Cerebral Palsy :

Or thop e dic A sp e c ts
and Rehabilitation
A. Nadire Berker, MDa,*, M. SelimYal|n, MDb

KEYWORDS
 Cerebral palsy  Rehabilitation  Orthopedics

Cerebral palsy (CP) is the most common chronic disability of childhood today. It is
ubiquitous, and it occurs all around the world. As a result of injury to the brain, children
have motor defects that may be progressive and may affect them for their entire
lifetime. In addition to movement and balance disorders, patients experience other
manifestations of cerebral dysfunction. Abnormal muscle tone, disturbance of balance
mechanisms, muscle weakness, and loss of selective motor control lead to an inability
to stretch muscles, contractures, and deformities. Treatment often starts when they
are infants and continues throughout their life, even into adulthood. Without proper
management, these children cannot become productive members of society.

DEFINITION

CP is a disorder of movement and posture that appears during infancy or early child-
hood. It is caused by nonprogressive damage to the brain before, during, or shortly
after birth. CP is not a single disease but a name given to a wide variety of static neuro-
motor impairment syndromes occurring secondary to a lesion in the developing brain.
The damage to the brain is permanent and cannot be cured, but the consequences
can be minimized. Progressive musculoskeletal pathologic findings occur in most
affected children.1,2

EPIDEMIOLOGY

CP is the most common cause of childhood disability in Western societies. The

incidence is 2 to 2.5 per 1000 live births.3

a
Department of Physical Medicine and Rehabilitation, American Hospital, Anka Med Bagdad
Cad. 333/8 Erenkoy, 34728 Istanbul, Turkey
b
Department of Orthopaedics and Traumatology, Marmara University School of Medicine,
Bagdad Cad. 333/8 Erenkoy, Istanbul, Turkey
* Corresponding author.
E-mail address: nadireftr@yahoo.com (A.N. Berker).

Pediatr Clin N Am 55 (2008) 1209–1225

doi:10.1016/j.pcl.2008.07.011 pediatric.theclinics.com
0031-3955/08/$ – see front matter ª 2008 Elsevier Inc. All rights reserved.
1210 Berker & Yalcın

ETIOLOGY

Any nonprogressive central nervous system (CNS) injury occurring during the prenatal,
perinatal, or postnatal period during the first 2 years of life is considered to be CP. The
exact etiology can be identified only in 40% to 50% of the cases.4 Certain factors
increase the risk for neurologic injury. Prematurity and low birth weight are the two
most important risk factors for CP universally.5 A clear association exists between
premature delivery and spastic diplegia. Rubella, herpes simplex, toxoplasma, and
cytomegaloviruses cross the placenta to infect the fetus and have severe effects on
the developing CNS. Eclampsia or another severe maternal illness and hypothermia
or hypoglycemia of the neonate cause a reduction in the levels of oxygen and nutrients
available to the fetus or an increase in the levels of toxins or waste products, adversely
affecting the developing CNS. Multiple pregnancies or breech presentation also can
increase the risk. An excess of bilirubin resulting from hemolytic disease of the
newborn is clearly associated with CP.6,7

PATHOLOGIC FINDINGS

Specific brain lesions related to CP occur in regions that are particularly sensitive to
disturbances in blood supply and are grouped under the term hypoxic ischemic
encephalopathy.8

MANIFESTATIONS

CP is essentially a movement and balance disorder. The child has abnormalities of

muscle tone and reflexes, is delayed in achieving developmental milestones, and
cannot control his or her balance or movements. There is muscle weakness, spastic-
ity, loss of coordination, and persistence of primitive reflexes interfering with the
development of gross and fine motor control. Moreover, advanced postural reactions
for balance and equilibrium that are a prerequisite for sitting and walking are delayed
or nonexistent. Apraxia, defined as the inability to plan and execute motor function, is
present. Even though superficial sensation is generally normal, cortical sensation,
proprioception, and sensation of movement may be impaired.9 In addition, patients
experience other manifestations of cerebral dysfunction (Table 1).

