Congenital Scoliosis

Author: Tracy Jones

Topic updated on 04/04/13 10:04pm

Introduction

Congenital scoliosis is defined as 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
- prevalence in general population estimated at 1% to 4%
Genetics
- most cases occur spontaneously
Associated conditions
- up to 61% of patients have associated systemic anomalies
  - cardiac defects - 10%
  - genitourinary defects - 25%
  - VACTERL syndrome- 38% to 55%
  - limb abnormalities
  - auditory deficits
  - facial asymmetry
  - dysraphism
  - Goldenhar syndrome
  - thoracic insufficiency syndrome (TIS)
    - associated with severe congenital scoliosis
      - also caused by
        
        severe idiopathic scoliosis
        
        Jarcho-Levin syndrome
    - caused by shortening of the thorax and rib fusions
    - result is thorax is unable to support lung growth
    - result is respiratory decompensation
  - Jarcho-Levin syndrome
    - a condition characterized by extensive congenital fusions
    - two subtypes include
      - spondylothoracic dysplasia
        
        primarily involves vertebral bodies
      - spondylocostal dysplasia
        
        involves fused or missing ribs
Prognosis
- progression
  - progression is more rapid in the first 3 years of life
  - risk of progression is determined by the morphology of vertebrae
    - unilateral unsegmented bar with contralateral hemivertebra >
      - has greatest potential for rapid progression (5 to10 degrees/year)
    - unilateral unsegmented bar >
    - fully segmented hemivertebra >
    - unsegmented hemivertebra >
    - incarcerated hemivertebra >
    - unincarcerated hemivertebra >
    - block vertebrae
      - have 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)
Failure of Segmentation	Bar body (unilateral unsegmented bar is common and likely to progress)
Mixed	Unilateral unsegmented bar with contralateral hemivertebra (most rapid progression)

Imaging

Radiographs
- AP and lateral plain films usually sufficient to confirm diagnosis
CT
- 3D CT useful to better delineate posterior bony anatomy and define type
  - judicious routine use recommended due to radiation exposure
MRI
- indications
  - all patients with congenital scoliosis to evaluate for
    - need to evaluate for neural axis abnormality (found in 20-40%)
      - Chiari malformation
      - tethered cord
      - syringomyelia
      - diastematomyelia
      - intradural lipoma
  - prior to any surgical intervention
- technique
  - sedation required in infants so may be delayed if no surgery is planned and no neuro deficits
Additional studies
- important to obtain studies for associated abnormalities
  - renal ultrasound or MRI
  - echocardiogram if suspicion for cardiac manifestations
  - pyelography

Treatment

Nonoperative
- observation & bracing
  - indications
    - absence of documented progression as usually seen with
      - incarcerated hemivertebrae
      - nonsegmental hemivertebrae
      - some partially segmented hemivertebrae
  - bracing
    - is not indicated in the treatment of congenital scoliosis (no effectiveness shown)
    - may be used to control supple compensatory curves
Operative
- in situ posterior fusion
  - indications
    - older patients with significant progression, neurologic deficits, or declining respiratory function
      - girls > 10 yrs
      - boys > 12 yrs
- 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
    - nutritional status of patient must be optimized
- 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
- VEPTR (vertical expanding prosthetic titanium rib)
  - indications
    - thoracic insufficiency syndrome
  - outcomes
    - long-term follow up is needed to determine efficacy

Techniques

Spinal arthrodesis +/- vertebrectomy/osteotomy
- in situ arthrodesis, anterior/posterior or posterior alone
  - indications
    - unlateral unsegmented bars with minimal deformity
- in situ anterior and/or posterior contralateral hemiepiphysiodesis with hemiarthrodesis
  - indications
    - progressive fully segmented hemivertebrae
    - patients less than 5 yrs. with < 40 degree curve
    - no signs of truncal imbalance
- hemivertebrectomy
  - indications
    - 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
- vertebrectomy/anterior and/or posterior osteotomy
  - 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
- risk factors include
  - overdistraction during surgery
  - overcorrection during surgery
  - harvesting of segmental vessels
- somatosensory and motor evoked potentials important during surgery
Soft-tissue compromise
- nutritional aspects of care essential to ensure adequate soft tissue healing

