Cerebral Palsy

Key Points:

  • Non-progressive disorder related to a brain insult in the prenatal, perinatal, or postnatal times of development. 
  • Multiple forms of presentation including quadriplegic, diplegic, hemiplegic, and mixed.  
  • In a spastic patient with the potential for ambulation, the hallmark of treatment is bracing and stretching through growth spurts to diminish spasticity. Surgery may be needed for cases refractory to bracing. 
  • Hip contractures may lead to hip subluxation or dislocation, and pelvis radiographs should be obtained and followed closely in at risk patients. Hip dislocation in CP is different than developmental dysplasia of the hip. Surgical intervention may be indicated in order to contain the hips.
  • Neuromuscular scoliosis is common in patients with spastic quadriplegia and is different from adolescent idiopathic scoliosis. Surgical intervention for quadriplegic CP is debated. 

Description:

Cerebral Palsy is a broad diagnosis with a wide range of clinical manifestations that primarily involves the musculoskeletal, neurological, gastrointestinal, and genitourinary systems (Karol, 2013; Renshaw, 2006; Flynn, 2002; McCarthy, 2006; Karol, 2004; Lomita, 2010). Although some patients with spastic or dystonic movements are grouped into the cerebral palsy category, a true diagnosis of cerebral palsy involves the presence of a brain lesion that is fixed and nonprogressive (Karol, 2013; Renshaw, 2006; Flynn, 2002; McCarthy, 2006; Karol, 2004; Lomita, 2010). The hallmark of CP is uncoordinated movements with the presence of spasticity (Renshaw, 2006).  
 
Recent research on spastic paraparesis has led to an increased understanding of this neuromuscular subtype and its identification as a separate entity from CP (Karol, 2013). Some spastic paraparesis patients have been inaccurately classified as CP. Most spastic paraparesis patients have a genetic etiology for the disease, will usually have a normal brain MRI, may walk with a head bob, and have gait patterns that respond differently than a CP patient (Karol, 2013). 

Epidemiology:

The incidence of CP has most recently been reported as between 2.4 and 2.7 per 1000 live births (Karol, 2013; Renshaw, 2006; Flynn, 2002; McCarthy, 2006; Karol, 2004; Lomita, 2010). In a fullterm baby, the risk is about 1 in 2000 live births (Karol, 2013). About half of patients born with CP will have a low birth weight, and just over a quarter percent will weigh under 1500g (Karol, 2013). 

Clinical Findings:

Cerebral palsy is associated with a wide array of musculoskeletal manifestations that include spastic, dystonic, or hypotonic movements. There are a number of specific types of CP that are based on the neuropathic or anatomic patterns.  
 
Neuropathic types include spastic, athetoid, ataxic, or mixed. The most common type is spastic, which involves upper motor neuron abnormalities that lead to weakness and rigidity. Athetoid is a writhing type motion that is due to an extrapyramidal brain lesion. Ataxic subtypes lead to incoordination with tremors usually due to a dysfunction in the cerebellum. (Karol, 2013; Renshaw, 2006). 
 
Anatomic patterns are defined as quadriplegic (all four limbs), diplegia (usually both lower extremities), and hemiplegic (with the upper limb more involved than the lower limb). Patients with quadriplegia often have mental retardation, difficulty sitting, and lack of head control; however, specific manifestations of quadriplegia differ across each patient. Diplegic CP is often associated with the presence of an abnormal third ventricle and periventricular leukomalacia on a brain MRI. Diplegic CP patients are usually intellectually normal. Hemiplegic patients are more likely to have had a focal event leading to the disorder; ranging from a vascular to traumatic event. 
 
CP patients are commonly given a classification from 1 to 5 on the Gross Motor and Functional Classification System (GMFCS).  
* GMFCS 1 – ambulates independently without an assistive device, able to jump and climb stairs. Speed and balance may be limited 
* GMFCS 2 – independent ambulator with difficulty navigating uneven surfaces or stairs 
* GMFCS 3- ambulates with the use of an assistive device such as a crutch or walker. May use a wheelchair for long distances 
* GMFCS 4- primarily mobilize with a wheelchair, but able to use an assistive device in short distances with help 
* GMSCF 5-  wheelchair bound, difficulty with head and trunk control, total care required
 
* Milestones: Patient’s with CP may have delayed milestones; adjustments in milestone timing should be made in a premature patient. Apparent handedness in a young child may be an early sign that CP is present.  
 
