Three-dimensional analysis of the vertebral rotation associated with the lateral deviation in Marfan syndrome spinal deformity.

Abstract

Marfan syndrome (MFS) is a genetic disease often marked with the presence of scoliosis, which is poorly described in the literature. No three-dimensional analysis of the deformity in the literature is observed. Thirty patients diagnosed with MFS were prospectively included in our series. Each patient was proposed to undergo a stereoradiographic examination of the spine. Personalized three-dimensional reconstruction from T1 to L5 of the spine were made. The Cobb angle of each curve and the axial rotation of each vertebra were assessed. Our aim was to assess whether there is a correlation between the Cobb angle and the axial vertebral rotation at the apex of the curves associated with MFS. Fourteen females and 16 males were included. The mean age was 25.9 years, ranging from 4 to 65 years. Eleven patients were under 16 years. Nineteen patients were defined as scoliotic (Cobb angle over 10 degrees ), and 11 were defined as nonscoliotic (Cobb angle under 10 degrees ). A strong correlation (Pearson) between the Cobb angle and the axial vertebral rotation at the apex of the curves associated with MFS was observed. This is the first step of a three-dimensional analysis of the scoliosis associated with MFS. This study pointed out that the vertebral axial rotation in scoliosis associated with MFS compared with that observed in adolescent idiopathic scoliosis and in scoliosis associated with cerebral palsy patients. II (Diagnostic Study).