Tethered Cord Syndrome Unleashed: A Comprehensive Guide

Introduction

Tethered Cord Syndrome (TCS) is a condition where the spinal cord gets stuck or pulled too tightly, either from birth or due to other problems. This can cause harm to the spinal cord and make it difficult for the spinal cord to move freely when a person bends or stretches their back. This issue can be related to problems such as spina bifida, tumors, fatty lumps, or infections.

This can result in damage to the spinal cord, which can make it harder for the affected person's body to function properly. Depending on the person's age and what is causing the problem, they might experience pain in their legs or lower back, difficulty moving their legs, problems with urination, issues with their bones, or changes in their skin.

What Are the Signs and Symptoms of Tethered Cord Syndrome?

Symptoms of tethered cord syndrome can vary widely from person to person, and the severity and progression of the condition differ as well. Typically, symptoms tend to appear during childhood, but in some cases, they may remain stable during childhood and only become noticeable in adulthood. Many children with tethered cord syndrome exhibit physical signs like:

• Tufts of hair on the skin.

• Skin tags.

• Dimples.

• Benign fatty tumors.

• Skin discoloration.

• Hemangiomas.

• Lower back pain that worsens with activity.

• Leg pain or numbness.

• Difficulty walking.

• Foot and spine deformities.

• High-arched feet.

• Hammertoes.

• Problems with bladder and bowel control, including involuntary urination or defecation and recurring urinary tract infections, are also common.

• Occasionally, there may be differences in leg strength.

These symptoms in children usually progress slowly. While tethered cord syndrome was once believed to be rare in adults, more cases have been reported recently due to advances in neurological examinations and imaging.

In adults, the condition often presents with constant and severe back and leg pain, which may extend to the rectum and genital area in some cases. Progressive sensory and motor deficits can affect the legs, potentially causing numbness, weakness, or muscle wasting. Over half of affected individuals experience bladder and bowel dysfunction, leading to increased urgency or frequency of urination and constipation. In some instances, a fluid-filled cavity called a syrinx may be present, and this can result in symptoms similar to syringomyelia, such as burning anal pain, decreased motor function, muscle loss, or occasional headaches.

What Are the Causes of Tethered Cord Syndrome?

Tethered cord syndrome happens when the spinal cord gets stuck or stretched too much. It can be there from birth or happen later.

• From Birth:

  • Some babies are born with spine problems like spina bifida. It is when the back does not close properly, and part of the spinal cord sticks out.

  • If this problem is near the end of the spine, it can cause tethered cord syndrome.

  • There are different types of spina bifida that can lead to tethered cord syndrome, like a weird connection to the spinal cord, a skin tract, a split spinal cord, or a fatty lump next to the spinal cord.

• Later in Life:

  • For some people, tethered cord syndrome happens because a part called the "filum terminale" becomes thick and stiff. It is like a bridge between the spinal cord and the tailbone.

  • Normally, this part is stretchy, but if it gets stiff, it can pull and "tether" the spinal cord.

  • This usually happens in kids and not so much in teenagers or adults.

• Other Reasons: Sometimes, tumors, infections, or scars from surgery can cause tethered cord syndrome. It can also be linked to spinal injuries, but some experts think the problem was there before the injury made it worse.

How Is Tethered Cord Syndrome Diagnosed?

Tethered cord syndrome diagnosis relies on identifying specific signs and symptoms, which help locate the problem in the spine. To do this, doctors need to gather a detailed patient history, conduct a thorough clinical evaluation, and use advanced imaging like MRI (magnetic resonance imaging) or CT (computed tomography) scans. In kids, these scans show features like a low-lying spinal cord and a thickened filum terminale. Detecting spina bifida, a bone issue in the spine also supports the diagnosis. For teenagers and adults, doctors look for the filum being out of place behind the cauda equina, a bundle of nerve roots.

• MRI (Magnetic Resonance Imaging): This diagnostic test employs strong magnets and advanced computer technology to create three-dimensional images of internal body structures. It is particularly useful for visualizing the spinal cord, nerve roots, and their surroundings. MRI can detect conditions such as spinal cord enlargement, degeneration, and displacement.

