Updated: 8/29/2021

Infantile Idiopathic Scoliosis

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  • 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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(SAE07PE.37) A 6-year-old girl has a painless spinal deformity. Examination reveals 2+ and equal knee jerks and ankle jerks, negative clonus, and a negative Babinski. The straight leg raising test is negative. Abdominal reflexes are asymmetrical. PA and lateral radiographs are shown in Figures 15a and 15b. What is the next most appropriate step in management?

QID: 6097
FIGURES:
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MRI of the spinal axis

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(268/420)

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Physical therapy

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A brace for scoliosis

7%

(28/420)

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Observation, with reevaluation in 6 to 12 months

29%

(120/420)

5

Posterior spinal fusion from T6 to T12

0%

(1/420)

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