Table 1
Manifestations of cerebral palsy

Neurologic Associated Problems

Muscle weaknesss Intellectual impairment
Abnormal muscle tone Epilepsy
Balance problems Visual problems
Loss of selective control Hearing loss
Pathologic reflexes Speech and communication
Loss of sensation problems
Swallowing difficulty
Feeding difficulty, failure to thrive
Respiratory problems
Incontinence
Musculoskeletal —
Contractures deformities —
 Cerebral Palsy 1211

The Movement Problem

The motor impairment in CP is classified as primary, secondary, or tertiary. The
primary impairments are muscle tone, balance, strength, and selectivity problems
that are directly related to damage in the CNS. Secondary impairments are muscle
contractures and deformities that develop over time in response to the primary prob-
lems and musculoskeletal growth. Abnormal muscle tone, disturbance of balance
mechanisms, muscle weakness, and loss of selective motor control lead to an inability
to stretch muscles, resulting in contractures. Contractures in the presence of muscle
weakness and spasticity result in abnormal skeletal forces that cause bone deformity
as the child grows older. The distal biarticular muscles are more affected, because
selective motor control is worse distally and the biarticular muscles are more abnormal
than are the monoarticular muscles. Tertiary impairments are adaptive mechanisms
and coping responses that the child develops to adapt to the primary and secondary
problems. One typical example is gastrocnemius spasticity as a primary impairment
leading to secondary ankle plantar flexion contracture and knee hyperextension in
stance as an adaptive mechanism.10,11
Early signs suggestive of CP in the infant are abnormal behavior, oromotor prob-
lems, and poor mobility. The infant is irritable, too docile, or difficult to handle. He or
she does not suck well, sleeps poorly, vomits frequently, and has poor eye contact.
Head control may be poor, with normal or increased tone in the limbs and persistent
or asymmetric fisting. Instead of crawling, the child moves by creeping or hopping like
a bunny. Hand preference during the first 2 years of life is a sign of hemiplegic CP.
The movement problem becomes apparent as the baby grows, and the typical
clinical picture is established toward the end of the first year of life. Primitive reflexes
persist, advanced postural reactions do not appear, and abnormal movement patterns
emerge. Delays in the achievement of developmental milestones correlate with the
severity of the problem. Babies who have CP usually have a period of hypotonicity
during the early months of life. Muscle tone gradually increases toward 18 months
in those who are going to develop spasticity. Fluctuation in tone from hypotonicity
to hypertonicity is a characteristic of developing dyskinetic CP. Athetosis is obvious
after 18 to 24 months. Ataxia may not be apparent until even later.12

CLASSIFICATION

Classification is based on the change in muscle tone, anatomic region of involvement,

and severity of the problem (Fig. 1).

Fig. 1. Classification of CP.

1212 Berker & Yalcın

Spastic Cerebral Palsy

Spasticity is defined as an increase in the physiologic resistance of muscle to passive
motion. It is part of the upper motor neuron syndrome characterized by hyperreflexia,
clonus, extensor plantar responses, and primitive reflexes. Spastic CP is the most
common form of CP. Approximately 70% to 80% of children who have CP are
spastic.13

Hemiplegia
There is involvement in one side of the body, with the upper extremity generally more
affected than the lower. Seizure disorders, visual field deficits, astereognosis, and
proprioceptive loss are common. Twenty percent of children who have spastic CP
have hemiplegia. In many cases, the cause is a focal traumatic, vascular, or infectious
lesion. A unilateral brain infarct with posthemorrhagic porencephaly is apparent on
MRI. All hemiplegic children walk independently by the age of 3 years. Prognosis
for independent living is good. Seizures, mild mental retardation, learning difficulties,
and behavioral disturbances may complicate the management and integration into
society.

Diplegia
The lower extremities are severely involved and the arms are mildly involved in diplegic
patients. Fifty percent of children who have spastic CP have diplegia. Diplegia is
becoming more common as more low-birth-weight babies survive. Intelligence is usu-
ally normal, and epilepsy is not common. These children often have visual perceptual
deficits and strabismus. MRI may reveal mild periventricular leukomalacia (PVL). Most
diplegic children need various treatments to be able to walk independently. Children
who can sit by the age of 2 years can usually walk by the age of 4 to 7 years. Hand
dexterity and fine motor control are impaired. Most diplegic children need surgery
for contractures and deformities, and many use walking aids.14

Quadriplegia (Total Body Involvement or Tetraplegia)

All four limbs; the trunk; and muscles that control the mouth, tongue, and pharynx are
affected. The term triplegia is used when one upper extremity is less involved. Thirty
percent of children who have spastic CP have quadriplegia. Cerebral dysfunction is
more extensive, and the prognosis is worse.
Quadriplegics have severe motor impairment and other signs and symptoms of
CNS dysfunction, such as cognitive impairments, seizures, and speech and swallow-
ing difficulties. Primitive reflexes persist lifelong, and extrapyramidal signs are
common. Mental retardation, seizures, visual deficits, strabismus, bulbar dysfunction
manifested by drooling, dysphagia, dysarthria, and medical complications are
frequent. Swallowing difficulty combined with gastroesophageal reflux causes feeding
problems and can result in aspiration pneumonia. Growth is retarded, and many
children do not have bladder and bowel control. The spectrum of severity is variable,
from having no sitting ability or head control to being able to walk independently. Only
approximately 15% of quadriplegic children have the potential to walk, and the rest
are wheelchair bound. Most of them require lifelong all-day care by the family.15

Dyskinetic Cerebral Palsy

Athetosis, dystonia, and chorea are the main movement disorders seen in dyskinetic
children. Dysarthria, dysphagia, and drooling accompany the movement problem.
Dyskinetic CP accounts for approximately 10% to 15% of all cases of CP. Mental
 Cerebral Palsy 1213

status is generally normal; however, severe dysarthria makes communication difficult.

