Definition
CP is a permanent non-progressive disorder of movement and posture secondary to brain injury
Incidence
2-3 per 1000 live births
Etiology
Highly associated
- prematurity < 37 weeks
- low birth weight < 2.5 kg
| Prenatal | Perinatal | Postnatal |
|---|---|---|
|
Maternal infection (TORCHS) - Toxoplasmosis - Rubella - Cytomegalovirus - Herpes - Syphilis
Maternal exposure to drugs / alcohol |
Difficult delivery and hypoxia - prolong labor - vacuum / forceps delivery - meconium aspiration
Hypoxia |
Meningitis
Head injury
Immersion |
Classification
Highly variable depending on extent of brain injury
Spastic diplegia most common / 35% of all cases
Pathophysiology classification
| Spastic (80%) | Dyskinetic (15%) | Ataxic (5%) |
|---|---|---|
|
Increased muscle tone Increased reflexes Develop contractures
|
Involuntary uncontrolled movements May have hyper or hyptonia
|
Poor co-ordination Wide gait Hypotonia |
Anatomic
| Diplegia (60 - 70%) | Hemiplegia (20 - 30%) | Quadriplegia (10 - 15%) |
|---|---|---|
| Lower limbs > upper limbs |
One side of body Usually upper limb > lower limb |
Extensive involvement all limbs |
| Prematurity | Obstetric complication | Cerebral anoxia |
|
Delayed milestones Delayed walking |
Abnormal walking May have seizures |
Bulbar palsy / choking with feed Epilepsy Severe Intellectual abnormality |
| 75% walk | 100% walk | 25% walk |
GMFCS / Gross motor function classification system
| Level | Function |
|---|---|
| Level 1 | Normal but slow gait |
| Level 2 |
Difficulty walking long distances Hand rail up stairs |
| Level 3 |
Walker inside Wheelchair outside |
| Level 4 |
Wheelchair Can sit without support |
| Level 5 |
No head support Need supports to sit |
Presentation / Diagnosis
Quadriplegia
- rigidity
- difficulty feeding / swallowing
- seizures
Can be more difficult with hemiplegia / diplegia
- delayed milestones
- presence of primitive reflexes after normal period
- hyper / hypotonia
- not using one hand - hemiplegia
Pediatric neurologist / MRI
Examination
Back - scoliosis
Hypertonicity
- increased tone / clonus
- spasticity - difference between R1 (slow ROM) and R2 (fast ROM)
Hips
- tight psoas - FFD hip / Thomas test
- tight adductors
- ITB - Obers' test
Knee
- hamstrings - FFD knee
- quadriceps - Ely test: prone, unable to flex knee with hip extended
Foot
- tight tendoachilles / equinus
- Silverskiold test (if gastrocnemius tight, reduced ankle dorsiflexion with knee extended versus flexed)
Upper limb
- thumb in palm
- flexed fingers / swan neck deformity
- elbow and shoulder contactures
Gait patterns
Scissoring - tight adductors
Asymmetrical arm swing - hemiplegia
Short leg gait - hip dislocation
Jump gait: hip flexion / knee flexion / ankle equinus
Crouch gait: hip flexion / knee flexion / ankle dorsiflexion
Management
Guidelines
Cerebral palsy clinics / multidisciplinary teams
Single Event Multi Level surgery (SEML)
- avoid birthday syndrome (where children are operated on every year)
- surgery between 4-8 years before fixed deformity
Orthopedic issues
| Hip | Knee | Foot & Ankle | Upper limb |
|---|---|---|---|
|
Hip dislocation Adductor contractures Flexion contractures In-toeing Windswept hips |
Hamstring contractures Rectus femoris contractures |
Equinus Equinovarus Valgus |
Thumb - in palm deformity Fingers - swan-neck +/- flexed Wrist - flexed + pronated Elbow - flexed + pronated Shoulder - adducted and internally rotated
|
Scoliosis
- most common in spastic quadriplegia
- sitting issues / hygiene / respiratory issues / feeding issues
www.boneschool.com/pediatrics/neuromuscular-scoliosis
Nonoperative management
Physiotherapy
Clutterbuck et al Disability Rehabilitation 2019
- systematic review of active exercises in ambulant / semi-ambulant CP
- evidence of improved gross motor function
Bracing
Ankle Foot Orthosis (AFO)
- systematic review of AFOs in CP
- strong evidence for improvements in gait and gross motor function
Hand splints
Jackman et al Dev Med Child Neurol 2014
- systematic review of hand splints for CP
- evidence of small benefit of wearing splint
- benefit disappears with removal of splint
Botox
Indication
Dynamic or spastic deformity (R1 v R2)
Technique
Botulinum Toxin A every 6 months to musculotendinous juntion
- peaks at 4 weeks
- lasts 3-4 months
Results
- systematic review of Botox for lower limb spasticity in CP
- evidence of reduction in spasticity
- unclear evidence with regards functional gains
- systematic review of 15 RCTs using Botox for upper limb spasticity in CP
- evidence of reduction in spasticity
- unclear evidence with regards functional gains
Intrathecal Baclofen
Indication
GABA analogue via implantable pump
Aid to reduce spasticity
Results
- systematic review of intra-thecal baclofen in CP
- 40% reduction in spasticity
- risk of infection / pump complications / increased seizures
Selective dorsal rhizotomy
Indication
Spastic diplegia / ambulatory patients
- cut sensory nerves and decrease feedback from stretch receptors
- aim to reduce spasticity and aid walking
? benefit in spastic quadriplegia / GMFCS 4 and 5
Technique
Laminectomy at level of conus medullaris
- stimulate rootlets
- cut 50 - 70% of dorsal rootlets / L1 L2 sensory nerves
Results
Dudley et al J Neurosurg Pediatr 2013
- selective dorsal rhizotomy in 102 patients with cerebral palsy
- evidence of long lasting improvement in GMFCS Level 1, 2 and 3
Complications
Mishra et al Neurochirurgie 2023
- systematic review of complications after selective dorsal rhizotomy
- scoliosis / kyphosis
- constipation / hip dislocation
- sensory change / incontinence