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Cerebral Palsy

Ashish Anand Consultant Orthopedic surgeon

Cerebral Palsy
A non-progressive

disorder Caused by brain injury pre (70-80%), peri, or post natally Injure occurs before CNS reaches maturity Patients often have great potential masked by their

Etiology


Congenital cerebral defects Anoxia at birth Hemorrhage at birth Pre-maturity (3 1/3 pounds are up to 30 times more likely to develop cerebral palsy than full-term babies) Infection rubella (German measles), cytomegalovirus and toxoplasmosis Toxemia of pregnancy Rh incompatibility Developmental abnormalities

Manifestations
Malfunction of motor

centers Postural and balance difficulties Normal life expectancy possible Early death respiratory involvement

Characteristics
Impaired movements 65% speech defects 50% are mentally

retarded 50% ocular defects 25% hearing impairment 40% seizure disorders 20% seriously

Head and Neck findings


24% inability to chew 20% inability to swallow easily 20% frequent dental caries High rate of temporo-mandibular
disorders

Cerebral Palsy-Spastic Type

Findings in Spastic CP
52-70% of all CPs Hyperirritability of muscles Arms flexed, legs internally rotated Difficulty bending into a sitting position Difficulty with head control Postural difficulty May not have protective extension

Cont,d
Speech impairment Swallowing impairment/drooling Spastic tongue thrust Primitive reflexes

Athetoid or Dyskinetic type

Findings
25- 30% of CPs Uncontrollable writhing movements of opposing
muscle groups All four extremities involved Neck and face involved Voluntary movements are flailing Difficulty uprighting and balancing May lack protective extension

Cont,d
Grimacing Drooling Speech defects Continuous mouth breathers Excessive head movements Tongue protrusion Primitive reflexes of varying severity

Ataxic type
5 to 10 % Affects balance and coordination. They may walk with an unsteady gait

with feet far apart, and they have difficulty withmotions that require precise coordination ,such as writing

Other types of CP
Tremors (rare form) of CP Rigid 5 -10% of CPs Flaccid (Hypotonicity) Mixed 15 - 40% of cases

Limb Involvement
Can be single or multiple(except in
athetoid) Monoplegia Hemiplegia Diplegia Quadriplegia

Early Diagnosis
Primitive Reflexes Automatic Movement Reactions Walking

Preventing Impairment
Orthotics: Braces and Splints Positioning Oral Medication Nerve Blocks, Motor Point Blocks, and Botulinum Toxin Neurosurgery Orthopedic Surgery

Preventing Diasability: Promoting optimal function


Early Intervention Services Therapy Options Functional Mobility and Equipment Physical Activity and Sports Assistive Technology School

Goals of Surgery
surgery is best reserved for children over
3 yr with spastic CP, good intelligence, and voluntary muscle control; - muscle imbalance yields later to bony changes, so goal is to perform soft tissue procedures early and, if necessary, bony procedures later;

Goals of Treatment
Facilitate function in the areas of the brain
that control muscle coordination and movement Improve motor control and functional movement patterns Optimize health and well-being

Enhance Brain Function


No proven treatments to enhance brain
function This is an active area of research Three areas of current interest Replace non-functioning brain cells Repair cell connectors and Dendrites and axons Promote alternative brain pathways

Enhance Brain Function


Replacing injured cells Stem cells (primitive cells that can develop into any cell

line) exist in human brains throughout life Animal and human studies few stem cells develop,join normal brain, and survive after brain injury Currently stem cell implants not useful Many questions remain How to expand and sustain regeneration of brain cells Promoting stem cell population in the right place at the right time Potential cancer risks

Enhance Brain Function



Repair cell-cell connectors (wiring system) Dendrites: Short arm connectors Axons: Long arm connectors Periventricular Leukomalacia Common finding on MRI in children with cerebral palsy Pathology-poor myelination of nerve cell connectors Results in poor transmission of nerve cell impulse Research in potential treatment Develop cell growth factor to improve myelination

Enhance Brain Function


Brain plasticity and the development of
alternative pathways Brain constantly reorganizing structurally and functionally in response to sensory input and injury . Most plasticity occurs before 2 years

Enhance Brain Function



Types of neuroplasticity Compensatory masquerade One body part compensates for loss of function in another body part Functional map expansion Area of healthy brain grows into an area of injury Homologous region adoption One area of the brain takes over the job of a distant injured area Cross model reassignment One sensory input replaces another (braille

