Syringomyelia

It’s often unclear why and how syringomyelia happens, but some theories propose that a blockage of cerebrospinal fluid is a likely cause. The two main syringomyelia causes are congenital (present at birth) and acquired.

Congenital syringomyelia

Congenital syringomyelia is present at birth and is most commonly caused by birth defects like Chiari malformation type 1. If present in children, symptoms may include back pain or scoliosis.

The different causes of congenital syringomyelia include:

• Chiari malformations. Chiari malformations are structural defects in the lower part of the brain, where the tonsils, part of the cerebellum, enter the spinal canal. Chiari malformations can result in a blockage of cerebrospinal fluid. The most common form is Chiari malformation type 1, in which the cerebellum pushes into the foramen magnum.
• Spina bifida is a neural tube defect in which the neural tube, which forms the early brain and spine in pregnancy, does not close all the way. Without a closed neural tube, the backbone doesn’t form properly in an infant, and can result in spinal cord malformation and resultant syringomyelia.
• Tethered cord syndrome is another structural condition where the movement of the spinal cord is restricted. The condition can cause the spinal cord to stretch abnormally. It’s progressive, resulting in symptoms like lower back or leg pain, spinal deformities, or bowel and bladder dysfunction.

Acquired syringomyelia

Acquired syringomyelia refers to cases in which the condition develops later in life, or is triggered by an injury to the spinal cord.

Causes of acquired syrinogmyelia include:

• Spinal cord injuries. Spinal cord injuries can lead to post-traumatic syringomyelia, either immediately after the injury, or months or years afterward. The buildup of scar tissue after an injury can block the flow of cerebrospinal fluid, resulting in syringomyelia.
• Meningitis. In this disease, the meninges – or protective layer around the brain and spinal cord – get infected. Meningitis symptoms include fever, headache, neck stiffness, and seizures. Meningitis can also lead to syringomyelia, though it may not appear until years after the infection.
• Arachnoiditis is a condition in which the arachnoid, a membrane that surrounds the spinal cord nerves, is inflamed. Arachnoid inflammation can be caused by chemicals, bacterial or viral infections, acute spinal injuries, or complications from spinal surgery. Arachnoiditis can lead to syringomyelia by triggering the formation of cavities, and thus cysts, in the spine.
• Spinal cord tumor. Tumors that develop in the spinal canal or bones can lead to neurological disorders or problems, including paralysis. They can also interfere with the flow of cerebrospinal fluid, triggering syringomyelia.
• Tethered cord syndrome While tethered cord syndrome can be present at birth, it can also occur in adults.