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Spinal Deformity in Russell-Silver Syndrome
Kent T Yamaguchi1, Jennifer B Salem1, Karen S Myung1
1Children's Orthopaedic Center, Children's Hospital of Los Angeles, 4650 W Sunset Boulevard, Mailstop #69, Los Angeles, CA 90027, USA.
Patients with Russell-Silver syndrome (RSS) have a high prevalence of spinal deformities like scoliosis and kyphosis. Many individuals with RSS may require corrective spinal surgery.
Area of Science:
- Medical research
- Genetics
- Orthopedics
Background:
- Russell-Silver syndrome (RSS) is a growth disorder characterized by body asymmetry.
- Previous research has not extensively evaluated spinal deformities in RSS patients.
Purpose of the Study:
- To determine the prevalence of scoliosis and kyphosis in individuals with Russell-Silver syndrome.
- To assess the need for surgical intervention for spinal deformities in RSS patients.
Main Methods:
- A cross-sectional analysis was conducted on 163 individuals with RSS.
- Participants completed surveys, and spinal radiographs were analyzed for scoliosis and kyphosis using Cobb angles.
Main Results:
- Overall, 21% of RSS patients exhibited spinal deformities (scoliosis and/or kyphosis).
- The average age of diagnosis for spinal deformities was 8 years.
- 18% of those with spinal deformities underwent or planned corrective surgery.
Conclusions:
- Individuals with RSS have a high prevalence of spinal deformities.
- A significant proportion of RSS patients with spinal deformities require surgical correction.
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