CP and Neuroplasticity
Brain reorganizing and restructuring continuous brain

function Restructuring may interfere with other brain functions Most possible when small specific areas of the brain involved Sensory input linked to motor performance important in improving desired outcome Example: Intermittent partial constraint therapy

Enhance Brain Function


Cerebral palsy is a complex disorder that can be

caused by several types of brain injury Failure of cell migration from origin to functional location Failure of oligodendrocytes to deposit myelin on connector cells Poor function at brain synapse Death of grey matter cells

Enhance Brain Function


Techniques to enhance brain function are

exciting areas of research Currently some evidence based treatment programs as well as complementary and alternative techniques are based in these theories Unlikely that a single theory will be effective for all children with cerebral palsy

Improve Motor Control



Common motor problems in children with cerebral palsy Muscle spasticity Muscle weakness and poor motor control Uncontrolled movements Common impairments Mobility Poor feeding skills Difficulty with speech and communication Poor self-help skills

Improve Motor Control



Treatment of muscle spasticity Bolulinum Toxin Most widely used treatment for focal spasticity Inhibits acelycholine released at the neuromuscular junction Permanent blockade, nerve sprowling and muscle reinnervation lead to functional recovery in a few months Used with intensive physical therapy following to maximize results Passive function improved, few studies show active function improvement (?poor study design

Improve Motor Control


Botulinum Toxin Type A and B FDA Early Communication about an

Ongoing Safety Review 2-8-2008 System adverse reactions including respiratory compromise and death suggestive of Botulism Occurred mostly in children with cerebral palsy

Improve Motor Control


Botulinum Toxin FDA recommendations Professionals understand unit designation not

comparable across products Be alert of potential systemic effects Effects may occur 1 day to several weeks after treatment Provide patients/families with signs and symptoms or systemic effects Tell patients to receive immediate attention for problems

Treatment of muscle spasticity


Treatment of muscle spasticity Medications oral Baclofen, tizanidine, benzaodiazepines,

dentrolene and gaberpentin All have side effects like drowsiness and weakness (Thompson, 2005) Surgical treatments Intralhecal Baciofen Selective Dorsal Rhizatomy Orthopedic surgery procedures

Improve Motor Control


Selective Dorsal Rhizolomy (SDR) Techniques evolved over the 20th century with current

techniques developed by Peacock and Arens in the 1980s Operative site lumbo-sacral or conus Balance between elimination of spasticity and preservation of strength MUST be followed by intensive therapy to optimize results Reducing spasticity without improving strength and coordination will not usually improve motor control

Improve Motor Control


Selective Dorsal Rhizotomy areas of improvement Gross motor Those with highest function pre-operatively did

best Several studies identify gains for at least 2 years Spasticity Many studies show decrease in spasticity after SDR Long term reduction significant (over 5 years)

Improve Motor Control




Selective Dorsal Rhizotomy area of improvement Strength Shown by several studies, hard to measure Seems stable over time, most gain in 1st year Range of motion Improved, mostly in the 1st year Fine motor skills Improved, especially Eye-hand coordination and manual dexterity

Improve Motor Control


Selective Dorsal Rhizotomy areas of

improvement Self-help skills improved Orthopedic procedures less common after SDR Complications CSF leaks, infection Bladder dysfunction Spinal deformities(Farmer 2007 )

Improve Motor Control


Physical therapy, Occupational therapy, and

Speech therapy All therapy modalities provide documented benefits for children with cerebral palsy Goals of treatment to improve function and prevent deterioration Little data exists to support which therapist, which therapies, timing, frequency, and duration

Improve Motor Control


Bower, et al examined motor function and
performance in 56 children with cerebral palsy by varying intensity of PT and collaborative vs therapist goal-setting 4 groups 1. Present PT levels 1-4 hours/month 2. Intensive PT 5 hour/wk (PT set goals) 3. Group 1 with collaborative goal setting 4. Group 2 with collaborative goal setting

Improve Motor Control


No statistical difference in motor scores
between intensive group vs routine group No difference in motor scores between PT set goals vs collaboratively set goals

Improve Motor Control


Trahan et al, examined a novel therapy
schedule with intermittent intensive physical therapy in 5 children with severe cerebral palsy Therapy schedule Physical therapy 4 days/week for 4 weeks, off for 8 weeks

