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13 Cards in this Set
- Front
- Back
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What is the inheritance?
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AD
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What is the mutation?
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Neurohbromin on chromosome 17
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What cells does the mutation affect?
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Neural crest cells
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What percentage of cases arise from new mutations?
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50%
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What anatomic site is most commonly affected?
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Spine
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What are the Eve radiographic features of the spine with neuroiibromatosis (NF)?
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Vertebral scalloping
Enlarged neuroforamina Penciling of transverse processes Short, tight curves Atlantoaxial instability |
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Penciling of transverse processes is a marker of what?
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Impending curve progression
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What are the two basic types of spine deformities with NF?
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Dystrophic (kyphoscoliotic)
Nondystrophic (same as AIS, adolescent idiopathic scoliosis) |
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What is the threshold for treatment of dystrophic curves?
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ASF/PSF (anterior/posterior spinal fusion) if over 20 degrees
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This is the same threshold as what disorder?
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Muscular dystrophy
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What surgical complication is especially common in the neurofibromatosis patient?
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Increased risk of pseudarthrosis
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Therefore, one might consider what intervention?
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Augmentation of fusion at 6 months
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What test must be ordered preoperatively? Why?
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MRI
To exclude neurotibromas and ectasia |
