Cerebral palsy Flashcards by Sally-Anne Phillips

Define Cerebral Palsy?

A Nonprogressive Upper motor neurone disease (Static encephalopathy) due to injury to immature brain
Onset must be before first 2 years of life
leads to muscle imbalance with a mixture of weakness and spasticity

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What is the aetiology of cerebral palsy?

Often not identifible
Prematurity - most common
anxoic injury
prenatal interuterine factors
perinatal infections - toxiplasmosis/rubella/CMV/Herpes simplex/ToRCH
Meningitis
Brain malformations

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What orthopaedic manifestations characterise cerebral palsy?

Primary
- Abnormal Tone
- Loss of motor control
- Impaired balance
Secondary ( growth and spasticity related)
- Muscle contractures
- bony deformities
- Joint subluxation/dislocation
- Scoliosis
- foot deformities

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What is the prognosis of cerebral palsy?

Most reliable predictor for walking is independent Sitting by age 2

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What is the physiological classification of cerebral palsy?

SMAASH
Spastic
- Most common
- velocity- dependent increased muscle tone and Hyperreflexia
- ** slow restricted movement due to simultaneous contraction of agonist/antagonist muscles**
- most amenible to operative tx
- increased muscle tone, hyperreflexia, tone increases with velocity
Mixed
- usually mixed spastic and athethoid
- involves entire body
Athetoid
- charactersed by slow, writhing, involuntary movements
Ataxic
- Characterised by inability to coordinate muscle movements-> unbalanced wide based gait
Hypotonic
- usually precedes spastic or ataxic for 2-3 yrs

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What is the anatomical classification of cerebral palsy?

Quadriplegic
- total body and nonambulatory
- low IQ
- High mortality
Diplegic
- Legs > than arms
- Still ambulatory
- IQ maybe normal ( injury in brain is midline)
Hemiplegic
- Arms and legs on **1 side of body **
- Usually with Spascitity
- Will eventually be able to walk, regardless of TX

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Describe your evaluation of a CP pt?

HX
- Perinatal hx
- Growth and development
- Prior medical tx
- functional status
  - Nutritional status
  - Respiratory function
  - Sitting/standing position
  - Upper/lower extremities function
  - Communication skils
  - Acuity of hearing/vision
EXam
- Musculoskeletal system
  - Motion, tone and strength
  - Hamstring contractures ->decrease lumbar lordosis
  - Hip contractures-> xs lumbar lordosis
- Spine exam
  - look at flexibility of curve
  - spinal balance and shoulder height
  - pelvic obliquity

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What does MRi of brain in cerebral palsy show?

Periventricular leukomalacia

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What is the general tx of cerebral palsy pts?

Non operative

PT, Bracing/orthotics and medication for spasiticity

Operative

Surgery to improve function should be considered in child > 3years with spasticity and volunary muscle control

Mutlilevel soft tissue procedures
- preform early < 5 years
- techniques
  - Tenotomies for continuously active muscles ( hip adductors)
  - Tendon lengthening - Achilles/hamstring
  - tendon transfers for muscles firing out of phase ( rectus tendon/tibialis posterior)
- Bony procedures- scoilosis/pelvic osteotomies later in life >5 yrs
- Rhizotomy
  - Neurosurigcal resection of dorsal roolets that do not show a myographic or clinical response to stimulation
  - for child 4-8 ambulatory spastic diplegic and stable gait pattern that is limited by lower extremity spasticity

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What medication is used to tx spasticity in cerebral palsy?

Botox
- Botulinum - toxin A ( acetylcholinesterase)
- Competitive inhibitor of presynaptic cholinergic receptors (decreases acetylcholine) with a finite lifetime ( lasts 2-3 months)
- used to maintain joint motion during rapid growth when a child is too young for surgery
- Often injected into gastronemius
Baclofen
- reduced tone via unknown mechanism
- though to act as a agonist presynaptic GABA ( gamma-aminobutyric acid) occurs primarly at spinal cord to inhibit monosynaptic ?polysynaptic reflexes
- intra-thecal administration is preferred route to avoid cognitive impairment seen with oral administration

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Describe the epidemiology of scoliosis in cerebral palsy?

Overall incidence 20%
More severe the cerebral palsy the more likely of scoliosis
Spastic quadriplegic highest risk- esp if no ablity to sit
bedridden children approach 100% scoliosis
spinal deformity rare in ambulatory cerebral palsy pt

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What is the difference between cerebral palsy scoliosis and idiopathic scoliosis?