• Myelogram: A myelogram involves taking an X-ray of the spinal canal after injecting contrast material into the thecal sac (a protective membrane is a sac-like covering that surrounds the spinal cord). This procedure helps reveal any pressure on the spinal cord or nerves caused by a tethered spinal cord.

• CT or CAT Scan (Computed Tomography): This diagnostic imaging technique generates detailed images by processing X-rays through a computer. It is sometimes used following a myelogram to depict how the contrast dye flows around the spinal cord and nerves.

• Ultrasound: During an ultrasound, a water-soluble gel is applied to the skin, where a handheld transducer device is placed. This gel facilitates the transmission of high-frequency sound waves to the spinal area. The ultrasound captures moving images of the spinal cord within the thecal sac.

How Is Tethered Spinal Cord Syndrome Treated?

In children, surgery can be recommended to address spinal cord issues that are causing progressive neurological symptoms. The specific type of surgery required depends on the mechanical causes of these issues, such as an inelastic filum, myelomeningocele (a form of spina bifida in which the spinal canal and spine of a fetus do not completely close before delivery), lipo myelomeningocele (an area of fat beneath the skin of the child's back), and dermal sinus. The prognosis after surgery varies based on the symptoms presented and the underlying tethering-producing anomalies. It is worth noting that the approach to treating tethered cord syndrome in adult patients can be a subject of debate. However, it is clear that for both pediatric and adult patients with clear evidence of tethered cord syndrome, prompt surgical intervention can often lead to the reversal or stabilization of symptoms in many cases.

Parents should discuss their child's specific spinal cord problems, associated symptoms, and spinal deformities with their physician and medical team. In cases where an individual has only minimal complaints, the physician may recommend non-surgical, conservative treatment while closely monitoring the condition to assess any progression of symptoms. Some experts advise against surgery for individuals who show MRI evidence of "cord elongation and thickened filum" but have no symptoms. Nevertheless, certain neurosurgeons may opt to cut the thickened filum in such cases for preventive purposes.

The outcomes of treatment for tethered cord syndrome, whether it involves repairing myelomeningocele or addressing scar tissue formation, can vary from person to person. Following the initial repair, there is a possibility of the spinal cord becoming ‘retethered,’ which may necessitate additional surgery.

In individuals with severe arachnoiditis (a condition where the covering of the spinal cord adheres to it) identified through MRI or CT scans, a careful evaluation of pain and neurological status is essential to determine whether surgical intervention is warranted. During surgery, the release of arachnoid adhesions must be carried out with meticulous precision. Failure to do so may result in re-adhesion or extensive scar formation after surgery. To mitigate this problem, two specialized surgical procedures have been advocated:

• Transection of the spinal cord to alleviate severe back and leg pain.

• Shortening of the spinal column by removing one or two vertebrae to relieve tension on the spinal cord.

What Happens After Tethered Spinal Cord Surgery?

After tethered spinal cord surgery, the patient or their child can typically resume light activities shortly after the procedure. However, they will need to avoid strenuous activity to allow the area to heal properly. This cautionary period typically lasts at least six to eight weeks, but the exact duration depends on the surgeon's recommendations.

In some cases, individuals may require physical or occupational therapy to help them regain function after surgery. The surgery aims to prevent ongoing nerve injury, but it is unlikely to reverse nerve damage that has already occurred. Therefore, some individuals may continue to experience muscle weakness or motor control problems (movement issues) even after receiving treatment.

Conclusion

Tethered cord syndrome is a condition in which the spinal cord becomes stuck or attached to the spinal canal due to tight tissue. This restricted movement can lead to various neurological and other problems as a person grows. Some individuals may have this condition from birth, while others may develop it during childhood or even in adulthood. Tethered cord syndrome is often associated with a congenital condition called spina bifida, where the spinal cord or canal does not develop properly. The good news is that early surgical treatment can often correct this issue. So, if they or their child exhibit symptoms of a tethered spinal cord, it is crucial to consult a healthcare provider promptly.