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 Classification Physiologic Classification Spastic Most common Velocity-dependent increased muscle tone and hyperreflexia with slow, restricted movement due to simultaneous contraction of agonist and antagonist muscles. Most amenable to operative treatments. Athetoid Characterized by a constant succession of slow, writhing, involuntary movements Ataxic Characterized by inability to coordinate muscle movements. Results in unbalanced, wide based gait. Mixed Usually mixed spastic and athetoid features and involves the entire body Hypotonic Usually precedes spastic or ataxic for 2-3 years Anatomic Classification Quadriplegic Total body involvement and nonambulatory Diplegic Legs more than arms but usually still ambulatory. IQ may be normal (injury in brain is midline) Hemiplegic Arms and legs on one side of the body, usually with spasticity Will eventually be able to walk, regardless of treatment Gross Motor Function Classification Scale (GMFCS) Type I Near normal gross motor function, independent ambulator Type II Walks independently, but difficulty with uneven surfaces, minimal ability to jump Type III Walks with assistive devices Type IV Severely limited walking ability, primary mobility is wheelchair Type V Nonambulator with global involvment, dependent in all aspects of care 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