SCOLIOSIS

I. Classification

  1. Functional
  1. Unequal leg length
  2. Muscle spasm
  3. Posturing
  4. Hysteria
  1. Structural
  1. Idiopathic scoliosis (80%)
    1. Infantile (0-3yo)
      1. Fairly rare
      2. Possibly related to intrauterine positioning
      3. Boys > girls
      4. Usually single thoracic curve convex to left
      5. 90% resolve spontaneously
      6. Risk factors for progression: > 1yo at onset; curve > 35'; thoracolumbar, lumbar, or double-curve pattern; marked diff. in rib-vertebral angle of convex c/w concave side of the curve
      7. Unlike in older kids, all should be x-rayed to r/o congenital spinal deformities
    2. Juvenile (3-10yo)
      1. 15% of all pts with idiopathic scoliosis
      2. Boys > girls
      3. Usually have single thoracic curve, convex to right, though other patters are seen
      4. Tend to progress more slowly than infantile or adolescent BUT 50% will progress to require surgical intervention
      5. Up to 20% may have syringomyelia; should have low threshold for doing MRI of spinal cord if progressive rapidly or neurologic signs/sx
      6. Risk factors for progression are unknown, so all need to be followed closely until skeletal maturity
    3. Adolescent (> 10yo)
      1. 80-90% of all pts with idiopathic scoliosis
      2. Girls: boys 7:1
      3. 98% have single thoracic curve, convex to right
      4. 5-25% will progress; risk of progression inversely related to physical maturity and degree of curve at presentation (risk of progression with curve > 20% and Risser score 0-1 is 68%)
      5. Note that some progression may occur after maturity (only curves > 30')
    4. Adult
  1. Nonidiopathic scoliosis (20%)
    1. Neurologic
  1. Upper motoneuron (cerebral palsy, spinocerebellar degeneration, spinal cord trauma, spinal cord tumor, syringomyelia)
  2. Lower motoneuron (myelomeningocele, poliomyelitis, spinal muscular atrophy, trauma)
  3. Dysautonomia (Riley-Day syndrome)
  4. Neurofibromatosis
  1. Myopathic
  1. Arthrogryposis
  2. Congenital hypotonia
  3. Muscular dystrophy
  4. Myotonic dystrophy
  1. Congenital abnormalities
    1. Hemivertebrae
    2. Wedge vertebrae
    3. Bloc vertebrae (nonsegmented)
    4. Unsegmented bar
    5. Meningomyelocele
    6. Spinal dysraphism
  2. Mesenchymal
    1. Ehlers-Danlos syndrome
    2. Homocyst
    3. Marfan's syndrome
  3. Osteochondrodystrophies
    1. Achondroplastic
    2. Diastrophic dwarfism
    3. Mucopolysaccharidoses
    4. Spondyloepiphyseal dysplasia
  4. Neoplasm
  5. Post-traumatic
  6. Postsurgical
  7. Spondylolysis

II. Misc. facts on idiopathic scoliosis

  1. Definition: > 10' lateral deviation as observed in a frontal plane x-ray, w/o identifiable cause (Scoliosis Research Society)
  2. Anatomy: Not only a lateral deviation but usually also involves deviation in the saggital plane (usually loss of kyphosis) and a rotational abnormality
  3. Etiology: unknown, possible familial predisposition
  4. Epidemiology: 1-5% of children
  5. Natural history
    1. Progression depends primarily on curve size and physical maturity at presentation--the more immature, the more likely that significant progression will occur.
    2. Severe scoliosis (> 60')can cause pulmonary compromise and consequent cor pulmonale
  6. Clinical presentation
    1. Should be completely asymptomatic--if there are symptoms, consider other dx, e.g. spinal neoplasm or infection
    2. Usually picked up on routine exam
    3. Pt or parents may notice shoulder asymmetry, differences in breast size or chest appearance, waist asymmetry, or rib hump

