DISCUSS SURGICAL MANAGEMENT

OF CEREBRAL PALSY

BY DR EZE KINGSLEY C
MODERATOR : DR AHAOTU F N
(CONSULTANT ORTHOPAEDIC
SURGEON)
 OUTLINE
Introduction
Epidemiology
Pathology
Management
History
Physical examination
Investigation
Treatment
Follow up
Prognosis
Prevention
Subregional problems
Conclusion
references
 INTRODUCTION
Cerebral palsy includes a group of
disorders that result from non-progressive
brain damage during early development
and are characterized by abnormality of
movement and posture.
The condition is extremely heterogenous.
The insult to the brain is believed to occur
between the time of conception and the
age of 2 years.
 It has varied clinical forms.
Although the insult is permanent and
non progressive, the effect on the
patient is dynamic.
Known etiologic factors are maternal
toxaemia, prematurity, perinatal
anoxia, kernicterus and postnatal
brain infection or injury.
 In our environment, the commonest
cause seems to be prolonged
obstructed labour, with associated
complication of perinatal anoxia.
Commonly associated with a spectrum
of developmental disabilities
The diagnosis is majorly clinical.
Management is challenging and should
be multidisciplinary.
 Specialized centres and dedicated
staff, as well as surgeons well
experienced in procedures are all
paramount for successful
management.
Follow up is for life.
Prognosis depends on the type
among other factors
There is no cure so prevention
cannot be overemphasized.
 EPIDEMIOLOGY
Incidence is about 2 2.5 / 1000 live
births in singletons
About 7.5/1000 live births in multiple
births
About 60/1000 live births in
prematurity (1500g or less).
The prevalence varies around the
world.
About 0.6 to 7 cases per 1000 live
birth.
 Spastic type is the most common
form of C P, approximately 80% of
cases
Diplegic is the most common
anatomical type, about 50% of cases.
 PATHOLOGY
Pathophysiology
Non progressive brain damage during early
development.
Mainly motor and posture disorder.
There is basically weakness of the affected
muscles with loss of selective motor control,
deficits in balance and coordination.
There is no known genetic component to
cerebral palsy.
 Hypertonia leads to dynamic
contractures.
Muscle growth is stimulated by muscle
stretch, which is impaired due to the
hypertonia.
Muscle growth fails to keep up with
skeletal growth resulting in static
contractures.
Developmental delay and the abnormal
muscle forces on the growing skeleton
leads to bony deformities and joint
instability and lever-arm dysfunction.
 This manifest in abnormal and
inefficient gait patterns and hip
displacement and spinal deformities
in those severely affected.
Although the primary lesion in the
brain is not progressive, the
secondary musculoskeletal
consequences become worse with
growth.
 Associated problems include
Mental retardation (40%)
Epilepsy (33%)
Visual(16%), hearing and speech defects
Strabismus(50%)
Cognitive dysfunction
Malnutrition and related conditions such as
gastroesophageal reflux, obesity and
undernutrition(15%)
Emotional and behavioral problems.
osteopaenia.