Sensorineural hearing dysfunction also impairs communication. The most probable
etiology is hyperbilirubinemia or severe anoxia that causes basal ganglia dysfunction.
The ambulation potential of dyskinetic children depends on the severity of involve-
ment. Most children who have severe dystonia are unable to walk. A percentage of
children who have athetosis can become ambulatory; however, they have a clumsy
and unstable gait.

Ataxic Cerebral Palsy

Ataxic children cannot coordinate their movements or maintain balance. Ataxia
becomes apparent toward the age of 2 to 3 years. Ataxia is associated with cerebellar
lesions and is generally seen in association with spastic diplegic CP.

Mixed Cerebral Palsy

Many children who have CP have mixed involvement, in which ataxia and dystonia
occur together with spasticity.

PROGNOSIS

The clinical picture in CP ranges from extremely mild to extremely severe depending
on the extent of the CNS lesion. Walking is usually possible between 2 and 7 years of
age. Approximately 85% of partially involved children have the potential to become
independent ambulators compared with only 15% of severely involved children.16
Spastic hemiplegic and diplegic children with good cognitive function generally
become independent walkers and productive members of the community. Most spas-
tic hemiplegic children are able to become independent adults even without therapy.
Most diplegic children need treatment. Physiotherapy, bracing, and efficient spasticity
management result in a more efficient gait with less contracture formation, even
though 80% of affected children still need orthopedic surgery in childhood or adoles-
cence. Approximately 85% of total body–involved children remain fully dependent on
a caregiver. Management should include assistive devices, special housing arrange-
ments, and continuous care. Physiotherapy, bracing, and drug treatment do not result
in functional gains in athetoid or dystonic patients. Children who are mildly affected
use assistive devices and mobility aids to ambulate, and severely involved children
remain totally dependent.

MANAGEMENT TEAM

A large team of experts needs to work together to manage the many impairments
associated with the primary neurologic lesion and the accompanying motor disorder
seen in CP. The team consists of physicians; surgeons; and allied health profes-
sionals, such as physiotherapists, occupational therapists, and child development
professionals, who are aware of all the needs and limitations of the child and know
what all other members of the team are doing for the child.

Goal of Management
The ultimate goal is minimizing disability while promoting independence and full
participation in society. All treatment efforts are directed to gain independence in
activities of daily living, ability to go to school, earn a living, and a have a social life.
Everyone is a child only once. The child should live a childhood as close to normal
as possible and grow up to be a happy and healthy adult.
1214 Berker & Yalcın

The treatment strategy is based on a realistic evaluation of the child’s present

functional status and possible future prognosis. The Gross Motor Function Classifica-
tion System is useful to determine prognosis (Table 2).17

Management strategy and rehabilitation of the child who has cerebral palsy

1. Support growth and nutrition

2. Therapy for vision: ophthalmologic therapy
Rehabilitation
Glasses
Surgery
3. Dental hygiene
4. Gastrointestinal problems
Medications for reflux
Gastrostomy
Antireflux surgery
5. Therapy for motor function
Physical therapy
Occupational therapy
Adaptive seating
Bracing
Wheeled mobility
Orthopedic surgery
Sports-recreation

Table 2
The gross motor classification system

Level Ability
Level I Children walk indoors and outdoors and climb stairs without limitations.
Children perform gross motor skills, including running and jumping, but
speed, balance, and coordination are reduced.
Level II Children walk indoors and outdoors and climb stairs holding onto a railing
but experience limitations walking on uneven surfaces and inclines and
walking in crowds or confined spaces. Children have at best only minimal
ability to perform gross motor skills, such as running and jumping.
Level III Children walk indoors or outdoors on a level surface with an assistive
mobility device. Children may climb stairs holding onto a railing.
Depending on upper limb function, children propel a wheelchair manually
or are transported when traveling for long distances or outdoors on
uneven terrain.
Level IV Children may maintain levels of function achieved before the age of 6 years
or rely more on wheeled mobility at home, school, and in the community.
Children may achieve self-mobility using a power wheelchair.
Level V Physical impairments restrict voluntary control of movement and the ability
to maintain antigravity head and trunk postures.
All areas of motor function are limited. Functional limitations in sitting
and standing are not fully compensated for through the use of adaptive
equipment and assistive technology. At level V, children have no means
of independent mobility and are transported.
 Cerebral Palsy 1215

