the hip. At younger ages, hip surgery

is often less extensive and may consist of only soft tissue

release, whereas in older children, additional osteotomies of

the femur and pelvis are required.

For other children who are nearly ambulatory but whose

progress has been halted by contractures in the lower

extremities, earlier surgery may allow them additional

range of motion to make walking less cumbersome. Adductor release for scissoring and
gastrocsoleus surgery with or

without hamstring lengthening in these younger children

may be indicated. Parents should be forewarned about

recurrent contractures requiring additional surgery in the

future.

Studies have found that the natural history of gait

disturbances in patients with CP is gradual deterioration

beginning at 12 years of age.318 Decreased ankle, knee,

and hip sagittal-plane motion, decreased cadence, and

slower walking speed have been documented over time in

patients who did not undergo intervening surgical procedures.57 It is common for crouch,
contractures, and outtoeing to worsen in the early teen years.501 Surgery may
offer some benefit, but realistic goals must be discussed.

Surgery to maintain ambulation can be successful in many

children who are GMFCS 3; however, those patients who

are GMFCS 4 (therefore nonambulatory) prior to surgery are unlikely to achieve maintenance or
restoration of

mobility, including even standing transfers, with extensive

surgery.72

A word of warning regarding tone and its effect on postoperative results is warranted. Best results
following SEMLS

surgery are seen in patients with pure spasticity. Those

patients with dystonia, although preoperatively resembling

their spastic counterparts, may experience unpredictable

results such as early recurrence or worsening of gait in a

subset of cases.71

Anesthetic Concerns

Patients with CP experience latex allergy with increased

frequency, and for those with suspected latex allergy, a

latex-free surgical environment is necessary. Allergy testing is available for latex products. At-risk
children are those

who have undergone multiple previous operations or who

have indwelling latex devices such as gastrostomy tubes or

ventriculoperitoneal shunts.160,181

Antiseizure medications may produce alterations in clotting. Increases in bleeding times and
decreased platelet

counts are known side effects of some antiepileptic agents.

Preoperative evaluation of clotting parameters is recommended for major operations such as hip
reconstruction

or spinal fusion. A routine clotting profile consisting of a

prothrombin time and partial thromboplastin time will miss

bleeding time abnormalities. Platelet function assays can

help identify these bleeding disorders.

Postoperative pain control is difficult in patients undergoing hip reconstruction, spinal surgery, and
multilevel

tendon surgery. We use short-term inpatient continuous

epidural infusions at our hospital and find that if the pain is

well controlled, problems with muscle spasm are lessened.

Oral medication often needs to include both pain control

medications and muscle relaxants such as diazepam.

Postoperative Management

Weakness is a frequent short-term sequela of lower extremity surgery in those with CP. Persistent
diminished muscle

strength has been documented 1 year following surgery.569

Plans should be made for frequent postoperative physical

therapy beginning shortly after surgery with attention given to

muscle strengthening. If at all possible, the patient should be

kept ambulatory or weight bearing after the operation. Prolonged time in a wheelchair adds to the
overall weakness of

the limbs. Patients with osteotomies are the exception to this

rule. In these children, weakness may be even more of a problem once casts are discontinued. A
few centers have exhibited

new enthusiasm for minimally invasive surgery to correct contractures in children with CP.
Percutaneous releases with safe

intramedullary fixation of rotational osteotomies may result in

less loss of strength in the postoperative period.632 However,

because it is imperative that safety be ensured, percutaneous

releases are not applicable in all situations.

A trend toward less immobilization after soft tissue surgery has emerged. In our practice, short-leg
casts are still

used after Achilles tendon lengthening, but knee immobilizers rather than casts are becoming
more frequently used after

hamstring lengthening, and removable abduction bars are

preferable to Petrie casts after adductor release.309 The goal

of soft tissue surgery is greater range of motion, so avoiding

overimmobilization and secondary stiffness is logical.