Madera Marci, Crawford Alvin, Mangano Francesco T
Department of Neurosurgery, University of Cincinnati College of Medicine Surgery, Cincinnati, Ohio, USA.
J Neurosurg Pediatr. 2008 Apr;1(4):320-4. doi: 10.3171/PED/2008/1/4/320.
Larsen syndrome is a rare genetic disorder of the connective tissue that is characterized by multiple joint dislocations, distinctive deformities of the hands and feet, characteristic facial features, kyphoscoliosis, and segmentation anomalies of the vertebrae. Diverse treatment options, including conservative observation and surgical correction, have been reported for patients who present with cervical spine pathophysiology. Differences in surgical approaches, timing of the correction, and pre- or postoperative bracing have been reported. According to the authors, the present case is the first report of a pediatric patient with Larsen syndrome in whom an asymptomatic cervical instability was treated before neurological deterioration with synchronous anterior decompression and fixation, posterior fusion and fixation, and halo placement. A review of the literature on similar patients reveals the variety of practices associated with a diagnosis of Larsen syndrome.
拉森综合征是一种罕见的结缔组织遗传性疾病，其特征为多关节脱位、手足的独特畸形、特征性面部特征、脊柱后凸侧弯以及脊椎节段异常。对于出现颈椎病理生理状况的患者，已有多种治疗方案被报道，包括保守观察和手术矫正。手术方式、矫正时机以及术前或术后支具治疗方面均存在差异。据作者称，本病例是首例关于拉森综合征儿科患者的报告，该患者在神经功能恶化前接受了同步前路减压固定、后路融合固定及头环置入术，以治疗无症状性颈椎不稳。对类似患者的文献回顾揭示了与拉森综合征诊断相关的多种治疗方法。