6. Oromotor therapy
Chewing
Swallowing
Speech
7. Seizure prevention
8. Spasticity and dyskinesia
Medical treatment
Botulinum toxin
Intrathecal baclofen
Selective dorsal rhizotomy (SDR)
Management between 0 and 2 years of age should include physiotherapy, infant
stimulation, positioning, and parent education. Ways to decrease muscle tone should
be encouraged during the age of 2 to 5 years when muscle tone becomes a problem
and dyskinesias manifest themselves. Orthopedic intervention may be necessary from
5 years onward. Hygiene, seating, and care issues, in addition to preventing pain and
discomfort secondary to spasticity, become predominant during the teenage years
(Table 3).

COMPONENTS OF REHABILITATION

Rehabilitation of the child who has CP consists of improving mobility, preventing

deformity, helping the child to learn the skills he or she needs in daily life, and educat-
ing the parents about the child’s problem. Rehabilitation should help to provide
schooling, sports, and recreation for the child. Methods used in CP rehabilitation
are physiotherapy, occupational therapy, bracing, assistive devices, adaptive technol-
ogy, and sports and recreation.
Physiotherapy aims to bring the child to an erect position, give the child indepen-
dent mobility, and prevent deformity (Table 4). It begins in early infancy and continues
throughout adolescence to facilitate normal neuromotor development. With the help of

Table 3
Timing of rehabilitative measures

Age Goal Methods

Infancy Supportive measures for Nutritional support
prolonging and optimizing Infant stimulation and
physical status and life positioning
Childhood Strategies to obtain maximum Medication to minimize
independent mobility spasticity
Exercise
Botulinum toxin
Bracing
Preschooler Maximum independent Minimize deformity
mobility Medication
Exercise
Botulinum toxin
Bracing
Orthopedics surgery
Adolescence Education, vocation, Schooling, sports, psychosocial
and integration into support
the community
1216 Berker & Yalcın

Table 4
Effects of physiotherapy

Physiotherapy Tries to Improve

Postural control Decreasing spasticity and contracture
Muscle strength Increasing muscle elasticity and joint laxity
Range of motion
Joint alignment Increasing coordination/agility
Motor control Balance
Muscular/cardiovascular endurance Transitions
and mobility skills Use of assistive devices

correct positioning, appropriate stimulation, and intensive exercise, the therapist tries
to gain head control, postural stability, and good mobility in the child. This is possible
only to the extent of the child’s neurologic capacity. Therapeutic exercises help the
child to learn how to sit, stand, and walk and how to use his or her upper extremity
for function. The child also learns how to use his or her remaining potential to compen-
sate for the movements he or she cannot perform. Decreasing spasticity, gaining
muscle strength, and improving joint alignment decrease deformity. The education
of caregivers involves gently coaching them to set reasonable expectations for their
child and teaching them to follow their child’s exercises at home. Parents should
encourage their children to participate in daily living activities by using the functional
skills they learned during therapy. Community and social support is another aspect
of rehabilitation.18
All therapy methods should support the development of cognitive, visual, sensory,
and musculoskeletal systems; involve play activities to ensure compliance; enhance
social integration; and involve the family. The basic element is having fun.
Occupational therapy aims to improve hand and upper extremity function in the
child through play and purposeful activity. Sports and physical and recreational activ-
ities play an important role in physical development, general fitness, and health of the
child who has CP. Physical and occupational therapy, combined with recreational
activities or adapted physical education, increase the efficiency of rehabilitation and
assist the disabled child to use his or her potential.19
Management should prevent disability by minimizing the effects of impairments,
preventing secondary disabilities, and maximizing motor function throughout the
child’s life. Functional goals change as the baby becomes a child and the child
matures into an adult, but the principles remain the same. Younger children focus
mainly on mobility, whereas adults put more emphasis on communication and activ-
ities of daily living. The first 4 years are spent in physiotherapy and bracing, orthopedic
surgical procedures are performed between 5 and 7 years of age, and education and
psychosocial integration become the main issues in adolescents. Mobilization goals
should be met by the time the child is ready to go to school. Aggressive physiotherapy
is needed in the growth spurt period and after orthopedic surgery.20

Bracing
Braces are devices that hold the extremities in a stable position. The goals of bracing
are to increase function, prevent deformity, keep the joint in the functional position,
stabilize the trunk and extremities, facilitate selective motor control, decrease spastic-
ity, and protect the extremity from injury in the postoperative phase. Indications differ
according to the age, selective motor control level, type of deformity, and functional
 Cerebral Palsy 1217

prognosis of the child.21 Various kinds of ankle-foot orthoses (AFOs) are the most
common braces used in CP (Fig. 2). Knee-immobilizing splints and hip abduction
splints are prescribed for nonambulatory and ambulatory children. Compliance with
night splints to prevent deformity is low. The indications of bracing in the upper
extremity are limited.

