Spinal Abnormalities Associated with Marfan Syndrome

Lee, Chong Suh; Chung, Sung Soo; Lee, Saeng Guk; Son, Han Seok; Kim, Sung Min

Abstract

STUDY DESIGN: This is a retrospective review on the spinal abnormalities in Marfan syndrome.
OBJECTIVES
To analyze clinical and radiological features of the spine in Marfan syndrome. SUMMARY OF LITERATURE: Scoliosis & kyphosis are common spinal abnormalities in Marfan syndrome. Other spinal abnormalities associated with Marfan syndrome are rarely reported in the literature.
MATERIALS AND METHODS
The spinal abnormalities which can be associated with Marfan syndrome were analyzed in 32 patients who were diagnosed as Marfan syndrome. Coronal and sagittal curvatures on whole spine standing film were evaluated with Cobb's method. Uncommon spinal abnormalities which can be associated with this syndrome, such as spondylolisthesis and dural ectasia were also evaluated.
RESULTS
Scoliosis was identified in 18 of 32 patients(56.3%). The mean scoliotic angle was 26 degrees and thoracic curve was most common. Thoracic kyphosis, lumbar lordosis and thoracolumbar kyphosis were measured as 27 degrees +/- 13 degrees ( 0 degrees ~ 53 degrees), -42 degrees +/- 13 degrees( -10 degrees ~ -70 degrees ) and 9 degrees +/- 12 degrees ( -5 degrees ~ 4 5 degrees) respectively. Spondylolisthesis was present in two cases(6.3%), one of which was 15% slip and the other was 50% slip. Two cases of dural ectasia were confirmed with MRI.
CONCLUSION
Marfan syndrome showed high incidence of scoliosis(56.3%) and thoracolumbar kyphosis(50%). The frequency of spondylolisthesis was only slightly higher than that in the general population. The dural ectasia is a common but little known abnormality. Therefore it has a significant diagnostic value without clinical importance. In Marfan syndrome, we should keep these common and uncommon spinal abnormalities in mind.