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目的:探讨马凡氏综合征的临床及影像学诊断。方法:收集X线平片、心动超声、眼科等临床资料完整的马凡氏综合征5例,年龄11~43岁,男性3例为同胞兄弟,女性2例为母女关系。病人主要症状表现为消瘦,其中3例有不同程度四肢细长。结果:均表现左心室不同程度扩大,超声心动示左心室或/和主动脉根部扩大,四肢细长,其中3例有扁平足,3例近视及复视,有1例伴晶状体脱位。结论:骨骼及心血管特征性影像学改变,结合临床症状及家族史可以做出明确诊断。
Objective: To investigate the clinical and imaging diagnosis of Marfan syndrome. Methods: Five cases of Marfan syndrome with complete clinical data of X-ray, echocardiography and ophthalmology were collected. The age ranged from 11 to 43 years old. Three males were siblings and two females were mother-daughter relationship. The main symptoms of patients showed weight loss, of which 3 patients with varying degrees of slender limbs. Results: The left ventricle was enlarged to different extents. The left ventricle and / or aortic root were enlarged by echocardiography, and the limbs were slender. Among them, 3 had flat feet, 3 had myopia and diplopia, and 1 had lens dislocation. Conclusion: The characteristic imaging changes of the skeleton and cardiovascular can make a definite diagnosis based on clinical symptoms and family history.