Assistive Devices
A child who has CP needs to move around, to explore his or her surroundings, and to
interact with his or her peers so that his or her mental, social, and psychologic skills
develop to the fullest. A variety of mobility aids and wheelchairs provide differing
degrees of mobility to these children. Transfer aids, such as lift systems, assist the
caregiver when performing transfers. Passive standing devices called standers allow
the child to get accustomed to standing erect and provide therapeutic standing. Some
ambulatory children use gait aids, such as walkers, crutches, and canes, in addition to
braces for efficient and safe ambulation. Gait aids mainly used to improve balance
also decrease energy expenditure, decrease the loads on the joints, and improve pos-
ture and pain. Nonambulatory children use wheelchairs for moving around. Wheel-
chairs must be properly fitted with seating aids, cushions, and other positioning
components.
Spasticity Management
Spasticity should be treated when it causes loss of function; produces contractures,
deformities, pressure sores, or pain; or causes difficulty in positioning or caring for
the total body–involved child. All treatment options aim to modulate the stretch reflex.
Basic measures, such as positioning, exercises, and bracing, may be combined with

Fig. 2. Child with AFO.

1218 Berker & Yalcın

oral medications or botulinum toxin injections to improve outcome. Orthopedic

surgery is an effective option in moderate to severe spasticity.

Oral management
Systemic oral antispastic drugs are useful for total body–involved children who have
severe spasticity, and for a short period after orthopedic surgery, but are not recom-
mended for ambulatory children because of side effects, such as drowsiness, seda-
tion, and generalized weakness.22

Botulinum toxin
Botulinum toxin inhibits acetylcholine release at the neuromuscular junction causing
reversible chemodenervation. The general indication for botulinum toxin injection in
CP is ‘‘the presence of a dynamic contracture, interfering with function, in the absence
of a fixed muscular contracture.’’23 If botulinum toxin injections are started at an early
age and repeated as necessary, they can help to prevent the development of muscle
contractures and bony deformities.23

Intrathecal baclofen
Intrathecal administration introduces baclofen directly into the cerebrospinal fluid
(CSF) through an implantable pump and catheter system, increasing efficacy and
minimizing side effects. Intrathecal baclofen administration is useful for the severely
involved spastic child, dystonic child, or child who has mixed disease to enable sitting
in the wheelchair, make transfers easier, decrease spinal deformity, and increase the
comfort level and ease of care.24

Selective dorsal rhizotomy

SDR involves sectioning of the dorsal column rootlets to interrupt the spinal reflex arc,
inhibiting afferent input from the muscle and tendons, and reducing efferent activity at
the level of the spinal cord. There is a permanent and global muscle tone reduction in
the lower extremities with loss of superficial and deep sensation. Hip instability and
spinal deformity are more common after the procedure.25,26

Corrective casting
Corrective casting is used for minor ankle equinus contracture that does not respond
to physical therapy or botulinum toxin injections and for knee flexion deformities that
involve more than just hamstring tightness. The compliance with serial casting is low
because of the difficulties of repeated casting and cast removals.18 Some researchers
propose that casting weakens the already weak spastic muscles, creates atrophy, and
does not allow the antagonist muscle to work.

ORTHOPEDIC SURGERY

Orthopedic surgery is widely used in the management of children who have CP to

prevent or correct certain musculoskeletal problems, such as muscle shortening
and bony deformities. The goal of orthopedic surgery in a child with walking potential
is to improve functional ambulation. For a good walking pattern in the ambulatory
child, the feet should be plantigrade and stable, the hips should extend well and be
stable, and the knees should have good extension. For nonambulatory children, the
goal of orthopedic surgery is to ease the burden of care through facilitating sitting,
improving hygiene, and preventing pain. Therefore, the spine should be straight, the
pelvis horizontal, and the hips stable.18
 Cerebral Palsy 1219

