SUMMARY

• Cerebral Palsy is a common congenital condition caused by injury to the immature brain that leads to upper motor neuron disease and presents with cognitive and musculoskeletal manifestations of varying severity.

• Diagnosis is made clinically with evaluation of developmental milestones, cognitive function, and musculoskeletal abnormalities including spasticity, loss of motor control, and impaired balance.

• Treatment involves a multidisciplinary approach to address spasticity, orthopedic manifestations, and cognitive function. 

EPIDEMIOLOGY

• Incidence

  • 2-3 per 1000

• Demographics

  • by definition onset must be before first two years of life, although diagnosis may be delayed in very mild cases

  • most common cause of chronic childhood disability

ETIOLOGY

• Nonprogressive upper motor neuron disease (static encephalopathy) due to injury to immature brain

  • orthopaedic manifestations

    • contractures (this topic)

    • fractures (this topic)

    • upper extremity deformities 

    • hip subluxation and dislocation 

    • spinal deformity 

    • foot deformities 

    • gait disorders 

• Pathophysiology

  • pathoanatomy

    • leads to muscle imbalance with a mixture of weakness and spasticity

    • the encephalopathy is static while the affected portion of the musculoskeletal system changes with growth

  • risk factors

    • prematurity (most common)

    • anoxic injuries

    • prenatal intrauterine factors

    • perinatal infections

      • toxoplasmosis

      • rubella

      • cytomegalovirus infection

      • herpes simplex

      • ToRCH

    • meningitis

    • brain malformations

    • brain trauma - NAT

• Associated conditions

  • orthopaedic manifestations characteristic of CP

    • primary

      • abnormal tone

      • loss of motor control

      • impaired balance

      • spasticity

      • (hypotonia)

      • (dyskenesias such as chorea and athetosis)

    • secondary (growth and spasticity related)

      • contractures

        • starts as dynamic contractures, become static with time (continuous muscle contraction results in shortening) and growth (growth of bones occurs at a faster longitudinal rate than muscles in spastic cerebral palsy)

      • upper extremity deformities

      • hip subluxation and dislocation

      • spinal deformity

      • foot deformities

      • gait disorders

      • fractures

        • often associated with non-ambulators secondary to low bone mineral density

        • bisphosphonates may be useful

          • IV pamidronate considered with >3 fractures and a DEXA z-score <2 SD

EVALUATION

• History

  • clinical history

    • perinatal history

    • growth & development

    • prior medical treatments

  • functional status 

     

    • sitting/standing posture

    • upper and lower extremities function

    • communication skills

    • acuity of hearing and vision

• Physical exam

  • general musculoskeletal exam

    • motion, tone, and strength

    • Rotational limb profiles for torsional deformities

  • gait

    • gait lab analysis

    • plantigrade feet

    • crouch

    • stiff knee gait

  • spine exam 

    • presence and flexibility of scoliosis

    • spinal balance and shoulder height

    • pelvic obliquity

    • resting head posture

    • hamstring contractures (lead to decreased lumbar lordosis)

  • hips 

    • hip contractures

      • flexion contracture (lead to excessive lumbar lordosis)

      • adduction contracture

    • hip instability and dislocations are common, may be looked over as a contracture alone

      • observe thigh length in sitting, leg length when supine, or galleazzi test

      • adductor contracture can make examination difficult

  • foot and ankle 

    • equinovarus and planovalgus deformities common

    • observe wear patterns, callouses

    • note hypertonicity

    • toe walking or absent heel strike during gait secondary to gastrosoleus spasticity and contracture

      • may hyperextend knee to obtain heel contact 

    • provacative Silverskiold test to differentiate gastrocnemius contracture vs achilles contracture

IMAGING

• Radiographs

  • standard radiographs should include

    • AP and lateral of hips

    • standing spine radiographs as baseline

• MRI

  • MRI of brain shows a spectrum of changes including

    • periventricular leukomalacia (PVL) white matter lesions most frequent (56%)

    • while grey matter lesions (18%)

    • brain malformations are less frequent (9%)

TREATMENT SPASTICITY & DEFORMITY

• Nonoperative

  • physical therapy, bracing/orthotics, medications for spasticity

    • spasticity control

      • Botox (botulinum - A toxin)

        • competitive inhibitor of presynaptic cholinergic receptors with a finite lifetime (usually lasts 2-3 months) 

           

        • used to maintain joint motion during rapid growth when a child is too young for surgery

        • often injected into gastrocnemius

        • helpful treatment in dynamic contractures; little benefit with static contractures

      • baclofen

        • reduces tone via unknown mechanism

          • thought to act as GABA agonist

          • intra-thecal administration is preferred route to avoid cognitive impairment seen with oral administration (poor bioavailability leads to difficulty with oral dosing)

          • Intra-thecal baclofen tends to be used for non-ambulatory CP (GMFCS IV and V)

        • side effects

          • baclofen withdrawal

            • can cause severe fevers, hyperkalemia, and elevated CPK levels 

               

• Operative

  • soft tissue procedures/releases

    • indications

      • to improve function in child from 3-5 years of age with spasticity and voluntary muscle control

    • techniques

      • tenotomies for continuously active muscles (e.g. hip adductor)

      • tendon lengthening for continuously active muscles (e.g. achilles tendon or hamstring)

      • tendon transfers for muscles firing out of phase (e.g. rectus tendon or tibialis posterior)

        • tendon transfers in the upper extremity show the best improvement in function in patients with voluntary motor control 

           

  • selective dorsal rhizotomy

    • indications 

       

      • ages 4 to 8, ambulatory spastic diplegia, and a stable gait pattern that is limited by lower extremity spasticity

    • neurosurgical resection of dorsal rootlets that do not show a myographic or clinical response to stimulation

    • contraindications

      • athetoid CP

      • nonambulatory patients with spastic quadriplegia (associated with significant spinal deformities)

    • falling out of favor due to limited functional gains and no reduced risk of subsequent musculoskeletal surgeries

  • bony procedures/deformity correction

    • indications

      • usually performed in later childhood / adolescence

      • static contractures, progressive joint breakdown, and certain patterned gait-deterioration can be treated with combinations of myotendonous unit lengthening, tendon transfers, and osteotomies

    • SEMLS surgery (Single-Event, Multi-Level Surgery)

      • concept arose to limit multiple surgeries, anesthetics, and rehabilitation time for children

      • most successful when combined with a thorough gait lab assessment that predicts improvemenet in function with multiple level surgical interventions

      • simple lengthenings can cause deterioration in gait when other contractures are "uncovered"; SEMLS management seeks to avoid these iatrogenic complications

      • can be done on bilateral lower extremities in efforts to improve gait

    • specific procedures

      • see Cerebral Palsy Upper Extremity Conditions 

      • see Cerebral Palsy Spine Conditions 

      • see Cerebral Palsy Hip Conditions 

      • see Cerebral Palsy Gait Disorders 

      • see Cerebral Palsy Foot Disorders 

PROGNOSIS

• Most reliable predictor for ability to walk is independent sitting by age 2