Contd
Compliance during intensive therapy 93% Motor scores improved an average of 10% Children maintained their performance

during rest periods Authors suggest intensive treatment may be less tiring and may provide practice conditions for consolidating motor skills

Improve Motor Control


Constraint-induced Movement Therapy Technique developed to help adults with

hemiparesis after stroke gain upper extremity function Involves constraint of non-paretic arm and intensive motor shaping of paretic extremity Based on principles of neuroplasticity Theoretically should be helpful in young children

CIMT


Several small studies have examined Constraint-Induced movement therapy (CIMT) in children Many protocols and outcome measures Results promising Deluca et al(2006) used a randomized controlled cross-over design with a total of 18 children CIMT produced significantly greater gains than conventional therapy

Optimize Health and Well Being


Quality of Life (QoL) Defined by the WHO as an individuals

perception of their position in life in the context of culture and value systems in which they live and in relation to their goals It incorporates physical health, psychological state, level of independence, social relationships, and personal beliefs Simplistically, well-being

Optimize Health and Well Being


Dickinson (2007)et al surveyed 500 children with cerebral palsy in 6

European countries (8-12 years old) Type and severity of impairments not associated with QoL for 6 domains Psychological well-being, self-perception, social support and peers, school environment, financial resources, and social acceptance Specific impairments associated with poorer QoL in 4 domains Poor walking poorer physical well-being, intellectual impairment-lower mood and emotions and less autonomy,speech difficulty poorer relationship with parents, pain associated with poorer QoL

Optimize Health and Well Being


Shields et al(2007) survey 47 children with

spastic diplegia or hemiplegia and matched typical peers Children with CP: lower scores on scholastic competence, social competence, and athletic competence No difference in global self-worth, physical appearance, or behavioral conduct

Contd
Aran et al 2007)surveyed 39 children with CP

ages 6-18 years old, their siblings and parents to assess the impact of parenting style on QoL Children reported that their QoL had more to do with their parents parenting style than the severity of their motor handicap 2 factors in parenting had the biggest impact Parents acceptance of the disability Parents willingness to foster autonomy

Optimize Health and well Being


Recreation Informal survey of children show interest in sports and

recreation Only limited research available to examine benefits Exercise and sports regimens can improve strength,balance, and coordination (small case studies) Often recreational activities more motivating and enjoyable than intensive therapy For older children, activities more developmentally appropriate and more social than individual therapy

Recreation


Recreation Wide range of adaptive sports possible Swimming Hippotherapy (therapeutic horseback riding) Adaptive skiing Martial arts Wheelchair sports

Optimize Health and Well Being




Websites for adaptive sports Disabled Sports USA http://dsusa.org Wheelchair Sorts USA http://www.wsusa.org National Sports Ability Center for the Disabled http://www.nscd.org Boundless Playgrounds p://www.boundlessplaygrounds. org Kidsource http://www.kidsource.com

Knee
most thoroughly analyzed joint in cerebral palsy has
been knee; - most common deformity of the knee involves flexion contractures and decreased range of motion; - early changes: - short stride gait and reduced popliteal angle; - knee flexed at the initiation of stance phase and throughout gait cycle; - late changes: - knee flexion and hip extension contracture; - patellae alta;

Contd
role of quadriceps:
- although knee-flexed postures are most easily conceived of as being due to spastic hamstring muscles, spasticity of the quadriceps (co-spasticity) is often associated with this deformity; - spasticity of the quadriceps (most often the rectus) limits the 35 degrees of initial flexion and the 70 degrees of total flexion of the knee during the swing phase of gait and results in a less-than-optimum, energy-consuming, stiff-legged gait; - crouched gait: - gait pattern characterized by hip-flexion, knee-flexion, and ankledorsiflexion posture throughout stance phase; - either the psoas or the hamstrings may be responsible for the flexion posture of the hip and knees; - in some cases crouched gait pattern is precipitated by lengthening of the Achilles tendon, without adressing hamstring contractures;

