Cerebral Palsy Orthopedic Manifestations and Treamtnes

Introduction
 The term ‘cerebral palsy’ includes a group of disorders that result
from permanent non-progressive brain damage during early
development and are characterized by abnormalities of
movement and posture.
 CP was first described by William Little in 1862.
 CP is the result of a brain lesion; therefore, the spinal cord and
muscles are structurally and biochemically normal.

 Incidence of 1 to 3 per 1,000 live births.
 the highest rates in premature babies and those of multiple births.
 78 per 1,000 infants weighing less than 1,000 g.
 9 to 12 per 1000 in twins
 31 to 45 per 1000 in triplets
 111 per 1000 in quadruplets.
Epidemiology

 Known causal factors: maternal toxaemia, prematurity, perinatal
anoxia, kernicterus and postnatal brain infections or injury like
birth injury.
 CP can affect childhood motor development, speech, cognition,
and sensation.
 The main consequence is the development of neuromuscular
incoordination, dystonia, weakness and spasticity
Etiology

 Cerebral palsy is usually classified according to the type of
motor disorder, with subdivisions referring to the topographical
distribution of the clinical signs.
 TYPE OF MOTOR DISORDER:
 Spasticity
 Hypotonia
 Athetosis
 Dystonia
 Ataxia
 Mixed palsy
Classification

 Spasticity is the commonest muscle movement disorder and is
associated with damage to the pyramidal system in the CNS. It
is characterized by increased muscle tone and hyper-reflexia
with passive stretch . “clasped knife”.
 Hypotonia Infants are described as floppy.it is usually a phase,
lasting several years during early childhood before the features
of spasticity become obvious.
Classification

 Athetosis manifests as continuous, involuntary, purposeless
movements that may be exacerbated by environmental
stimulation. It is caused by damage to the extrapyramidal
systems of the CNS. In pure athetoid cerebral palsy, joint
contractures are unusual and muscle tone is not increased.
 Dystonia There is a more generalized increase in muscle tone
and abnormal positions induced by activity “lead pipe”. This
occurs in the absence of hyperreflexia, spasticity, and clonus
and it may occur with athetosis.
Classification

 Ataxia appears in the form of muscular incoordination during
voluntary movements. It is usually due to cerebellar damage.
Balance is poor and the patient walks with a characteristic
wide-based gait.
 Mixed palsy appears as a combination of spasticity and
athetosis. The presence of both types of motor disorder can
make the results of surgical intervention unpredictable.
Classification

TOPOGRAPHIC DISTRIBUTION
 Hemiplegia
 Diplegia
 Total body involvement
(Quadriplegia)!!
 Monoplegia

 Hemiplegia: is the commonest. This usually appears as a
spastic palsy on one side of the body with the upper limb more
severely affected than the lower. Most of these children can
walk and they respond reasonably well to treatment.
 Diplegia: involves both sides of the body, with the lower limbs
always most severely affected. Side to side involvement may
be asymmetrical, Intelligence is often normal.

 Total body involvement: general and often more severe
disorder affecting all four limbs, the trunk, neck and face with
varying degrees of severity. Patients usually have a low IQ,
they may have epilepsy, they are often unable to walk and the
response to treatment is poor.
 Monoplegia: very rare, occasionally appears in an upper
limb.

gross motor function classification
system (GMFCS)
 GMFCS is most commonly used to describe the patient’s level
of function before and after an intervention.
 The GMFCS scale which was developed by Palisano et al.
in 1997 , has five levels:
 GMFCS 1: ambulates without aids on all surfaces and keeps up
with peers.
 GMFCS 2: fully ambulatory, may use lower extremity orthoses,
and does not keep up fully with peers.
 GMFCS 3: uses ambulatory aids such as a walker or crutches
and may use a wheelchair for longer distances.
 GMFCS 4: nonambulatory patients who are able to propel their
own wheelchair.