More likely to progress
- 1-2^o per month starting at 8/10 years
Curve begins at earlier age
Curve is long, C shaped
Bracing is less effective

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What is the aetioogy of scoliosis in cerebral palsy?

Muscle weakness and truncal imbalance implicated but little evidence
Pelvic obliquity -> deforming forces on spine and scoliosis

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What is the natural hx of scoliosis in cerebral palsy?

Larger the curve mre likely to progress
larger curves associated with pelvic deformity and obliquity

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What is the classification of cerebral palsy scoliosis?

Weinstein
Group 1- Double curves with thoracic/lumbar component & minimal pelvic obliquity
Group 2- large lumbar or thoracolumbar curves with marked pelvic obliquity

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What imaging is useful in cerebral palsy scoliosis?

Do standing and erect films wherever possible
Standing AP and lateral
- look for rib deformity, wedging, spinal rotation
- look for spondylolithesis on lateral (4-21% w spastic diplegia)
Bending films
- evaluate flexibility of curve
MRI- not routinely used for spinal surgery unless sudden progression in scoliosis/changes in neuro exam

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What is the non op tx for scoliosis in cerebral palsy?

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Non operative

Observation, custom seat/bracing, botox injections
- for non progressing curves <50^o
- early stages in pts <10 years
- goal is to delay surgery until older
- outcomes
  - custom seat orthosis- helpful but not change Natural course of disease
  - bracing
    - TLSO helpful to improve sitting balance, doesn’t effect course of disease
    - slow progression in skeletal immature pts
  - Botox
    - short term benefit, lasts 2-3 mo
    - presynaptic cholinergic receptor inhibitor

What are the surgical tx of scoliosis in cerebral palsy?

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Goals of surgery
- obtain painless solid fusion with well corrected , well balanced spine with level pelvis
- decision must inlcude family goals and risk- benefit analysis
Posterior spinal fusion +/- extension to pelvis
- group 1 curves 50^o-90^o in ambulators that is progressive and interfering with sitting
- Pt > 10 years
- adequate hip rom
- stable nutritional ad medical status
PSF +/- ASF +/- pelvic extension
- Group 1 curves >90o + non ambulators
- group 2 curves

When would fusion be extended to include the pelvis?

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Pelvic Obliquity >15o
required due to increased pseudoarthrosis rate if you don’t!!

Describe the surgical technique for PSF in cerebral palsy pt with scoliosis?

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Proximal fusion should extend to T1/T2 - otherwise risk of proximal thoracic kyphosis
Distal fusion level depends on curve pattern
- due to long curve often extends to L4/5
- extends to pevis when obliquity is >15^o
Pedicle screw fixation technique
- may provide better correction & eliminate need for anterior surgery
unit rod with sublaminar wire technique

What are the complications of spinal surgery in cerebral palsy scoliosis?

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Implant failure
- maybe asymptomatic and may not require tx
- includes penetration of pelvic limb of unit rod into pelvis
pulmonary complications
- Chronic aspiration
- pulmonary insufficiency most common
- pneumonia
GI complications
- GERD
- poor nutrition and dleayed growth
Neurological complications
- Seizures
Wound Infection
- More common in CP than in idopathic scoliosis
- occurs in 3-5% and usually can be tx with local wound debridment alone
Death 0-7%

What is the epidemiology of cerebral palsy hip conditions?

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Progressive hip subluxation occus in up to 50% of children with spastic quadriparesis

What is the pathoanatomy of cerebral palsy hip conditions?

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Subluxation
- Strong tone in hip adductor and flexor-> scissoring and prediposes to hip subluxation and dislocation
Dislocation
- typically posterior and superior
Degeneration
- in time dysplastic and erosive changes in cartilage of femoral head can develop -> pain

What is the prognosis of hip conditions in cerebral palsy?

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Grade of hip subluxation is correlated with GMFCS level ( gross motor function classification system)
- minimal in level 1
- 90% level V-
Natural hx studies have shown that hips will dislocate in the absence of tx if Reimers index >60-70%

What is the tx for a hip at risk, Hip abduction \<45 ^o, reimers index \<33%?

* Attempt to prevent dislocation with ADDUCTOR release + poas release + abduction bracing avoid obturator neurectomy

what is the tx for hip subluxation, in cerebral palsy, Reimer's index \>33%, disrupted Shenton's line?

* Tx with ADDUCTOR tenotomy if abduction restricted * Consider proximal femur and pelvic osteotomies if significant dysplasia present

What is the tx of spastic dislocation, Reimer index \> 100%, frankly dislocated hip?