Treatment
Hamstring Lengthening:
- hamstring lengthening is helpful to relieve excessive contractures, esp when they have a significant effect on gait; - hamstring lengthening is often performed along with Achilles tendon lengthening (in order to avoid crouched gait) - the threshold for performing hamstring lengthening varies, but many surgeons use a popliteal angle of 90-100 deg as a threshold for performing lengthening in non ambulating patients and an angle of 135 deg as a threshold in ambulators; - distal hamstring lengthening is preferred (over proximal lengthening) for ambulatory patients; - complications: - may result in knee recurvatum if distal lengthening is performed in the face of uncorrected equinus; - may increase lumbar lordosis (if hamstring lengthening is performed proximally);

Rectus Transfer
Rectus Transfer:
- transfer of the distal part of the rectus femoris tendon may be indicated inorder to partially reduce the spasticity of the quadriceps; - rectus transfer (to the gracilis) allows better knee function and foot clearance in the swing phase of gait; - distal part of rectus femoris tendon is freed from vastus lateralis and medialis muscles, and the flat tendon is separated from underlying vastus intermedius tendon and is sectioned distally; - resultant free rectus femoris tendon is then transferred to belly of the sartorius muscle so that it can act as initial flexor of the knee and external rotator of the hip

Hip Joint
may occur in upto 30% of patients w/ spastic CP, and may occur in
upto 50% of patients w/ spastic quadriparetic CP; - more severe contractures with unilateral or bilateral hip dislocation occur in the nonambulatory child with spastic quadriplegia; - hip subluxation may be more likely in asymmetric in spastic quadriplegia and w/ pelvic obliquity and scoliosis; - spastic deformities are initially the result of muscle tone alone; - degree of muscle tone is difficult to quantitate, as it varies with position of adjacent joints and body posture; - hips usually dislocate posteriorly, due to the overactivity of the adductors and flexors of hip; - hip on the high side of the pelvis is usually dislocated, adducted, & internally rotated, whereas hip on the opposite side is located, abducted, and externally rotated;

Natural history

most often, the hip dislocates when the child is between 5 - 7 years of age, taking approximately two years from first evidence of subluxation; - often the first clinical indication of subluxation is heralded by inability to abduct more than 45 deg; - untreated dislocations may produce pelvic obliquity, pain, and problems w/ seating, decubiti, and hygiene problems; - in the report by Raymond Knapp Jr MD and Hector Cortes, the authors evaluate the problems associated with hip dislocation in adults with cerebral palsy; - 29 subjects with dislocated hips and no prior hip surgery were identified; - there were a total of 38 dislocated hips; - age range was 21 to 52 years (average 34); - 7 dislocated hips (18%) were definitely painful and four hips (11%) produced only mild or intermittent pain; - 27 hips (71%) were not painful; - 7 painful hips underwent proximal femoral resection, resulting in excellent range of motion and no pain; - authors conclude that if a dislocated hip becomes painful in adulthood or develops an adduction contracture interfering with perineal care, a proximal femoral resection can be performed with reliably good success;

Examination
check hip abduction in both extension and flexion (less than 45 deg
indicates contracture, and less than 30 deg abduction indicates a significant contracture); - Treatment: - usually requires both a bony and a soft tissue procedure; - both hips are done with soft tissue procedures; - for child with spastic diplegia, adductor tenotomy or posterior transfer, with iliopsoas lengthening when the adduction deformity is accompanied by flexion contracture, typically is done between 4 and 9 years of age;

Early Treatment: (w/ > 40% subluxation);


soft tissue releases may be effective if performed before significant
dysplasia is present; - soft tissue releases should not be expected to improve osseous deformity; - spastic deformities can result in fixed contractures that can progress to fixed joint contractures; - contracture of psoas muscle is cause of flexion deformity in spastic diplegia as well as in spastic quadriplegia; - treatment: - soft tissue releases w/ post operative abduction bracing may prevent dislocation when performed before femoral head is 50% uncovered

Contd
iliopsoas procedures:
- poas lengthening (resection) may be indicated with hip flexor contracture (positive Thompson test) - preservation of the iliacus maintains the strength of hip flexors; - appreciable weakness of flexion of hip occurrs after iliopsoas tenotomy, and therefore avoid this procedure in patients who can walk; - bilateral adductor tenotomy; - beaware, unilateral soft-tissue surgery may have negative effect on opposite hip; - w/ bilateral hip flexion contractures, unilateral soft tissue release may cause the contralateral hip to undergo progressive subluxation; - hip that continues to subluxate after adductor release may require pelvic or femoral procedure and should be watched closely;

