Patients with a double curve pattern in which the thoracic curve is >35 degrees and the LPR angle is >12 degrees are significantly more likely to demonstrate curve progression. In-brace correction for double curves of at least 25% and a patient's ability to wear the orthosis >18 hours/day significantly increased the likelihood of success.
Spinal deformities in patients with neurofibromatosis 1 should be regarded as deformities in evolution. One should resist assigning these evolving deformities to either the dystrophic or nondystrophic end of the spectrum without considering the possibility of modulation across the spectrum. A spinal deformity that develops before 7 years of age should be followed closely for evolving dystrophic features (i.e., modulation). When a curve acquires either three penciled ribs or a combination of three dystrophic features, clinical progression is almost a certainty.
C-1 lateral mass screw placement is generally feasible and safe in pediatric patients. With a nearly 2-year average follow-up, C1-2 rigid screw/rod fixation has proven to be an effective treatment modality for pediatric atlantoaxial instability in our series.
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