Written by Chad Birt
A tethered spinal cord (or tethered spinal cord syndrome) is a developmental abnormality that occurs in about 2 of every 1,000 births. The condition is primarily identified in infancy and early childhood but sometimes isn’t diagnosed until adulthood. Certain medical conditions, like spina bifida, seem to increase the risk of tethered spinal cord syndrome, but researchers haven’t found a genetic link.
The severity of tethered spinal cord syndrome varies. For some, it causes occasional or persistent pain and mobility issues. For others, it can be severely crippling, with additional symptoms such as incontinence.
Children with minor spinal cord tethering don’t always need intervention, but in most cases, surgery is the only treatment option. Untethering surgery detaches the tissue that hampers spine movement, relieving symptoms and improving mobility. If you’re making this choice for your child, familiarizing yourself with tethered spinal cord syndrome and untethering surgery can help you make the right decision.
WHAT IS TETHERED SPINAL CORD SYNDROME?
Tethered spinal cord syndrome is a condition that impacts the spine’s ability to grow and move. It occurs for different reasons but is commonly associated with conditions such as Ehlers-Danlos syndrome, a connective tissue disorder, and spina bifida, a birth defect that affects the spine.
“The spinal cord is anchored in the spine in a way that it can float freely,” explains Petra Klinge, MD, PhD, a pediatric neurosurgeon and spinal cord untethering expert based in Providence, RI. “Anything that holds the spinal cord unnaturally to the spinal canal causes tethering.” For instance, spinal cord tethering can occur if your child has a thickened filum terminale—a piece of tissue located between the tip of their spinal cord and tailbone. Tethering can also occur due to medical conditions or scar tissue that forms after spine surgery.
Tethering can occur in any part of the spine, but it’s most common in the lower back near the tailbone. It isn’t always noticeable at first. As a child grows, spinal cord tethering can place excess pressure on surrounding tissue and nerves, impacting the spine’s flexibility and increasing the risk of more severe problems, such as neurological damage.
SYMPTOMS OF TETHERED SPINAL CORD SYNDROME
Identifying tethered spinal cord syndrome in infants and young children can be challenging. Symptoms include:
Credit: Controversies in Dermatology, Segmental lumbosacral hemangioma shown in a 2-year-old
Difficulty walking
Increased fussiness when sitting up and/or standing
Scoliosis – an abnormal spinal curve
Hemangiomas – discolored patches of skin on the lower back (see photo for one example; note hemangiomas vary)
Numbness in the legs and back
Muscle atrophy – loss of muscle mass
Incontinence – loss of bladder and bowel control
Symptoms affect children differently. Mark Proctor, MD, a pediatric neurosurgeon at Boston Children’s Hospital, says growth spurts tend to exacerbate them. As the spine lengthens, the spinal cord tether stretches like a rubber band, which can increase the presence of symptoms. Untethering surgery relieves this tension and reduces the risk of lasting complications.
RISK FACTORS FOR TETHERED SPINAL CORD SYNDROME
Anyone can be born with tethered spinal cord syndrome—it isn’t always related to an underlying condition. However, medical conditions associated with tethered spinal cord include:
Ehlers-Danlos syndrome
Spina bifida (between 20%-50% of children with spina bifida also have a tethered spinal cord)
Lipomyelomeningocele (a birth defect that causes fat to attach to the spinal cord and its membranes)
Lipoma (a benign, fatty growth on the spine)
Benign tumors present at birth
A thickened or tight filum terminale (a delicate piece of tissue that connects the spinal cord to the tailbone)
Split spinal cord
Severe spinal trauma
Scar tissue that develops after a spinal surgery
Dermal sinus tract (a congenital abnormality that causes the skin to fold inward toward the spine)
Syringomyelia (a neurological disorder that causes fluid-filled cysts to form in the spinal cord)
And, other genetic conditions, such as Trisomy 18 (Edwards syndrome)
HOW TO TEST FOR TETHERED SPINAL CORD SYNDROME
Pediatricians may have limited experience with tethered spinal cord syndrome and may not be aware of the increased risk associated with certain co-occurring conditions. This can complicate diagnosis for children who have more than one medical diagnosis.
Tiffany Pasillas, whose daughter, Aiyana, had untethering surgery, says getting a diagnosis was one of the biggest obstacles to treatment. “It took me about a year of pretty vigorous advocating before I got Aiyana’s care team to take me seriously,” Pasillas says. Since Aiyanna has multiple diagnoses, many of her symptoms, like difficulty sitting and lying down, were attributed to other health issues.
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