Treatment in older children


- varus femoral osteotomies;
- this may correct femoral valgus and anteversion abnormalities, but will not be expected to correct acetabular dsyplasia; - neck shaft angle of more than 145 deg is an indication for trochanteric osteotomy, with a goal of reducing the neck shaft angle to less than 125 deg; - derotational osteotomy may produce more normal gait but does not necessarily increase stride length; - this operation can be done at the supracondylar level, but if there is significant valgus or subluxation of the hip, it should be done in intertrochanteric region; - salter osteotmy; - may have better success in DDH rather than spastic dislocation; - chiari osteotomy; - some authors feel that this osteotomy is contra-indicated w/ spastic dislocation;

Contd
pemberton pericapsular osteotomy:
- indicated for acetabular dysplasia, subluxation, and dislocation; - may not provide optimal posterior coverage; - spastic dislocation: - acute dislocations (rare) may benefit from open reduction, femoral shortening, varus derotation osteotomy, and Chiari osteotomy; - VDRO may be successful in 8-10 years olds w/ femoral anteversion and hip dislocation / subluxation; - late dislocations may best be left out or treated with a Shanz abduction osteotomy; - windswept hips: - characterized by abduction of one hip and adduction of contralateral hip; - treatment is best directed at attempting to abduct the adducted hip with bracing or tenotomies; - scoliosis is treated similarly to idiopathic scoliosis;

Foot and Ankle


Discussion:
- most common problem; - in those w/ diplegia, it is bilateral & almost always flexible in child under three years; - diff dx: toe walking

- Exam: - it is essential that the ankle and hindfoot be held in varus while the amount of dorsiflexion is measured; - be sure to exam the popiteal angle for hamstring contracture;
- Non Operative Rx: -

Contd
anterior tibial tendon f(x) determines whether child will
be brace free; - ankle position can be controlled with an orthosis until myostatic contracture occurs or when child is closer to 5 years of age; - despite widespread use of plastic AFO (and similar braces) prospective evidence which proves long term efficacy (for prevention of contracture) is not available;

Operative treatment

method of correcting spastic equinus deformity in older children (above age 5) remains operative; - open Hoke Method: - open Z lengthening: - aponeurotic lengthenings of gastrocnemius (Olney et al.) - advantage is that this procedure preserves the strength of the soleus muscle was achieved; - performed in 156 patients (219 procedures); - there were no instances of over-lengthening resulting in pes calcaneus; - equinus deformity recurred in 48 % of ankles; - there were more recurrences in children who were operated on before the age of five years; - duration of postop immobilization, did not affect result or rate of recurrence; - botox injections: - has been recommended for temporary reduction of muscle tone; - in the report by Dimitrios Metaxiotis et al, the efficacy of repeated botulinum toxin A injections in the calf muscles was evaluated in a clinical trial involving 21 children with cerebral palsy and dynamic equinus foot deformity who were able to ambulate; - mean age of the children was 5.7 years; - all patients received at least 2 injections, 6 patients received at least 3 injections, and 3 patients received 4 injections. - significant improvements of the gait parameters were observed at 6 and 18 weeks after the first and second injections; - botulinum toxin A injections in gastrocnemius and soleus muscles may change the natural history of equinus foot deformity in patients with spastic diplegia; - ref: Repeated Botulinum Toxin A Injections in the Treatment of Spastic Equinus Foot Dimitrios Metaxiotis, MD Clin Orthop 2002 January;2002(394):177-185 -

Contd

postop care: - immobilization in above-knee cast for six weeks, followed by use of a night splint or orthosis; - recurrence may occur in approx 20%; - in the study by Katz et al 2000, the authors evaluated the long-term result of postop immobilization for two weeks in a below-knee cast and early weight bearing, without the use of a splint or orthosis; - 36 children (52 feet) with spastic cerebral palsy underwent sliding Achilles tendon lengthening; - at five to ten years, there was recurrence rate of 19.2%; - most recurrences occured in children operated on before five years of age; - Complications: - overlengthening and crouched gait: - overlengthening is to be avoided at all costs; - crouched gait is the classic iatrogenic error in which the child walks w/ the ankles in maximum dorsiflexion and the knees flexed throughout the gait cycle; - results from Achilles tendon overlengthening and neglect of hamstring contracture; - in CP, hamstring contracture should be corrected at the same time as the equinus contracture; - non operative treatment of crouched gait, involves an anterior floor reaction AFO which limits dorsiflexion during stance phase;

