Background

Tethered cord syndrome (TCS) is a neurological disorder characterized by abnormal tension or fixation of the spinal cord within the spinal column. In this condition, the spinal cord is unnaturally stretched or pulled, usually due to an abnormal attachment of tissue, scarring, or other anatomical abnormalities.

This tension restricts the normal movement of the spinal cord and can lead to various neurological symptoms and complications. Tethered cord syndrome can be congenital, meaning present at birth, or acquired later in life.

Congenital TCS often occurs due to improper development of the spinal cord during fetal growth, while acquired TCS can result from spinal cord injury, spinal surgery, spinal tumors, or spinal infections.

Epidemiology

The exact prevalence of tethered cord syndrome (TCS) is not well-established as it is a relatively rare condition. However, it is estimated to occur in approximately 1 to 5 per 1,000 live births. TCS can affect individuals of all ages, including infants, children, and adults.

Here are some key points regarding the epidemiology of tethered cord syndrome:

• Congenital TCS: Tethered cord syndrome is often present at birth (congenital). It is commonly associated with other underlying congenital conditions, such as spina bifida, lipomyelomeningocele, or other spinal dysraphisms. The prevalence of congenital TCS varies depending on the specific population studied and the presence of associated conditions.
• Acquired TCS: While TCS is commonly associated with congenital conditions, it can also develop later in life as an acquired condition. Acquired TCS can occur due to spinal cord trauma, spinal surgeries, spinal tumors, spinal infections, or other factors leading to scar tissue formation or abnormal tissue attachments.
• Age Distribution: TCS can affect individuals of all ages, but the presentation and underlying causes can differ. In infants and young children, TCS is often associated with congenital abnormalities and may present with symptoms such as delayed motor development or signs of neurogenic bladder or bowel dysfunction. In older children and adults, TCS may be acquired and may present with symptoms such as back pain, leg weakness, or changes in gait.
• Gender Distribution: There is no significant gender predilection for tethered cord syndrome. It affects males and females equally.
• Associated Conditions: TCS is commonly associated with other spinal cord abnormalities, such as spina bifida, lipomyelomeningocele, or spinal dysraphism. Additionally, conditions like scoliosis, Chiari malformation, and spinal cord tumors may also be found in individuals with TCS.

Anatomy

Pathophysiology

The pathophysiology of tethered cord syndrome (TCS) involves abnormal tension or fixation of the spinal cord within the spinal column, resulting in functional impairment and potential neurological deficits. The exact mechanisms leading to tethering of the spinal cord can vary depending on whether it is congenital or acquired.

• Congenital TCS: In congenital TCS, the pathophysiology is often related to incomplete spinal cord development during fetal growth. Several underlying anatomical abnormalities can contribute to the tethering of the spinal cord, including:
• Spina Bifida: This is a condition where the spinal column fails to close completely, resulting in a defect through which the spinal cord and its covering structures may protrude. The abnormal neural tube development can lead to abnormal tethering and stretching of the spinal cord.
• Lipomyelomeningocele: This is a type of spina bifida characterized by an abnormal fatty mass (lipoma) located within the spinal canal. The lipoma can cause tension on the spinal cord, leading to its fixation or tethering.
• Tethered Cord Skin Lesion: Some individuals with congenital TCS may have visible signs on the skin, such as a tuft of hair, dimple, or fatty mass over the lower back. These skin lesions may be associated with underlying abnormalities that contribute to spinal cord tethering.
• Acquired TCS: Acquired TCS can occur due to various factors that lead to abnormal tissue attachments, scarring, or tension on the spinal cord. Some common causes include:
• Spinal Cord Trauma: Injury to the spinal cord, such as from a traumatic event or surgery, can result in scar tissue formation or abnormal tissue attachments that tether the spinal cord.
• Spinal Surgeries: Certain spinal surgeries, such as those involving the removal of spinal tumors or the correction of spinal deformities, can lead to scar tissue formation or unintended tissue attachments that cause spinal cord tethering.
• Spinal Infections: Infections of the spinal cord or surrounding tissues can lead to inflammation, scarring, and tissue adhesions that may tether the spinal cord.

