Discover symptoms, treatment options and causes of Tethered Cord Syndrome. Learn about diagnosis in adults and babies to address this neurological condition.

Tethered Cord Syndrome

Humza Khizer

Overview

Tethered cord syndrome is a rare neurological condition in which the spinal cord abnormally attaches to the surrounding tissues, restricting its movement. 
Tethered cord syndrome can lead to significant disability if left untreated. Options typically involve surgery to release the tethered spinal cord and correct any associated spinal abnormalities. 
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Typically, the spinal cord moves freely within the spinal canal. However, in tethered cord syndrome (TCS), the spinal cord is abnormally attached to the wall of the spinal canal. The abnormal traction or pulling of the spinal cord leads to dysfunction in various bodily functions, such as the nervous system, gastrointestinal system, musculoskeletal system, and urinary system. It is estimated to affect approximately 0.25 per 1,000 births<a href="#accordion">[1]</a>. 
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Tethered cord syndrome can occur as an isolated condition. However, it is often associated with other spinal dysraphism conditions. It is most commonly diagnosed in infants or young children who exhibit physical signs, such as skin abnormalities, lower limb deformities, bowel or bladder dysfunction, progressive weakness or numbness in the limbs, or progressive scoliosis. The condition can also be discovered incidentally during other medical evaluations. 
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Tethered cord syndrome is typically divided into two: primary and secondary<a href="#accordion">[6]</a>. 
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<h5>Primary Tethered Cord Syndrome</h5>

This type is due to congenital causes. The most common cause is a thickened &lsquo;fatty&rsquo; filum terminale. The filum terminale connects the bottom of the spinal cord to the tailbone. However, during fetal development, the thickening of the tissue can result in an abnormally elongated spinal cord. This then leads to tethering of the spinal cord. Lipomas, spina bifida, and spinal cord malformations have also been linked to primary TCS. 
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<h5>Secondary Tethered Cord Syndrome</h5>

This is an acquired form of TCS and can result from several factors that cause the thickening of the filum terminale, such as tumors, infections, and fibrotic scarring. Some people may also develop fibrotic thickening after spinal surgery. 
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Signs and Symptoms

The signs and symptoms of tethered cord syndrome can vary depending on the severity of the condition and the age of the person affected. Some common signs and symptoms of tethered cord syndrome may include:

<ul>
	<li><a href="https://merisehat.pk/disease/chronic-back-pain">Back pain</a></li>
	<li>Leg pain, weakness, or numbness</li>
	<li><a href="https://merisehat.pk/disease/scoliosis">Scoliosis</a></li>
	<li>Bowel or bladder dysfunction, including urinary incontinence or difficulty with bowel movements</li>
	<li>Abnormal foot or leg position or gait abnormalities</li>
	<li>Difficulty standing, walking, or running</li>
	<li>Decreased or absent reflexes in the legs or feet</li>
	<li>Muscle stiffness or spasticity</li>
	<li>Skin changes such as dimpling or hairy patches on the lower back or near the spine</li>
	<li>Muscle wasting or atrophy 
	&nbsp;</li>
</ul>

If left untreated, these symptoms may worsen over time. In infants and young children, the condition may be associated with developmental delays, delayed walking, or abnormal crawling.

Note: If you or someone you know experiences any of these symptoms, it is important to seek medical attention to determine the underlying cause and appropriate treatment. 
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Causes

The two main causes of tethered cord syndrome are myelomeningocele and lipomyelomeningocele. 
&nbsp;

<ul>
	<li>Myelomeningocele: A type of spina bifida, myelomeningocele develops when the spinal column does not form properly, causing a section of the spinal cord to be exposed in a fluid-filled sac located at the baby&rsquo;s back. In infants with myelomeningocele, as the child grows, the spinal cord fails to separate from the skin at the back. This leads to the storage of fat in the lower end of the spinal cord.</li>
	<li>Lipomyelomeningocele: It involves the intertwining of the fat in the spinal cord with that of the thecal sac; thus, restricting the movement of the spinal cord.</li>
</ul>

Tethered cord syndrome can occur either at birth or later in life. 
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<h5>Congenital Causes</h5>

<ul>
	<li>Abnormally Thick Filum Terminale: The filum terminale is a thin strand of tissue that anchors the spinal cord to the sacrum. In some cases, this tissue can become abnormally thick and cause the spinal cord to be stretched or compressed, leading to tethered cord syndrome.</li>
	<li>Dermal Sinus Tracts: These are abnormal connections between the skin and spinal cord. 
	&nbsp;</li>
</ul>

