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Syringomyelia ("Syrinx")

Also known as hydromyelia as well as by the common simpler name of syrinx, a syringomyelia is a fluid collection in the spinal cord.

Causes

There are many possible causes of this, such as:

  • Hydrocephalus
  • Chiari Malformation
  • Trauma
  • Tethered spinal cord
  • Tumor
  • A very large number without any clear cause, the so-called idiopathic.

Symptoms

These collections are usually similar to spinal fluid. They can cause symptoms in two main ways.

  • Due to the direct pressure of the fluid collection on the spinal cord. The symptoms will then be related to the level of the spinal cord at which the collection is. It may cause weakness, numbness, stiffness, pain, scoliosis, and incontinence to name a few. These symptoms usually come on quite gradually.
  • Due to the underlying cause of the syrinx. For instance, a syrinx may be caused by a Chiari Malformation, and the symptom may be one of headaches or neck pain even though the syrinx may be lower in the spinal cord. The important thing is for the physician to recognize the possibilities and perform the appropriate tests.

Diagnosis

The availability of MRI has greatly improved your physician's ability to both diagnose and follow these collections. Once one is identified it is usually prudent to study the entire nervous system looking for associated abnormalities which may influence treatment. When it is determined that the syrinx is of clinical significance (causing problems such as injury to the spinal cord or progressive scoliosis), the only treatment available is surgery.

Treatment

There are many different opinions as to how best treat a syrinx, including, in the broadest sense:

  • If there is an obvious cause (such as hydrocephalus or Chiari Malformation) treat the cause with the expectation that the syrinx will then resolve on its own.
  • Treat the syrinx as the initial problem with some form of drain (see below).
  • Combine both of these options and treat both the cause and the syrinx at the same operation.

The drains used to treat syrinxes are quite variable. Some surgeons prefer to place a small tube or "stent" in the syrinx. This is a rather short length of hollow tubing that extends from inside the spinal cord collection to just outside the spinal cord where the spinal fluid space is. Others prefer to use a shunt-type system, similar to that used in hydrocephalus to divert the fluid to other body cavities such as the chest or abdomen.

The fact that there are so many options tells us that no one is superior to all the others. It is important to discuss these issues with your neurosurgeon. Whatever method is chosen, it is likely that a follow-up MRI will be performed at some point after surgery to show adequate drainage of the collection. As with any of the processes that effect the nervous system, careful follow-up is essential. Most children should show some if not total improvement of their symptoms.

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