III. Screening for idiopathic scoliosis

  1. Physical exam
    1. Spine exam
      1. General observation from behind with pt standing: for truncal asymmetry, level of shoulders, and centering of base of neck over midline of sacrum
      2. Make sure pelvis is level to floor (by palpating posterior superior iliac spines) so as not to be fooled by pelvic obliquity
      3. Observe child from behind, asking him/her to lean forward from the waist--rotation of vertebral bodies is such that rib/musculature prominence is toward the convex side of the curve
      4. Examination from the side may reveal loss of normal kyphosis, i.e. flattening of thoracic spine or exaggeration of lumbar lordosis
      5. Consider non-idiopathic causes if there is a thoracic curve that is convex to the left
      6. Mobility of spine--should be normal
    2. Neurologic exam--including abdominal reflex
    3. Straight leg raise test--should evoke no sx suggestive of radiculopathy
    4. Look for cafe-au-lait spots which may indicate neurofibromatosis
    5. Assess physical maturity
      1. Tanner staging
      2. Menarchal status in girls--end of spinal growth usually occurs 1.5-2y after menarche
      3. Skeletal maturity--correlates most closely with risk of progression of scoliosis
        1. KS recommends using the "Risser sign," a 0-5 scale of ossification of the iliac apophysis as seen on AP x-ray of the pelvis. Risser 5 correlates with end of spinal growth
  1. Scoliometer testing
    1. Hand-held device that easures angle of trunk rotation
    2. Pt bends forward and examiner places on child's back, measuring the tilt
    3. Using 5' as cutoff is 99.9% sensitive for scoliosis; however, more recent studies suggest that 7' is as sensitive and more specific
  1. X-rays
    1. Indications: any of the below; if none are present consider re-examination in 9-12mos if physically immature
      1. Any symptoms (pain, paresthesias, etc.)
      2. Any abnormalities on visual exam other than mild truncal asymmetry
      3. Any abnormalities on neurologic exam
      4. Scoliometer reading > 7'
    2. For initial evaluation, KS recc's standing AP and lateral views of the thoracic and lumbar spine and pelvis; for subsequent comparisons in girls, consider PA films to minimize breast irradiation.
    3. Estimate degree of scoliosis using the "Cobb method"

IV. Follow-up in children with idiopathic scoliosis

  1. Pts with < 10' curve on x-ray do not require specific f/u unless there is a marked change on exam or in sx
  2. Pts with 10-20' curve and skeletal immaturity (Risser scores 0-1 or premenarchal)--Q6mo exam with scoliometer until progress to > 7' in trunk rotation'
  3. Pts with 20-30' curve and skeletal immaturity--consider referral; followup x-rays Q4-6months until skeletal maturity; brace if progression > 5'/yr
  4. Pts with curves > 30' and skeletal immaturity--brace at initial visit
  5. Pts with "intermediate skeletal maturity"--postmenarchal girls or Risser score > 1--if curve < 20', do repeated scoliometer exam; if 20-30', x-ray Q6-9 months; > 30', x-ray Q6mos
  6. Pts with < 40' curve at skeletal maturity--no specific f/u unless there is a marked change on exam or in sx
  7. Pts with > 40' curve at skeletal maturity--yearly evaluation (inc. x-rays?)

V. Treatment of idiopathic scoliosis

  1. No proof as of 1993 that tx of curves < 10' alters risk of progression
  2. Treatment modalities
    1. No proven benefit: physical therapy, spinal manipulation, electrical stimulation of paraspinous mm., exercises
    2. Bracing--continue until Risser 4 (or if a girl, until 1.5-2y pastmenarchal). Different types include Milwaukee brace (oldest), Boston underarm thoracolumbosacral orthosis (TLSO), Charleston bending brace. May not reverse the scoliosis but does seem to prevent progression
    3. Surgery (spinal fusion)--provides some reversal of scoliosis and prevention of progression

(Source: Song K. and Herring J.A., J. Musculoskel. Med. 4/93, p. 63 & 11/93, p. 40)