目的

    分析比较马方综合征并脊柱侧凸（Marfan syndrome scoliosis, MSS）患者与青少年特发性脊柱侧凸（Adolescent idiopathic scoliosis, AIS）患者行后路矫形手术治疗效果的差异。

方法

    分析MSS患者与AIS患者的临床及影像学资料，所有患者均采用单一后路矫形手术，对手术时间、手术失血量、输血量、手术前后侧凸参数、固定节段、矫形率、身高变化。

结果

    入选MSS患者42例，男11例，女31例，平均年龄15.2岁，侧凸类型单弯10例，双弯24例，三弯8例。按照1:4配比，入选AIS患者168例，男34例，女134例，平均年龄14.5岁，侧凸类型单弯40例，双弯96例，三弯32例。MSS组23例患者得到随访，AIS组94例得到随诊，平均随访时间分别为18.4月和18.5月。二组患者术前侧凸主弯冠状面Cobb角、末次随诊时主弯冠状面Cobb角及矫形率、手术融合节段、手术时间、手术失血量、输血总量平均分别为60.4°和57.5°、14.6°和15.2°、76.4%和74.1%、11.5和11.0个椎体、4.6和4.0小时、845ml和698ml、1151ml和894ml。。

结论：

马方综合征脊柱侧凸患者后路手术矫形效果与青少年特发性脊柱侧凸患者无明显差异，但手术时间更长、手术失血量更多。

关键词：马方综合征；特发性脊柱侧凸；手术；矫形。

English Abstract

Comparison of posterior correction results between Marfan syndrome scoliosis and adolescent idiopathic scoliosis

Aims：To analyze whether there are differences of posterior correction surgery in Marfan syndrome scoliosis(MSS) with that of adolescent idiopathic scoliosis(AIS).

Methods：All the patients included underwent posterior correction surgery.The radiographic data, operation duration, estamited blood loss, transfusion, fusion levels and correction rate，mental health scores of Scoliosis Research Society-22(SRS-22) questionnaire were analyzed between the 2 groups.

Results：Group MSS included 42 patients, 11 male and 31 female with an average age of 15.2 years old. Ten patients had a single curve, and 24 patients had 2 curves and the other 8 patients had 3 curves. Group AIS included 168 patients(ratio,1:4), 34 male and 134 female with an average age of 14.5 years old. Forty patients had a single curve, and 96 patients had 2 curves and the other 32 patients had 3 curves. Twenty-three patients in group MSS and 94 patients in group AIS followed up, with an average time of 18.4 months and 18.5 months, respectively. The mean coronal Cobb angle of the major curve before operation and at final follow-up, the correction rate, fusion level, operation duration, estimated blood loss(EBL) during operation, transfusion bwtween the 2 groups were 60.4° and 57.5°, 14.6° and 15.2°, 76.4% and 74.1%, 11.5 vertebra and 11.0 vertebra, 4.6 hours and 4.0 hours, 845ml and 698ml, and 1151ml and 894ml, respectively. The age, gender ratio, curve type and coronal Cobb angle of the major curve were all matched(all P>0.05). Group MSS had a longer operation duration and more EBL compared with those of group AIS(both P<0.05), while there was no significant difference in terms of fusion level, transfusion, coronal Cobb angle of the major curve at final follow-up and correction rate(all P>0.05).

Conclusions：When perform scoliosis correction, the patients with Marfan syndrome scoliosis had a more EBL, and longer operation duration than those of AIS patients, while the correction rate was similar. In surgically treated patients, MSS patients had lower mental health scores of SRS-22 questionnaires compared with those of AIS patients.

[Key words] Marfan syndrome，idiopathic scoliosis, surgery，deformity correction