Tethered Spinal Cord: Symptoms, Types, Causes and Treatment

Tethered spinal cord syndrome (TCS) is a complex neurological disorder that can affect both children and adults, often leading to a progressive decline in motor, sensory, and autonomic functions. Understanding the symptoms, types, causes, and treatment options for TCS is essential for early diagnosis and optimal management. This comprehensive article synthesizes current research to provide a detailed overview of TCS, with practical insights for patients, caregivers, and healthcare professionals.

Symptoms of Tethered Spinal Cord

Tethered spinal cord syndrome can present with a wide variety of symptoms, which often depend on the patient’s age, the underlying cause, and the severity of cord tethering. Early recognition is critical to prevent irreversible neurological damage.

Symptom	Description	Frequency/Prevalence	Source(s)
Pain	Back, leg, or anorectal pain, often diffuse	32–81%	2 5 12
Motor Deficit	Weakness, gait deterioration, spasticity	54–63%	5 9 12
Sensory Loss	Numbness, tingling in legs/feet	61%	1 2 12
Bladder/Bowel	Incontinence, urgency, retention, constipation	6–56%	1 2 5 9 12
Orthopedic	Scoliosis, foot/spinal deformities	11–51% (mainly children)	5 7
Dermatologic	Skin stigmata over lower back	Variable	4 7

Table 1: Key Symptoms of Tethered Spinal Cord

Pain and Sensory Changes

Pain is the most common presenting symptom in both adults and children, often described as diffuse, non-dermatomal, and sometimes referred to the anorectal area. Sensory symptoms such as numbness or tingling in the lower extremities are also frequent and can progress over time if not addressed 2 12.

Motor Decline

Progressive weakness, difficulty walking, and muscle spasticity are hallmark signs. In children, these may manifest as delayed milestones or a decline in previously acquired skills, while in adults, there can be a gradual or sudden onset of weakness, often triggered by trauma or physical stress 1 2 5 9 12.

Bladder and Bowel Dysfunction

Bladder and bowel symptoms range from mild urgency to severe incontinence. These symptoms are particularly concerning as they often indicate advanced neurological compromise. Notably, resolution after surgery is less predictable for urological symptoms compared to pain and motor issues 1 2 5 9 12.

Orthopedic and Dermatologic Findings

Children may develop scoliosis or foot and spinal deformities due to chronic abnormal tension on the cord 5 7. Dermatologic markers—such as hairy patches, dimples, or lipomas over the lumbosacral region—can serve as external clues to underlying tethering, particularly in pediatric cases 4 7.

Types of Tethered Spinal Cord

Tethered cord syndrome encompasses a spectrum of anatomical and clinical conditions. Identifying the specific type of tethering is crucial for targeted management.

Type/Variant	Key Features	Distinguishing Factors	Source(s)
Congenital	Present at birth; associated with dysraphism	Often linked to neural tube defects	4 5 7
Acquired	Develops after birth (e.g., post-surgical)	Follows repair of spinal defects	5 10
Occult/Normal Conus	Conus appears normal but symptoms present	Radiology may be misleading	3 4 11
Retethered Cord	Recurrent symptoms after prior surgery	Scar/adhesion formation	10 13

Table 2: Types of Tethered Spinal Cord

Congenital Tethered Cord

This is the most common type and is often associated with spinal dysraphic conditions such as myelomeningocele, lipomyelomeningocele, split cord malformation, and fatty filum terminale. These anomalies prevent the normal ascent of the conus medullaris during development, leading to chronic cord tension and neurological sequelae 4 5 7.

Acquired Tethered Cord

Acquired tethering usually follows surgical intervention for neural tube defects or trauma, where postoperative scarring or adhesions cause the spinal cord to become fixed. Up to 30% of children with repaired myelomeningocele may develop symptomatic TCS later in life 5 10.

Occult Tethered Cord (Normal Conus)

In some individuals, classical imaging may not show a low-lying conus, yet clinical features of tethering are present. These cases challenge traditional radiological definitions and underscore the need for clinical vigilance 3 4 11.

Retethered Cord

Retethering refers to the recurrence of symptoms after prior detethering surgery, usually due to new adhesions or scarring. This complicates management and sometimes requires innovative surgical approaches to prevent further retethering 10 13.

Causes of Tethered Spinal Cord

Understanding the underlying causes of tethered cord syndrome is essential for prevention, diagnosis, and treatment planning. Causes are typically categorized as congenital or acquired.

Cause	Description	Mechanism/Association	Source(s)
Thickened Filum	Fatty, short, or inelastic filum terminale	Limits cord movement	1 2 3 4 7
Lipoma	Fatty mass within or near spinal canal	Physically tethers cord	2 4 7 9
Myelomeningocele	Neural tube defect with cord exposure	Fixation at defect site	4 5 7
Scarring/Adhesion	Post-surgical or traumatic cord attachment	Loss of cord mobility	5 10 13
Split Cord Malformation	Two hemicords, often with bony/fibrous spur	Structural tethering	4 7 9
Tumors/Infections	Intraspinal lesions causing fixation	Compression or scarring	4

Table 3: Causes of Tethered Spinal Cord

Congenital Causes

• Thickened or Fatty Filum Terminale: The most common congenital cause, where the filum terminale (a thin filament anchoring the cord) is thickened, shortened, or infiltrated with fat, preventing normal spinal cord movement 1 2 3 4 7.
• Lipomas and Lipomyelomeningoceles: Fatty masses that physically tether the cord, often associated with other spinal anomalies 2 4 7 9.
• Myelomeningocele and Other Dysraphisms: Neural tube defects create abnormal attachments between the spinal cord and surrounding tissues, leading to chronic tension 4 5 7.
• Split Cord Malformations: Characterized by a duplicated spinal cord, often separated by a bony or fibrous spur, leading to mechanical tethering 4 7 9.