Equinovarus
more common in spastic hemiplegia, and is caused by overpull of of the tibialis
posterior and/or anterior tibial tendons; - try to determine which of these muscles is more pathologically involved; - remember that the tibialis anterior is normally active at heel strike and thru out swing phase where as the tibialis posterior normally activates just after heel strike and stays active thru out stance phase; - children w/ hemiplegia often display equinovarus foot deformity as result of posterior tibial tendon dominance, although anterior tibial tendon is occassionally at fault; - dynamic varus deformities of foot are most often due to spasticity of tibialis posterior (active in stance phase) or anterior tibial muscle (active in the swing phase); - etiology in adults: - stroke; - parkinson's disease; - focal dystonia; - may present as torticollis, writer's cramp, and less often it involves the leg (as equinovarus);

Treatment
transfer of an entire muscle (posterior or anterior tibialis)
is rarely indicated; - entire transfer of posterior tibial tendon to dorsum of foot should be avoided unless EMG show that it is active only during the swing phase of gait; - transfer of a PT tendon w/ normal phase activity (active in stance), will produce a calcaneovalgus foot from chronic tonic activity; - split muscle transfers are helpful, especially when the affected muscle is spastic in both stance and swing phases of gait;

Split Transfer of Tib Post


split posterior tibialis transfer involves rerouting half of the tendon dorsally
to the peroneus brevis; - advantage is that the strength of plantar flexion is preserved; - split posterior tibial tendon transfer, combined with heel cord lengthening, is used in cases w/ spacicity of muscle, flexible varus foot, and weak peroneals; - it remains an alteranative to the traditional lengthening of posterior tibial tendon; - lengthening of the posterior tibialis is now rarely indicatated because of recurrence and development of a calcaneovalgus foot; - alternative procedure involves transfer of posterior tibial tendon through interosseous membrane to dorsum of lateral portion of cuneiform bone; - most poor results will result from residual varus deformity; - complications include decreased foot dorsiflexion;

Split Transfer of Tib Ant

indicated when the tibialis anterior is active both in stance and swing phase (normally on active in swing phase) - combined split anterior tibial tendon trasfer and IM lengthening of posterior tibial tendon (Barnes and Herring) has been recommended for dynamic varus of hindfoot and adduction of forefoot in both stance and swing phase of gait; - split anterior tibialis transfer is used when forefoot supination is predominant, and usually is combined with posterior tibial tendon lengthening; - split anterior tibialis transfer (rerouting 1/2 of tendon posteriorly to the cuboid) is used in patients with spasticity of muscle and flexible varus deformity; - usual approach is lengthening of Achilles tendon, often w/ split transfer of the anterior tibial tendon; - tendo-achilles lengthening addresses the equinus deformity; - split tibialis anterior transfer adresses hindfoot varus deformity; - occassionally the posterior tibial tendon must be lengthened; - complications: - patients who demonstrate a strong contraction of the TA during swing phase before surgery may develop a drop foot following surgery; - this occurs because the TA transfer reduces dorsiflexion; - recurrence of varus deformity will occur in aboutt 15%; - complications of this procedure include over correction;

Contd
extrinsic toe flexor release:
- release of the FHL and FDL lessens the toe flexion that occurs with dorsiflexion or the foot; - anterior transfer of long toe flexors (FHL and FDL): - indicated for adult patients who have had a stroke; - procedure is often combined with tendo Achilles lengthening; - motivation for this procedure is based on the EMG observation that the long toe flexors in these patients shows a relatively high incidence of muscle actively inthe swing phase; - using this procedure, about 75% of patients will be able to walk without an AFO; - tendons of the FHL and FDL are transferred anteriorly to the 4th metatarsal through the interosseous membrane of the lower leg; - FHL tendon is passed around the base of the 4th metatarsal to make a loop by suturing it to the FDL tendon under maximum tension; - when the hindfoot varus deformity is severe, consider lengthening the posterior tibial tendon; - recurrence of varus deformity will occur in aboutt 15%;

