Congenital Scoliosis Flashcards Preview

Orthobullets/ FRCSC Canadian Board > Congenital Scoliosis > Flashcards

Flashcards in Congenital Scoliosis Deck (13)
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1
Q

Congenital Scoliosis

A

Congenital scoliosis is the failure of normal vertebral development during 4th to 6th week of gestation

  • caused by developmental defect in the formation of the mesenchymal anlage
2
Q

Epidemiology

A

prevalence in general population estimated at 1% to 4%

3
Q

Causes

A
  • most cases occur spontaneously
  • maternal exposures
    • diabetes
    • alcohol
    • valproic acid
    • hyperthermia
  • genetic
    • uncertain
4
Q

Associated conditions

A
  • 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
    • Klippel-Feil syndrome

short neck, low posterior hairline, and fusion of cervical vertebrae

5
Q

Prognosis

A
  • most rapid in the first 3 years of life
  • 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
6
Q

Classification of Congenital Scoliosis

A
7
Q

Radiographs

A
  • 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
8
Q

MRI Indications

A

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

9
Q

Additional medical studies

A

important to obtain studies for associated abnormalities

  • renal ultrasound or MRI
  • echocardiogram if suspicion for cardiac manifestations
10
Q

Nonoperative

A

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
11
Q

Operative

A
  1. in situ arthrodesis, anterior/posterior or posterior alone
  2. hemiepiphysiodesis
  3. osteotomy
  4. hemivertebrectomy
  5. spinal column shortening resection
12
Q

operative treatment measures

A
  • 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
13
Q

Complications

A
  • 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

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