Forst R, Forst J
Orthopädische Klinik RWTH Aachen, Germany.
Arch Orthop Trauma Surg. 1995;114(2):106-11. doi: 10.1007/BF00422837.
A total of 123 patients with Duchenne muscular dystrophy (DMD) was surgically treated during two different periods of their course by hip and knee release, aponeurectomy of the iliotibial band and z-shaped Achilles' tendon lengthening. In 57 patients (group I) this was carried out prophylactically as retractions of the lower limb joints were just beginning at the age of 6.4 +/- 1.43 years and in 66 patients (group II) as mild contractures of the joints at the end of walking ability were already manifest with an average age of 9.27 +/- 1.86 years. The average follow-up was 3.7 +/- 1.2 years in both groups. To be able to assess the interindividual course of both groups, we defined "joint and motor quotients", which allowed a complex assessment of joint function and motoric capacity. In addition, both groups were compared with a control group (natural history) consisting of 100 non-operated DMD patients. In both groups a significant release of the contractures could be obtained primarily. Patients in group I showed a much better long-term effect than those in group II. The motor quotient in group I was significantly better over the whole follow-up period (P < 0.001) than in group II or the control group. The prolongation of walking ability by about 2 years compared with the natural history is in our opinion not the central goal of this surgical treatment concept of lower limbs in DMD, but rather the additionally achieved prolongation of an assisted standing ability with the lower limbs free from contractures and deformities.(ABSTRACT TRUNCATED AT 250 WORDS)
共有123例杜氏肌营养不良症(DMD)患者在病程的两个不同阶段接受了手术治疗,手术包括髋膝关节松解、髂胫束腱膜切除术和Z形跟腱延长术。57例患者(第一组)在6.4±1.43岁下肢关节挛缩刚开始时进行了预防性手术,66例患者(第二组)在行走能力末期关节出现轻度挛缩时接受了手术,平均年龄为9.27±1.86岁。两组的平均随访时间均为3.7±1.2年。为了评估两组患者的个体病程,我们定义了“关节和运动商”,以便对关节功能和运动能力进行综合评估。此外,将两组患者与由100例未接受手术的DMD患者组成的对照组(自然病程组)进行了比较。两组患者的挛缩均主要得到了显著缓解。第一组患者的长期效果明显优于第二组。在整个随访期间,第一组的运动商显著优于第二组或对照组(P<0.001)。与自然病程相比,行走能力延长约2年,我们认为这不是DMD下肢手术治疗理念的核心目标,而是额外实现的下肢无挛缩和畸形的辅助站立能力的延长。(摘要截选至250字)