Hydrocephalus (14%)
 Etiology
Prenatal
Iron deficiency, poor nutrition
Infection( TORCH), UTI, high fever
Chorioamniotis
HTN, DM
Teratogens
Poor ANC
Alcohol exposure
Multiple pregnancy
Fetal vasculopathy
IUGR
Developmental malformation
Perinatal
Birth asphyxia
Premature / LBW
Hyperbilirubenemia
Birth trauma e.g IVH
Sepsis, pneumonia, meningitis
 Postnatal
CNS infections
Trauma with head injuries
Seizures
Hypoxic damage
Hyperpyrexia damage
Cerebral malaria
 Classification
There is no universally accepted
classification scheme
Can be classified based on
Clinical physiological pattern
The region of the body affected
Neuroanatomical region of the brain
injured
 Geographical
Hemiplegia upper limb >
lower limb
Diplegia most common
type, common in
prematurity, lower limbs >
upper limbs
Monoplegia very rare, one
limb involved
Quadriplegia 4 limbs
equally involved
Total body loss of head and
neck control
Other types double
hemiplegic, paraplegic
 Physiological
Spastic type commonest
Athetoid pure type joint
contractures are
uncommon
Choreiform continual
purposeless movements
Rigid cogwheel or lead
pipe muscle stiffness
Ataxic disturbance of
coordinated movement
Hypotonic low muscle
tone + normal DTR
Mixed more than one
 Neuroanatomical region
Site of brain injury Pathology
Cortical Periventricular
leucomalacia spastic
Sub-cortical diplegia
Periventricular Stroke in utero
Basal ganglia hemiplegia
Multifocal
Cerebellum
encephalomalacia
Brain stem quadriplegia
Cerebellar ataxia
Basal
ganglia,thalamus,puta
men - dyskinetic
20
 Differential diagnosis
Spinal cord injury
Spina bifida
Transient dystonia of prematurity
Poliomyelitis
Head injury
Brachial plexopathy
Arthrogryposis
Motor neurone diseases
 Management
This should be multidisciplinary in
approach
Involve the paediatrician,
orthopaedic surgeon,
physiotherapist, psychologist, speech
and occupational therapist, orthotist,
nutritionist and social worker.
Diagnosis is mainly clinical
Start by taking a detailed history
 Biodata - age of the patient
Presenting complaint
Infants difficulty in suckling/swallowing, drooling
of saliva, the baby may feel stiff or wriggle
awkwardly, delayed developmental milestones
Later childhood gait abnormality, repeated
falling
History of presenting complaint
Cause, course ,care and complications
Associated problems
 Perinatal history
ANC, Maternal toxaemia, infections,
prolonged labour
Developmental milestones
Nutritional history
Past medical history
Meningitis, encephalitis, intracerebral bleeds
Family and social history
Review of systems
 Physical examination
General exam
Regional/systemic exam
Evaluation of the patient is done in
different positions sitting ,
standing , walking and lying.
It is done using the look, feel and
move approach.
 Standing
Spastic diplegic with the hips flexed,
adducted and internally rotated, knees
flexed and the feet in equinus.
Difficulty standing unsupported in cases
of tight hamstrings.
Scoliosis/ pelvic obliquity
Poor balance reactions.
Walking
Gait analysis ( clinical / computerized)
Scissors, crouched etc
With or without shoes/ orthotic support
Dystonic, athetoid, ataxic movements
 Neuromuscular exam
Muscle tone
Muscle power
Deep tendon reflexes
Plantar response
Ankle clonus
ROM at each joints
 Deformity assessment
Done for all region of the body limbs,
trunk and spine.
Identify primary/ secondary deformities
Spinal deformity is common in total
body CP, usually scoliosis with pelvic
obliquity, kyphosis/ hyperlordosis may
occur
Muscle contractures are common
Bony deformity is due to failure of
normal modelling leading deformities in
the hips, knees, ankle, elbow or wrist
 Some of the notable deformoties in
different regions of the body
Upper limb
deformities
Elbow flexion Spine/ pelvis
deformity Scoliosis
Forearm pronation Kyphosis
deformity
Kyphoscoliosis
Wrist flexion
Hyperlordosis
deformity
Pelvic obliquity
Finger flexion
deformity
Thumb adduction
(thumb in hand)
deformity
 Lower limb deformities
Hip adduction
Hip flexion
Hip internal rotation
Hip subluxation/dislocation
Knee flexion with upward displacement of patella
Spastic knee extension
External tibial torsion
Equinus foot
Foot varus
Planovalgus deformity of the foot
Special test done at different regions
of the body.
Thomass test
Stahelis prone hip
extension test
Elys test
Hamstring test
Silfverskiold test
 Colemans block test
Fixed varus deformity present in the
hind foot.
