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Cerebral Palsy - Spinal Disorders

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Topic updated on 07/31/16 10:17am
Introduction
  • See Cerebral Palsy General 
  • 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
  • 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
  • Etiology
    • 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
  • 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
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
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
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
    • 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
    • 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
      • 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)
    • extension to pelvis
      • indications
        • pelvic obliquity > 15°
        • required due to increased pseudoarthosis rate if you do not do it
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
Surgical Techniques
  • 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%)

 

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