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Qbank (3 Questions)

TAG

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

Review Topic

FIGURES: A

1. Illustration 1
2. Illustration 2
3. Illustration 3
4. Illustration 4
5. Illustration 5

PREFERRED RESPONSE ▶

DISCUSSION: Congenital scoliosis is caused by anatomic anomalies of the vertebral bodies. The different type of anomalies can be divided into failure of formation (hemivertebrae, wedge vertebrae, butterfly vertebrae) and failure of segmentation (block vertebrae, bar body). The worst situation is when there is failure of formation with contralateral failure of segmentation. An example of this is a hemivertebrae with a contralateral bar body as depicted in Illustration 5. This has the worst prognosis and is an indication for surgery.

REFERENCES:

2. Hedequist D, Emans J. Congenital scoliosis. J Am Acad Orthop Surg. 2004 Jul-Aug;12(4):266-75. Review. PMID:15473678 (Link to Abstract)

Question Authors:

Derek Moore, Derek Moore, Patrick McCulloch MD,

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TAG

(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? Topic

Review Topic

FIGURES: A

1. HLA-B27
2. Echocardiography
3. Bone scan with SPECT images
4. Skeletal survey
5. Electromyogram

PREFERRED RESPONSE ▶

DISCUSSION: The radiograph demonstrates congenital scoliosis with defects in formation (resulting in the hemivertebrae) and segmentation. Basu et al assessed the incidence of intraspinal anomalies and other organic defects associated with different types of congenital spinal deformity using MRI, echocardiography, renal ultrasound, and a thorough clinical assessment. Intraspinal abnormalities were found in 37% of patients - more commonly in those with congenital kyphosis and scoliosis resulting from mixed and segmentation defects. Cardiac defects were detected in 26% and urogenital anomalies in 21% of patients. Based on their finding, they argue magnetic resonance imaging and echocardiography should be an essential part in the evaluation of patients with congenital spinal deformity. Renal ultrasound should be considered, although a complete spine MRI may detect renal pathology as well.

REFERENCES:

1. Basu PS, Elsebaie H, Noordeen MH. Congenital spinal deformity: a comprehensive assessment at presentation. Spine (Phila Pa 1976). 2002 Oct 15;27(20):2255-9. PMID:12394903 (Link to Abstract)

Question Authors:

Ben Taylor MD, Derek Moore, Eric Shirley MD,

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Groups

ABOS II: Congenital Scoliosis

General - Oral Boards Review

7/29/2011

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Evidence & References Show References

Level of Evidence 4 (Case Series)

Basu PS, Elsebaie H, Noordeen MH. Congenital spinal deformity: a comprehensive assessment at presentation. Spine (Phila Pa 1976). 2002 Oct 15;27(20):2255-9. PMID:12394903 (Link to Abstract)

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012

Undefined

King AG, MacEwen GD, Bose WJ. Transpedicular convex anterior hemiepiphysiodesis and posterior arthrodesis for progressive congenital scoliosis. Spine (Phila Pa 1976). 1992 Aug;17(8 Suppl):S291-4. PubMed PMID: 1523515. PMID:1523515 (Link to Abstract)
Holte DC, Winter RB, Lonstein JE, Denis F. Excision of hemivertebrae and wedge resection in the treatment of congenital scoliosis. J Bone Joint Surg Am. 1995 Feb;77(2):159-71. PubMed PMID: 7844121. PMID:7844121 (Link to Abstract)
Orthopaedic Knowledge Update 8. Alexander R. Vaccaro (Editor). Published by American Academy of Orthopaedic Surgeons, Rosemont, IL. Copyright 2005
Hedequist D, Emans J. Congenital scoliosis. J Am Acad Orthop Surg. 2004 Jul-Aug;12(4):266-75. Review. PMID:15473678 (Link to Abstract)

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Congenital Scoliosis

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