Rotational profile
assessment
 Investigation
Plain x-ray
Shows joint
subluxation/dislocat
ions, bony
deformities
 Electromyography
Documents the activity of various
muscles during the gait cycle

Computerized gait analysis

Gait patterns, energy consumption
during walking, pedobarography( foot
pressure)
 Neuroimaging (CT Scan / MRI )
This is frequently obtained when:
There is a history of complications during
pregnancy , labour , and delivery
The infant is born very premature (< 32
weeks)
Neurological symptoms or findings are
present on neonatal examination.
 Investigations to work up patient for
surgery
FBC + ESR
Urinalysis
SEUC
FBC
Group and crossmatch blood
 Treatment
Expectations of the parent / patient must be
matched with reaity
Thus, there is need for adequate
communication and counselling.
Four basic principles of treatment exist :
Although the CNS injury is non progressive , the
deformities are progressive
Treatment options currently available correct the
secondary deformities only
The deformities become worse during times of rapid
growth
Operative or non operative treatment should be
done to minimize the negative effect on the patient.
 Aims of treatment for all CP patient
To achieve the ability to communicate
To achieve the ability to cope with
activities of daily living including
personal hygiene
To achieve independent mobility , which
may mean wheelchair mobilization.
 Options of treatment include
Non operative
Operative
Goals of tratment include :
Strengthening of weakened muscles
Contracture prevention/ release
Gait and balance training
For severely affected patients is to
improve sitting balance and hygiene and
ease of care for caregivers.
 Non operative treatment
Anticonvulsants for seizures
Trihexyphenadryl for dystonia
Baclofen an agonist of gamma-
aminobutryic acid(GABA), acts by inhibiting
reflex activity, decrease substance p levels.
Dantrolene produces weakness without
much reduction in spasticity, rarely used.
Botulinium toxin(BTX-A) neurotoxin, has 7
serotypes, blocks acetylcholine at
neuromuscular jxn. Effects after few days.
Effective for about 10 12 weeks.
 Continuous intrathecal baclofen
infusion

48
 Other agents like phenol and alcohol can
be used.
Physiotherapy most useful in early
childhood up to 7 8 years and essential
for maximizing the effects of surgery.
Positioning/splinting ensure the child has
good posture at all times. Adjustments may
be made to chairs and wheelchairs . Splints
may be corrective or adoptive in function.
Manipulation/serial casting have limited
role in improving muscle and joint
contractures. Relapse is frequent.
 Operative measures
Indications include
Spastic deformities not amenable to non
operative treatment.
Fixed deformities that interfer with
function, cause pain or interfere with
activities of daily living.
Secondary complications like bony
deformities, hip dislocations and joint
instability.
 Important points to note while embarking on
operative treatment:
In CP , all muscles are weak
Muscle lengthening surgeries are muscle weakening
Improving a patients nutritional status before surgery,
may decrease the overall complication rate.
Timing of surgery is important, many advocate
around 4 8 years.
Birthday surgery should be avoided in favor of
simultaneous multilevel surgery.
Every case is considered on its own merit.
 Surgical options include ;
Upper limb surgeries :
Elbow flexion deformity
if the elbow can flex up to 900 , no
treatment is required.
more marked flexion contracture is
treated by fractional lengthening of
biceps / brachialis tendons with
release of brachialis origin.
 Forearm pronation deformity
Treated by release of pronator teres or re-
routing the tendon round the back of the
forearm, to act as a supinator.
Wrist flexion deformity
Improved by lengthening or release of flexor
carpi ulnaris
Severe cases are treated by wrist arthrodesis
with excision of proximal carpal row.
Flexion deformity of the fingers(clawing)
Treated by individual lengthening of the flexor
tendons.
 Thumb adduction deformity (thumb-
in-palm)
Mild cases can be treated by splinting
the thumb away from the palm or by
operative release of the adductor pollicis
and 1st dorsal interosseous muscle.
