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Updated: Jun 24 2021

Congenital Scoliosis


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Images - block and hemivertebrae_moved.jpg xray_moved.jpg with contralateral bar_moved.jpg
  • summary
    • Congenital Scoliosis is a congenital spinal deformity that occurs due to the failure of normal vertebral development during 4th to 6th week of gestation.
    • Diagnosis is made with AP and lateral full spine radiographs. MRI is required to assess for neural axis abnormalities. 
    • Treatment can be observation or surgical management depending on the specific anatomical anomaly, and curve progression.
  • Epidemiology
    • Prevalence
      • estimated at 1% to 4% in the general population
  • Etiology
    • Mechanism
      • caused by a developmental defect in the formation of the mesenchymal anlage
    • Causes
      • most cases occur spontaneously
      • maternal exposures
        • diabetes
        • alcohol
        • valproic acid
        • hyperthermia
    • Genetic
      • uncertain
    • Associated conditions
      • may occur in isolation or with associated conditions
      • with associated systemic anomalies, up to 61%
        • cardiac defects - 10%
        • genitourinary defects - 25%
        • spinal cord malformations
      • with underlying syndrome or chromosomal abnormality
        • VACTERL syndrome
          • in 38% to 55%
          • characterized by vertebral malformations, anal atresia, cardiac malformations, tracheo-esophageal fistula, renal, and radial anomalies, and limb defects
        • Goldenhar/OculoAuricularVertebral Syndrome
          • hemifacial microsomia and epibulbar dermoids
        • Jarcho-Levin Syndrome/Spondylocostal dysostosis
          • short trunk dwarfism, multiple vertebral and rib defects and fusion
          • most commonly autosomal recessive
          • often associated with thoracic insufficiency syndrome (TIS)
            • caused by shortening of the thorax and rib fusions
            • result is thorax is unable to support lung growth and respiratory decompensation
        • Klippel-Feil syndrome
          • short neck, low posterior hairline, and fusion of cervical vertebrae
        • Alagille syndrome
          • peripheral pulmonic stenosis, cholestasis, facial dysmorphism
  • Classification
      • Classification of Congenital Scoliosis
      • Failure of Formation
      • Fully segmented hemivertebra
      • -has normal disc space above and below
      • Semisegmented hemivertebra
      • -hemivertebra fused to adjacent vertebra on one side with disk on the other
      • Unsegmented hemivertebra
      • -hemivertebra fused to vertebra on each side
      • Incarcerated hemivertebra
      • -found within lateral margins of the vertebra above and below
      • Unincarcerated hemivertebra
      • -laterally positioned
      • Wedge vertebra
      • Failure of Segmentation
      • Block vertebra
      • (bilateral bony bars)
      • Bar body
      • (unilateral unsegmented bar is common and likely to progress)
      • Mixed
      • Unilateral unsegmented bar with contralateral hemivertebra
      • (most rapid progression)
  • Imaging
    • Radiographs
      • recommended views
        • AP and lateral plain films usually sufficient to confirm diagnosis
    • CT
      • indications
        • judicious use recommended due to radiation exposure
        • 3D CT useful to better delineate posterior bony anatomy and define type for surgical planning
    • MRI
      • indications
        • all patients with congenital scoliosis prior to surgery to evaluate for neural axis abnormality (found in 20-40%) including
          • Chiari malformation
          • tethered cord
          • syringomyelia
          • diastematomyelia
          • intradural lipoma
      • technique
        • sedation required in infants so may be delayed if no surgery is planned and no neuro deficits
    • Additional medical studies
      • important to obtain studies for associated abnormalities
        • renal ultrasound or MRI
        • echocardiogram if suspicion for cardiac manifestations
  • Treatment
    • Nonoperative
      • observation and bracing
        • indications for observation
          • absence of documented progression, ie:
            • incarcerated hemivertebrae
            • nonsegmental hemivertebrae
            • some partially segmented hemivertebrae
        • bracing
          • not indicated in primary treatment of congenital scoliosis (no effectiveness shown)
          • may be used to control supple compensatory curves, but effectiveness is unproven
    • Operative
      • posterior fusion (+/- osteotomies and modest correction)
        • indications
          • hemi-vertebrae opposite a unlateral bar that does not require a vertebrectomy at any age. this otherwise will relentlessly progress until fused.
          • older patients with significant progression, neurologic deficits, or declining respiratory function
          • having many pedicle screws may decrease crankshaft phenomenon adn obviate the need for an anterior fusion.
      • anterior/posterior spinal fusion +/- vertebrectomy
        • indications
          • young patients with significant progression, neurologic deficits, or declining respiratory function
            • girls < 10 yrs
            • boys < 12 yrs
          • patients with failure of formation with contralateral failure of segmentation at any age that requires hemi-vertebrectomy and/or significant correction. This may be done from a posterior approach
        • technique
          • nutritional status of patient must be optimized prior to surgery
      • distraction based growing rod construct
        • indications
          • may be used in an attempt to control deformity during spinal growth and delay arthrodesis
        • outcomes
          • need to be lengthened approximately every 6 months for best results
      • osteotomies between ribs
        • indications
          • mulitple (>4) fused ribs wit potential for thoracic insufficiency syndrome
        • outcomes
          • long-term follow up is needed to determine efficacy. the downside is this may make the chest stiff and hurt pulmonary function.
      • Hemi-Vertebrectomy - usally done from a posterior approach, particularly with kyphosis.
        • indications - age 3-8 years (younger is difficult to get good anchor purchase)
        • progressive or significant deformity
  • Techniques
    • Spinal arthrodesis +/- vertebrectomy/osteotomy
      • in situ arthrodesis, anterior/posterior or posterior alone
        • indications
          • unilateral unsegmented bars with minimal deformity
      • hemiepiphysiodesis
        • indications
          • intact growth plates on the concave side of the deformity
          • patients less than 5 yrs. with < 40-50 degree curve
          • mixed results
      • osteotomy
        • osteotomy of bar
      • hemivertebrectomy
        • hemivertebrae with progressive curve causing truncal imbalance and oblique takeoff
          • often caused by a lumbosacral hemivertebrae
        • patients < 6 yrs. and flexible curve < 40 degrees best candidates
      • spinal column shortening resection
        • indications
          • deformities that present late and have severe decompensation
          • rigid, severe deformities
          • pelvic obliquity, fixed
  • Complications
    • Crankshaft phenomenon
      • a deformity caused by performing posterior fusion alone
    • Short stature
      • growth of spinal column is affected by fusion
        • younger patients affected more
    • Neurologic injury
      • surgical risk factors include
        • overdistraction or shortening
        • overcorrection
        • harvesting of segmental vessels
      • somatosensory and motor evoked potentials important
    • Soft-tissue compromise
      • nutritional aspects of care essential to ensure adequate soft tissue healing
  • Prognosis
    • Dependent on potential for progression and early intervention
    • Progression
      • most rapid in the first 3 years of life
      • anterior failure of formation is rapidly progressive and often results in paralysis; anterior failure of segmentation can be rapidly progressive but rarely results in paralysis
      • determined by the morphology of vertebrae. Rate of progression from greatest to least is:
        • unilateral unsegmented bar with contralateral hemivertebra >
          • greatest potential for rapid progression (5 to10 degrees/year)
        • unilateral unsegmented bar >
        • fully segmented hemivertebra >
        • unincarcerated hemivertebra >
        • incarcerated hemivertebra >
        • unsegmented hemivertebra >
        • block vertebrae
          • little chance for progression (<2 degrees/year)
      • presence of fused ribs increases risk of progression
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