Congenital Scoliosis

  • Embryologically the spine develops at 5-8 weeks
  • Vertebral anomally present at birth
  • Usually presents much earlier than adolescent idiopathic scoliosis
  • Curves tend to be rigid
  • Many cases early fusion preferred

Classification

  • Failure of segmentation
  • Failure of formation
  • Combined
  • 25% non-progressive, 25% midly progressive, 50% very progressive

Features of Scoliosis

  • Still watch for progression
  • Bracing generally is ineffective
  • Progression in greater than 75%
  • Worst prognosis with thoracic & especially with unilateral bar & contralateral hemivertebrae
  • Generally no genetic link

Presentation

  • Pregnant mother with ultrasound
  • Incidental chest xray
  • Diagnosis of deformity
  • Hairy patch, midline angioma, sacral dimple
  • Neurlogic findings including a small foot
  • Intraoperative – i.e. when fixing “idiopathic”

Inves>Investigations

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  • For Other abnormalities
    • Urologic ~ 20%
    • Cardiac ~ 10-15%
    • Spinal dysraphism – 20% look for dimples, hairy patches, skin pigment
    • Foot abnormalities
    • Get MRI

    • XrayXray

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  • Want to see entire spine from cervical to sacral

    • Treatme>Treatment

    class="wp-block-heading">Nonoper>Nonoperativeclass="wp-block-list">
  • Need to follow these kids with serial xrays approximately every 6 months
  • Xray & clinical exam tell if progressing
  • Observation is used for non progressive curves
  • Orthosis
    • Infrequently indicated

    • Operati>Operative

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  • Severe & progressive
  • Options
    • Posterior fusion
    • Posterior & anterior fusion
    • Anterior hemiepiphysiodesis & anterior hemifusion
    • Hemivertebral resection

    • Strateg>Strategy of Surgery

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  • Prophylactic
    • In situ fusion
      • ant, posterior or combined
    • Hemiepiphysiodesis
      • done before age 5, one level above & below the pathologic area leading to correction by the intact concave growth plates
  • Corrective without resection
    • Posterior spine fusion may lead to crankshaft
    • Posterior spine  fusion with instrumentation will be OK in older patient with no risk of crankshaft – beaware of overcorrection leading to neurologic deficit or even pseudoarthrosis
    • Anterior & Posterior  – again beaware that distraction can lead to neurologic injury
  • Corrective surgery with excision
    • Best indicated in a sub cord level – e.g. lumbosacral hemi
    • A combined anterior – first with excision & then posterior excision & instrumentation
  • More extreme would be spinal column resection
  • Posterior
    • Not for correction
    • Frequently used in past with preoperative traction
    • Instrumentation is supplemental
    • Need MRI & Wakeup Test
  • Combined
    • Common procedure if there is significant convex growth potential
  • Convex Growth Arrest
    • For use in single convex hemivertebrae with nearly normal concave side
  • Hemivertebral Excision
    • Usually in the lumbosacral area