A 360-Degree Surgical Approach for Correction of Cervical Kyphosis and Atlantoaxial Dislocation in the Case of Larsen Syndrome.

Abstract

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.