Tethered Cord Syndrome – A Syndrome Associated with Spina Bifida Occulta and the Neurological Diagnosis of Tethered Cord Syndrome

The human spinal cord within the spine becomes thinner and more cone-shaped toward its lower end. At the tip of this region, known as the conus medullaris, the spinal cord tapers to a thread-like point.

This terminal filament can be compressed by

• pathological (abnormal) fatty tissue,
• connective tissue, or
• scar tissue

on the wall of the spinal canal or the surrounding tissue. This causes excessive tension on the nerve fibers, which can have various consequences.

In some cases, symptoms are barely noticeable, and the patient remains symptom-free for a long time or even permanently. More commonly, however, neurological, motor, and/or orthopedic problems occur to varying degrees. When symptoms do occur, the condition is referred to as “tethered cord syndrome.”

The course of the condition also varies from patient to patient. In most cases, however, the symptoms worsen over time.

Possible signs of the condition include, for example:

• sensory disturbances on the inner thighs, ranging up to complete numbness in this area,
• abnormal reflexes,
• problems with urination, urinary incontinence (with possible kidney damage as the disease progresses),
• sexual dysfunction, erectile dysfunction,
• scoliosis (curvature of the spine),
• pain in the legs or lower back,
• deformed feet, knees, or hips,
• spasticity (paralysis caused by muscle spasms) in the legs, and
• motor disorders ranging up to paralysis.

In cases of congenital tethered cord, noticeable skin symptoms may also occur. These include, for example, excessive hair growth or prominent birthmarks in the areas where the cord is attached.

Significant differences in foot size may also occur.

Furthermore, a malformation of the cerebrum may occur. Consequences include, for example, hydrocephalus (“water on the brain”) or a malposition of certain parts of the brain. This can lead to various problems in later years, such as

• pain,
• neurological deficits, or
• disorders of vital functions

.

Primary tethered cord refers exclusively to conditions resulting from congenital malformations. For example, this condition may be part of the spinal malformation known as spina bifida (open spine).

Secondary tethered cord refers to conditions that develop later in life. Causes may include surgeries or accidents. For example, tethered cord may first occur when the aforementioned spina bifida malformation closes, or it may result from adhesions following injuries.

Tethered cord may be suspected as early as during pregnancy. Abnormal findings in amniotic fluid tests or ultrasound examinations may indicate tethered cord.

After birth, three different diagnostic methods are available.

• Sonography, or an ultrasound examination (this diagnostic method is primarily used for newborns),
• myelography, a specialized form of computed tomography designed specifically to visualize the spinal canal and surrounding structures
• magnetic resonance imaging (MRI).

These procedures are roughly equally reliable. They can also be used to detect other malformations or possible involvement of the surrounding tissue.

Surgery is the only way to effectively treat the underlying cause of a tethered cord. During the procedure, the surgeon releases as many adhesions and scar tissue as possible to eliminate the tension on the nerve fibers. This improves neurological, motor, and orthopedic problems—or at least prevents them from worsening further.

The surgical procedure is called myelolysis, which translates to “loosening of the spinal cord.”

Opinions vary on who is a suitable candidate for the surgery. In children, the surgery is often performed as a preventive measure to ensure that more serious problems do not develop during growth. However, some doctors believe that surgery should only be performed when symptoms requiring treatment arise.

Myelolysis is a time-consuming surgery that can take several hours, depending on the individual severity of the tethered cord.

In a microsurgical procedure, the surgeon completely frees and isolates the spinal cord along with its roots. The terminal filament is severed. The surgeon then removes all excess tissue, such as

• scar tissue,
• pathological connective tissue, or
• fat deposits.

To prevent new adhesions, the spinal cord should be able to move within as large a cerebrospinal fluid space as possible. The cerebrospinal fluid space is the area filled with cerebrospinal fluid.

It is difficult to predict the success of the surgery in advance, as the benefits often only become apparent over time. In most cases, the surgery prevents further deterioration. However, it is also possible that existing problems will improve significantly. The chances of this are particularly high when the surgery is performed in early childhood.

However, there are also cases in which symptoms worsen as a result of the surgery.

Myelolysis surgery always requires an inpatient hospital stay. This typically lasts between seven and 12 days. This hospital stay is followed by a recovery period at home lasting approximately 14 to 28 days.

On average, the total time off from work or school is 32 days.

Depending on the individual case, it takes six weeks to three months for the patient to return to the state of health they were in before the surgery. During this time, they usually undergo physical therapy. This can significantly improve the healing process and the final outcomes.