Indications
The main indication for orthopedic surgery is to correct contractures and deformities
of the spine and extremities that disturb sitting, standing, and walking. Orthopedic
surgery reduces muscle tone by lengthening the spastic muscles and decreasing
the sensitivity of the stretch reflex. Balance is decreased immediately after surgery
but improves in the long run because of the plantigrade stable feet providing a better
base of support. Muscles usually get weak, but they can be strengthened. Tendon
transfers change the direction of deforming forces that create muscle imbalance,
thus preventing deformity and allowing the child to use his or her muscle strength
more efficiently. Decreased spasticity and improved balance may indirectly improve
selective motor control; however, primitive reflexes do not change after surgery.27

Surgical Methods
Orthopedic surgical procedures used in CP are muscle releases and lengthenings,
split tendon transfers, osteotomies, and arthrodeses. Muscle-tendon lengthening
weakens spastic and shortened muscles and decreases the unopposed pull of spastic
muscles, thereby balancing the forces acting on the joint. Even severe contractures
can be treated effectively with muscle lengthening. Osteotomy corrects varus and
valgus deformities of the foot and flexion deformities in the lower extremity. Hip
osteotomy stabilizes the subluxated or dislocated hip, preventing pelvic obliquity
and pain, to improve sitting balance. Rotational osteotomies correct the torsional
deformities in the tibia or the femur and help to transfer the malaligned muscle force
into the correct plane of movement to make it easier for the child to walk. Arthrodesis
corrects deformity and stabilizes the joint. Spinal fusion and instrumentation correct
spinal deformity.
The correction of joint alignment makes walking easier, and the child may stop using
coping mechanisms and adaptive responses that he or she developed because of his
or her contractures and deformities.
Timing of surgery depends on CNS maturation, ambulation potential, and rate of
deformity development. The nervous system matures around the age of 4 to 6 years
when the physician can assess muscle imbalance more accurately; predict a func-
tional prognosis; and make sure that no other abnormalities, such as athetosis or dys-
tonia, are present. Therefore, soft tissue procedures are generally performed between
4 and 7 years of age, hand surgery between 6 and 12 years of age, and bony proce-
dures after 8 years of age although it is the needs of the child that determine the exact
timing. Exceptions are progressive hip instability and deformities and contractures
interfering with function.18,27
Orthopedic surgery is usually performed to correct pes equinus and pes varus in
hemiplegia and jump, scissoring, and crouch gait in diplegia. In the child with total
body involvement, spinal deformity and hip instability are treated with surgical
methods.
Certain patients benefit a lot from orthopedic surgery, whereas others may not ben-
efit at all. Spastic diplegic and hemiplegic children improve more compared with spas-
tic total body–involved, dyskinetic, and mixed types. Fewer operations are performed
and the gains are limited in dyskinetic cases. Results are best in children who have
a higher degree of selective motor control.
Lack of balance, cognitive deficits, and visual impairments are not by themselves
contraindications for orthopedic surgery and do not affect the surgical outcome unless
they are extremely severe. Surgical procedures requiring postoperative intensive
physiotherapy or long-term cast immobilization should be avoided in children who
1220 Berker & Yalcın

have severe cognitive deficits, however.28 Children who have sensory deficits are not
candidates for upper extremity surgery for function.

Postoperative Care
The focus in the immediate postoperative period is on analgesia and muscle relaxa-
tion. Usually, a combination of a narcotic analgesic and diazepam helps to control
the immediate pain. Early mobilization and early weight bearing in addition to strength-
ening of the trunk and upper extremities should be encouraged. Casts, splints, plastic
AFOs, or knee ankle foot orthosis (KAFO) are used depending on the age and coop-
eration of the child and surgical stability. Weight bearing is allowed on the second
to fourth day after soft tissue surgery, whereas it depends on the quality of internal fix-
ation of the bones in combined soft tissue and bony operations. Adequate nutrition
and skin care are necessary to prevent complications, such as pressure sores.
Range-of-motion and strength exercises begin as early as possible after surgery.
The lengthened muscles need to be strengthened with proper exercises. It usually
takes approximately 3 months to regain the preoperative muscle strength after multi-
level surgery.29 The child then begins to acquire new skills. Changes in function are not
extremely obvious for up to 1 year after the operation. Postoperative physiotherapy
should be combined with sports and purposeful play to increase the benefits of surgi-
cal intervention. The physician must also monitor for new dynamic or fixed contrac-
tures or orthotic problems, prescribe accurate braces, and provide guidance for
adaptive equipment.