* Hips: patients with CP are usually born with normal hips,but they develop abnormalities as a result of their muscle imbalance (Flynn, 2002). The muscle imbalance leads to alterations in bony development, which creates worsening dysplasia. CP hip dysplasia is different from developmental dysplasia of the hip. Hip dysplasia in CP may be found in 2-75% of patients, with those patients having more involved forms being most likely to develop hip abnormalities (Flynn, 2002). The femur is typicallyanteverted with coxa valga, and the spastic hip adductors and hip flexors along with the femur abnormalities lead to mispositioning in the acetabulum. A misshapen femoral head develops as a result of the osseous and muscular alterations. Most commonly, there will be a deficiency in the acetabulum along the posterosuperior aspect. On exam, hip abduction, extension, flexion, and adduction should be assessed. The Galeazzi sign should be tested. Sitting balance and pelvic obliquity should also be assessed. 
 
* Spine: Scoliosis occurs on average in 20% of patients with CP. In non-ambulatory patients, the incidence is closer to 60% and it approaches 100% in totally involved children (McCarthy, 2006). Whereas patients with adolescent idiopathic scoliosis generally have S-shaped curves, CP scoliosis is usually a long, sweeping, C-shaped curve that leads to rapid progression with deteriorating function (McCarthy, 2006). Unlike idiopathic scoliosis, neuromuscular scoliosis with spasticity cannot be controlled with brace treatment. The presence of scoliosis in CP may worsen hip subluxation/dislocation and pelvic obliquity. Surgical indications for CP are highly debated. If surgery is performed, pelvic fixation is usually included to improve sitting balance.  
 
* Lower extremity and gait: Ankle equinus is common in patients with CP. Further, equinovarus is more prevalent in the hemiplegic subtype and equinovalgus in the spastic diplegic or quadriplegic subtypes [Karol, 2004]. Hamstring, adductor, and hip flexor contractures are also common. The Silfverskiold Test should be performed to assess for gastrocnemius versus Achilles tightness. The popliteal angles should also be measured to quantify hamstring tightness. The transmalleolar axis or thigh foot axis can also be assessed to determine if concomitant bony abnormalities are contributing to the deformities along with the muscle tightness. Surgical intervention may be indicated in order to improve contractures, including gastrocnemius recession, adductor lengthening, and hamstring lengthening (Karol, 2004). Surgical decision making is complex. Botox injections may also be used, in addition to serial casting, to improve heel-cord contractures (Koman, 2000). Botox can also be used for hamstring and adductor contractures. The use of Botox is controversial, and the long-term risks are unknown. 
 
* Upper extremities: when assessing the CP patient, it is important to look at active and passive upper extremity motions. Patients commonly present with internal rotation contractures of the shoulder, and flexion contractures of the elbow and wrist. Forearm pronation and thumb-in-palm of the hand are also common (Lomita, 2010). Sensory exams and proprioception are also important to assess, as deficits are often present (Lomita, 2010). Surgical planning is complex and related to the contractures that are present, the muscles that are functioning, and the age of the patient. Sometimes Botox may be injected to assist with decreasing contractures (Lomita, 2010). The use of Botox in the upper extremity is controversial, as it may exacerbate muscle weakness (Van Heest, 2015). 

Imaging Studies:

Patients presenting to the orthopedic office will likely have had an MRI ordered from a pediatrician or neurologist. The brain MRI may show periventricular leukomalacia, brain malformations, or white or gray matter lesions.  
 
Radiographs of the hips and spine should be obtained during growth to assess for hip subluxation or dislocation and scoliosis. Hip radiographs should be obtained twice a year from age 2 to eight (Flynn, 2002).  On a pelvic x-ray the acetabular index and the migration index should be assessed. There is no consensus regarding the appropriate frequency with which to take a spine radiograph, or the best positioning (sitting, standing, supine) to take a spine radiograph [McCarthy, 2006]. Radiographs should, however, be repeated in the same positioning each time to assess for comparable changes.  
 
Gait lab analysis is useful for surgical planning in the ambulatory CP patient.

