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”

Investigations

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

Xray

• Want to see entire spine from cervical to sacral

Treatment

Nonoperative

• 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

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 – 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