Beals R K, Mason L
Radiology. 1981 Sep;140(3):723-5. doi: 10.1148/radiology.140.3.7280241.
The classic clinical features of Marfan syndrome include ectopic lens, valvular heart disease and dissecting aneurysm, and long, narrow extremeties associated with tall stature. When the classic features are incomplete, diagnosis is uncertain. This study presents some radiographic measurements of the skull in patients with the Marfan syndrome, in an attempt to aid diagnosis by quantitating the features defining the syndrome. Compared with the normal skull, the Marfan skull was found to be longer, taller, thicker, and to have have increased frontal sinus area. The most consistent and therefore diagnostic abnormality ws increased height.
马凡氏综合征的典型临床特征包括晶状体异位、心脏瓣膜病和夹层动脉瘤,以及与身材高大相关的四肢细长。当典型特征不完整时,诊断就不确定。本研究展示了一些马凡氏综合征患者颅骨的影像学测量结果,试图通过对定义该综合征的特征进行量化来辅助诊断。与正常颅骨相比,发现马凡氏综合征患者的颅骨更长、更高、更厚,额窦面积增大。最一致因而具有诊断意义的异常是高度增加。