Syringomyelia is a major pathology of the spinal cord. The diagnostic and therapeutic approach must be fast and effective by the neurosurgeon, as the disease causes the spinal cord to fill with fluid and can leave the patient invalid.
Syringomyelia: what is it and what causes it?
Syringomyelia is a pathology of the spinal cord in which the spinal cord fills with fluid, forming a liquid collection that compresses the cord from the inside out, and can leave a patient tetraplegic or paraplegic.
Syringomyelia appears when there is a conflict of flow or pressure in the spinal cord space. In the embryonic period we have a central canal in the medulla through which the cerebrospinal fluid passes. This canal closes before birth but certain diseases may cause it not to close or later, in juvenile or adult life, it may reopen.
When this happens, the medulla remains full of fluid that compresses it. This damages the surrounding structures with:
- Muscle weakness
- Muscle atrophy
- Fasciculations
- Release of reflexes
- Loss of sphincter control
- Varying levels of loss of balance and sensation.
Rarely, the disease reaches the brainstem (medulla oblongata), which is then called Syringobulbia. This condition is very serious, as it can seriously compromise the patient’s breathing and, if not treated promptly, lead to death.
Relationship of Syringomyelia with Arnold-Chiari Syndrome
Arnold-Chiari syndrome also corresponds to a pathology where, due to a defective development of the skull, there is a conflict of the cerebrospinal fluid and its pulsation, which does not pass adequately from the cranial to the spinal compartment. In some of these cases there is dilatation of the cranial cerebrospinal fluid cavities (hydrocephalus) and, in others, it occurs at the spinal level, dilating the ependymal canal (Syringomyelia). In these cases syringomyelia is associated with Arnold-Chiari syndrome.
Treatment of syringomyelia
Syringomyelia that is not related to Arnold-Chiari syndrome can be caused by multiple factors: trauma, tumors, post-surgery, etc. In some cases no causative agent can be identified (idiopathic) and, in others, it can appear up to more than 30 years after a severe dorso-lumbar fracture (ascending syringomyelia), for example.
In cases where there is a clear causal factor, it will be necessary to operate this first and wait for the resolution of the syringomyelia afterwards. If this is not the case, the neurosurgeon must specifically intervene in the cavity created with its opening and place a silicone tube that goes from inside the medullary cavity to the space surrounding the medulla (subarachnoid space) where it empties the liquid and where it will be reabsorbed. On other occasions the tube is brought to the peritoneum in the patient’s abdomen, where it is also reabsorbed into the general circulation. In this way, the excess fluid and its pulsation are cushioned out of the spinal cord, thus nullifying the spinal cord damage caused by the fluid retention. As a result, more than 50% of syringomyelia are halted or even improved.
Syringomyelia associated with Arnold-Chiari syndrome, on the other hand, is not usually treated specifically. With the decompression of the foramen magnum at the base of the skull, which is performed in Chiari, it is possible to open the foramen for the passage of cerebrospinal fluid, so that in 70% of cases, pressure is no longer transmitted to the spinal cavity, which is reduced or disappears.
Quality of life of the patient with syringomyelia
In order for syringomyelia not to significantly damage the spinal cord, it is necessary to act quickly at the onset of symptoms and to intervene in the patient. Although in the initial stages the cavity is not very dilated, nowadays, with intraoperative ultrasound and neurophysiological monitoring, we can more easily find the way to the liquid collection and drain it out. With this, at least, we will stop the process in a phase of little deterioration and the patient will maintain an acceptable quality of life, especially if the sphincters are preserved, since this is an important prognostic factor.