Pes Valgus
Discussion:
- 64% w/ spastic diplegia & quadriplegia had pes valgus; - spastic pes valgus foot is a flexible deformity until adolescence; - spastic peroneal muscles pull the forefoot laterally and with it supporting plantar ligaments for the talar head, resulting in plantar flexed talus; - left untreated, the natural history may result in midfoot sag and lateral near complete break down in the midfoot. - anatomical relationships: - plantar flexion of the talus and calcaneus; - excessive valgus, external rotation and dorsiflexion of the calcaneus in relationship to the talus; - navicular dorsiflexed and abducted on the head of the talus; - forefoot is supinated in relationship to the hindfoot;

Exam
Exam:
- note correctability of the hindfoot in relation to the forefoot; - look for equinus contracture (w/ the hindfoot placed in varus); - skin changes may occur over the medial aspect of the talar head;

- Non Operative Treatment: - patient's w/ spastic deplegia will often not tolerate orthotic management;

- Treatment Methods: - Grice Arthrodesis: - subtalar extra-articular arthrodesis that was originally devised by Grice for the management of pes valgus due to flaccid paralysis muscle imbalance (polio); - modifications of the Grice procedure have also been widely used for treating hindfoot valgus deformity in CP; - Sub-talar Arthrodesis: - Triple Arthrodesis:

Calcaneal Lengthening

modified Evan's calcaneal lengthening technique; - indicated for chronic pain, brace intolerance, and skin changes; - surgical approach: - similar to Sub-talar Arthrodesis using modified Ollier incision; - care is taken not to damage the calcaneal-cuboid joint; - bluntly dissect over the dorsum of the calcaneus, and then dissect medially (just distal to the middle facet; - perform similar dissection under the plantar surface of the calcaneus; - two curved retractors are inserted dorsally and plantarly inorder to protect the calcaneus during the procedure; - insert two Steinman pins on either side of the proposed osteotomy site; - the calcaneal osteotomy courses obliquely across the calcaneus between the anterior and middle facets; - begin about 1.5 cm proximal to the calcaneal cuboid joint; - insert a lamina spreader to distract the osteotomy site; - trapezoidal shaped tricortical graft (10-12 mm in width) is then inserted laterally, inorder to lengthen the lateral column of the foot; - ensure that there has not been subluxation of the calcaneal cuboid joint; - the graft may then be held in place with Steinman pins; - w/ concomitant Equinus contracture, Achilles tendon lengthening may be required;

Equinovalgus
is more common in children w/ diplegia and spastic quadriplegia;
- in ambulatory children, mild deformity can be corrected w/ peroneal tendon lengthening (transfer of brevis to posterior tibialis is unreliable) combined w/ achilles tendon lengthening; - if heel valgus is pronouned, medial displacement os calcis osteotomy can be added; - if heel valgus and forefoot pronation are marked, opening wedge osteotomy of the anterior calcaneal process can be performed; - if hindfoot is in rigid valgus, it is best to combine an extra-articlar subtalar fusion with the tendon lengthening; - more common in spastic diplegia; - caused by spastic peroneals, contracted heel cords; - peroneus brevis lengthening is helpful to correct moderate valgus; - subtalar arthrodesis is reserved for severe valgus deformities;

Hand
Discussion:
- most pts w/ hemiplegic cerebral palsy have functionless hand marked by: - flexion of the elbow with pronation of the forearm; - flexion of the wrist and fingers: spasticity, weakness, flexion deformity of the wrist & fingers - thumb in palm deformity; - loss of sensation and proprioception; - Physical Exam: - note the degree of finger flexion deformity w/ the wrist flexed and extended;

Treatment Considerations
treatment of upper extremity lesions needs to be directed toward improving specific
functions, but care must be taken not to diminish established skills; - for instance, a patient w/ the typical wrist flexion deformity may be able to sweep the floor (using the hand as a hook), but may be unable to sweep the floor when the hand is braced in the functional position (w/ wrist extended and MP joints flexed); - it is also possible to worsen the finger flexion deformity by taking the wrist out of its flexed position and placing it in extension; - preoperative voluntary control and stereogenesis help predict postoperative functional gains; - surgery may also be indicated to improve the appearance of hand; - cautions: a patient w/ a severe elbow contracture and wrist flexion contracture may not benefit from correct of the wrist flexion deformity alone; - in this situation, correction of the wrist flexion deformity will position the extended wrist right in front of the patient's face;

