Juvenile Idiopathic Scoliosis

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

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

Complications