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Paediatrics FCRS > Cerebral palsy > Flashcards

Cerebral palsy Flashcards

1
Q

Define Cerebral Palsy?

A
  • 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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2
Q

What is the aetiology of cerebral palsy?

A
  • 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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3
Q

What orthopaedic manifestations characterise cerebral palsy?

A
  • 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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4
Q

What is the prognosis of cerebral palsy?

A
  • Most reliable predictor for walking is independent Sitting by age 2
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5
Q

What is the physiological classification of cerebral palsy?

A
  • 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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6
Q

What is the anatomical classification of cerebral palsy?

A
  • 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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7
Q

Describe your evaluation of a CP pt?

A
  • 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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8
Q

What does MRi of brain in cerebral palsy show?

A
  • Periventricular leukomalacia
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9
Q

What is the general tx of cerebral palsy pts?

A

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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10
Q

What medication is used to tx spasticity in cerebral palsy?

A
  • 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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11
Q

Describe the epidemiology of scoliosis in cerebral palsy?

A
  • 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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12
Q

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

A
  • More likely to progress
    • 1-2o 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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13
Q

What is the aetioogy of scoliosis in cerebral palsy?

A
  • Muscle weakness and truncal imbalance implicated but little evidence
  • Pelvic obliquity -> deforming forces on spine and scoliosis
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14
Q

What is the natural hx of scoliosis in cerebral palsy?

A
  • Larger the curve mre likely to progress
  • larger curves associated with pelvic deformity and obliquity
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15
Q

What is the classification of cerebral palsy scoliosis?

A
  • 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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16
Q

What imaging is useful in cerebral palsy scoliosis?

A
  • 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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17
Q

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

A

Non operative

  • Observation, custom seat/bracing, botox injections
    • for non progressing curves <50o
    • 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
18
Q

What are the surgical tx of scoliosis in cerebral palsy?

A
  • 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 50o-90o 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
19
Q

When would fusion be extended to include the pelvis?

A
  • Pelvic Obliquity >15o
  • required due to increased pseudoarthrosis rate if you don’t!!
20
Q

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

A
  • 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 >15o
  • Pedicle screw fixation technique
    • may provide better correction & eliminate need for anterior surgery
  • unit rod with sublaminar wire technique
21
Q

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

A
  • 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%
22
Q

What is the epidemiology of cerebral palsy hip conditions?

A
  • Progressive hip subluxation occus in up to 50% of children with spastic quadriparesis
23
Q

What is the pathoanatomy of cerebral palsy hip conditions?

A
  • 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
24
Q

What is the prognosis of hip conditions in cerebral palsy?

A
  • 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%
25
Q

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

A
  • Attempt to prevent dislocation with ADDUCTOR release + poas release + abduction bracing avoid obturator neurectomy
26
Q

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

A
  • Tx with ADDUCTOR tenotomy if abduction restricted
  • Consider proximal femur and pelvic osteotomies if significant dysplasia present
27
Q

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

A
  • Open reduction with varus derotational osteotomy +/- femoral shortening and pelvic osteotomies ( dega)
  • varus osteotomy to correct increased valgus and anteversion
28
Q

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

A
  • Brace adducted hip with/w out tenotomy
  • release abduction contracture of abducted hip
29
Q

What is another tx of a painful dislocation?

A
  • 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
30
Q

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

A
  • Prevent hip subluxation/dislocation
  • maintain comfortable seating
  • facilate care and hygiene
  • in older children soft tissue corection not enough need bony correction
31
Q

Describe the 3 types of gait pattern in cerebral palsy

A
  • 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
32
Q

What is the tx of toe walking gait?

A
  • 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 10o Dorsiflexion
33
Q

What is the tx of crouching gait?

A
  • 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
34
Q

What is the tx of stiff knee gait?

A
  • Transfer of rectus femoris tendon
  • EMG with gait analysis
  • *shows quadriceps activity from terminal stance throughout swing phase**
35
Q

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

A
  • Hygenic procedures
  • Functional procedures
36
Q

Decribe the characteristic CP upper limb deformities?

A
  • Shoulder internal rotation contracture
  • forearm pronated/ elbow flexion deformity
  • wrist-flexion deformity
  • thumb in palm deformity
  • finger flexion deformity
37
Q

What is the tx for glenohumeral internal rotation contracture?

A
  • Shoulder derotational osteotomy +/- Subscapularis and pectoralis lengthening with biceps/brachialis capsulotomy
  • for severe contractures >30 degrees interfering with hand function
38
Q

Describe the tx of elbow flexion contractures in CP?

A
  • Lacertus fibrosis release
  • biceps and brachialis lengthening
  • brachioradialis origin release
39
Q

What is the tx of forearm pronation in CP?

A
  • Pronator teres release
  • transfer to an anterolateral position that can lead to supination
40
Q

How is wrist flexion tx in CP?

A
  • 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
41
Q

How is thumb in palm deformity tx in CP?

A
  • 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
42
Q

What are the tx aims in foot surgery for CP?

describe the types of foot deformity in CP?

A
  • 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

Paediatrics FCRS (27 decks)

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