Question:
9 years ago, my daughter had a C1/C2 traction, fusion and eventually anterior decompression of C1. A few years ago, I felt like her energy levels were very low so after sleep studies, CBC’s, thyroid tests we re-visited her cervical spine. It shows significant impingement again and a slightly stable fusion. We have met with two surgeons and they both wanted to wait, then thought we needed to have surgery asap, then both again changed their minds and said to monitor. If her current fusion fails – we will be lucky if she survives as a quadriplegic. Just looking for advice on different hospitals/surgeons you may know of that may specialize/have experience with this.
Answer:
I am sorry to hear of your daughter’s difficulties.
The neck includes 7 vertebrae (bones) that are stacked nicely one upon the other. These are called cervical vertebrae. They are connected by ligaments and muscles. The vertebrae have a solid body that lies in the front (anteriorly). In the back (posteriorly), the bone surrounds and protects a hollow area in which the spinal cord is contained.
Subluxation is slippage of one vertebra on the next. When they “slip,” one of the vertebrae typically slides or moves forward. As it moves forward, the bones can put pressure on the spinal cord. This can cause spinal cord injury. When there is concern for spinal cord injury or pending spinal cord injury, surgery is required. This may include traction (a procedure used to stretch the neck to help the bones to re-align), decompression (surgical procedure to relieve the pressure on the spinal cord), and fusion (surgical procedure to prevent the vertebrae from slipping forward again).
This can occur with any cervical vertebrae, but it is most common at the joint between the first and second vertebrae. The first vertebrae is called the atlas and the second is called the axis. Therefore, this is known as atlanto-axial subluxation or instability.
Whether to operate at all and when to operate depends on several factors such as age, whether there are symptoms, evidence of injury to the spinal cord (usually seen on an MRI), co-occurring health conditions, and others.
We have had many people with DS undergo surgery from our practice. We worked with Spine Orthopedic Surgery and Neurosurgery to help determine the need for and timing of the surgery.
We recommend contacting your local DS clinic to see who they recommend for surgery for cervical subluxation. We have knowledge of our local providers but not of those across the country.
Clinics for people with DS can be found at these sites: Global Down Syndrome Foundation, National Down Syndrome Congress, or National Down Syndrome Society.