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.
Copyright
Association for Helping Neurosurgical Sick People. This is an open
access article published by Thieme under the terms of the Creative
Commons Attribution-Non Derivative-Non Commercial License, permitting
copying and reproduction so long as the original work is given
appropriate credit.
Contents may not be used for commercial purposes, or
adapted, remixed, transformed or built upon. ( https://creativecommons.org/licenses/by-nc-nd/4.0/
)
This is an open-access article distributed under the terms of the
Creative Commons Attribution-Non Commercial-No Derivatives License, which
permits unrestricted reproduction and distribution, for non-commercial
purposes only; and use and reproduction, but not distribution, of
adapted material for non-commercial purposes only, provided the original
work is properly cited.
