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Cerebral Palsy

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Ovid: 5-Minute Orthopaedic Consult

Editors: Frassica, Frank J.; Sponseller, Paul D.; Wilckens, John H.
Title: 5-Minute Orthopaedic Consult, 2nd Edition
> Table of Contents > Cerebral Palsy

Cerebral Palsy
Paul D. Sponseller MD
Basics
Description
  • Cerebral palsy is a general term covering a wide range of presentations.
    • The common theme is a static neuropathy developing in the first 3 years of life.
    • The cause may vary, and typically only the result is seen, through its effect on the trunk and extremities.
    • Although the neurologic lesion does not change, the functional level may change as the person grows older (1).
  • Classification is by anatomic and physiologic types; a patient should be classified according to both parameters.
    • Anatomically: Hemiplegic (ipsilateral arm and leg), diplegic (both legs), and “totally involved”
    • Physiologically: Spastic, athetoid, mixed, and dystonic (1).
Epidemiology
Incidence
This disorder affects 1–2% of all children (1).
Risk Factors
  • Difficult delivery
  • Prematurity
  • Postnatal central nervous system injury
Pathophysiology
  • Peripheral nerves are normal.
  • Brain involvement depends on the specific cause.
  • Muscles of the affected limb show some degree of fibrosis.
  • Typically, no biopsy is indicated.
Etiology
  • Prenatal brain dysplasia
  • Maternal infection
  • Fetal hypoxia
  • Vascular event
  • Encephalitis
  • Meningitis
  • Trauma
  • Kernicterus
Associated Conditions
Associated defects in some patients may include visual or hearing impairment, seizures, osteoporosis, and learning disability (2).
Diagnosis
Signs and Symptoms
  • Hypotonia followed by spasticity is the most common pattern.
  • Milestones may be delayed, and gait is abnormal.
  • Deep tendon reflexes are increased after the 1st year of life, and clonus may develop in certain muscles.
  • Contractures develop, especially in the Achilles tendon, hamstrings, and adductors.
  • Typical findings:
    • Diplegic type: Normal intelligence with equinus or equinovalgus feet
    • Hemiplegic type: Normal intelligence with equinovarus feet and upper extremity spasticity or flexion
    • Totally involved form: Scoliosis, hip dysplasia, and contractures, usually with some cognitive deficit.
    • Athetoid form:
      • Contracture does not develop, and irregular movements occur in the extremities.
      • Normal or mildly impaired intellect (1)
Physical Exam
  • Examine:
    • Upper extremity for deformity, sensation, and use (3)
    • Spine for scoliosis or dysraphism
  • Measure limb lengths and document:
    • Contractures at all joints
    • Muscle excursion at all levels, including ankle dorsiflexion, popliteal angle, and hip abduction
  • Observe the gait several times, if possible, with and without braces.
Tests
Imaging
  • MRI:
    • Indicated if a diagnostic dilemma presents
    • In a patient with spastic diplegia:
      • Typical finding is periventricular leukomalacia, the end result of ventricular hemorrhages.
      • However, the MRI scan may be normal in such patients.
    • In a patient with spastic hemiplegia, typical findings are focal infarct or cyst.
  • Radiography:
    • The cervical spine may be imaged with radiographs in some patients with severe diplegia.
    • Routine films of the hip should be obtained for the child with severe diplegia or total involvement (4).
    • If a deformity is apparent, scoliosis films should be obtained for patients with the totally involved variant.
Differential Diagnosis
  • Brain or upper spinal cord tumor
  • Instability of the upper cervical spine
  • Neurodegenerative disorder
  • Metabolic disorder
  • Familial spastic paraparesis
  • Early stage of myopathy or neuropathy
  • Rett syndrome
Treatment
General Measures
  • Infant stimulation and encouragement of mobility are the most important measures.
  • At-risk muscles (hamstrings, gastrocnemius-soleus) should be stretched if no improvement occurs.
  • Intrathecal baclofen may help to decrease peripheral spasticity.
  • Diazepam (Valium) has little benefit except in the year of any surgery.
  • Physical therapy is best suited to
    address specific, short-term goals; to monitor changes over time; and
    to advise on adaptive equipment.
Special Therapy
Physical Therapy
Physical therapy is useful for gait training, for
instruction in stretching to prevent contractures and the use of
braces, and for aid in activities of daily living.
Surgery
  • Foot deformities almost always should be corrected if the patient has limited ambulatory potential.
    • Lengthening contracted muscles decreases their spasticity or triggering during gait.
    • This principle applies especially to the Achilles tendon, hamstrings, and adductors, but the rectus femoris also may be tight.
  • Hand muscle tightness or dysfunction rarely benefits from surgery because of the lack of sensory integration (3).

