Pang D, Hoffman H J
Neurosurgery. 1980 Aug;7(2):118-26. doi: 10.1227/00006123-198008000-00002.
In the past, sacral agenesis was thought to be a form of bony malformation associated with static neurological deficits. The majority of therapeutic recommendations involved orthopedic correction of foot and leg deformities. Myelographic studies were seldom obtained, and surgical exploration was rarely attempted. We recently encountered two cases of sacral agenesis with progressive neurological deficits; both patients had myelographically demonstrable lesions and both responded favorably to surgical treatment. We believe that patients with caudal agenesis can be divided into two groups: one with static neurological deficits that probably would not improve after operation, and the other with progressive neurological findings probably associated with specific neuropathological lesions that would be amenable to operative therapy. We therefore endorse a more aggressive approach toward the management of patients in the second group. Upon the diagnosis of caudal agenesis, a detailed documentation of the base line neurological status, the bladder and bowel functions, and the degree of lower limb deformities should be obtained. A diligent follow-up program is essential to detect progression which, when present, should be followed promptly by myelography and surgical intervention. The etiology, classifications, and clinical features of sacral agenesis are discussed, and the associated neuropathological abnormalities are analyzed.
过去，骶骨发育不全被认为是一种与静态神经功能缺损相关的骨骼畸形形式。大多数治疗建议涉及对足部和腿部畸形进行骨科矫正。很少进行脊髓造影检查，也很少尝试手术探查。我们最近遇到了两例伴有进行性神经功能缺损的骶骨发育不全病例；两名患者的脊髓造影均显示有病变，且对手术治疗反应良好。我们认为，尾椎发育不全患者可分为两组：一组有静态神经功能缺损，术后可能无法改善；另一组有进行性神经学表现，可能与特定的神经病理学病变有关，适合手术治疗。因此，我们支持对第二组患者采取更积极的治疗方法。在诊断为尾椎发育不全后，应详细记录基线神经状态、膀胱和肠道功能以及下肢畸形程度。积极的随访计划对于发现病情进展至关重要，一旦出现进展，应立即进行脊髓造影和手术干预。本文讨论了骶骨发育不全的病因、分类和临床特征，并分析了相关的神经病理学异常。
