Daeubler Bernd F, Carrel Thierry, Kujawski Tomasz, Schnyder Aurelia, Zurmuehle Pia, Vock Peter, Anderson Suzanne E
Department of Radiology, University Hospital, Inselspital, Freiburgstrasse 20, Berne CH-3010, Switzerland.
AJR Am J Roentgenol. 2006 May;186(5):1246-51. doi: 10.2214/AJR.05.0071.
The purpose of this study was to determine if the thoracic vertebral elements are altered in patients with Marfan's syndrome.
Thirty patients underwent helical CT of the thorax because of suspected thoracic aortic dilatation and acute dissection. Thirteen had Marfan's syndrome and 17 did not. Two reviewers, unaware of the final diagnosis, evaluated the images by consensus for laminar thickness, foraminal width, dural sac ratios, and vertebral scalloping for T2-T12.
At T9-T12, dural sac ratios at the midcorpus level (p = 0.031) and foraminal width (p = 0.0124) were significantly greater in the patients with Marfan's syndrome than in the patients without. Dural sac ratios at lower endplate levels (p = 0.0685), laminar thickness (p = 0.951), and vertebral scalloping (p = 0.24) were not significantly greater in the patients with Marfan's syndrome than in the patients without.
Because the phenotypic expression of Marfan's syndrome is variable, information on the spine from thoracic studies in combination with major criteria may be helpful clinically.
本研究旨在确定马凡综合征患者的胸椎结构是否发生改变。
30例因疑似胸主动脉扩张和急性夹层而接受胸部螺旋CT检查的患者。其中13例患有马凡综合征，17例未患。两名不知最终诊断结果的评估者通过共识对T2 - T12的椎板厚度、椎间孔宽度、硬膜囊比例和椎体扇贝样变进行图像评估。
在T9 - T12，马凡综合征患者在椎体中部水平的硬膜囊比例（p = 0.031）和椎间孔宽度（p = 0.0124）显著大于非马凡综合征患者。马凡综合征患者在下终板水平的硬膜囊比例（p = 0.0685）、椎板厚度（p = 0.951）和椎体扇贝样变（p = 0.24）与非马凡综合征患者相比无显著差异。
由于马凡综合征的表型表达具有变异性，结合主要标准的胸部研究中关于脊柱的信息在临床上可能会有所帮助。
