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Dystrophic spinal deformity in neurofibromatosis
1Department of Surgery, Dartmouth-Hitchcock Medical Center, Hanover, New Hampshire.
Journal of Pediatric Orthopedics
|July 1, 1990
Summary
Neurofibromatosis often causes dystrophic spinal deformities. Isolated posterior fusion in these patients leads to high rates of pseudarthrosis and curve progression, necessitating revision surgery.
Area of Science:
- Orthopedics
- Genetics
- Neurosurgery
Background:
- Neurofibromatosis is a genetic disorder that can cause significant spinal deformities.
- Dystrophic spinal curves are a common manifestation in patients with neurofibromatosis.
Purpose of the Study:
- To investigate spinal deformity patterns in neurofibromatosis patients.
- To determine the incidence of pseudarthrosis and curve progression after spinal fusion in this population.
Main Methods:
- Retrospective review of 32 patients with neurofibromatosis and spinal deformity.
- Analysis of curve patterns, fusion techniques, pseudarthrosis rates, and curve progression.
Main Results:
- 72% of patients exhibited dystrophic curve patterns.
- 38% incidence of pseudarthrosis in the dystrophic group undergoing isolated posterior fusion.
- Average curve progression of 12.7 degrees and 1.7 revision procedures required for solid fusion.
Conclusions:
- Dystrophic spinal curves in neurofibromatosis are associated with high rates of pseudarthrosis and progression after isolated posterior fusion.
- Combined anterior/posterior fusion is recommended for kyphoscoliotic curves in neurofibromatosis.