The tethering of the spinal cord in TCS can result in several pathophysiological consequences:

• Restricted Spinal Cord Movement: The tension on the spinal cord restricts its normal movement and mobility within the spinal column. This can interfere with the spinal cord’s ability to respond to changes in position or stretch, compromising its normal function.
• Impaired Blood Flow: Tethering of the spinal cord can compress or kink the blood vessels supplying the spinal cord, leading to impaired blood flow. Reduced blood flow can result in ischemia (lack of oxygen and nutrients) and damage to the spinal cord tissue.
• Disrupted Neural Signaling: Tethering can disrupt the normal transmission of neural signals along the spinal cord. This can interfere with sensory, motor, and autonomic functions, leading to various neurological symptoms.

Etiology

Tethered cord syndrome (TCS) can have various etiologies, both congenital and acquired, that contribute to the abnormal tethering or fixation of the spinal cord. The underlying causes of TCS can differ depending on whether it is present at birth (congenital) or develops later in life (acquired). Here are some common etiological factors associated with TCS:

Congenital Causes:

• Spina Bifida: TCS is commonly associated with spina bifida, a neural tube defect characterized by incomplete closure of the spinal column during fetal development. Spina bifida can lead to abnormal tethering of the spinal cord due to the presence of an underlying myelomeningocele, lipomyelomeningocele, or other spinal dysraphisms.
• Lipomyelomeningocele: This is a specific type of spina bifida in which a fatty mass (lipoma) is attached to the spinal cord. The lipoma can cause tethering of the spinal cord, leading to TCS.
• Other Congenital Anomalies: Certain congenital abnormalities, such as dermal sinus tracts, diastematomyelia (split spinal cord), or abnormal bony structures in the spinal column, can contribute to spinal cord tethering and the development of TCS.

Acquired Causes:

• Spinal Cord Trauma: Injury to the spinal cord, such as from trauma or surgery, can cause scar tissue formation or the development of adhesions that tether the spinal cord. Trauma-related causes of TCS can include spinal fractures, penetrating injuries, or surgical interventions.
• Spinal Surgeries: Some surgical procedures performed on the spine can inadvertently lead to the development of TCS. For example, procedures to correct spinal deformities or remove spinal tumors can cause scar tissue formation or tissue adhesions that result in spinal cord tethering.
• Spinal Infections: Infections affecting the spinal cord or surrounding tissues, such as meningitis or spinal abscesses, can cause inflammation, scarring, and tissue adhesions that lead to TCS.
• Spinal Tumors: Tumors within or near the spinal canal can exert pressure on the spinal cord and cause it to become tethered or fixed in place, resulting in TCS.
• Radiation Therapy: Radiation treatment directed at the spinal cord or nearby areas can cause fibrosis, scarring, and tissue adhesions that contribute to spinal cord tethering and the development of TCS.

It’s important to note that TCS can have a multifactorial etiology, and some cases may involve a combination of congenital and acquired factors. Additionally, in some cases, the cause of TCS may remain unknown (idiopathic).

If TCS is suspected, a comprehensive evaluation by a healthcare professional, such as a neurosurgeon or pediatric neurologist, is necessary to determine the underlying cause and guide appropriate management.

Diagnostic imaging, such as magnetic resonance imaging (MRI), is typically used to visualize the spinal cord and identify any tethering or associated abnormalities.

Genetics

Prognostic Factors

The prognosis of tethered cord syndrome (TCS) can vary depending on several factors, including the underlying cause, severity of symptoms, age at diagnosis, and timely intervention. With appropriate treatment and management, many individuals with TCS can experience improvement in their symptoms and have a good long-term prognosis. However, it’s important to note that individual outcomes can differ, and some patients may continue to experience residual symptoms or complications. Here are some key points regarding the prognosis of TCS:

• Early Intervention: Early diagnosis and timely surgical intervention for TCS tend to yield better outcomes. Prompt surgical release of the tethered cord can help alleviate symptoms and prevent further neurological deterioration or complications.
• Symptom Improvement: Following surgical detethering, many individuals experience improvement or resolution of their symptoms. This can include relief from back pain, leg pain, sensory abnormalities, muscle weakness, and bladder/bowel dysfunction.
• Residual Symptoms: In some cases, individuals may have residual symptoms even after surgical treatment. The extent and persistence of residual symptoms can vary and may depend on factors such as the severity of the initial cord tethering, the presence of associated spinal abnormalities, and the duration of symptoms prior to treatment.
• Complications and Recurrence: While surgical release of the tethered cord can be successful in alleviating symptoms, complications and recurrence can occur in some cases. Complications may include surgical site infections, cerebrospinal fluid leaks, or neurological deficits. Recurrence of tethering can also happen, particularly in cases where the underlying cause (such as spina bifida) cannot be completely resolved.
• Long-Term Monitoring: Individuals with TCS often require long-term monitoring to assess their neurological status and identify any potential recurrence or new symptoms. Regular follow-up visits, imaging studies (such as MRI), and consultations with the healthcare team are essential for ongoing management and evaluation.
• Functional Outcomes: The impact of TCS on an individual’s functional abilities can vary. With appropriate management, including physical therapy and rehabilitation, many patients can improve their mobility, strength, coordination, and overall functional abilities.