<h5>Acquired Causes</h5>

<ul>
	<li>Spinal Cord Surgery: Scar tissue can form around the spinal cord after surgery, which can cause it to become tethered.</li>
	<li>Infections: Certain infections can cause inflammation around the spinal cord, which can lead to tethering.</li>
	<li>Severe Spinal Trauma: Trauma to the spinal cord can cause damage that leads to tethering.</li>
	<li>Tumors: Tumors can grow on or near the spinal cord and cause it to become tethered. 
	&nbsp;</li>
</ul>

<h5>Possible Genetic Factors</h5>

Spina bifida and myelomeningocele are both known to have genetic components, but the genetics of tethered cord syndrome is not as well understood. Some studies have suggested that certain genes may increase the risk of developing tethered cord syndrome, but more research is needed to confirm this. 
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Diagnosis

There are currently no specific screening tests for tethered cord syndrome. However, maternal blood tests such as the serum alpha-fetoprotein (AFP) test and fetal ultrasound scans can help screen for spina bifida, which is a common cause of TCS.

Diagnosis of tethered cord syndrome is based on the identification of characteristic signs and symptoms that can locate the lesion above the attachment of anomalies to the spinal cord. 
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A detailed patient history and a thorough clinical evaluation are necessary for diagnosis.

Special imaging techniques such as magnetic resonance imaging (MRI) or computed tomography (CT) scan and ultrasound studies help confirm a diagnosis in children.

 
Some commonly used imaging tests are:

MRI: This is the gold standard for diagnosis. It helps identify the location of the tethering, the presence of a tumor or a lipoma, or whether the base of the spinal cord is lower than normal.

Myelogram: This procedure involves the injection of a contrast dye into the thecal sac that surrounds the spinal cord. An X-ray is then used to detect any tethering of the spinal cord.

CT: A CT scan can be used in people who cannot undergo MRI scans. It may also be conducted after a myelogram to see how the contrast dye flows around the spinal cord.

Ultrasound: In young infants, an ultrasound is used to visualize the movement of the spinal cord within the spinal canal and the presence of any lipomas.

X-Rays: These can help detect bone anomalies associated with neural tube defects.

Electromyography (EMG): This nerve function test can help assess the patient&rsquo;s nerve function, especially if urinary dysfunction is present.&nbsp; 
&nbsp;

Spina bifida is a common imaging feature that supports a diagnosis of tethered cord syndrome.

In teenagers and adults, a consistent finding of displacement of the filum located posterior to the cauda equina is observed.

The combination of MRI, endoscopy, and surgical findings is used to confirm the diagnosis. 
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Treatment

The most common treatment for tethered cord syndrome is surgery, which aims to release the spinal cord from any tissue that is restricting its movement. This surgery called a laminectomy, involves removing bone spurs or tissue from the lower part of the spinal cord, which allows surgeons to access the area around the spinal cord and enlarge the spinal canal. Thus, reducing pressure on the spinal cord and the nerves around it and relieving symptoms. During the surgery, the spinal cord may be freed by gently moving it or by completely cutting it away from the spinal fat that restricts its motion. After the surgery, the area is usually patched up to prevent blood and cerebrospinal fluid loss. 
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In some cases, the spinal cord may reattach itself to the tissue as the child grows, which can lead to a recurrence of symptoms. Additional surgeries may be necessary to release the spinal cord again. Around 1 in 5 children with this condition require more than one surgery due to the occurrence of symptoms during growth phases<a href="#accordion">[4]</a>.

Children can typically return to their normal activities within a few weeks of successful surgery. The amount of muscle and bladder function regained after surgery depends on the severity of the condition before surgery. However, there may be complications such as infections, bleeding, or damage to the spinal cord, which may lead to reduced muscle strength or limited bladder and bowel function. Physical therapy may be necessary to help children recover and regain muscle strength in the areas of the body that were previously affected by the condition. 
&nbsp;