Complications of Surgery
Common complications are pressure sores attributable to prolonged bed rest or cast,
sciatic nerve traction injury after surgery to relieve knee flexion contracture, and frac-
ture in the immobilized and osteoporotic children who are wheelchair-bound spastic
quadriplegics. Heel cord and hamstring tendon overlengthening can lead to pes
calcaneus and knee recurvatum, respectively.30

The ‘‘Birthday Syndrome’’

One group of complications related to a chain of operations over the years is social
isolation, loss of motivation, frustration, and psychosocial problems termed the birth-
day syndrome.31 Instead of performing one operation at a time over many years, all
necessary surgical interventions should be done at the same time in a single setting
if the child’s medical and social status permits. This spares the child the burden of
multiple consecutive surgical interventions throughout his or her life. Treatment plans
must be individualized for each child according to his or her specific needs.

Limitations
Surgery is not the single solution to the wide variety of problems caused by CP. It is
only a momentary pause in the long journey of CP management. The need for rehabil-
itation measures, such as bracing, physiotherapy, sports, and antispastic medication,
still remains after surgery.
Because family cooperation is essential for the success of treatment, realistic goals
should be set after a thorough evaluation of the expectations and limitations of the
family. The expected functional gains after surgery must be thoroughly and realistically
decided on, and surgery should not be attempted unless one is certain that the oper-
ation is going to create considerable functional gains not obtainable by other means.
 Cerebral Palsy 1221

PROBLEMS SPECIFIC TO THE TYPE OF CEREBRAL PALSY

Problems and Management in Hemiplegia
Musculoskeletal problems of the upper extremity are shoulder abduction, internal
rotation, elbow flexion and pronation, wrist and finger flexion, and thumb in palm.
Typical lower extremity problems are hip flexion, internal rotation, knee flexion or
extension, ankle plantar flexion and foot varus, although valgus may also be seen.
The hemiplegic side may be slightly atrophic and short.
Physiotherapy may prevent contractures of the involved side and strengthen the
weak muscles, thus enabling better use of the upper extremity and a more efficient
gait pattern.
Botulinum toxin injections reduce gastrocnemius-soleus and rectus femoris spas-
ticity in the young child. Early relief of spasticity may prevent shortening of the gastroc-
nemius muscle, help to establish a close-to-normal gait, and delay or eliminate the
need for surgical intervention.23 Injection to the upper extremity may minimize forearm
pronation and relax wrist, finger, and thumb flexors so that the child may gain forearm
supination and wrist stabilization. Hand splints in hemiplegia are used to prevent
deformity and to improve function. The child’s compliance with night splints is gener-
ally poor. Splints prevent sensory input in the already compromised hand. AFOs
stabilize the ankle and foot and keep it in the plantigrade position for weight bearing.
All fixed contractures must be corrected before applying braces.
The usual indications for orthopedic surgery in the hemiplegic child are pes equinus,
pes varus, and stiff knee. Thumb-in palm and wrist flexion deformity may also respond
to surgery. Soft tissue procedures are usually performed in children around 5 to 6 years
of age. Bone procedures are delayed until children are at least 8 years of age unless
the deformity is causing a functional problem.32 Upper extremity surgery for function
should be delayed until the child is mature enough to cooperate with rehabilitation.

Problems and Management in Diplegia

Problems in maintaining balance; muscle imbalance; and spasticity leading to
contractures and deformities of hips, knees, and ankles contribute to the specific pos-
ture and gait patterns typical for diplegic CP. The frontal plane pathologic condition is
scissoring, which occurs as a result of persistent femoral anteversion and hip adduc-
tor and medial hamstring spasticity. A scissoring gait may accompany sagittal plane
pathologic findings, such as jump or crouch knee gait. Jump gait, defined as exces-
sive hip flexion, knee flexion, and equinus in stance, is the most common sagittal plane
pathologic finding in young diplegic children. The crouch gait that occurs in the older
diplegic child is defined as excessive knee flexion and ankle dorsiflexion throughout
stance. Common causes are short or spastic hamstrings, hip flexor tightness, and
excessive ankle dorsiflexion. Stiff knee gait is characterized by limited or lack of
knee flexion in swing as a result of rectus femoris spasticity or unopposed rectus
femoris function after hamstring lengthening. Genu recurvatum, conversely, is hyper-
extension of the knee at midstance, which is generally associated with mild equinus
caused by triceps surae spasticity, excessive spasticity in the quadriceps, weak ham-
string muscles, or hip flexion contracture.32
Positioning, strengthening, and stretching exercises preserve joint range of motion,
increase strength, and help to improve gait. They should be combined with bracing,
walking aids, and antispastic treatments. The risk for contracture formation in the
more vulnerable biarticular hamstring and gastrocnemius muscles increases during
the prepubertal growth spurt. Intensive physiotherapy and botulinum toxin injections
to lengthen the spastic muscles may prevent contractures then. Exercises should
1222 Berker & Yalcın

be integrated into play, particularly in toddlers and in noncompliant children. Oral

