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Congenital Kyphosis

- Discussion:
    - deformity is characterized by severe angular deformity with a prominent gibbus at the apex of the curve;
    - etiology involves either failure of segmentation or defect in formation of vertebral body elements;
    - defect of formation:
          - failure of formation of the anterior elements produces the worst deformity;
          - prognosis for defect in formation is considerably worse;
          - natural history involves relentless progression of deformity;
          - sequelae:
                 - paraplegia commonly results if untreated;
                 - breakdown of the overlying soft tissues;
                 - compression of the abdominal viscera;
                 - impairment of pulmonary function;
                 - poor sitting posture;
    - defect of segmentation:
           - slightly better prognosis;
           - produces a more rounded kyphotic shape;
           - deformity progresses more slowly and paraplegia is uncommon;

- Associated Conditions:
      - congenital scoliosis:
      - mucopolysaccharidoses:
      - spondyloepiphyseal dysplasia congenita:

- Posterior Fusion:
     - in children < 5 yrs w/ curves < 55 deg, only an situ posterior arthrodesis is required since some spontaneous correction of kyphosis 
           will occur with continued growth;
     - posterior fusion may have wider indications w/ kyphosis due to failure of segmentation (as opposed to failure of formation);
     - posterior kyphectomy & arthrodesis involve meticulous care of tissues;
     - resection of the non-functioning cord at apex of the deformity;
     - water-tight dural closure, w/ care being taken not to occlude terminal end of normal spinal cord at site of transection of cord;
     - bivalve total-contact orthosis is used to support trunk until fusion is solid;

- Anterior & Posterior Fusion:
     - combined anterior & posterior fusion is indicated for children > 5 years, and curves > 55 deg;
     - paralytic deformity:
             - reconstruction begins w/ anterior release w/ interbody arthrodesis;
             - decompression of spinal cord anteriorly is required;;
             - as a staged procedure, posterior arthrodesis is followed by segmental spinal fixation to pelvis;
     - reduction:
            - correction of kyphotic defomrity carries a high risk of paraplegia, esp when it is tried preoperatively w/ traction;
                   - this will stretch cord over fixed kyphotic deformity;
            - congenital kyphosis may be corrected intraoperatively if intra operative cord monitoring if available;
      - anterior arthrodesis:
            - requires strut graft as well as interbody arthrodesis;
                   - anterior unsegmented bar will develop in growing child if interbody arthrodesis is not added to procedure since unfused 
                           vertebral bodies will continue to grow & contribute to the deformity;
            - strut graft is placed as close to vertebral bodies as possible to reduce the risk of pseudoarthrosis;
      - vertebral resection:
            - w/ severe deformity present at birth, vertebral resection and a short arthrodesis may be required for closure of overlying soft tissues;
            - loss of correction with growth is common, and arthrodesis of entire spine may be required by age 10 yrs

Congenital kyphosis due to defects of anterior segmentation.

Kyphosis in childhood and adolescence.

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