Congenital Scoliosis

  • Embryologically the spine develops at 5-8 weeks
  • Vertebral anomaly 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% mildly 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 X-ray
  • Diagnosis of deformity
  • Hairy patch, midline angioma, sacral dimple
  • Neurologic findings including a small foot
  • Intraoperative – i.e. when fixing “idiopathic”

Investigations

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

X-ray

  • Want to see entire spine from cervical to sacral

Treatment

Non-operative

  • Need to follow these kids with serial X-rays approximately every 6 months
  • X-ray & clinical exam tell if progressing
  • Observation is used for non progressive curves
  • Orthosis
    • Infrequently indicated

Operative

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

Strategy of Surgery

  • 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 – be aware of overcorrection leading to neurologic deficit or even pseudoarthrosis
    • Anterior & Posterior  – again be aware 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
  • Convex Growth Arrest
    • For use in single convex hemivertebrae with nearly normal concave side
  • Hemivertebral Excision
    • Usually in the lumbosacral area

Reviewed by Professor Phong Tran, Head of Orthopaedic Surgery, Western Health. Last updated 10 October 2026.