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Introduction
  • Defined as scoliosis that presents in patient 3 years or less
    • considered a type of early-onset scoliosis
  • Epidemiology
    • incidence
      • 4% of idiopathic scoliosis cases 
    • demographics
      • males > females
    • location
      • usually left thoracic
    • risk factors
      • family history
  • 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°
  • 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
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 < 5 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
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
 

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