Jones Kevin B, Erkula Gurkan, Sponseller Paul D, Dormans John P
Department of Orthopaedic Surgery, Johns Hopkins Medical Institutions, Baltimore, MD 21287-0881, USA.
Spine (Phila Pa 1976). 2002 Sep 15;27(18):2003-12. doi: 10.1097/00007632-200209150-00008.
Retrospective review of records and radiographs with postoperative questionnaire of patients with Marfan syndrome receiving surgical correction for spinal deformity.
To characterize and analyze the challenges of surgical correction for spinal deformity in Marfan syndrome and to present recommendations that may reduce morbidity and increase success for these procedures.
Spinal deformities are common in Marfan syndrome and usually refractory to conservative management. The few, smaller studies of surgical intervention reported on earlier surgical techniques but suggested increased complication rates.
Records and radiographs of 39 patients with confirmed Marfan syndrome who underwent surgical management at either of two institutions for primary scoliosis (n = 26), kyphosis (n = 7), or deformity secondary to previous surgery elsewhere (n = 6) were reviewed. Presentation features, complications, and therapeutic results were analyzed. Low back outcome scores were generated from a questionnaire given to patients after surgery.
Increased blood loss and rates of infection (10%), dural tear (8%), instrumentation fixation failure (21%), pseudarthrosis (10%), and coronal (8%) and sagittal (21%) curve decompensation were noted. Infection was often associated with dural tear and decompensation with extreme correction. One patient died of valvular insufficiency 11 weeks after surgery.
The cardiopulmonary condition of patients with Marfan syndrome should be evaluated and planned for before surgery. Preoperative computed tomograph to assess bony adequacy for fixation and magnetic resonance imaging to evaluate dural ectasia are indicated. Attention paid to the sagittal profile, extension of fusion to vertebrae neutral and stable in both planes, minimization of soft tissue dissection, and avoidance of extreme correction may prevent curve decompensation.
对接受脊柱畸形手术矫正的马凡综合征患者的病历、X线片及术后问卷进行回顾性研究。
描述和分析马凡综合征脊柱畸形手术矫正的挑战，并提出可能降低发病率和提高手术成功率的建议。
脊柱畸形在马凡综合征中很常见，通常对保守治疗无效。少数规模较小的手术干预研究报道了早期的手术技术，但提示并发症发生率增加。
回顾了39例确诊为马凡综合征的患者的病历和X线片，这些患者在两家机构之一接受了原发性脊柱侧凸（n = 26）、后凸畸形（n = 7）或其他部位先前手术后继发畸形（n = 6）的手术治疗。分析了临床表现、并发症和治疗结果。通过术后向患者发放问卷得出腰痛结局评分。
发现术中失血增加，感染率（10%）、硬脊膜撕裂（8%）、内固定失败（21%）、假关节形成（10%）以及冠状面（8%）和矢状面（21%）的曲线失代偿情况。感染常与硬脊膜撕裂相关，而失代偿与过度矫正有关。1例患者术后11周死于瓣膜功能不全。
术前应评估和规划马凡综合征患者的心肺状况。建议术前行计算机断层扫描以评估固定的骨量充足情况，并行磁共振成像以评估硬脊膜扩张情况。注意矢状面形态，将融合范围扩展至在两个平面均中立且稳定的椎体，尽量减少软组织剥离，并避免过度矫正，可能预防曲线失代偿。