Severe cases may need combined
lengthening of the thenar muscle plus
tendon transfers to reinforce abduction
and extension.
 Lower limb surgeries
Spastic hemiplegia
Equinovarus foot deformity
Gastrocnemius/ soleus complex muscle recession
Dynamic varus foot deformity
Split tibialis anterior tendon transfer to the outer side
of the foot to avoid risk of over correction into valgus.
Split posterior tibial tendon transfer.
Pes valgus
Subtalar arthrodesis
Leg length discrepancy
Epiphyseodesis of the contralateral distal femoral and
or proximal tibial physis
Hip/knee deformity
Surgery not ususally required
 Fixed varus
deformity
Patient > 12 years
Triple arthrodesis
 Spastic diplegia
Hip adduction deformity
Adductor releases if passive abduction is < 20
deg. Done by tenotomy of adductor longus /
gracilis
Hip flexion deformity
Intramuscular lengthening of psoas tendon at
the pelvic brim.
Hip internal rotation deformity
Derotation femoral osteotomy after adductor
release / psoas lengthening.
 Hip subluxation/dislocation
Varus derotation femoral
ostetomy with acetabular
reconstruction.
Excision of proximal end of
femur for long standing
cases
Hip arthrodesis
Total hip replacement
Knee flexion deformity
Fractional lengthening of the
hamstring, posterior
capsular release.
Severe flexion deformities >
25 30 deg, treated by
extension osteotomy of the
distal femur or by physeal
plating anteriorly.
 Spastic knee
extension
Simple tenotomy of
the proximal end of
the rectus femoris
External tibial
torsion
Supramalleolar
osteotomy
 Equinus of the foot
Selective fractional
lengthening of the
gastrocnemius or
percutaneous lengthening
of the Achilles tendon.
Foot varus deformity
Gastrocnemius/ soleus
complex muslce recession
Equinovalgus & rocker
bottom foot
Calcaneal lengthening or
displacement osteotomy
plus subtalar fusion
 Total body involvement
Pain free hip subluxation / dislocation
No active treatment
Hip subluxation
Femoral varus derotation osteotomy with acetabular
reconstruction
Hip dislocation
Open reduction, release of soft tissue and bony realignment
Alternative option is resection of proximal femur
Spine / pelvis
For long C shaped scoliosis, operative correction / spinal
stabilization using pedicle screws/ rods extending from
thoracic spine to the pelvis
Other joints
Surgery as required
 Selective dorsal rhizotomy
A technique to reduce spasticity and
balance muscle tone in carefully
selected patients
Division of selected dorsal nreve roots
from L1 S2.
Gives good result in children 3 8 years
who are walking but have significant
spasticity, born prematurely, have good
intellectual function and good voluntary
control
 Follow up
This is for life.
 Prognosis
Prognosis
This depends on the type and severity of the
disease.
Bleck used the presence or absence of
promitive reflexes to determine prognosis for
ambulation for children with cerebral palsy
Sitting independently by 2 years of age is a
good predictor of independent ambulation
If a child cannot sit independently by 4 years
or has not learned to walk by 8 years, he/she
is unlikely to ever walk at all.
 Prevention
Cannot be overemphasized
Cheaper / better than treatment.
Preventive strategies include ;
Public enlightenment programmes / health
education.
Avoidance of known etiologic factors
Good ANC
Safe delivery practises
Immunization and screening
Genetic counselling
Drugs
 Subregional problems
Poverty
Ignorance
Cultural beliefs
Late presentation
 CONCLUSION
Cerebral palsy is a heterogenous disorder
with characteristic abnormality of
movement and posture.
It poses a great challenge to the managing
team
Adequate knowledge of its pathology and
prognosis is important for a successful
surgical management
The importance of prevention cannot be
overemphasized.
Thanks for
listening
References