Clinical History

Clinical history

When taking a clinical history of a patient suspected of having tethered cord syndrome (TCS), healthcare professionals typically focus on gathering information related to the patient’s symptoms, medical history, and developmental milestones. Here are some key aspects to consider when obtaining a clinical history for TCS:

• Presenting Symptoms: Inquire about the specific symptoms the patient is experiencing. Common symptoms associated with TCS include:
• Back pain: Ask about the location, duration, severity, and exacerbating factors of the back pain.
• Leg pain: Determine the presence, location, radiation pattern, and aggravating factors of any leg pain or sciatica-like symptoms.
• Sensory changes: Inquire about any numbness, tingling, or abnormal sensations in the lower back, buttocks, or legs.
• Muscle weakness: Ask about any weakness or difficulty in moving the legs, feet, or toes.
• Bladder/bowel dysfunction: Determine if there are any changes in urinary or bowel function, such as urinary frequency, urgency, hesitancy, incontinence, or constipation.
• Gait abnormalities: Inquire about any changes in walking pattern, such as limping, clumsiness, or difficulty with balance.
• Developmental Milestones: In children, assess their developmental history, particularly milestones related to motor function. Inquire about any delays in crawling, walking, or other motor skills compared to their peers.
• Past Medical History: Gather information about any relevant medical conditions, surgeries, or known congenital abnormalities. Conditions such as spina bifida, lipomyelomeningocele, or previous spinal surgeries may increase the risk of TCS.
• Family History: Ask about any family history of spina bifida, TCS, or other congenital spinal abnormalities. Some cases of TCS can have a genetic component.
• Trauma or Surgery History: Inquire about any history of spinal trauma or previous spinal surgeries, as these can contribute to acquired TCS.
• Neurological Symptoms: Assess for the presence of any additional neurological symptoms, such as headaches, changes in vision, weakness or sensory changes in other parts of the body, or symptoms suggestive of Chiari malformation.
• Functional Impact: Evaluate how the symptoms of TCS are impacting the patient’s daily activities, mobility, and overall quality of life.

Physical Examination

Physical examination

When performing a physical examination for a patient suspected of having tethered cord syndrome (TCS), healthcare professionals typically focus on assessing neurological function and looking for signs of spinal abnormalities. Here are some key aspects to consider during the physical examination for TCS:

Neurological Examination:

• Motor Function: Evaluate muscle strength and tone in the lower extremities, assessing for any weakness, atrophy, or spasticity.
• Sensory Function: Test for sensation in the lower back, buttocks, and legs, checking for any areas of numbness, tingling, or abnormal sensory perception.
• Reflexes: Assess deep tendon reflexes, such as the patellar reflex (knee jerk) and Achilles reflex, which may be altered in TCS.
• Coordination and Balance: Observe the patient’s gait, looking for any abnormalities in balance, coordination, or unsteady walking.
• Bladder/Bowel Function: Evaluate for signs of neurogenic bladder or bowel dysfunction, such as urinary retention, incontinence, or abnormal bowel habits.

Dermatological Examination:

• Skin Lesions: Inspect the lower back for any visible skin abnormalities, such as a tuft of hair, dimple, or fatty mass, which can be associated with underlying spinal cord tethering.

Spinal Examination:

• Spinal Alignment: Observe the patient’s posture and assess for any signs of scoliosis, kyphosis, or other spinal deformities.
• Spinal Column Palpation: Palpate the spine for any bony abnormalities, such as spina bifida defects or abnormalities along the vertebral column.
• Spinal Range of Motion: Assess the patient’s ability to flex, extend, and laterally bend the spine, noting any restrictions or discomfort.

