Gayet L E
Service d'orthopédie-traumatologie adulte et infantile H2A, hôpital Jean-Bernard, CHRU de la Miléterie, Poitiers, France.
Chirurgie. 1999 Sep;124(4):423-31. doi: 10.1016/s0001-4001(00)80016-1.
The purpose of this retrospective study is to demonstrate the advantages of early surgical operation for patients suffering from Duchenne muscular dystrophy scoliosis.
Since 1992, 37 patients suffering from Duchenne muscular dystrophy were operated on for scoliosis. Mean age was 12 years. Vital capacity was 62 +/- 17% and left ventricular ejection fraction 55 +/- 7%. Insertion of flexible vertebral instrumentation included a pedicular screwing system in the lumbo-sacral area and transversal attachments with steel threads at the thoracic level. A sub-laminar fastening was placed at L1. Bone bank arthrodesis was performed only at lumbo-sacral level, in order to maintain flexibility in the thoracic part of the assembly and to enable growth.
Assisted ventilation was necessary in three children during 1.5 month. Superficial sepsis was treated locally with an antibiotherapy without the removal of material in four patients. There was one stem rupture two years after operation, caused by a road traffic accident. No further procedure was necessary for technical reasons. There was no death during the longest follow-up period among the first 24 patients (mean follow-up: 57 months). In the frontal plane, the preoperative Cobb angle, which was 19 degrees, was brought to 5.2 degrees at the postoperative stage, and 9.5% at the latest measurement, i.e., a loss of angular correction of 4.3 degrees. In the sagittal plane, there were physiological curvatures. Pelvic balancing was correct and results have held over time. Vital capacity was reduced by 3.6% per year.
These results encourage early operation on these patients in order to avoid anaesthetic, peri- and postoperative complications. Likewise, giving support to minor curves reduces mechanical constraints during the first postoperative years. The absence of thoracic arthrodesis enables growth of about 5 cm when patients are operated on at about the age of 12 years. Stabilization of the myopath's spine enables the child to remain in an upright sitting position. The assembly's thoracic suppleness enables an increase in the range of movement in the upper limbs. It seems appropriate to operate on such patients when they cease walking, around the age of 12 years. Cardiorespiratory function and life expectancy are not improved, but most patients and families are very satisfied by the comfort brought about by the surgical operation.
本回顾性研究旨在证明早期手术治疗杜氏肌营养不良症脊柱侧弯患者的优势。
自1992年以来，37例杜氏肌营养不良症患者因脊柱侧弯接受手术。平均年龄为12岁。肺活量为62±17%，左心室射血分数为55±7%。柔性脊柱内固定装置的植入包括腰骶部的椎弓根螺钉系统和胸段的带钢丝横向连接。在L1处进行椎板下固定。仅在腰骶部进行骨库植骨融合，以保持组件胸段的柔韧性并促进生长。
3名儿童在1.5个月内需要辅助通气。4例患者局部应用抗生素治疗浅表脓毒症，未取出植入物。术后两年有1例棒体断裂，由交通事故引起。因技术原因无需进一步手术。在前24例患者最长随访期内（平均随访：57个月）无死亡病例。在额状面，术前Cobb角为19度，术后降至5.2度，最新测量时为9.5度，即角度矫正丢失4.3度。在矢状面，存在生理曲度。骨盆平衡良好，结果随时间保持稳定。肺活量每年降低3.6%。
这些结果鼓励对这些患者进行早期手术，以避免麻醉、围手术期和术后并发症。同样，对轻度侧弯给予支撑可减少术后头几年的机械应力。不进行胸段植骨融合，在患者约12岁时进行手术，可使脊柱生长约5厘米。稳定肌病患者的脊柱可使儿童保持直立坐姿。组件胸段的柔韧性可增加上肢的活动范围。在患者约12岁停止行走时对其进行手术似乎是合适的。心肺功能和预期寿命并未改善，但大多数患者和家属对手术带来的舒适度非常满意。
