This 2010 narrative review by Feldman et al. synthesizes the orthopedic manifestations of NF-1 — an autosomal dominant disorder affecting 1 in 3,000 people — focusing on the evaluation and management of dystrophic and nondystrophic scoliosis, congenital pseudarthrosis of the tibia, metabolic bone disease, and soft-tissue tumor complications.
When you see scoliosis in an NF-1 patient, immediately classify it as dystrophic vs. nondystrophic: count penciled ribs and dystrophic features to stratify progression risk, obtain MRI to exclude intraspinal pathology, and treat any curve >20° with dystrophic features surgically rather than with bracing.
For NF-1 patients with anterolateral tibial bowing, counsel families early about the high CPT rate, the near-universal need for eventual surgery, and the significant refracture risk even after achieving union.
This 2010 narrative review by Feldman et al. synthesizes the orthopedic manifestations of NF-1 — an autosomal dominant disorder affecting 1 in 3,000 people — focusing on the evaluation and management of dystrophic and nondystrophic scoliosis, congenital pseudarthrosis of the tibia, metabolic bone disease, and soft-tissue tumor complications.
When you see scoliosis in an NF-1 patient, immediately classify it as dystrophic vs. nondystrophic: count penciled ribs and dystrophic features to stratify progression risk, obtain MRI to exclude intraspinal pathology, and treat any curve >20° with dystrophic features surgically rather than with bracing.
For NF-1 patients with anterolateral tibial bowing, counsel families early about the high CPT rate, the near-universal need for eventual surgery, and the significant refracture risk even after achieving union.