Scoliosis in Neurofibromatosis


- See:
      - Neurofibromatosis
      - Scoliosis

- Discussion:  
    - 2 subtypes: idiopathic and dystrophic;
    - dysplastic type: (dystrophic)
          - short, sharply angulated curve which involve only few vertebra;
          - associated w/:
                   - neural foramina enlargement
                   - rib penciling
                   - kyphosis;
                   - vertebral body scalloping
                   - dural ectasia
                   - soft tissue masses;
                   - references:
                         - Pathophysiology of spinal deformities in neurofibromatosis. An analysis of 71 patients who had curves associated with dystrophic changes
                         - Modulation of spinal deformities in patients with neurofibromatosis type 1.

- MRI: evaluates dystrophic forms to characterize soft tissue masses;

             

- Treatment: of Dysplastic Scoliosis;
    - one can expect relentless progression that is refractory to bracing;
    - surgical fusion is therefore required;
    - w/ scoliosis alone: posterior arthrodesis (w/ internal fixation);
    - w/ associatted kyphosis there is increases risk of paraplegia & pseudo-arthrosis following surgery;
    - anterior fusion combined w/ posteiror instrumentation & fusion is indicated when kyphosis > 50 deg or scoliosis is > 80 deg



Pitfalls of spinal deformities associated with neurofibromatosis in children.

Scoliosis surgery in neurofibromatosis.

Cotrel-Dubousset instrumentation in neurofibromatosis spinal problems.

Cervical spine abnormalities in neurofibromatosis.

Spine deformity in neurofibromatosis. A review of one hundred and two patients.

Pathophysiology of spinal deformities in neurofibromatosis. An analysis of seventy-one patients who had curves associated with dystrophic changes.

Neurofibromatous scoliosis. Natural history and results of treatment in thirty-seven cases.



Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Wednesday, June 20, 2012 2:12 pm