* Open reduction with varus derotational osteotomy +/- femoral shortening and pelvic osteotomies ( dega) * varus osteotomy to correct increased valgus and anteversion

What is the tx of windswept hips, abduction of one hip & adduction of contralteral hip?

* Brace adducted hip with/w out tenotomy * release abduction contracture of abducted hip

What is another tx of a painful dislocation?

* Abduction osteotomy or girdlestone procedure * abduction osteotomy pulls proximal femur further away from pelvis and decreases the pain produced from forces on the ilium * girdlestone - proximal femoral resection-\> floppy but painless leg

What are the goals when doing an adductor and posas release and abductor bracing?

* Prevent hip subluxation/dislocation * maintain comfortable seating * facilate care and hygiene * in older children soft tissue corection not enough need bony correction

Describe the 3 types of gait pattern in cerebral palsy

* **Toe walking gait** * common in **hemiplegic** * **crouched gait** * common in **diplegic** * **hamstring contracture-\> hip flexion, knee flexion and ankle equinus** * **stiff- knee gait** * **common in spastic diplegic CP** * limited _knee flexion in swing phase due to rectus femoris firing out of phase_

What is the tx of toe walking gait?

* [http://www.orthobullets.com/video/view?id=49](http://www.orthobullets.com/video/view?id=49) * Non operative * **Ankle foot orthosis** * _flexible deformities_- foot is passively correctable to neutral * posterior leaf spring ankle-foot orthoses used in presence of **absent heel strike and minimal dorsiflexion** , it controls excessive ankle plantar flexion in the swing phase, allows ankle dorsiflexion midstance- see pic * Surgery * **Tendo-achilles lengthening** * for _rigid deformities_- foot not passively correctable to neutral * Gastronemius recession vs TA lengthening * Silfverskiold test * goal to obtain 10^o Dorsiflexion

What is the tx of crouching gait?

* [http://www.orthobullets.com/video/view?id=50](http://www.orthobullets.com/video/view?id=50) * **Multiple simultaneous soft tissue releases** - hip, knee, ankle * \*\*\*isolated heel cord lengthening in presence of tight hamstrings and hip flexors-\> progressive flexion of hips and knees so worsening the crouching gait\*\*\*\* * Complications * hamstring contracture most likely * patella alar

What is the tx of stiff knee gait?

* **Transfer of rectus femoris tendon** * **EMG with gait analysis** * *shows quadriceps activity from terminal stance throughout swing phase**

What can tx of CP upper extremity conditions be divided into?

* Hygenic procedures * Functional procedures

Decribe the characteristic CP upper limb deformities?

* **Shoulder internal rotation contracture** * **forearm pronated/ elbow flexion deformity** * **wrist-flexion deformity** * **thumb in palm deformity** * **finger flexion deformity**

What is the tx for glenohumeral internal rotation contracture?

* **Shoulder derotational osteotomy +/- Subscapularis and pectoralis lengthening with biceps/brachialis capsulotomy** * for severe contractures \>30 degrees interfering with hand function

Describe the tx of elbow flexion contractures in CP?

* **Lacertus fibrosis release** * **biceps and brachialis lengthening** * **brachioradialis origin release**

What is the tx of forearm pronation in CP?

* **Pronator teres release** * **transfer to an anterolateral position** that can lead to supination

How is wrist flexion tx in CP?

* The wrist is flexed and ulnarly deviated * **Wrist Arthrodesis** * as hygenic procedure in low funtioning pts * **FCU to ECRB transfer** or * **FCU to EDC** transfer * as functional procedure in pts with voluntary control, IQ 50-70 or higher/ better sensibility

How is thumb in palm deformity tx in CP?

* Fixed thumb into palm prevents grasping and pinching activites * can preclude to inappropriate hygiene * doen in pt w IG 50-70 or higher, voluntary control * Release or Lengthening * adductor pollicis * first dorsal interosseus * Flexor pollicis brevis * flexor pollcis longus * web space deepening plasty

What are the tx aims in foot surgery for CP? describe the types of foot deformity in CP?

* A **plantigrade, painless, braceable foot** **** * **Equinovarus** * **Equinovalgus** * **cavus foot and hallux clawing** * **hallux valgus** * **1st MT arthrodesis** * highest success rate cf other surgeries in ambulatory/ nonambulatory children w CP * **proximal akin osteotomy**

Cerebral palsy Flashcards

(42 cards)