antispastic medications are valuable if spasticity interferes with sleep. Botulinum toxin
is useful to relieve spasticity of the lower extremities and prevent contractures of the
young diplegic child when it is too early for orthopedic surgery. It must be combined
with physiotherapy and bracing. Most diplegic children need variations of the AFO to
provide a stable base for standing and maintaining good joint alignment during
walking. Resting and night splints are used to prevent knee and ankle contractures;
however, the compliance is poor in the child who has severe spasticity.
Most deformities of diplegics can be prevented or corrected with appropriate ortho-
pedic intervention. The ideal age of surgery is between 5 and 7 years of age or when
the child is able to cruise holding onto furniture or walk holding hands. Early surgery
may be necessary in cases with hip instability; knee flexion contracture because of
spastic hamstrings; and contracture of gastrocnemius-soleus unresponsive to phys-
iotherapy, botulinum toxin, or serial casting. It is necessary to define clearly all the
musculoskeletal problems of the lower extremities before surgery and address them
in a single setting if possible to obtain a successful result.14,33 All orthopedic interven-
tions should be timed so as not to interfere with the child’s education and social life.
Sports activities and play with peers are essential during school years. Swimming and
horseback riding are beneficial for the poorly developed balance reactions of the
diplegic.34 These activities restore a sense of well-being and self-confidence in the
child. Occupational therapy is helpful to improve hand function.

Problems and Management in Quadriplegia

Spine and hip deformities, such as hip instability, pelvic obliquity, and scoliosis, are
common and interfere with sitting balance. Knee and ankle deformities seen in hemi-
plegic and diplegic children may also exist. The incidence of lower extremity contrac-
tures increases with severity of the motor impairment. Elbow flexion-pronation
contracture creates problems when using forearm crutches, and severe flexion
contractures in the hand impair hygiene and cosmesis.
Hip instability and spinal deformity of the nonambulatory quadriplegic child do not
respond to conservative measures and generally require orthopedic surgery. Scoliosis
can develop as early as 5 years of age and continues to progress after skeletal matu-
rity, especially if the curve exceeds 40 .35 Hyperkyphosis is common in the young
child with weak spinal extensor muscles. The hips are usually normal during the first
years of life. Progressive instability occurs later because of a combination of muscle
imbalance, persistent primitive reflexes, faulty posture, and absence of weight-
bearing stimulation on bone to progressive instability. Dislocation can occur as early
as 18 months of age, and most hips dislocate by the time the child is 6 years of age
if they are going to do so.36,37 Hip dislocation affects hygiene, sitting, and gait of
the total body–involved child. It causes pain by early adulthood. Secondary scoliosis
and contralateral adduction deformity causing ‘‘windswept hips’’ further worsen the
situation.
The aim of rehabilitation in quadriplegic children is to obtain and maintain sitting bal-
ance. Infant stimulation, positioning, and parent education are important between
0 and 2 years of age. Ways to decrease muscle tone should be emphasized from
the age of 2 years, when muscle tone becomes a problem and dyskinesias manifest
themselves. In the nonambulatory child, orthopedic surgery is usually reserved for
hips at risk and spinal deformity. Hygiene, seating, and care issues in addition to
preventing pain and discomfort secondary to spasticity become predominant during
the teenage years. The knee and ankle deformities should also be corrected in mildly
involved quadriplegic children who have the potential to stand independently and take
 Cerebral Palsy 1223

a few steps to enable efficient transfers and limited ambulation. The aim is to obtain
a comfortable posture in lying, sitting, and the standing frame. The knee should flex
to 90 for sitting and extend to at least 20 for transfers. Stretching and range-of-
motion exercises may prevent knee flexion deformity early on. Regular exercises,
night splints, and standing in the stander to protect the range of motion gained by
surgical intervention are necessary.

Problems and Management in Dyskinesia

Hyperkinetic or choreoathetoid children have purposeless, often massive involuntary,
movements that increase when the child is excited or frightened. Dystonic children
manifest abnormal shifts of general muscle tone induced by movement, leading to
an abnormal posture. Contractures are almost never seen. Degenerative hip disease
and acetabular dislocation are common complications during the adolescent growth
spurt, particularly in children who have athetoid CP.30,38 Scoliosis is common. The
complication rate of spine surgery is high. The aim is to minimize muscle contractions
and unwanted movements to ease the burden of care and the child’s discomfort.

SUMMARY

The concept of management rather than cure forms the basis of intervention. Therapy
programs should be integrated with summer camps, home activities, and school,
enabling the child and the family to live as close to normal as possible. Successful
rehabilitation should prevent additional problems, minimize disability, and create
a happy child.

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