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Introduction
  • 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
  • Epidemiology
    • incidence
      • prevalence in general population estimated at 1% to 4%
  • Causes
    • most cases occur spontaneously 
    • maternal exposures
      • diabetes
      • alcohol
      • valproic acid
      • hyperthermia
    • genetic
      • uncertain
  • Associated conditions    
    • 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
      • Goldenhar/OculoAuricularVertebral Syndrome
        • hemifacial microsomia and epibulbar dermoids
      • Jarcho-Levin Syndrome/Spondylocostal dysostosis
        • short trunk dwarfism, multiple vertebral and rib defects and fusion
        • most commonly autosomal recessive
        • often associated with thoracic insufficiency syndrome (TIS)
          • caused by shortening of the thorax and rib fusions 
          • result is thorax is unable to support lung growth and respiratory decompensation
      • Klippel-Feil syndrome
        • short neck, low posterior hairline, and fusion of cervical vertebrae
      • Alagille syndrome
        • peripheral pulmonic stenosis, cholestasis, facial dysmorphism
  • Prognosis
    • progression
      • 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
  • Outcomes
    • dependent on potential for progression and early intervention
Classification
 
 Classification of Congenital Scoliosis
Failure of Formation Fully segmented hemivertebra
(has normal disc space above and below)
Semisegmented hemivertebra
(hemivertebra fused to adjacent vertebra on one side with disk on the other)
 
Unsegmented hemivertebra
(hemivertebra fused to vertebra on each side) 
Incarcerated hemivertebra
(found within lateral margins of vertebra above and below)
Unincarcerated hemivertebra
(laterally positioned)
Wedge vertebra
Failure of Segmentation Block vertebra
(bilateral bony bars)

Bar body
(unilateral unsegmented bar is common and likely to progress)
Mixed q Unilateral unsegmented bar with contralateral hemivertebra
(most rapid progression)

 
Imaging
  • 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
  • MRI
    • 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
  • Additional medical studies
    • important to obtain studies for associated abnormalities q q
      • renal ultrasound or MRI
      • echocardiogram if suspicion for cardiac manifestations
Treatment
  • Nonoperative
    • 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
  • Operative
    • posterior fusion (+/- osteotomies and modest correction)
      • indications
        • hemi-vertebrae opposite a unlateral bar that does not require a vertebrectomy at any age.  this otherwise will relentlessly progress until fused.
        • older patients with significant progression, neurologic deficits, or declining respiratory function
        • having many pedicle screws may decrease crankshaft phenomenon adn obviate the need for an anterior fusion.
    • anterior/posterior spinal fusion +/- vertebrectomy
      • indications
        • young patients with significant progression, neurologic deficits, or declining respiratory function
          • girls < 10 yrs
          • boys < 12 yrs
        • patients with failure of formation with contralateral failure of segmentation at any age that requires hemi-vertebrectomy and/or significant correction. This may be done from a posterior approach 
      • technique
        • nutritional status of patient must be optimized prior to surgery
    • distraction based growing rod construct
      • indications
        • may be used in an attempt to control deformity during spinal growth and delay arthrodesis
      • outcomes
        • need to be lengthened approximately every 6 months for best results
    • osteotomies between ribs.
      • indications
        • mulitple (>4) fused ribs wit potential for thoracic insufficiency syndrome
      • outcomes
        • long-term follow up is needed to determine efficacy. the downside is this may make the chest stiff and hurt pulmonary function.
    • Hemi-Vertebrectomy - usally done from a posterior approach, particularly with kyphosis. 
      • indications - age 3-8 years (younger is difficult to get good anchor purchase)
      • progressive or significant deformity
Techniques
  • Spinal arthrodesis +/- vertebrectomy/osteotomy
    • 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
Complications
  • 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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(OBQ12.14) The parents of a 14-month-old boy bring their child into your office. They state the child has reached developmental milestones at appropriate ages, but noticed he was leaning to one side when standing or walking. A radiograph was obtained demonstrating a non-flexible 40-degree curve with multiple vertebral anomalies, highlighted by a convex segmented hemivertebra associated with a concave unilateral bar. After ensuring that the patient has no other associated anomalies in other organ systems, an MRI of the spine revealed no intraspinal abnormalities. What treatment would you recommend to the family? Review Topic

QID: 4374
1

Continued observation with annual follow up

8%

(304/3633)

2

Risser Casting

6%

(201/3633)

3

VEPTR

4%

(163/3633)

4

Instrumentation with growing rods without fusion

9%

(335/3633)

5

Excision of the hemivertebra with short segment posterior instrumented fusion

72%

(2607/3633)

Select Answer to see Preferred Response

PREFERRED RESPONSE 5

(OBQ07.220) Figure A demonstates different anatomic patterns in congenital scoliosis. Which pattern has the worst prognosis and is an indication for surgery. Review Topic

QID: 881
FIGURES:
1

Illustration 1

6%

(138/2384)

2

Illustration 2

1%

(16/2384)

3

Illustration 3

1%

(13/2384)

4

Illustration 4

3%

(71/2384)

5

Illustration 5

90%

(2142/2384)

Select Answer to see Preferred Response

PREFERRED RESPONSE 5

(OBQ05.116) A 2-year-old girl presents to the office for evaluation of spinal deformity. A radiograph is shown in Figure A. What additional work-up should be done? Review Topic

QID: 1002
FIGURES:
1

HLA-B27

1%

(14/2188)

2

Echocardiography

93%

(2025/2188)

3

Bone scan with SPECT images

1%

(27/2188)

4

Skeletal survey

4%

(81/2188)

5

Electromyogram

1%

(30/2188)

Select Answer to see Preferred Response

PREFERRED RESPONSE 2
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