Updated: 6/24/2021

Cerebral Palsy - Spinal Disorders

Review Topic
  • summary
    • Spinal Disorders in Cerebral Palsy are thought to be caused by muscle weakness and truncal imbalance and most commonly present with progressive scoliosis.
    • Diagnosis is made with full-length spine radiographs.
    • Treatment can range from observation, bracing, or surgical management depending on the severity of underlying medical conditions, skeletal maturity of the patient, magnitude of deformity, and curve progression.
  • Epidemiology
    • Scoliosis common in children with cerebral palsy
      • overall incidence is 20%
      • the more involved and severe the cerebral palsy, the higher the likelihood of scoliosis
        • spastic quadriplegic at highest risk, especially if no ability to sit independently.
        • for bedridden children incidence approaches 100%
        • spinal deformity is rare in children who are able to ambulate
  • Etiology
    • See Cerebral Palsy General
    • Pathophysiology
      • muscle weakness and truncal imbalance has been implicated as primary etiology (little evidence to support)
      • pelvic obliquity leads to deforming forces on spine and scoliosis
    • Associated conditions
      • Scoliosis in patients with cerebral palsy differs from idiopathic scoliosis in that
        • curves are more likely to progress
          • (scoliosis progresses 1° to 2° per month starting at age 8 to 10 years)
        • curve begins at earlier age
        • curve is a long, stiff C-shaped curve
          • left sided curves are not uncommon
        • curve has greater sagittal plane deformity (kyphotic or lordotic)
        • associated with pelvic obliquity,
        • skeletal maturity is delayed in CP
        • bracing is less effective
        • longer fusions to the pelvis are often necessary
        • patients are more medically fragile and a multi-disciplinary approach is often necessary
  • Classification
    • Weinstein classification
      • Group I - double curves with thoracic and lumbar component and minimal pelvic obliquity
      • Group II - large lumbar or thoracolumbar curves with marked pelvic obliquity
  • Evaluation
    • Treatment of cerebral palsy spine disorders requires a careful risk-benefit analysis. Therefore it is important to have a thorough understanding of the medical history and functional status.
    • History
      • clinical history
        • perinatal history
        • growth & development
        • all medical treatment
      • functional status
        • patients nutritional status
        • respiratory function
        • sitting / standing posture
        • upper and lower extremities function
        • communication skills
        • acuity of hearing and vision
    • Physical Exam
      • muscular-skeletal exam
        • motion, tone, and strength
        • hamstring contracture's (lead to decreased lumbar lordosis)
        • hip contractures (lead to excessive lumbar lordosis)
      • spine exam
        • look at flexibility of curve
        • spinal balance and shoulder height
        • pelvic obliquity
  • Imaging
    • Radiographs
      • introduction
        • important just to use same radiographic technique in patients over time
          • technique often determined by functional status of patient
          • do standing or erect films whenever possible
      • standard AP & lateral
        • look for rib deformity, wedging, and spinal rotation
        • be sure to evaluate for spondylolisthesis on lateral (incidence of 4-21% in patients with spastic diplegia)
      • bending films
        • important to evaluate flexibility of curve
        • use push-pull radiographs or fulcrum bending radiographs if patient can not cooperate
    • MRI
      • preoperative MRI is not routinely performed for patients undergoing spinal deformity surgery
      • indications for MRI include
        • rapid curve progression
        • change in neurologic exam
  • Treatment
    • Nonoperative
      • observation, custom seat and/or bracing, botox injections
        • indications
          • nonprogressive curves < 50°
          • early stages in patients < 10 years of age
            • goal is to delay surgery until an older age
        • outcomes
          • custom seat orthosis
            • helpful with seating but does not affect natural course of disease
          • bracing
            • TLSO is helpful to improve sitting balance but does not affect natural course of disease
            • some studies have supported use as a palliative measure to slow progression in skeletally immature patients only
          • botox
            • competitive inhibitor of presynaptic cholinergic receptor with a finite lifetime (usually last 2-3 months)
            • provide some short term benefit in patients with spinal deformity
