Congenital Scoliosis Flashcards Preview

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Flashcards in Congenital Scoliosis Deck (9):
1

Define congential scolisosi?

  • Failure of the normal vertebral development during 4th-6th wk of gestation
  • caused by developmental defect in the formation of the mesenchymal anlage

2

What is the epidemiology of congenital scoliosis?

  • Prevalence in general population 1-4%

3

What are the causes of congential scoliosis?

  • Most occur spontaneously
  • Maternal exposure
    • diabetes
    • alcohol
    • Valproic acid- in anticovulsants/ mood stabilisers
    • Hypethermia
  • Genetics
    • Notch gene

4

What are the associated conditions of congential scoliosis?

  • Associated conditions in 61%
  • Cardiac defects- 10%
  • Genitourinary defects- 25%
  • Spinal cord malformations
  • w underlying syndrome/dhromosomal abnormality
    • VACTERL syndrome
    • 38-55%
    • Vertebral malformations, anal atresia, cardiac malformations, tracheo-oephageal fistula, renal and radial abnormalities, limb defects
    • Klippel- Feil sydrome
      • short neck, low hairline, fusion c cpine
    • Jarcho- Levin syndrome/Spondylocostal Dysostosis
      • Short trunk dwarfism, multiple vertebral, rib defects and fusion
      • most commonly autosomal recessive
      • often assoc w thoracic insufficiency syndrome
      • shortening of thorax/rib fusion
      • results in thorax unable to support lung growth/resp decompensation

5

What is the progression with congenital scoliosis?

  • Most rapid in first 3 years of life
  • Rate of progression- **greatest to least
    • Unilateral Unsegmental Bar with contralateral hemivertebra ** 5-10o pa
    • Unilateral unsegmental bar
    • Fully segmented Hemivertebra
    • Unsegmented Hemivertebra
    • Incarcerated hemivertebra
    • Unicarcerated hemivertebra
    • Block vertebra- least chance of progression
  • Presence of fused ribs increases risk of progression

6

Can you describe the classification of congenital scoilosis?

  • Failure of formation
    • Fully Segmented Hemivertebra
    • Semisegmented Hemivertebra
    • Unsegemented Hemivertebra
    • Incarcerated Hemivertebra
    • Unicarcerated hemivertebra
    • Wedge Vertebra
  • Failure of Segmentation
    • Block Vertebra- bilat bony bars
    • Bar Body- unilat unsegemented bar
  • Mixed
    • Unilateral unsegmented bar with contralateral hemivertebra

7

What investigations are helpful in dx of congenital  scoliosis?

  • X rays
    • AP and lateral films usually enough for dx
  • CT
    • 3d CT better to delinate bony anatomy
  • MRI
    • in all pt with congential scoliosis prior to surgery to evaluate neural axis abnormality and 
      • chiari malformation
      • tethered cord
      • syringomyelia
      • diastematomyelia
      • intrdural lipoma
    • Renal USS
    • Echo- cardiac manifestations

8

What is the tx for congenital  scoliosis?

Non operative

  • Observation 
    • for non progression
    • incarcerated hemivertebra
    • nonsegmental hemivertebra
    • some partially segmented hemivertebra
    • bracing not indicated in primary tx of congential scoliosis

Surgery

  • Anterior/posterior spinal fusion +/- Vertebrectomy
    • pt w failure of formation with contral failure of segmentation- any age
    • fusion is short distance after hemivertebrectomy
    • young pt with significant progression, neurological deficits or declining respiratory function
  • Growing rod construct
    • used in an attempt to control deformity during spinal growth/delay arthrodesis
    • may need to be lengthened every 6 months
  • Osteotomy of bar
  • Hemiepiphysiodesis
    • intact growth plates on concave side
  • Hemivertebrectomy- with progressive curve causing truncal imbalance and oblique take off
    • pt <6 yrs, flexible curve <40o
  • Spinal column shortening resection
    • ​those present late and fixed

9

What are the complications of congenital  scoliosis?

  • Crankshaft phenomenon
    • caused with posterior surgery alone
  • Short stature
    • growth of spinal column affected by fusion
    • younger pt more affected
  • Neurologic injury
    • overdistraction
    • overcorrection
    • harvesting of segmental vessels
  • Soft tissue compromise
    • nutritional aspects of care essential to esnure soft tissue healing