Additional Assessments:

• Cranial Nerve Examination: Perform a thorough assessment of cranial nerve function, particularly assessing for any signs of Chiari malformation, which can be associated with TCS.
• Developmental Milestones: In pediatric patients, assess developmental milestones, focusing on motor function, such as crawling, walking, or other motor skills.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Differential diagnosis

When evaluating a patient with suspected tethered cord syndrome (TCS), it is essential to consider other conditions that can present with similar symptoms. The following are some of the differential diagnoses to consider:

• Lumbar Disc Herniation: A herniated disc in the lumbar spine can compress nerve roots and cause symptoms similar to TCS, such as back pain, leg pain (sciatica), numbness, and muscle weakness. Imaging studies, such as MRI, can help differentiate between TCS and disc herniation.
• Spinal Cord Tumors: Tumors of the spinal cord or surrounding structures can lead to symptoms resembling TCS. These may include back pain, radicular pain, muscle weakness, sensory changes, and bowel/bladder dysfunction. Imaging studies and possibly a biopsy are necessary to differentiate between TCS and spinal cord tumors.
• Spinal Cord Injuries: Traumatic or non-traumatic injuries to the spinal cord can cause symptoms similar to TCS. However, the history of a traumatic event or clear evidence of spinal cord injury on imaging can help distinguish between the two conditions.
• Cauda Equina Syndrome: This condition occurs when the nerve roots of the cauda equina, located below the level of the spinal cord, are compressed. It can result in similar symptoms to TCS, such as back pain, leg pain, numbness, muscle weakness, and bowel/bladder dysfunction. Imaging studies and careful evaluation of clinical features can aid in differentiating between the two conditions.
• Arachnoiditis: Arachnoiditis refers to inflammation of the arachnoid membrane surrounding the spinal cord. It can cause symptoms resembling TCS, including back pain, radicular pain, numbness, muscle weakness, and bladder/bowel dysfunction. Imaging studies and clinical evaluation can help differentiate between the two conditions.
• Spinal Stenosis: Spinal stenosis refers to the narrowing of the spinal canal, which can lead to compression of the spinal cord or nerve roots. Symptoms may include back pain, leg pain, numbness, weakness, and difficulty with walking. Imaging studies, such as MRI or CT myelography, can help in distinguishing between TCS and spinal stenosis.
• Neurogenic Bladder/Bowel Dysfunction: Various neurological conditions, such as multiple sclerosis, spinal cord injury, or other spinal cord disorders, can cause bladder and bowel dysfunction similar to TCS. A comprehensive neurological evaluation and appropriate imaging can aid in making the correct diagnosis.

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

The treatment of tethered cord syndrome (TCS) typically involves surgical intervention to release the tethered spinal cord and alleviate symptoms. The specific approach and surgical technique may vary depending on the underlying cause, severity of symptoms, and individual patient characteristics. Here are some common treatment options for TCS:

• Surgical Release of Tethered Cord: The primary treatment for TCS is surgical detethering of the spinal cord. The procedure aims to free the spinal cord from any abnormal attachments, scar tissue, or adhesions that are causing the tethering.
• The surgery is typically performed by a neurosurgeon or pediatric neurosurgeon, and the specific technique may vary depending on the underlying cause and individual patient factors. In some cases, additional procedures may be performed concurrently, such as addressing associated spinal abnormalities or removing spinal tumors if present.

Symptomatic Management:

• Pain Management: Medications such as nonsteroidal anti-inflammatory drugs (NSAIDs), muscle relaxants, or neuropathic pain medications may be prescribed to manage back pain or radicular pain associated with TCS.
• Bladder and Bowel Management: If TCS has led to neurogenic bladder or bowel dysfunction, a urologist or gastroenterologist may be involved to provide appropriate management strategies, such as catheterization, medications, or bowel management programs.
• Rehabilitation and Physical Therapy: After surgical intervention, rehabilitation and physical therapy may be recommended to help improve strength, mobility, and overall function. Physical therapy exercises and interventions can assist in restoring muscle strength, improving coordination, and optimizing gait and balance.
• Monitoring and Follow-up: Regular follow-up visits with the healthcare team are important to monitor the patient’s progress, assess for any recurrence of symptoms, and address any new concerns that may arise. Imaging studies, such as MRI, may be performed periodically to evaluate the status of the spinal cord and monitor for any potential re-tethering.

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References

https://www.ncbi.nlm.nih.gov/books/NBK585121/