If left untreated, tethered cord syndrome can cause serious consequences such as deteriorating motor and sensory functions, paralysis of the lower body, and total loss of bowel and bladder functions. Therefore, early treatment is essential to prevent long-term damage. 
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Complications of tethered cord syndrome can include a range of issues that may occur during or after treatment. Surgery, which is the primary treatment for tethered cord syndrome, can result in complications such as infections, bleeding, and damage to the spinal cord. In some cases, surgery may not be completely successful, and the spinal cord may reattach to the tissue as the child grows, requiring additional surgeries. 
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Before treatment, tethered cord syndrome can cause a variety of complications depending on the severity of the condition. These can include problems with motor function, such as weakness or numbness in the legs or feet, or difficulties with bowel and bladder function, such as urinary or fecal incontinence. If left untreated, tethered cord syndrome can lead to more serious complications, including deterioration of motor and sensory functions, as well as paralysis of the lower body, and total loss of bowel and bladder functions. 
&nbsp;

Following surgery, physical therapy may be necessary to help the child recover and regain muscle strength and function in areas that were affected by the condition. The degree of recovery will depend on the severity of the condition before treatment, as well as the success of the surgery and the effectiveness of any post-surgical therapy or rehabilitation. 
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The exact cause of tethered cord syndrome is not known, but several risk factors can increase the likelihood of developing this condition. Some of the risk factors for tethered cord syndrome include: 
&nbsp;

<ul>
	<li>Spina Bifida: This is a birth defect in which the spinal cord doesn&#39;t develop properly. Children born with spina bifida are at a higher risk of developing tethered cord syndrome.</li>
	<li>Chiari Malformation: This is a structural defect in which the brain tissue extends into the spinal canal. This can cause the spinal cord to be pulled downwards and become tethered.</li>
	<li>Previous Spinal Surgery: People who have had previous spinal surgery are at a higher risk of developing tethered cord syndrome.</li>
	<li>Trauma: Trauma to the spine, such as a spinal cord injury, can cause the spinal cord to become tethered.</li>
	<li>Tumor: A tumor or other abnormal growth in the spinal cord or spinal canal can cause tethered cord syndrome.</li>
	<li>Infection: Infections that affect the spinal cord, such as meningitis, can cause the spinal cord to become tethered.</li>
	<li>Connective Tissue Disorders: Certain connective tissue disorders, such as Ehlers-Danlos syndrome, can weaken the connective tissues in the spinal cord and increase the risk of tethered cord syndrome. 
	&nbsp;</li>
</ul>

There are no known preventative measures for tethered cord syndrome. However, some risk factors, such as spina bifida, may be prevented with proper prenatal care, including taking folic acid supplements and avoiding certain medications or substances during pregnancy.

Additionally, early detection and treatment of tethered cord syndrome can prevent further damage and potential complications. It is important for individuals with known risk factors or symptoms of tethered cord syndrome to seek medical attention and follow up with their healthcare provider for appropriate evaluation and management. 
&nbsp;

Key Points

<ul>
	<li>Tethered cord syndrome is a neurological disorder that results from the abnormal attachment of the spinal cord to the tissues surrounding the spine.</li>
	<li>The condition can be caused by a birth defect, spinal cord injury, or spinal cord tumors.</li>
	<li>Symptoms of tethered cord syndrome include back pain, leg pain, muscle weakness, and bowel and bladder dysfunction.</li>
	<li>Treatment for tethered cord syndrome usually involves a surgical intervention to release the spinal cord from the tissue.</li>
	<li>Surgery for tethered cord syndrome is called a laminectomy and may involve removing bone spurs or tissues from the lower part of the spinal cord.</li>
	<li>In some cases, patients may need multiple surgeries to release the spinal cord.</li>
	<li>Complications after surgery include infections, bleeding, and damage to the spinal cord.</li>
	<li>Physical therapy may be necessary after surgery to improve muscle strength and regain function.</li>
	<li>Preventative measures for tethered cord syndrome include monitoring for symptoms and prompt intervention with surgery. 
	&nbsp;</li>
</ul>

[1] Bhimani AD, Selner AN, Patel JB, et al. Pediatric tethered cord release: an epidemiological and postoperative complication analysis. J Spine Surg. 2019;5(3):337-350. doi:10.21037/jss.2019.09.02.

[2] Tethered cord syndrome - symptoms, causes, treatment: Nord. National Organization for Rare Disorders.

[3] Tethered spinal cord: What it is, symptoms & treatment. Cleveland Clinic.

[4] What is tethered cord syndrome? WebMD

[5] Strong MJ, Thompson EM, Roundy N, Selden NR. The use of lumbar laminoplasty vs. laminotomy for transection of the filum terminale does not affect early complication rates or postoperative course. Childs Nerv Syst. 2015;31(4):597-601. doi:10.1007/s00381-015-2615-8