Acquired Causes

• Scarring and Adhesions: Surgery for spinal dysraphism or trauma can result in scar tissue that re-tethers the cord, especially in children with repaired myelomeningocele 5 10 13.
• Intraspinal Tumors or Infections: These can cause direct fixation or induce scarring around the cord 4.

Pathophysiology: How Tethering Causes Damage

Tethering exerts abnormal longitudinal traction on the spinal cord, especially during growth or movement. This leads to vascular compromise (reduced blood flow), impaired oxidative metabolism, and ultimately, progressive neurological dysfunction. The degree of metabolic and vascular disturbance correlates with the severity of symptoms 1 3 6 7.

Treatment of Tethered Spinal Cord

Effective management of tethered spinal cord syndrome focuses on relieving cord tension and preventing further neurological deterioration. Treatment approaches have evolved, especially for challenging or recurrent cases.

Treatment	Description	Outcomes/Considerations	Source(s)
Surgical Detethering	Microsurgical release of tethered cord	Pain/motor improvement common; bladder recovery less predictable	5 9 11 12
Preventive Techniques	Use of barriers (e.g., Gore-Tex membrane)	Lowers risk of retethering	10
Spinal Column Shortening	Osteotomy to reduce cord tension	For recurrent/complex cases	13
Conservative	Observation in select cases	For mild or stable symptoms	11

Table 4: Treatments for Tethered Spinal Cord

Surgical Detethering

Microsurgical release of the tethered cord is the mainstay of treatment. This involves careful separation of the cord from surrounding tissues, removal of tethering lesions (e.g., fatty filum, lipoma), and restoration of cord mobility. Surgery often leads to stabilization or improvement of pain and motor symptoms; however, bladder and bowel dysfunction are less likely to resolve completely, especially if symptoms have been present for a long time 5 9 11 12.

• Early intervention is associated with better outcomes, especially before severe neurological deficits become irreversible 2 5 9.
• Risks include infection, cerebrospinal fluid leak, and (rarely) neurological worsening.
• Recurrence: Retethering can occur in 5–50% of cases, particularly after prior surgery for complex spinal dysraphism 13.

Preventing Retethering

To minimize the risk of the cord re-adhering post-surgery, some surgeons use synthetic barriers, such as a Gore-Tex membrane, to physically separate the cord from the surrounding tissue. Preliminary results suggest this technique is effective, though longer-term studies are needed 10.

Spinal Column Shortening

For patients with recurrent tethering or extensive adhesions where direct detethering is not feasible, posterior vertebral column subtraction osteotomy (removal of a segment of the spine to shorten it) can relieve tension on the cord indirectly. This is a technically demanding procedure reserved for select cases 13.

Conservative Management

In asymptomatic or mildly symptomatic patients, especially adults, careful observation and regular monitoring may be appropriate. Treatment decisions must balance risks and benefits, as unnecessary surgery can carry significant risks 11.

Diagnostics and Follow-up

• Imaging: MRI is the gold standard for diagnosis, but prone MRI can increase the sensitivity for detecting subtle tethering or retethering 8.
• Multidisciplinary Care: Children with TCS, especially after myelomeningocele repair, benefit from follow-up in clinics that include neurosurgery, orthopedics, and urology 5.

Conclusion

Tethered spinal cord syndrome is a multifaceted neurological disorder with potentially life-altering consequences if left untreated. Early recognition, accurate diagnosis, and timely intervention are key to optimizing outcomes.

Key Points:

• Varied Symptoms: TCS can present with pain, motor and sensory deficits, bladder/bowel dysfunction, orthopedic deformities, and skin stigmata, often varying by age and underlying cause 1 2 5 7 12.
• Multiple Types and Causes: It includes both congenital and acquired forms, with causes ranging from thickened filum terminale and lipomas to surgical adhesions and split cord malformations 2 4 5 7 10.
• Progressive Pathology: Chronic cord tension leads to impaired blood flow and metabolism, resulting in neurological decline 1 3 6 7.
• Surgical Treatment: Detethering is the mainstay, with early surgery offering the best chance of symptom improvement; preventive and alternative surgical techniques are evolving for complex cases 5 9 10 12 13.
• Comprehensive Care: Multidisciplinary follow-up and individualized management plans are crucial, especially in children and recurrent cases 5 11 13.

Timely intervention and coordinated care can significantly improve quality of life for individuals with tethered spinal cord syndrome. Ongoing research continues to refine both diagnosis and treatment strategies, offering hope for better outcomes in the future.