 GMFCS 5 : inability to transfer, propel a wheelchair, or support
the trunk.
 GMFCS levels:
gross motor function classification
system (GMFCS)
level 1
35%
level 2
16.4%;
level 3
14.2%
level 4
16.1%
level 5
18%

Diagnosis
 Diagnosis in infancy:
 The full-blown clinical picture may take months or even years to develop.
 history of prenatal toxaemia, haemorrhage, premature birth, difficult labour,
foetal distress or kernicterus should arouse suspicion.
 A neonatal ultrasound scan of the head may identify intracerebral bleeding
that would increase the likelihood of later problems.
 Early symptoms include difficulty in sucking and swallowing, with dribbling
at the mouth.
 Motor milestones are delayed.

 Diagnosis in later childhood:
 Most children have already had the diagnosis made.
 the child should be carefully observed sitting, standing, walking and lying.
 gross motor function classification system (GMFCS) is used to evaluate there
condition, which categorizes the child, relative to their age, in terms of
mobility and bases this on their average function
 There are no definitive laboratory studies
Diagnosis

 GAIT assessment: Gait should be observed with and without shoes or orthotic
supports.
 Clinical gait analysis; Each limb must be observed in both the stance and swing
phases of gait and in the coronal, sagittal and transverse planes.
 Computerized gait analysis; it supplements clinical gait analysis, includes
Kinematics, kinetics, EMG, pedobarography (foot pressures), and metabolic energy
analysis.
 Sutherland and Davids at (1993) described four pathological gait patterns based on
knee motion in the sagittal plane:
o Toe-walking (jump knee) gait
o crouch knee gait
o Stiff knee gait
o Recurvatum knee gait
Diagnosis

Diagnosis
A. Toe-walking (jump knee) gait: due to
tightness in the hamstring muscles, hip
adductors and gastronomies muscles
B. crouch knee gait; due to long lasting of the
knee extensor muscles mainly rectus femoris
in stance phase .this also causes pelvis to
bend forward and causes a crouch in body.
C. Stiff knee gait; due to stiffness in the
posterior capsule of knee in the flexed
position
D. Recurvatum knee gait; due to long lasting of
the knee flexor muscles mainly biceps
femoris in stance phase .

 Deformity assessment:
 MUSCLE CONTRACTURE: longstanding spasticity leads to relative shortening of
the muscles and hence fixed contractures and changes in joint congruity.
 BONY DEFORMITY: it is influenced by muscle contracture.
 STRUCTURAL SCOLIOSIS: Flexible curves are common in CP, but it may
become structural.
 Imaging studies:
 CT & MRI of the brain
 Electroencephalography: Important in the diagnosis of seizure disorders
 Electromyography and nerve conduction studies: Helpful when a muscle or
nerve disorder is suspected
Diagnosis

management
 There is no single ‘blueprint’ for the management of all patients
with cerebral palsy; each patient and his or her family provides a
different challenge.
 For all patients with cerebral palsy the priorities are:
(1) an ability to communicate with others
(2) an ability to cope with the activities of daily living (including personal
hygiene)
(3) independent mobility , which may mean a motorized wheelchair rather
than walking.

 For the child who from an early age is recognized to be ‘non-
walking’ realistic goals should be:
(1) a straight spine with a level pelvis
(2) located, mobile and painless hips that flex to 90 degrees (for
comfortable sitting) and extend sufficiently to allow comfortable sleeping
and participation in standing/swivel transfers
(3) knees that are mobile enough for sitting, sleeping and transferring
(4) plantigrade feet that fit into shoes and rest on the footplates of the
wheelchair comfortably.
management

 TONE MANAGEMENT:
 Medical treatment:
 anticonvulsants for seizures, short-term benzodiazepine use for postoperative
pain
 trihexyphenidyl for dystonia.
 oral tizanidine (muscle relaxant ) has been used to relieve spasticity
 Physical therapy; is considered to be most helpful in early childhood up to
the age of 7 or 8 years
 Positioning and splinting; Splints are used to prevent muscle contracture,
maintain joint position and improve movement and hence function.
 Manipulation and serial casting: have limited role.
management

