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Tethered Spinal Cord Syndrome and Surgery: What Parents Need to Know

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:

Segmental lumbosacral hemangioma shown in a 2-year-old
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.

Tests for tethered spinal cord syndrome typically include:

1. Bowel and bladder control assessment

Tethered spinal cord syndrome can damage the nerves responsible for bowel and bladder control, causing incontinence. Children with suspected spinal cord tethering are often referred to a pediatric urologist for bowel and bladder function tests.

2. Magnetic resonance imaging (MRI)

An MRI is an imaging technology that produces detailed images of anatomy inside the body, and can help identify common causes of tethered spinal cord syndrome, like tumors, fatty growths, and spinal trauma. Patricia Weltin, whose daughter, Olivia, underwent untethering surgery, says an MRI was all they needed to confirm a diagnosis. “Olivia’s tethering was very, very apparent,” she says.

But that isn’t always the case. Sometimes, MRIs fail to detect a tethered spinal cord. This is called occult tethered cord syndrome (OCTS) and can be challenging to diagnose. Parents of children with tethered spinal cord syndrome say OCTS is one of the reasons it’s so important to find a pediatric neurologist who specializes in tethered spinal cord syndrome if there’s a suspicion your child has it. These experts have the training and knowledge to identify more nuanced symptoms, such as leg and back numbness, that might otherwise be overlooked.

Untethering surgery

The complexity of untethering surgeries varies from quick and straightforward, such as untethering a thickened filum terminale, to long and complicated. For example, a child with spina bifida might need a major untethering operation to close up their back and reposition their spinal cord. “It’s a very wide range of procedures,” Proctor says.

Let’s take a closer look at scenarios at each end of the complexity spectrum.

Klinge says that straightforward untethering surgery typically takes about an hour and a half to two hours. During the operation, a pediatric neurosurgeon makes an incision in the part of the back where the tethering occurs and uses surgical tools to gently detach the tether, freeing the spinal cord from the spinal canal. After releasing the tether, the surgeon closes the incision and moves your child to a recovery room.

More complicated untethering procedures can take six to eight hours. These procedures take longer because more tissue connects the spinal cord to the spinal canal. To ensure patient safety, surgeons “really have to tease out every nerve and map out the spinal cord,” Klinge explains. Because of the complexity, this type of untethering surgery often comes with increased risks, such as bleeding, infection, and spinal cord injury.

Preparing for spinal cord untethering

Prior to surgery, your child’s care team will look at your child’s individual situation and perform relevant testing. They’ll also provide pre-op instructions, such as fasting before surgery.

To prepare your child for surgery, Pasillas recommends working with your child’s care team to address your child’s unique needs, such as how your child responds to pain and how they communicate (such as vocally or with a communication device). It may be helpful to discuss possible scenarios with your child so that communication is easier later, such as when feeling pain or thirst. The care team will also consider if your child uses a feeding tube or has other medical needs.

Recovery after spinal cord untethering surgery

Just as surgeries range in complexity, so do recovery experiences. Some kids spend only a few days in the hospital, while others stay for a week or more. While your child is in the hospital, you may want to ask if a child life specialist is available. A child life specialist can provide resources and emotional support both leading into the surgery and after.

After surgery, all children must lie flat on their backs for the first 24 hours to reduce the risk of cerebrospinal fluid (CSF) leaks. Klinge says these leaks occur in about 3% to 5% of cases. Even so, lying still for hours can be particularly challenging for infants and children with special needs. Many surgeons prescribe paralytic drips to promote relaxation and make this part of recovery easier.

After minor untethering surgery, Klinge says school-age kids typically take seven to ten days off school. They also need to limit physical activity (like contact sports) and avoid lifting anything over five pounds for at least three months. Your child’s team will recommend when your child can resume therapy sessions should your child participate in physical or occupational therapy, for example.

For children recovering from complex untethering surgery, healing is more complex. “These children often have significant side effects from the operation,” Proctor says. “There’s a good chance some of the nerves in the legs, bowels, and bladder could be injured.” In these instances, your doctor may require physical therapy and/or occupational therapy, when your doctor feels your child is ready. Your child may also require assistive mobility devices, like a wheelchair.

Gwen Langfahl
Credit: Elizabeth Langfahl | Gwen Langfahl

Kids generally experience a significant improvement in their symptoms after untethering surgery. Elizabeth Langfahl, whose daughter, Gwen, underwent a minor untethering procedure, says the difference was night and day. “It felt like a miracle. I just remember watching Gwen go up a set of stairs for the first time when I’d been used to watching her crawl up the stairs––this was within a week of surgery!”

However, not every child has the same positive experience. Some children continue to experience pain, weakness, and mobility problems after untethering surgery. These postoperative complications are more common in girls than boys.

Pasillas says that while untethering surgery prevented Aiyala from permanent nerve damage, it resulted in other complications. For example, before untethering surgery, Aiyala could stand up assisted and move around on her own. Afterward, those skills significantly diminished. “In one sense, we accomplished what we wanted,” Pasillas says. “But it came with a cost.”

Will my child need more than one untethering surgery?

Most children with tethered spinal cord syndrome do not need multiple operations. In cases of minor untethering surgery, “the chance of needing another operation is very small, about 1% or 2%,” Proctor says. “For more complex forms of untethering, the literature says there’s a one in five chance of needing more surgery, but in reality, it’s probably a little bit higher than that.” Across cases, the American Association of Neurological Surgeons says 10-20% of children with tethered spinal cord syndrome need more than one surgery.

The bottom line

Tethered spinal cord syndrome ranges in severity. The treatment is often surgery, but the complexity of the procedure varies based on your child’s individual situation.

“I think the most important thing for families to understand is that you shouldn’t panic or assume the worst,” Proctor says. Even though the idea of untethering surgery can be frightening, Klinge echoes this advice. “The procedure is very reasonable and can stop the progression of symptoms, salvage the spinal cord, and prevent neurological damage.”

References and Further Reading:

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