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Congenital Scoliosis and Vertebral Defects

- See:
       - Crankshaft Phenomenon:
       - Epiphysiodesis
       - Hemivertebrae:
       - Pathogenesis
       - Spine Development
       - Segmented Vertebrae:
       - Spina Bifida
       - Spondylosis
       - Unilateral Bar

- Associated Malformations:
    - spinal / neurologic:
          - dysraphic malformations: (40%) (diastemotmyelia);
          - tethered cord;
          - spinal cord tumor (esp w/ back pain & cavus foot)
          - kyphosis may result in paralysis from the stretch imposed upon spinal cord;
          - arnold chiari, syringomyelia;
          - ref: Prevalence of Neural Axis Abnormalities in Patients with Infantile Idiopathic Scoliosis.
    - genito-urinary:
          - relatively high incidence of renal/urogenital anomalies: 20%
          - need to get ivp or ultrasound prior to surgery;
          - references: 
                      Congenital abnormalities of the urogenital tract in association with congenital vertebral malformations
                      Magnetic Resonance Imaging of Renal Abnormalities in Patients with Congenital Osseous Anomalies of the Spine
    - cardio-pulmonary:
          - congenital heart defects: 10-15%
          - abnormal curvatures of the spine may, for mechanical reasons, result in problems that are not themselves malformations:
                - pulmonary dysfunction, cor pulmonale, and back pain;
          - references: Natural history of scoliosis in congenital heart disease.
    - maternal diabetic influence on the spine:
          - Caudal regression syndrome and popliteal webbing in connection with maternal diabetes mellitus: a case report and literature review.
          - Caudal Regression Syndrome in twin pregnancy with type II diabetes.

- Exam:
    - neurologic changes
    - dermal lesion
    - foot deformities (c/w diastematomyelia or spinal dysraphism)

- Radiographs:
    - rib vertebral angle:
          - angle formed by perpendicular line from apical vertebral end plate and a second line from the mid-neck to mid-head of the adjacent rib;
          - the rib vertebral angle difference is the difference between the two RVA on the concave and convex sides of the curve;
                 - a curve greater than 20 is considered progressive;
          - reference:
                 The rib vertebral angle in the early diagnosis betweeen resolving and and progressive infantile scoliosis

- MRI:
    - indicated prior to any planned surgical procedure to rule out spinal dysraphism, which might not be amenable to treatment once a fusion is performed;
    - ref: Routine use of magnetic resonance imaging in idiopathic scoliosis patients less than eleven years of age.

- Non Operative Treatment:
    - risk of progression:
           - note that 50% of patients will have significant curve progression;
    - bracing:
           - is primary treatment for pts w/ infantile & juvenile idiopathic scoliosis.
           - infantile & juvenile scoliotic curves that are relentlessly progressive beyond 50 degrees despite bracing are very difficult to treat.

- Surgical Treatment Options:
    - posterior fusion:
    - hemivertebrae:
          - epiphysiodesis:
          - hemivertebra excision:
                 - w/ sizable curve are likely to progress & do not regress, making surgical treatment necessary

One-stage anterior and posterior hemivertebral resection and arthrodesis for congenital scoliosis.

Congenital scoliosis: a study of 234 patients treated and untreated.  

Scoliosis in arthrogryposis multiplex congenita.

Occult intraspinal anomalies and congenital scoliosis.

Posterior spinal arthrodesis for congenital scoliosis. An analysis of the cases of two hundred and ninety patients, five to nineteen years old.

The results of spinal arthrodesis for congenital spinal deformity in patients younger than five years old.

Variability in Cobb angle measurements in children with congenital scoliosis.

Posterior spinal fusion for congenital scoliosis. analysis of 290 patients, 5 to 19 years old.   

The natural history of congenital scoliosis: a study of two hundred and fifty one patients

Growth of the Thoracic Spine in Congenital Scoliosis After Expansion Thoracoplasty.