 Intrathecal Baclofen:
 γ-aminobutyric acid agonist, acts at the spinal cord level to impede
release of the excitatory neurotransmitters that cause spasticity.
 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.
management

 Botulinum Toxin:
 blocks the release of acetylcholine at the neuromuscular junction.
 botulinum toxin injection is of use in the treatment of lower
extremity spasticity.
 It is injected at known anatomic sites of innervation, often guided
by EMG.
 The drug begins taking effect after 2 to 3 days, and its effect wears
off after approximately 3-6 months.
 botulinum toxin may allow tendon surgery to be delayed until a
later age, when the risk for recurrence is lower.
management

 Selective dorsal rhizotomy;
 Neurosurgical procedure in which selected dorsal nerve roots from L1
to S2 are severed at the level of the cauda equina to reduce
spasticity.
 Candidates must have purely spastic CP.
 Studies have shown decreased tone and increased joint range of
motion after rhizotomy
 Residual contractures, hip subluxation, and spinal
deformity, in general, may occur in 36% of selective
dorsal rhizotomy patients.
management

 Operative treatment:
The indications for surgery are:
(1) a spastic deformity which cannot be controlled by conservative measures
(2) fixed deformity that interferes with function
(3) secondary complications such as bony deformities, dislocation of the hip and
joint instability.
(4) Shoe wear problems
(5) Pain
(6) Perineal hygiene problems
management

Timing of Surgery in CP
 Delaying surgery is advocated until age of 7–8
years because development of the CNS and
the gait pattern matures around this age and
then doing all the necessary operations at one
or two sittings.
 some patients surgery cannot be delayed like
hip subluxation which should undergo surgery
when the problem is first recognized to
improve coverage of the hip.
 children who are nearly ambulatory but whose
progress has been halted by contractures,
earlier surgery may allow them additional
range of motion to make walking less
cumbersome.

 Management of Foot Involvement:
 Equinus deformity:
o selective lengthening of the Achilles tendon
• Lengthening may be performed by open or
percutaneous techniques.
• The tendon must be repaired with sufficient tension
to avoid postoperative calcaneus gait
• ankle is then immobilized in a short-leg cast for 6 weeks
o gastrocnemius fascial recession
• the aponeurosis of the gastrocsoleus is divided in chevron fashion
• the soleus muscle fibers are not disturbed
• Immobilization is minimized
The Role of Orthopedic Surgery
in the Management of CP
FIGURE 35-20 Lengthening of the
gastrocnemius by the Vulpius technique.

 Equinovarus deformity:
o Posterior Tibialis Tendon Lengthening:
• usually done in conjunction with an Achilles tendon–lengthening
• patient is placed in a short-leg cast for approximately 6 weeks.
o Transfer of the Posterior Tibialis Tendon to the Dorsum:
• Calcaneovalgus can be a disastrous result and occurs in up
to 68% of patients.
• The only indication is when the posterior tibialis is
Completely silent during stance phase and active
during swing phase.

o Split Posterior Tibialis Tendon Transfer:
• posterior half of the posterior tibialis tendon is detached
from its insertion, split proximally to a level just proximal to the
ankle, rerouted posterior to the tibia and fibula, and then re-
attached into the tendon of the peroneus brevis.

o Split Anterior Tibialis Tendon Transfer:
• the lateral half of the anterior tibialis is detached from
the base of the first metatarsal.
• The tendon is passed beneath the extensor retinaculum
and inserted through a bone tunnel into the cuboid bone.
• usually combined with an Achilles tendon–lengthening procedure.
o Bone Surgery:
• If the varus deformity of the foot is fixed. heel varus will respond
to calcaneal osteotomy.
• If the deformity is very severe, calcaneal osteotomy will be insufficient and triple
arthrodesis should be performed.

 Pes Valgus deformity:
o Valgus deformity of the foot occurs in up to 25% of patients with CP
o Conservative treatment should be vigorously pursued because shoe inserts
and orthosis modifications may be adequate to relieve the pain
o In valgus deformity, bone surgery is the only predictable alternative for full and
lasting correction. Surgical options are:
(1) the Grice extraarticular arthrodesis ( extraarticular fusion of subtalar joint with bone graft)
(2) Lateral column lengthening of the calcaneal neck
(3) Calcaneal osteotomy
(4) triple arthrodesis.

