Learning objectives

• Describe cerebral palsy
• Recognize the symptoms and signs of cerebral palsy
• Outline the anticipated challenges for airway management in patients with cerebral palsy
• Identify common intraoperative complications associated with cerebral palsy

Definition and mechanisms

• Cerebral palsy (CP) is a group of permanent neurodevelopmental disorders that affects an individual’s muscle tone, motor functions, movement, and posture
• CP is attributed to non-progressive disturbances that occur in the developing fetal or infant brain
• CP is the most common motor disability in childhood

Signs and symptoms

• Signs and symptoms vary among people
• CP can affect the whole body or might be limited primarily to one or two limbs, or one side of the body
• Symptoms get more noticeable over the first few years of life, but underlying problems do not worsen over time

Movement and coordination

• Stiff muscles and exaggerated reflexes (spasticity)
• Variations in muscle tone (too stiff or floppy)
• Stiff muscles with normal reflexes (rigidity)
• Lack of balance and muscle coordination (ataxia)
• Tremors or jerky involuntary movements
• Favoring one side of the body (e.g., only reaching with one hand or dragging a leg while crawling)
• Difficulty walking (e.g., walking on toes, crouched gait, scissors-like gait with knees crossing, wide gait, or asymmetrical gait)
• Difficulty with fine motor skills

Speech and eating

• Delays in speech development
• Difficulty speaking
• Difficulty with sucking, chewing, or eating
• Excessive drooling or problems with swallowing

Development

• Delays in reaching motor skills milestones (babies with CP do not roll over, sit, crawl, or walk as early as other children of their age)
• Learning disability
• Intellectual disability
• Delayed growth (smaller size than expected)

Other problems

• Epilepsy
• Difficulty hearing
• Problems with vision and abnormal eye movements
• Abnormal touch or pain sensations
• Bladder and bowel problems (e.g., constipation and urinary incontinence)
• Mental health conditions (e.g., emotional disorders and behavioral problems)

Risk factors

Congenital CP (80%)

• Fetal pathogenic factors
  • Vascular maldevelopments 
  • Congenital genetic/metabolic disorders 
  • Microcephaly 
  • Fetal trauma 
  • Neonatal asphyxia in the peripartum period (6%) 
  • Low birth weight (<2.5 kg) 
  • Prematurity (<32 weeks) 
  • Low Apgar score 
  • Multiple births 
  • Prenatal “TORCH” infections (toxoplasmosis, rubella, cytomegalovirus, and herpes) 
• Maternal pathogenic factors
  • Breech presentation 
  • Pre-eclampsia 
  • Peripartum hemorrhage 
  • Maternal hyperthyroidism 
  • Fetal alcohol syndrome 

Acquired CP (20%) → develops during the first 2 years of life

• Intracerebral hemorrhage 
• Viral encephalitis 
• Bacterial meningitis 
• Hyperbilirubinemia (kernicterus) 
• Head injury 
• Neonatal seizures 

Complications

Muscle weakness, muscle spasticity, and coordination problems may contribute to complications during childhood or adulthood

• Contracture: Muscle tissue shortening due to severe muscle tightening as a result of spasticity → can inhibit bone growth, cause bones to bend, and result in joint deformities, dislocation, or partial dislocation (e.g., hip dislocation, scoliosis)
• Malnutrition: Swallowing or feeding problems limit the infant to get enough nutrition → impair growth and weaken bones 
• Mental health conditions: Depression and behavioral problems
• Cardiopulmonary disease
  • Increased risk of aspiration pneumonitis and subsequent chronic lung scarring due to swallowing difficulties, esophageal dysmotility, abnormal lower esophageal sphincter tone, and spinal deformity → gastroesophageal reflux disease (GERD)
  • Decreased immunity, poor nutrition, respiratory muscle hypotonia, and a weak cough in conjunction with GERD make patients more susceptible to recurrent chest infections, exacerbating the underlying chronic lung disease
  • Long-term truncal muscle spasticity can lead to scoliosis, restrictive lung defects, pulmonary hypertension, and ultimately cor pulmonale and respiratory failure
• Osteoarthritis: Pressure on joints or abnormal alignment of joints from muscle spasticity may lead to the early onset of osteoarthritis
• Osteoporosis: Fractures due to low bone density resulting from lack of mobility, inadequate nutrition, and anti-epileptic drug use
• Other complications: Sleep disorders, chronic pain, skin breakdown, intestinal problems, and issues with oral health

Pathophysiology

Treatment

Treatment is focused on 

• Improving posture and mobility by reducing spasticity, muscle spasms, and contractures
• Symptomatic relief of the associated medical problems (e.g., epilepsy, GERD, chest infections)

Combination therapies are more successful than single-treatment protocols: Physiotherapy, psychological counseling, occupational, speech, and behavioral therapy combined with

• Antispastic medications (e.g., baclofen)
• Neuromuscular denervation techniques (e.g., botulinum toxin injections, radiofrequency ablation of dorsal horn ganglia, and dorsal rhizotomy)
• Surgery (e.g., tenotomies, arthrodeses, osteotomies tendon transfer/lengthening, and multisegmental spinal fusion procedures)

Management

Keep in mind

• CP describes a spectrum of movement and posture disorders resulting from pathological injury to the developing fetal or infant brain
• Commonly associated comorbidities include dehydration, malnutrition, epilepsy, GERD, and impaired lung function
• Frequently encountered perioperative problems include difficulties with patient positioning and vascular access
• Regional analgesic techniques to reduce postoperative pain, muscle spasms, and respiratory complications are beneficial

Suggested reading

• Prosser DP, Sharma N. Cerebral palsy and anaesthesia. Continuing Education in Anaesthesia Critical Care & Pain. 2010;10(3):72-76.
• Miller B, Rondeau B. Anesthetic Considerations In Patients With Cerebral Palsy. [Updated 2022 Jun 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan. Available from: https://www.ncbi.nlm.nih.gov/books/NBK572057/ 

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