接受心脏手术的Marfan综合征患者合并脊柱侧凸的特点

General features of scoliosis in patients with Marfan syndrome undergoing cardiovascular surgery

目的:探讨接受心脏手术治疗的Marfan综合征患者合并脊柱侧凸的患病率、侧凸程度、类型及特点。方法:回顾性总结138例进行心脏外科手术的Marfan综合征患者的X线资料,测量冠状面和矢状面Cobb角,并对数据进行统计分析。结果:58例(42.03%)患者合并脊柱侧凸,男38例,女20例,男女患病率比例为1.18∶1,其中≤10岁6例,11～20岁12例,21～30岁19例,31～40岁11例,41～50岁7例,51～60岁3例;平均冠状面Cobb角为26.8°±27.8°;胸弯36例,胸腰弯11例,腰弯2例,双弯6例,三弯3例;单弯中顶椎凸向右侧38例,凸向左侧11例;矢状面胸椎后凸平均为14.3°±13.2°,其中胸椎前凸5例,胸椎后凸不足40例,胸椎正常后凸12例,仅1例胸椎后凸45°;11例患者冠状面Cobb角>40°,平均年龄15.9岁。结论:在接受心脏手术的Marfan综合征患者中脊柱侧凸患病率为42.03%;脊柱侧凸类型多样,冠状位畸形以胸弯和胸腰弯多见,胸椎凸向右侧发生率较高,矢状位畸形以胸椎后凸不足为主;在青少年表现较为严重,需要严密的随访及手术矫形。

Objective：To explore the prevalence and features of seoliosis in patients with Marian syndrome who accepted cardiovascular surgery.Method：Tbe radiographic data of 138 cases with Marfan syndrome undergoing cardiovascular surgery were reviewed and the Cobb angles in coronal and sagittal plane were measured and statistically analyzed.Result：Seoliosis was identified in 58 cases with Marian syndrome （38 males and 20 females） ,the prevalence rate was 42.03%,male-female sex ratio was 1.18：1,6 cases were in younger than 10-year-old group,12 cases were in 11～20-year-old group,19 cases were in 21～30-year-old group,11 cases were in 31-40-year-old group,7 cases were in 41～50-year-old group,3 cases were in 51～60-year-old group. Mean magnitude of Cobb angle in coronal plane was 26.8°±27.8° ,the types of seoliosis curve included thoracic curve （36 cases） ,thoracolumbar curve （11 cases） ,lumbar curve （2 cases）,double curve （6 cases） and triple curve （3 cases）,apex vertebraes were convex to the right side among single curves in 38 cases while 11 cases were convex to the left side.Mean magnitude of kyphosis in sagittal plane was 14.3°±13.2° ,5 patients had thoracic lordosis and 40 patients had hypokyphosis and 12 patients had normal kyphosis.Magnitude of Cobb angle ineoronal plane was beyond 40° in 11 adolescent cases with the mean ages of 15.9 years old. Conclusion：The prevalence rate in population with Marian syndrome who accepted cardiovascular surgery was 42.03%.The various types of seoliosis in above population with more thoracic or thoraeolumbar curve and more prevalence of thoracic curve to the right side in coronal plane and more hypokyphosis in saggital plane. The severity of scoliosis among adolescent with Marian syndrome was more than other groups and patients,the former should be followed up carefully and considered surgery ff necessary.