Deora Harsh, Singh Suyash, Sardhara Jayesh, Behari Sanjay
Department of Neurosurgery, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.
Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.
J Neurosci Rural Pract. 2020 Jan;11(1):196-201. doi: 10.1055/s-0039-3402624. Epub 2020 Mar 3.
Larsen syndrome is chronic debilitating disease that presents with multiple joint dislocations and severely affects the cervical spine in the form of cervical kyphosis and atlantoaxial dislocation. Children usually present in early with a myriad of deficits, compressive myelopathy being the most common. In addition to a bony compression, there is sometimes a soft tissue component, which is seldom addressed. We present here a case of atlantoaxial dislocation with cervical kyphosis due to Larsen syndrome, and along with our previous experience on syndromic atlantoaxial dislocations, we try to define an algorithm for the treatment approach of these onerous challenges. The importance of early intervention is also emphasized with a literature review of similar cases. In addition to the obvious physical damage, early intervention can also avoid the more sinister socioeconomic face of this debilitating disease.
拉森综合征是一种慢性致残性疾病,表现为多处关节脱位,并以颈椎后凸和寰枢椎脱位的形式严重影响颈椎。儿童通常早期就出现多种缺陷,其中压迫性脊髓病最为常见。除了骨质压迫外,有时还存在软组织成分,而这一点很少得到关注。我们在此介绍一例因拉森综合征导致寰枢椎脱位伴颈椎后凸的病例,并结合我们之前对综合征性寰枢椎脱位的经验,试图为应对这些艰巨挑战制定一种治疗方法的算法。通过对类似病例的文献综述,还强调了早期干预的重要性。除了明显的身体损伤外,早期干预还可以避免这种致残性疾病带来的更严重的社会经济问题。