    • Operative
      • PSF with/without extension to the pelvis
        • indications
          • Group I curves 50° to 90° in ambulators that is progressive or interfering with sitting position
          • patient > 10 yrs of age
          • adequate hip range of motion
          • stable nutritional and medical status
        • indications to extend to pelvis
        • technique
          • treated as idiopathic scoliosis with selective fusion
          • can result in worsening pelvic obliquity and sitting imbalance
      • PSF +/- ASF with/without extension to pelvis
        • indicated for
          • Group I curves >90° and in non-ambulators
          • Group II curves
          • children who have not yet reached skeletal maturity (avoid crankshaft phenomenon)
      • growing rod distraction
        • indications
          • young patient age
        • technique
  • Preoperative Assessment & Planning
    • Overview
      • treatment of cerebral palsy spine disorders is complicated by medical comorbidities
        • all patients should have a thorough multidisciplinary approach
    • Nutritional status
      • increase complications (infection, length of intubation, longer hospital stays)
        • associated with poor nutritional status (weight less than fifth percentile)
        • be sure patient has adequate nutrition before surgery (serum albumin > 3.5 g/dL, consider gastrostomy tube if not)
    • Respiratory status
      • difficult to do formal pulmonary functional capacity testing
      • can use respiratory history, clinical evaluation, and chest radiographs
    • GI evaluation
      • preoperative management of GERD is important in prevention of aspiration pneumonia
    • Neurologic function
      • if patients have seizure disorder (common) be sure it is under control
        • if patient taking valproic acid, obtain bleeding time as these patients may have increased risk of bleeding
  • Techniques
    • Goals of Surgery
      • obtain painless solid fusion with well corrected, well balanced spine with level pelvis
      • decision to proceed with surgery must include careful assessment of family's goals and careful risk-benefit analysis
    • Fusion levels
      • proximal fusion should extend to T1 or T2 (otherwise risk of proximal thoracic kyphosis)
      • distal fusion depends on curve pattern
        • due to long curves in CP often extends to L4 or L5
        • extend to pelvis whenever pelvic obliquity is > 15°
    • Posterior fixation techniques
      • Luque rod with sublaminar wires technique
      • Unit rod with sublaminar wires technique
      • Pedicle screw fixation technique
        • may provide better correction and eliminate need for anterior surgery
    • Pelvic fixation techniques
      • Galveston Technique
        • technique to fuse to pelvis with goal of a stability and truncal balance and a level pelvis
        • caudal ends of rods are bent from lamina of S1 to pass into the posterosuperior iliac spine and between the tables of the ileum just anterior to the sciatic notch
      • bilateral sacral screws
      • iliosacral screws
      • spinopelvic transiliac fixation
      • Dunn-McCarthy technique (S-contoured rod that wraps over sacral ala)
    • Anterior and Posterior Techniques
      • use of anterior procedures decreasing with improved posterior constructs
      • higher complication rate in anterior surgery in CP spinal deformity than idiopathic scoliosis
        • decrease complication rate if A/P done on same day verses staging procedure (improved nutritional status, decreased blood loss, short length of hospitalization)
    • Preoperative traction
      • may be option in severe and rigid curve
    • Postoperative bracing
      • usually not required
        • may be used in patients with osteoporosis or tenuous fixation
  • Complications
    • Implant failure
      • sometimes may be asymptomatic and not require treatment
      • includes penetration of pelvic limb of unit rod into pelvis
    • Pulmonary complications
      • chronic aspiration
      • pulmonary insufficiency most common complication in recent study
      • pneumonia
    • GI complications
      • GERD
      • poor nutrition and delayed growth
    • Neurologic complications
      • seizures
    • Wound infection
      • more common in CP than idiopathic scoliosis
      • occurs in 3-5% and usually can be treated with local wound debridement alone
    • Death (0-7%)
  • Prognosis
    • Natural history
      • the larger the curve the more likely it is to progress
        • larger curves are associated with pelvic deformity and obliquity
        • some studies show increase incidence of decubitus ulcer in patients with larger curves, other studies did not

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