What is Chiari Malformation?

Chiari Malformation (CM) also known as Hindbrain Hernia

Chiari Malformation is a rare condition involving the base of the brain and spinal cord. In this condition the cerebellar tonsils of the brain descend through an opening in the base of the skull (named the Foramen Magnum) into what should be a space alongside the spinal cord. This can cause compression of the brain stem and disruption of the flow of cerebrospinal fluid (CSF) at the top of the spinal canal. This can cause a number of symptoms: pain and tingling in the limbs, dizziness, nausea, balance problems, visual disturbances, problems swallowing, pressure headaches, which worsen when straining, laughing or coughing.

How is Chiari Malformation Diagnosed?

Chiari Malformation is diagnosed by an MRI scan, usually requested by a consultant after discussing symptoms with the patient.

Treatment

A decompression surgery may be offered to try to relieve symptoms and stop the condition progressing.

Before any surgery is undertaken, detailed discussions need to take place between the patient and the neurosurgeon, as to the potential benefits of surgery as well as the inconveniences, discomforts and risks that go with an operation on the brain or spinal cord.

What is Syringomyelia?

Syringomyelia is a disorder affecting the nervous system where fluid-filled cavities develop inside the spinal cord. The spinal cord is normally a solid structure which passes down the back inside the spinal canal. It connects the brain to the rest of the body, passing signals to and fro, enabling an individual to move his or her limbs at will, to feel objects and to control various bodily functions.

Within the spinal canal, the spinal cord is bathed with a clear, watery fluid, known as cerebrospinal fluid - a term usually abbreviated to CSF. The fluid from which Syringomyelia cavities are formed is identical to CSF.

Normally CSF flowing in the spinal canal communicates freely with CSF circulating inside the head but when CSF is trapped inside the spinal canal some of it begins to accumulate within the substance of the spinal cord.

What Causes Syringomyelia?

Next to Chiari Malfromation, the most common cause of Syringomyelia is spinal injury. A minority of victims of severe spinal cord trauma, who are already severely disabled as a result of their original injury, go on to develop additional problems as a result of Syringomyelia. Scar tissue within the spinal canal, developing as a consequence of the initial injury, obstructs CSF movement causing, once again, fluid to accumulate within the substance of the spinal cord.

There are other causes of Syringomyelia beyond the scope of these pages.

How is Syringomyelia diagnosed?

Patients who have Syringomyelia present with a variety of symptoms ranging from neck and arm pain through to fairly severe disability, with muscle weakness and paralysis. Fortunately nowadays, most cases of Syringomyelia are detected at an early stage before major disability develops.

Any of the symptoms of Syringomyelia can have other causes. Indeed, it is much more likely that somebody presenting with, say, neck and arm pain, will have a more common disorder and not Syringomyelia.

It is only when more common diseases are excluded that Syringomyelia may be considered as a diagnosis. In most cases the diagnosis of Syringomyelia is made by a specialist, usually a Neurologist.

Once the diagnosis of Syringomyelia is suspected, it is readily confirmed by an MRI scan.

How is Syringomyelia treated?

In many cases of Syringomyelia all that a patient needs is an explanation and reassurance, together with periodic review by a specialist.

The symptoms arising from Syringomyelia can be treated with drugs, other measures, or simply tolerated.

In some instances, however, if the cavity in the spinal cord is enlarging and threatens to cause significant disability, there may be a place for surgical intervention. In these circumstances the services of a neurosurgeon may be required. Before any surgery is undertaken, detailed discussions need to take place between the patient and the neurosurgeon, as to the potential benefits of surgery as well as the inconveniences, discomforts and risks that go with an operation on the brain or spinal cord.