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Review Question - QID 6094

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QID 6094 (Type "6094" in App Search)
A 10-year-old girl who is Risser stage 0 has back deformity associated with neurofibromatosis type 1 (NF1). She has no back pain. Examination shows multiple cafe-au-lait nevi with normal lower extremity neurologic function and reflexes. Standing radiographs of the spine show a short 50-degree right thoracic scoliosis with a kyphotic deformity of 55 degrees (apex T8). A 10-degree progression in scoliosis has occurred during the past 1 year. There is no cervical deformity. MRI shows mild dural ectasia, primarily in the upper lumbar region. Management should consist of

observation with repeat radiographs in 6 months.

4%

25/704

a thoracolumbosacral orthosis (TLSO).

14%

97/704

in situ posterior spinal fusion without instrumentation, followed by full-time TLSO bracing.

9%

63/704

anterior spinal convex hemiepiphysiodesis.

3%

21/704

combined anterior and posterior spinal arthrodesis with instrumentation.

70%

491/704

Select Answer to see Preferred Response

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Scoliotic deformities in patients with NF1 are often dysplastic with short, angular curves. Posterior arthrodesis is made more difficult by the presence of kyphosis and of weak posterior elements caused by dural ectasia. Combined anterior and posterior spinal arthrodesis is generally preferred for progressive dysplastic curves to maximize deformity correction and to decrease the risk of pseudarthrosis. Anterior fusion may also prevent crankshaft phenomenon in young children. Brace treatment is not effective for large, rigid, or dysplastic curves.

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