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Reduced volume and altered composition of paraspinal muscles in Marfan syndrome: A retrospective cohort study
Yuichi Yoshida1, Toru Doi2, Hiroyuki Oka3
1Department of Orthopaedic Surgery, The University of Tokyo Hospital, Tokyo, Japan.
Abstract:
Retrospective cohort study. Spinal deformities in patients with Marfan syndrome (MFS) are distinct from those in patients with idiopathic scoliosis (IS). It is more prone to progression and more likely to present with sagittal malalignment than IS. However, the etiology of this characteristic spinal deformity in MFS remains unclear. This study aimed to determine the spinal musculature characteristics in patients with MFS on the hypothesis that the paraspinal muscles of patients with MFS would be qualitatively or quantitatively different from those of patients with IS. Seventeen consecutive patients with MFS aged 25 years or younger undergoing surgery for scoliosis in our hospital were compared with age- and sex-matched patients with IS undergoing surgery for scoliosis. The body size-adjusted relative cross-sectional area (rCSA), fatty infiltration ratio (FI%), and relative functional cross-sectional area (rFCSA) of the psoas muscles (PM) and paravertebral muscles (PVM) at L3/4 and L4/5 were measured using preoperative T2-weighted magnetic resonance imaging. Functional CSA was defined as total CSA minus the fatty infiltration area of each muscle and rFCSA was calculated as the body size-adjusted functional CSA. The rCSA of the PM at L3/4 and L4/5 was significantly smaller in the MFS group than in the IS group (L3/4, P = .021; L4/5, P = .002). The FI% of the PM at L4/5 was significantly higher in the MFS group (P = .044). Consequently, the rFCSA of the PM at L3/4 and L4/5 and the rFCSA of the PVM at L3/4 in the MFS group were significantly smaller than those in the IS group (PM at L3/4, P = .021; PM at L4/5, P = .001; PVM at L3/4, P = .025). Compared with patients with IS, patients with MFS exhibited significantly decreased body-size-adjusted CSA of the PM and reduced body-size-adjusted functional CSA of the PVM and PM. These findings may partially explain the characteristics of distinctive spinal deformities in patients with MFS.
Insights
Patients with Marfan syndrome (MFS) show distinct spinal deformities due to reduced paraspinal muscle size and function compared to idiopathic scoliosis (IS). This study found smaller muscle cross-sectional areas and reduced functional capacity in MFS patients, potentially explaining their unique spinal issues.
Area of Science:
- Orthopedics
- Radiology
- Genetics
Background:
- Spinal deformities in Marfan syndrome (MFS) differ from idiopathic scoliosis (IS), exhibiting higher progression rates and sagittal malalignment.
- The underlying causes of these distinct spinal deformities in MFS are not fully understood.
Purpose of the Study:
- To investigate the characteristics of spinal musculature in patients with MFS.
- To test the hypothesis that paraspinal muscles in MFS patients differ quantitatively or qualitatively from those in IS patients.
Main Methods:
- Retrospective cohort study comparing 17 MFS patients (<25 years) with age- and sex-matched IS patients undergoing scoliosis surgery.
- Preoperative T2-weighted MRI was used to measure relative cross-sectional area (rCSA), fatty infiltration (FI%), and relative functional cross-sectional area (rFCSA) of psoas (PM) and paravertebral muscles (PVM) at L3/4 and L4/5.
Main Results:
- MFS patients had significantly smaller rCSA of PM at L3/4 and L4/5 compared to IS patients.
- Higher FI% in PM at L4/5 was observed in the MFS group.
- Significantly smaller rFCSA of PM (L3/4, L4/5) and PVM (L3/4) were found in MFS patients compared to IS patients.
Conclusions:
- Marfan syndrome patients exhibit reduced body-size-adjusted cross-sectional area of psoas muscles and diminished body-size-adjusted functional cross-sectional area in both psoas and paravertebral muscles compared to idiopathic scoliosis patients.
- These muscular differences may contribute to the characteristic spinal deformities observed in Marfan syndrome.
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