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马凡综合征中的脊柱侧弯。35例患者的特征及治疗结果

Scoliosis in Marfan's syndrome. Its characteristics and results of treatment in thirty-five patients.

作者信息

Robins P R, Moe J H, Winter R B

出版信息

J Bone Joint Surg Am. 1975 Apr;57(3):358-68.

PMID:1123390
Abstract

Of sixty-four patients with Marfan's syndrome, thirty-five had scoliosis, which in 44 per cent began in the infantile and juvenile age periods. The curve patterns were double major right thoracic-left lumbar in 48 per cent and single right thoracic in 33 per cent. At the start of treatment the curves were severe (average, 72 degrees) and quite structural (average bending correction, 36 per cent). Seventy-four per cent of the thirty-five patients had back pain. Inadequately treated and untreated curves usually progressed to severe and painful deformity. Milwaukee brace treatment was generally unsuccessful because of the severity and rigidity of the curves or the poor maintenance of correction obtained. Spine fusion combined with Harrington rod instrumentation (fourteen patients) gave an average correction of 41 per cent with an average loss of 7 degrees (average follow-up, 2.3 years). Operative treatment of scoliosis is indicated in most patients with Marfan's syndrome.

摘要

在64例马方综合征患者中,35例患有脊柱侧弯,其中44%始于婴幼儿期和青少年期。侧弯类型中,48%为双主弯(右胸弯-左腰弯),33%为单右胸弯。治疗开始时,侧弯严重(平均72度)且相当僵硬(平均弯曲矫正度为36%)。35例患者中有74%有背痛。未得到充分治疗和未治疗的侧弯通常会发展为严重且疼痛的畸形。由于侧弯严重且僵硬或矫正维持不佳,密尔沃基支具治疗通常不成功。脊柱融合术联合哈灵顿棒内固定术(14例患者)平均矫正度为41%,平均丢失7度(平均随访2.3年)。大多数马方综合征患者的脊柱侧弯需手术治疗。

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