Elbow Contracture
the study by Paul R. Manske et al, the authors evaluated anterior elbow release for
spastic elbow flexion deformity in children w/ CP; - 42 consecutive surgical procedures are reported in 40 children with a minimum of 1 year of follow-up; - procedure included incision of the lacertus fibrosus, fractional lengthening of the brachialis aponeurosis, and denuding the peritendinous adventitia from the biceps tendon to remove afferent nerve fibers and receptors; - flexion posture angle improved from 104 deg before surgery to 55 deg after surgery, a reduction of 49 active extension improved from 43 deg to 27 deg; - there was no significant change in elbow flexion. - before surgery, the average percentage use of the arm was 12%, which improved significantly to 44% after surgery; - ref: Anterior Elbow Release of Spastic Elbow Flexion Deformity in Children With Cerebral Palsy Paul R. Manske, M.D. Journal of Pediatric Orthopaedics 2001;21:772777

Wrist Flexion deformity

pts may benefit from transfer of FCU around ulnar border of wrist to ECRB to restore active dorsiflexion of the wrist; - note that correction of this deformity may worsen the finger flexion deformity; - by releasing the flexor pronator origin, one allows the wrist to come to come to neutral w/o causing flexion problems in the fingers; - after relaxing, wrist is extended & fingers can be actively extended; - releasing the flexor pronator origin improves appearance & function of the hand w/ severe flexion deformities of wrist and fingers; - in the report by El-Said (JBJS 2001), the author performed a transfer of flexor carpi ulnaris combined with selective release of the flexor pronator origin in 35 patients with hemiplegic CP for a pronation flexion deformity of the forearm, hand and wrist; - procedure reduces the power of wrist and finger flexion by release of the flexor pronator origin, and reinforces the strength of extension and supination of the wrist by transfer of FCU; - after a mean follow-up of four years the appearance of the hand and forearm improved in all patients; - none lost movement and all gained improved mobility of the forearm, wrist and hand. - there was no overcorrection; - ref: Selective release of the flexor origin with transfer of flexor carpi ulnaris in cerebral palsy N. S. El-Said. J Bone Joint Surg [Br] 2001;83-B:259-62. - wrist fusion: - procedures involving inlay iliac-crest graft are most successful, and wrist should be held in neutral position by a large Kirshner wire placed in third metacarpal across the wrist and into radius;

Finger Flexion deformity


finger & thumb flexors remain tight, sublimis tendons are divided at wrist,
profundus tendons are lengthened, & thumb is released; - in the report by Takashi Matsuo et CORR 2001, the authors followed 32 deformed hands of 31 patients with cerebral palsy were treated with combined release of the flexor digitorum profundus, FDS, and intrinsic muscles; - of these 31 patients, 26 patients (27 hands) were followed up after treatment; - improvements of more than one level on an average were observed in the modified classification of Zancolli et al and the classification of House et al. - ability to grasp, pinch, and release increased with improvement of 2.5 points in the object handling score, and activities of daily living were enhanced with improvement of 2.4 points; - ref: Release of Flexors and Intrinsic Muscles for Finger Spasticity in Cerebral Palsy. Takashi Matsuo, MD. CORR. 2001;2001:162-

Thumb in Palm deformity


/ IP joint contracture, consider FPL release and transfer
to the EPB performed along with IP joint fusion; - brachioradialis transfer to the abductor pollicis longus; - release of the thumb adductor (w or w/o release of 1st dorsal interosseous); - abductor pollicis longus and EPB may be plicated, & EPL may be rerouted to a more radial position; - fusion of the metacarpophalangeal joint of thumb

Spine
Non Operative Treatment:
- many patients can achieve functional sitting with body jackets or molded wheelchair inserts; - custom molded seat insertes allow better positioning but do not prevent curve progression; - Surgical Treatment: - w/ spastic quadriplegia scoliosis can be especially severe, and is associated with an increased incidence of pseudoarthrosis if treated with conventional posterior fusion; - anterior and posterior fusion or segmental instrumentation is favored in patients who can no longer sit properly; - curves > 80 deg, rigid curves, and those w/ severe pelvic obliquity require combined anterior & posterior arthrodesis; - fusion to the sacrum is required with a fixed pelvic obliquity (eg. with Luque rods to the pelvis - Galveston technique); - kyphosis is also common and may require fusion & instrumentation;