 Ankle valgus:
o Surgical correction of ankle valgus is performed by either:
• Hemiepiphysiodesis of the distal medial aspect of the tibia provides
gradual correction
• When immediate correction is desired, distal tibial osteotomy is useful.
 Hallux Valgus
o develops in response to an equinovalgus deformity of the hindfoot
• first MTP arthrodesis is preferred for the surgical treatment of
hallux valgus in patients with CP

 Management of knee Involvement:
o Hamstring Lengthening:
• The hamstrings are nearly always affected in patients with CP.
• hamstring spasticity can be measured via the popliteal angle.
• Normal popliteal angles are variable, with a mean value of 26 degrees in children 4
years and older. Values greater tha 50 degrees are considered abnormal.
1) Orthotic Management: Mild tightness in the hamstrings
may respond to orthotic management, usually with ground
reaction AFOs.
2) Surgical lengthening of the distal hamstrings is the preferred
surgical treatment of crouched-knee gait

 Management of knee Involvement
o Rectus Femoris Transfer:
• Surgical treatment of stiff-knee gait and inability to flex the knee in swing
phase consists of rectus femoris transfer.
• It is often performed simultaneously with hamstring lengthening
• The tendon is divided transversely just proximal to the superior pole of the patella, then
passed medially and sewn into the stump of the gracilis tendon
o Bone surgery:
• Bone surgery has become accepted for patients with CP who have fixed
flexion contractures or those who have previously undergone hamstring
lengthening.
• distal femoral extension osteotomy +/- patellar tendon reefing is useful in
older children and teens.

 Management of Rotational Abnormalities of the Femur and Tibia
o Spasticity in the lower extremities over time leads to the development of
rotational abnormalities in the femur and tibia (increased femoral anteversion
and external tibial torsion ).
o Derotational Osteotomy :
• Femoral anteversion is treated by femoral osteotomy, either
proximally at the intertrochanteric or subtrochanteric level or
distally at the supracondylar level.
• In tibial rotational deformities, surgical correction should be
performed at the distal level. Proximal osteotomies are associated
with a higher risk for neurovascular injury.

 Management of Hip Involvement in CP:
 Hip Flexion Contracture:
o Hip flexion contractures are nearly always seen in combination with increased
hip adduction and internal rotation, with knee flexion secondary to hamstring
spasticity, and equinus, or valgus deformities of the feet. Hence, surgery to
improve hip flexion contractures is part of the SEMLS( Single Event Multilevel
Surgery) approach in conjunction with other soft tissue or bony procedures in
patients with CP.
o It is caused by increased tone in the hip flexors, primarily
the iliopsoas muscle.
o The contracture is identified by performing the Thomas
and Staheli maneuvers
o The recommended procedure is psoas tenotomy
performed over the pelvic brim.

 Adduction Contracture:
o Spasticity in the adductor muscles in CP results in a narrow base of gait and
scissoring.
o The muscles leading to the adduction contracture are the adductor longus,
adductor brevis, adductor magnus, gracilis, and occasionally the pectineus.
o Surgery to improve adduction contractures is limited to adductor release, with
or without obturator neurectomy, and posterior adductor transfer.

 Hip Subluxation or Dislocation:
o Hip dysplasia or instability is a common problem in patients with CP and
occurred in approximately 21%
o Because bony deformity develops in response to muscular spasticity, bone
surgery in the absence of soft tissue release is ineffective in correcting
subluxation or dislocation secondary to CP.
o The goals of treatment include a painless hip that allows stable sitting and
positioning in a non-ambulatory patient and full hip reduction in an ambulatory
patient.
o Categories of Surgical Treatment. divided into three categories
(1) soft tissue release for subluxation or a hip at risk
(2) reduction and reconstruction of the subluxated or dislocated hip
(3) salvage surgery for long-standing painful dislocations.