Etiology:

Specific causes of cerebral palsy are unknown; however, the etiology of the diagnosis is associated with many risk factors (Renshaw, 2006). Cerebral palsy commonly occurs in the prenatal, perinatal, or postnatal environments [Karol, 2013; Renshaw, 2006]. Prenatally, cerebral palsy may result from infections, maternal ingestion of illicit or non-illicit drugs, non-compatible rhesus blood group, epilepsy, alcohol abuse, hyperthyroidism, incompetent cervix or congenital brain malformations (Karol, 2013; Renshaw, 2006; Flynn, 2002; McCarthy, 2006; Karol, 2004; Lomita, 2010). The TORCHES group of infections (toxoplasmosis, rubella, cytomegalovirus, herpes, and syphilis) affect a child in-utero, and may lead to orthopedic manifestations of cerebral palsy (Karol, 2013).  
 
Perinatal complications are a second time period where complications may lead to cerebral palsy. Some specific associations with CP in the perinatal time period include an anoxic brain injury, multiple births, kernicterus, premature birth, placental abruption, neonatal sepsis, and extracorporeal membrane oxygenation (Karol, 2013; Renshaw, 2006). 
 
Similar to prenatal causes, postnatal causes of CP include anoxic brain injuries from trauma, drowning, or infection (Karol, 2013; Renshaw, 2006). 

Treatment:

Reviewed above in Clinical Findings

Complications:

Close monitoring of a CP patient is important during growth, when contractures may become worse. A lack of orthopedic monitoring, or interventions when indicated, may lead to progressive contractures that affect sitting ability or ambulation.  This may ultimately increase caregiver burden due to suboptimal trunk, leg, and arm positioning. Painful hip pressure points, inability to provide perineal care, or wear warm shoes/boots in colder climates can be difficult for caregivers. The primary principles of treatment are to balance non-surgical and surgical interventions with the purpose of improving or maintaining a patient’s baseline function. Goals are to prevent complications associated with contractures by stretching, bracing, maintaining supple and plantigrade feet in an ambulator; maintaining or obtaining  reduced hips that are nonpainful; spinal intervention to allow for erect sitting posture; and ultimately to enable a patient to be as high functioning as their diagnosis  allows at the conclusion of growth. 

Video Links:

POSNAcademy
Multiple Videos avaialble at this link
   -VDO
   -Rectus Tranfer
   -UE transfers
   -Femoral Extension Osteotomy
   -Correcting Pelvic Obliquity

References:

  1. Flynn, JM. Management of Hip Disorders in Patients with Cerebral Palsy. Journal of the American Academy of Orthopaedic Surgeons. 2002; 10:198-209 
  2. Karol JA. Surgical Management of the Lower Extremity in Ambulatory Children with Cerebral Palsy. Journal of the American Academy of Orthopaedic Surgeons June 2004;12:196-203 
  3. Karol J. Disorders of the Brain. In: Herring JA., ed. Tachdjian’s pediatric orthopaedics. Ed 5. Elvsier , 2013. E2-e127 
  4. Koman, L, et al. Botulinum Toxin Type A Neuromuscular Blockade in the Treatment of Lower Extremity Spasticity in Cerebral Palsy: A Randomized, Double-Blinded, Placebo-Controlled Trial. Journal of Pediatric Orthopaedics 2010;20(1):108 
  5. Lomita C. Upper Extremity Surgery in Children with Cerebral Palsy. Journal of the American Academy of Orthopaedic Surgeons 2010;18:160-168 
  6. McCarthy JJ. Scoliosis in the Child with Cerebral Palsy. Journal of the American Academy of Orthopaedic Surgeons June 2006; 14(16): 367-375 
  7. Renshaw TS, Deluca PA. Lovell and Winter’s Pediatric Orthopaedics. Ed 6. In: Raymond T. Morrissy, Stuart L. Weinstein, ed. Lippincott Williams & Wilkins; 2006: Chapter 15 Cerebral Palsy. 1-99. 
  8. Van Hest et al. Tendon Transfer Surgery in Upper-Extermity Cerebral Palsy is More Effective Than Botulinum Toxin Injections or Regular, Ongoing Therapy. The Journal of Bone and Joint Surgery. 2015;97(7):529-536 

Top Contributors:

Ashley Startzman DO
Jaclyn Hill MD