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Updated: Aug 29 2021

Infantile Idiopathic Scoliosis

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https://upload.orthobullets.com/topic/2055/images/infantileidiopathic.jpg
https://upload.orthobullets.com/topic/2055/images/rib_phase.jpg
https://upload.orthobullets.com/topic/2055/images/rvad2.jpg
https://upload.orthobullets.com/topic/2055/images/mheta_cast_patient_on_table.jpg
  • summary
    • Infantile Idiopathic Scoliosis is a coronal plane spinal deformity which most commonly presents in children ages 3 years or less.
    • Diagnosis is made with full-length PA and lateral spine radiographs. MRI studies are indicated to rule out syrinx, tumor, or tethered cord. 
    • Treatment can be observation, bracing, or surgical management depending on the skeletal maturity of the patient, magnitude of deformity, and curve progression.
  • Epidemiology
    • Incidence
      • 4% of idiopathic scoliosis cases
    • Demographics
      • males > females
    • Anatomic location
      • usually left thoracic
    • Risk factors
      • family history
  • Etiology
    • Pathophysiology
      • pathoanatomy
        • may adversely affect growth of alveoli and normal development of the thoracic cage
    • Genetics
      • autosomal dominant with variable penetrance
    • Associated conditions
      • plagiocephaly (skull flattening)
      • congenital defects
      • neural axis abnormalities
        • 22% of patients with curves > 20° will be affected
          • 80% of these patients will need neurosurgical involvement
      • thoracic insufficiency syndrome
        • characterized by decreased thoracic growth and lung volume
        • leads to pulmonary hypertension and cor pulmonale
        • pulmonary function impairment associated with curves > 60°
        • cardiopulmonary issues associated with curves > 90°
  • Anatomy
    • Osteology
      • the T1-L5 spinal segment grows fastest in the 1st five years of life
        • the height of the thoracic spine increases by 2 times between birth and skeletal maturity
  • Classification
    • Infantile Idiopathic Scoliosis consists of
      • resolving type
      • progressive type
    • Early onst scoliosis
      • early-onset scoliosis is a broader category that includes scoliosis in children < 10 years. It includes
        • infantile idiopathic scoliosis (this topic)
        • juvenile idiopathic scoliosis
        • congenital scoliosis
        • neurogenic scoliosis
        • syndromic scoliosis
          • Marfan's
          • Down's syndrome
  • Presentation
    • History
      • age deformity was first noticed and any observed progression
      • perinatal history
      • developmental milestones
    • Presentation
      • most present with deformity
      • excessive drooling may reflect neurologic condition
    • Physical exam
      • inspection
        • cafe-au-lait spots (neurofibromatosis)
        • patches of hair
        • dimpling over the spinal region
          • dimpling outside of the gluteal fold is usually benign
        • nevi or other tumors may be indicative of spinal dysraphism
        • plagiocephaly (skull flattening)
      • neurologic
        • motor
          • document developmental milestones
          • evaluate for cavovarus feet
        • upper and lower extremities exam
        • reflexes
          • abnormal abdominal reflexes
            • associated with the presence of a syrinx
          • clonus
          • Hoffman sign
          • Babinski
      • gait analysis
  • Imaging
    • Radiographs
      • recommended views
        • standing PA and lateral
          • supine in infants unable to stand (will make curve appear less severe)
      • findings
        • look for congenital vertebral defects
      • measurements
        • cobb angle
          • > 20 degrees associated with progression
        • rib phase
          • technique
            • convex rib head position with respect to the apical vertebrae
          • findings
            • phase 1 - no rib overlap
            • phase 2 - rib overlap with the apical vertebrae
              • high risk for curve progression
        • RVAD (rib vertebrae angle difference, Mehta angle)
          • technique
            • measure angle between the endplate and rib (line between midpoint of rib head and neck)
            • RVAD = difference of 2 rib-vertebral angles
          • findings
            • > 20° is linked to high rate of progression
            • < 20° is associated with spontaneous recovery
    • MRI
      • obtain MRI of spine first to rule out
        • tether
        • cyst
        • tumor
        • syrinx (20% incidence)
  • Treatment
    • Nonoperative
      • observation alone (most resolve spontaneously)
        • indications
          • Cobb angle < 30°
          • RVAD < 20°
        • 90% will resolve spontaneously
      • serial Mehta casting (derotational) or thoracolumbosacral orthosis (TLSO)
        • indications
          • flexible curves
          • Cobb angle > 30°
          • RVAD > 20°
          • phase 2 rib-vertebrae relationship (rib-vertebral overlap)
        • mechanism
          • functions to straighten the spine in young patients
          • in older patients it serves as an adjunctive measure prior to definitive treatment
      • bracing
        • indications
          • incompletely corrected curves after Mehta casting
          • late presenting cases where the spine is still flexible
    • Operative
      • growing rod construct (dual rod or VEPTR)
        • indications
          • Cobb > 50 to 60 degrees
          • failed Mehta casting or bracing
        • fusion
          • delay until as close to skeletal maturity as possible
          • fusion before age 10 years results in pulmonary compromise
        • outcomes
          • improvement in FVC pulmonary function with implementation of growing construct
  • Techniques
    • Growing rod construct (dual rod or VEPTR)
      • permits growth of affected part of spine up to 5 cm
      • dual rods or VEPTR
        • use anchors proximally and distally
        • serial lengthening
          • required every six to eight months
  • Complications
    • High rate of complications with surgical treatment
  • Prognosis
    • Progression
      • most resolve spontaneously
      • if progressive by age 5, >50% of children will have a curve > 70°
    • Mehta predictors of progression
      • Cobb angle > 20°
      • RVAD > 20°
      • phase 2 rib-vertebral relationship (rib-vertebral overlap)
    • Prognosis
      • progressive curves have poor outcomes and must be treated
      • can be fatal if not treated appropriately
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