For details on Neurofibromatosis Type 1 (NF-1), I will refer
you to Wikipedia: https://en.wikipedia.org/wiki/Neurofibromatosis_type_I
As this blog focuses on spinal deformity in children and adolescents
following is a brief summary as it relates to the musculoskeletal system (spine
1. NF-1 causes tumors along nervous system which
can grow anywhere on the body.
2. Musculoskeletal abnormalities:
a. Spine: Meningocoeles, dural ectasia, scoliosis,
b. Skeletal muscle weakness
c. Long bones: pseudarthrosis (most commonly
tibia), limb hypertrophy
3. Approximately 20% of NF-1 patients will have
4. There are two types of spinal deformity in NF-1
Idiopathic-like: looks and behaves more like
i. Sharp, angulated spine deformity (kyphosis, scoliosis and kyphoscoliosis)
ii. More common in the thoracic spine
iii. Spines can start out having a more idiopathic-like deformity, which can change into a dystrophic type.
iv. Causes penciling or thinning of the rib heads which can migrate into the spinal canal
9 year old female
5. Dural Ectasia: Circumferential enlargement or
ballooning of the thecal sac, nerve root sleeves and spinal canal.
More common in lumbar spine
Causes vertebral body scalloping
Also thins the pedicles
For the idiopathic-like deformities, lower
magnitude deformities are amenable to bracing. Surgical treatment mirrors
guidelines for idiopathic scoliosis.
For the dystrophic deformities:
is limited in effectiveness
is performed for lower magnitude deformities due to the sharper-angulated
deformities, increasing difficulty in achieving necessary spinal fixation and
the risk of neurologic changes (such as weakness, sensory changes,
Next several blog posts actual surgical cases of NF-1 will be presented.