o First category: hip at risk is defined as a hip that has significant adduction and
flexion contractures but minimal subluxation, Surgical treatment is aimed at
preventing dislocation ofthe hip. The procedure consists of adductor release
and iliopsoas lengthening or release.
o Second category: The most frequently performed procedure in this setting is
femoral varus derotation osteotomy (VDRO).
o Third category: Indications for surgery in this group of patients include hip pain,
inability to sit in a modified wheelchair, and difficulty with perineal hygiene. Four
surgical options are available: proximal femoral resection, valgus osteotomy of
the proximal end of the femur, hip arthrodesis, and total hip arthroplasty.

varus derotation osteotomy Valgus osteotomy
proximal femoral resections

 Management of Upper Limb Involvement in CP:
o Operative treatment of the upper limb can improve the function of children with CP.
o Basic goals are improvement in function and improvement in appearance
o upper limb deformities:
(1) Elbow flexion deformity: no treatment is needed, occasionally
fractional lengthening of the biceps and brachialis tendons with
release of the brachialis origin is done.
(2) Forearm pronation deformity: fairly common, release of pronator
teres may improve the position.
(3) Wrist flexion deformity: it is usually in an ulnar direction it can be
improved by lengthening or releasing flexor carpi ulnaris. In severe cases wrist arthrodesis is
done.

 Management of Upper Limb Involvement in CP:
(4) Flexion deformity of the fingers: The flexor tendons can be lengthened
individually, but if the deformity is severe a forearm muscle slide
may be more appropriate.
(5) Thumb-in-palm deformity: due to spasticity of the thumb adductors
and/or flexors. but late there is also contracture of flexor pollicis longus.
In mild cases, function can be improved by splinting. Resistant deformity
may need combined lengthening of flexor pollicis longus and release of
the thenar muscles.

 Management of Spinal Deformity in CP:
 Scoliosis:
o Scoliosis is a significant problem in children with CP that
affects between 25% and 68% of patients.
o Scoliosis leads to difficulty sitting, the functional position
needed by a wheelchair-bound child.
o Nonoperative treatment of scoliosis with adaptive seating and orthoses has not
met with success.
o Indications for spinal fusion are:
1. Curves greater than 40 degrees in ambulatory patients
2. Progressive curves greater than 50 degrees in communicative patients
3. Curves that interfere with seating and nursing in patients whose families desire surgical
correction

 Management of Spinal Deformity in CP:
 Spondylolysis and Spondylolisthesis:
o hip flexion contractures and increased lumbar lordosis may lead to an
increased incidence of spondylolysis and back pain in patients with CP which
account for in 21% of patients.
o Dorsal rhizotomy may predispose patients to spondylolisthesis inasmuch as
12% of patients
o Treatment is surgical.

references
• Solomon L., Warwick D. , Nayagam S.,[2010] Apley’s System of Orthopaedics and
Fractures, 9th ed. Hodderarnold comp.,London, UK.
• Miller M. , Thompson S. , Hart J. ,[2012] REVIEW OF ORTHOPAEDICS [PDF], 6th
ed. by Saunders, an imprint of Elsevier Inc. , Philadelphia, USA.
• Canale S. , Beaty J. , [2007] Campbell’s Operative Orthopaedics [PDF], 11th ed. By
Mosby, An Imprint of Elsevier , Tennessee, USA.
• John A. Herring, [2014] Tachdjian’s Pediatric Orthopaedics [PDF], 5th ed. . by
Saunders, an imprint of Elsevier Inc. , Philadelphia, USA.
• John M. Flynn, [2011] American Academy of Orthopaedic Surgeons, Orthopaedic
Knowledge Update OKU 10 [PDF] , Printed in the USA.
• ORTHOPAEDIC REVIEW [2015] by orthobullets, [PDF], Collected By Islam Gomaa
Beltage.

Christy Brown
Jhamak Ghimire Stephen Hopkins;
"My hand trembles, my heart does not."
Maysoon Zayid
RJ Mitte

Cerebral Palsy Orthopedic Manifestations and Treamtnes

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