What is Tethered Cord Syndrome?

The "cord" in tethered cord syndrome refers to the spinal cord. The spinal cord is a long tube-like structure that extends from the brain to the lower back. It sends nerve signals out to the body and back to the brain. It floats in a fluid called cerebrospinal fluid and is surrounded by three protectives layers of tissue called meninges. The dura matter is the next layer out, which forms a protective barrier. (These layers also extend into the brain.) Finally, the spinal column--the tall stack of vertebrae running the length of the neck and back--provides a safe enclosure for all the softer material. (The skull does the same thing for the brain.) Since the spinal cord is the direct communicator to the brain from most of the body, it's important to keep it protected and moving smoothly in the cerebrospinal fluid.

In tethered cord syndrome,(1) the spinal cord gets tethered, or stuck, to the sides of the spinal canal. This usually happens in the caudal region of the spine--the part that's right above the tail bone and extends up into the lower back. As a child grows, the spinal cord can stretch, causing damage to the delicate cord. Scar tissue can also form around the place where it is tethered, blocking the cord even further and causing more nervous system problems.

Image courtesy of Seattle Children’s Hospital website (2)

Tethered cord syndrome (TCS) is associated with spina bifida (also called myelomeningocele), and it is estimated that 25-50% of children treated for spina bifida will need surgery in the future to untether their spinal cord, according to the American Association of Neurological Surgeons.

Causes of Tethered Cord Syndrome

• Myelomeningocele and lipomyelomeningocele (spina bifida)

• Dermal sinus tract--an abnormal, small passage from the skin into the spinal cord

• A split spinal cord near the end (Diastematomyelia3 or diplomyelia4)

• Lipoma (a fatty, non-cancerous growth)

• Tumor

• The filum terminal5--the very end of the spinal cord--is held too tightly

• A history of spine surgery and/or trauma

Symptoms of Tethered Cord Syndrome

TCS symptoms usually appear in children.(1) In rare cases, an adult may be undiagnosed and show symptoms of strain on the spinal cord, such as sensory and motor problems, loss of bladder and bowel control.

• Lesions on the lower back

• Fatty tumor, deep dimple, or birthmark in/on the lower back

• Skin discoloration on the lower back

• Hairy patch on the lower back

• Anorectal malformations

• Back pain, made worse with activity and better at rest, that may shoot into the legs

• Numbness, tingling, or weakness in the legs

• Changes in leg strength

• Deterioration in gait

• Inability to walk

• Tremors, spasms, or contractures in the leg muscles

• Leg deformities

• Spine tenderness

• Scoliosis--a curvature of the spine--that may change or get worse

• Worsening or loss of bowel and bladder control

• Repeated bladder infections

• Foot problems, like high arches or curled toes

Diagnosis for and Treatment of Tethered Cord Syndrome

To diagnose tethered cord syndrome, a medical provider will ask parent(s)/guardian(s) and the child in question about symptoms the child has been experiencing or things the parent(s)/guardian(s) have noticed. Usually a magnetic resonance image (MRI) is done so the physician can see where the cord may be tethered and any scar tissue that has formed.

The only treatment for tethered cord syndrome is a surgery called a laminectomy. During this surgical procedure, the goal is to free the spinal cord from where it is tethered. The neurosurgeons remove one or part of one of the vertebrae near where the cord is tethered. They then, very carefully and gently, cut the spinal cord away from spinal canal. There is a chance that future surgery may be needed as the child grows, but this is more likely in children with myelomeningocele and lipomyelomeningocele. In any event, children (if possible), parent(s)/guardian(s), and their physicians can discuss and ultimately pursue the treatment options that are appropriate for them.

1.     https://www.aans.org/en/Patients/Neurosurgical-Conditions-and-Treatments/Tethered-Spinal-Cord-Syndrome

2.     https://www.seattlechildrens.org/conditions/tethered-spinal-cord/

3.     https://en.wikipedia.org/wiki/Diastematomyelia

4.     https://radiopaedia.org/articles/diplomyelia?lang=us

5.     https://radiopaedia.org/articles/filum-terminale?lang=us