Updated: 9/14/2021

Juvenile Idiopathic Scoliosis

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  • summary
    • Juvenile Idiopathic Scoliosis is a coronal plane spinal deformity which most commonly presents in children between ages 4 and 10.
    • Diagnosis is made with full-length standing PA and lateral spine radiographs. MRI studies are indicated in children <10 years old with a curve > 20°.
    • Treatment can be observation, bracing, or surgical management depending on the skeletal maturity of the patient, magnitude of deformity, and curve progression.
  • Epidemiology
    • Incidence
      • 15% of all idiopathic scoliosis cases
    • Demographics
      • females > males
    • Anatomic location
      • most commonly appear as a right main thoracic curve
  • Etiology
    • Associated conditions
      • high incidence of neural axis abnormalities (18-25%)
        • syringomyelia
          • cyst or tubular cavity within spinal cord
          • can be seen in a scoliotic curve without rotation
          • can manifest as an asymmetric umbilicus reflex
        • Arnold-Chiari syndrome
          • cerebellar tonsil are elongated and protruding through the opening of the base of the skull and blocking CSF flow)
        • tethered cord
        • dysraphism
        • spinal cord tumor
  • Classification
    • Early onset scoliosis (EOS)
      • early-onset scoliosis is a broader category including scoliosis in children <10 years old. It includes
        • infantile idiopathic scoliosis
        • juvenile idiopathic scoliosis
        • congenital scoliosis
        • neurogenic scoliosis
        • syndromic scoliosis
          • Marfan's
          • Down's syndrome
  • Presentation
    • History
      • important to determine when deformity was first noticed and any observed progression
      • get perinatal history
    • Presentation
      • failure to develop bowel and bladder control by age ~ 3 or 4 may indicate neurologic involvement
      • patients often referred from school screening where a 7° curve on scoliometer during Adams forward bending test is considered abnormal
        • 7° correlates with 20° coronal plane curve
    • Physical exam
      • general inspection
        • cafe-au-lait spots (neurofibromatosis)
        • leg length inequality
        • shoulder height differences
        • truncal shift
        • waist asymmetry and pelvic tilt
        • foot deformities (cavovarus)
          • can suggest neural axis abnormalities and warrant a MRI
      • spine inspection
        • midline skin defects
          • hairy patches
          • dimples (signs of spinal dysraphism)
          • nevi
        • rib rotational deformity (rib prominence)
        • Adams forward bending test
          • axial plane deformity indicates structural curve
        • forward bending sitting test
          • can eliminate leg length inequality as cause of scoliosis
      • neurologic
        • motor
          • upper and lower extremities exam
        • reflexes
          • abnormal abdominal reflexes
            • associated with the presence of a syrinx
            • gently stroking each abdominal quadrant should cause contraction of the abdominal muscles
          • clonus
          • Hoffman sign
          • Babinski
      • gait analysis
  • Imaging
    • Radiographs
      • PA and lateral upright images are used to assess curve severity
        • treatment based on Cobb angle
      • Cobb angle
        • > 10° defined as scoliosis
        • intra-interobserver error of 3-5°
        • bending radiographs can help determine which curves require fusion
      • indicated in children <10 years old with a curve > 20°
        • even in the absence of neurologic symptoms
        • must rule out neural axis abnormalities (e.g., syringomyelia)
  • Treatment
    • Nonoperative
      • observation
        • indications
          • curves < 20°
        • technique
          • frequent radiographs to observe for curve progression
      • bracing
        • indications
          • curves 20 - 50°
          • designed to prevent curve progression, not correct the curve
          • relative contraindication to bracing is thoracic hypokyphosis
        • technique
          • 16-23h/day until skeletal growth completed or surgery indicated
    • Operative
      • non-fusion procedures (growing rods, VEPTR)
        • indications
          • curves > 50° in small children with significant growth remaining
            • allows continued spinal growth over unfused segments
            • definitive PSF + ASF performed when the child has grown and is closer skeletal maturity
        • traditional growing rods associated with greater curve correction and truncal height gain than VEPTR constructs 
      • anterior / posterior spinal fusion
        • indications
          • curves > 50° in younger patients
          • required in order to prevent crankshaft phenomenon
      • posterior spinal fusion
        • indications
          • curve > 50° in older patients near skeletal maturity
          • remains gold standard for thoracic and double major curves (most cases)
      • anterior spinal fusion
        • indications
          • curve > 50°
          • best for thoracolumbar and lumbar cases with a normal sagittal profile
  • Complications
    • Crankshaft phenomenon
  • Prognosis
    • High risk of progression
      • 70% require treatment (50% bracing, 50% surgery)
    • Very few experience spontaneous resolution
    • Can be fatal if not treated appropriately

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