    • P.61


  • Before degenerative changes occur, hip subluxation should be treated (4).
    • Adductor muscle lengthening, femoral osteotomy, and possibly an iliac osteotomy
    • Rarely, femoral head removal is indicated if the subluxation has been detected after severe pain develops.
  • If the child has trunk imbalance with difficulty in sitting or back pain, scoliosis treatment is indicated.
    • A brace may help the patient to sit more comfortably, but it does not keep the curve from worsening.
    • Surgical correction and fusion are the best choices for those who are having trouble sitting in a stable, comfortable fashion.
  • Other surgical treatments for patients with cerebral palsy include rhizotomy and an intrathecal baclofen pump.
    • Dorsal rhizotomy:
      • A procedure to decrease spasticity at the more central level of the spinal cord
      • The dorsal afferent rootlets are checked
        for the ability to generate a spastic reflex arc, and the most abnormal
        ones are sectioned.
      • This treatment works for patients <10 years old with ambulatory diplegia.
    • Intrathecal baclofen:
      • Interrupts spasticity at the spinal level by pharmacologic means
      • Used mostly for nonambulatory patients
Follow-up
Disposition
Issues for Referral
  • It is best for children with cerebral palsy to be seen on a routine basis at least once a year during growth.
  • This monitoring allows detection of
    deformities and hip dysplasia or scoliosis at a time when they can be
    treated most effectively.
Prognosis
  • The prognosis depends on the extent of cerebral palsy.
    • Patients with totally involved cerebral
      palsy have a shortened life expectancy, but those with other types show
      no decrease in life expectancy (1,3).
    • Most children with cerebral palsy show
      some decline in walking ability near maturity because, for them,
      walking demands much energy.
Complications
  • Weight gain is a common problem in both childhood and adulthood and contributes to debility.
  • Fractures are common in patients with the totally involved type of cerebral palsy.
  • Patients with totally involved cerebral palsy are likely to have respiratory problems.
Patient Monitoring
Children are seen at least once a year to check on ambulatory ability and status of activities of daily living.
References
1. Renshaw
TS, Deluca PA. Cerebral palsy. In: Morrissy RT, Weinstein SL, eds.
Lovell and Winter’s Pediatric Orthopaedics, 6th ed. Philadelphia:
Lippincott Williams & Wilkins, 2006:552–603.
2. Henderson
RC, Lark RK, Kecskemethy HH, et al. Bisphosphonates to treat osteopenia
in children with quadriplegic cerebral palsy: a randomized,
placebo-controlled clinical trial. J Pediatr 2002;141:644–651.
3. Chin TYP, Duncan JA, Johnstone BR, et al. Management of the upper limb in cerebral palsy. J Pediatr Orthop B 2005;14:389–404.
4. Presedo A, Oh CW, Dabney KW, et al. Soft-tissue releases to treat spastic hip subluxation in children with cerebral palsy. J Bone Joint Surg 2005;87A:832–841.
Miscellaneous
Codes
ICD9-CM
343.9 Infantile cerebral palsy, unspecified
Patient Teaching
  • Patients are encouraged to remain as active as possible and to keep their weight down.
  • It is ideal if they can be referred to a sports program for special-needs children.
  • Patients should understand that the
    condition cannot be totally cured, and they should beware of any claims
    that seem too optimistic.
  • They should learn that oral medications offer no substantial help for the disease.
Activity
To be encouraged
Prevention
Regular follow-up can prevent advanced hip dislocation.
FAQ
Q: What is the cause of cerebral palsy?
A: A number of different causes exist, but all have in common a 1-time injury to the brain during early childhood.

Q: How much physical therapy should a child with cerebral palsy have?
A:
Physical therapy should be individualized. Its main role is to help
children adapt to their own motor abilities. Rarely does a child need
intensive therapy throughout childhood.

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