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Cerebral Palsy - General

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

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