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Introduction
  • Defined as idiopathic scolioisis in children 4-10 years of age
    • considered on type of early-onset scoliois
      • infantile idiopathic scoliosis is other type 
  • Epidemiology
    • incidence
      • 15% of all idiopathic scoliosis cases
    • demographics
      • females > males
    • location
      • most commonly appear as a right main thoracic curve
  • 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
  • Prognosis
    • high risk of progression
      • 70% require treatment (50% bracing, 50% surgery)
    • very few experience spontaneous resolution
    • can be fatal if not treated appropriately
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 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
        • 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
  • MRI 
    • 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
    • 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
 

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