Sagittal balance in scoliosis associated with Marfan syndrome: A stereoradiographic three-dimensional analysis

Abstract

Purpose Marfan syndrome (MFS) is a genetic disease often marked by the presence of scoliosis. There is no three-dimensional analysis of the deformity in the literature. Our aim was to determine what kind of sagittal balance defines scoliosis associated with MFS, namely a flexion deformity, as it is in scoliosis associated with Chiari I or an extension deformity, as in adolescent idiopathic scoliosis (AIS). To address this issue, we compared the presence or absence of a thoracic scoliosis with the presence or absence of a segment in extension in the thoracic spine. Methods In our series, 30 patients diagnosed with Marfan syndrome were prospectively included. In each patient, personalized three-dimensional reconstruction from T1 to L5 of the spine was made using stereoradiography. The patients were first separated based on the presence or absence of thoracic scoliosis, in order to compare this with the presence or absence of a segment in extension in the thoracic spine. They were then classified into two groups based on the presence or absence of the segment in extension (meaning containing negative values of intervertebral sagittal rotation) in the thoracic spine.Results Among scoliotic patients with a thoracic scoliosis (17 cases), there were 13 (76.5% cases) with a segment in extension in the thoracic spine and 4 with no segment in extension.Conclusions Our results showed that scoliosis associated with MFS is somehow original, demonstrating a sagittal balance in extension (as AIS) in about 80% of thoracic curves, but without this characteristic feature in about 20%.

Estudo morfológico e hemodinâmico da aorta e análise do remodelamento das microfibrilas nos órgãos do sistema circulatório do modelo mg∆lpn sob a terapêutica com Losartan®

“…Pela ampla distribuição da proteína fibrilina-1 na MEC, encontra-se na MFS alterações em seus sítios de predominância, como: dismorfologia óssea, escolioses, enfisema pulmonar, edema pulmonar, ruptura dos alvéolos pulmonares, pneumotórax espontâneo, glomerulonefrite com membrana proliferativa, glomeruloesclerose segmentar e focal, dilatação da aorta, aneurismas, dissecções da aorta, ectopia da lente, ceratocone, córnea plana e hérnias no sistema digestório (ROBINSON, GODFREY, 2000;SALLUM et al 2002;NEPTUNE et al 2003;BOSEMAN et al 2008; GLARD et al 2008;BALDWIN et al, 2013;PYERITZ, 2018).…”

“…Os pacientes apresentam altura acima da média, membros longos e com alta mobilidade, escoliose e malformações do esterno e do palato (GLARD et al 2008;PYERITZ, 2018). Essas alterações causam dores musculares, nos ossos e nas articulações.…”

“…As malformações e fragilidade da coluna espinhal podem acarretar graves danos e alguns casos de óbito já foram relatados em pacientes que sofreram subluxação espontânea atlanto-axial (GLARD et al 2008;PYERITZ, 2018).…”