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.

Medicine
|September 29, 2023
PubMed

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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