Syndromic Scoliosis
Scoliosis can occur as part of many syndromic diseases (both genetic and non-genetic syndromes). Examples include:
- Marfan syndrome
- Ehlers-Danlos syndrome
- Other connective tissue disorders
- Muscular dystrophy
- Osteochondrodystrophy (dwarfism)
- Neurofibromatosis (NF)
- Noonan Syndrome
- VATER/VACTERL Syndrome
- Angelman Syndrome
- Rett
- Prader Willi
- Osteogenesis Imperfecta
- Trisomy 21 (Down's Syndrome)
Symptoms
Highly variable based on underlying syndrome and size of scoliosis. Usually not painful, but when severe can cause discomfort and/or pain with seating.
Diagnostics
When a child is diagnosed with a syndrome known to cause scoliosis, screening for scoliosis will begin fairly early. Examinations of the back, including x-rays, will be performed on a regular basis.
Prognosis
Because the natures of the causal syndromes are so varied, the curves that they cause in the spine progress at varied rates. Close assessment and follow-up with your pediatrician and your spine surgeon will help to manage effects of scoliosis and ensure that intervention is arranged when necessary.
Treatments
Bracing
Bracing or casting programs may help by allowing growth while minimizing increases in the scoliosis. The need for surgery may be delayed and, in some instances, avoided.
Observation
The behavior of the curve may be monitored via repeated clinic visits and x-ray examinations at various times during development for worsening or progression of the scoliosis.
Surgery
Surgery is generally recommended if brace or cast treatment should fail to keep the scoliosis from progressing, or if the curve pattern does not appear amenable to brace or cast treatment.
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