Phillips W A, Cooperman D R, Lindquist T C, Sullivan R C, Millar E A
J Bone Joint Surg Am. 1982 Dec;64(9):1282-94.
Twenty-two patients with lumbosacral agenesis were treated at the Shriners Hospital for Crippled Children, Chicago Unit, from 1953 to 1979. At the time of this study, ten of the patients were skeletally mature after an average follow-up of 24.1 years. Two patients had died, twelve could be examined, and eight who were unable to return for examination responded to a questionnaire. Eleven of the patients had diabetic mothers. Of the orthopaedic problems in these patients, knee-flexion contractures with popliteal webbing were the most difficult to correct. These deformities varied in severity with the level of the agenesis and the resulting loss of motor power. Other problems were dislocations and flexions contractures of the hips, scoliosis, equinovarus deformities of the foot, and instability at the spinal-pelvic junction. When there was inadequate quadriceps function it was difficult to correct knee-flexion contractures and to prevent them from recurring. For severe knee deformity, knee disarticulation and prosthetic fitting were the most effective treatment. Spinal-pelvic instability was not a problem in eighteen of the twenty surviving patients. Unreduced dislocated hips also did not cause any problems.
1953年至1979年期间，22例腰骶部发育不全患者在芝加哥施莱宁儿童医院接受了治疗。在本研究开展时，平均随访24.1年后，其中10例患者骨骼已成熟。2例患者已死亡，12例患者接受了检查，8例无法前来接受检查的患者回复了问卷。这些患者中有11例患者的母亲患有糖尿病。在这些患者的骨科问题中，伴有腘窝蹼的膝屈曲挛缩最难矫正。这些畸形的严重程度因发育不全的程度以及由此导致的运动能力丧失而异。其他问题包括髋关节脱位和屈曲挛缩、脊柱侧弯、马蹄内翻足畸形以及脊柱骨盆交界处不稳定。当股四头肌功能不足时，很难矫正膝屈曲挛缩并防止其复发。对于严重的膝关节畸形，膝关节关节离断术和假肢安装是最有效的治疗方法。20例存活患者中有18例不存在脊柱骨盆不稳定问题。未复位的髋关节脱位也未引发任何问题。
