When picturing what happens when a child is affected by tethered spinal cord syndrome, it may help to imagine a balloon attached to a long string. If the string of the balloon becomes stuck or tangled—perhaps on the overhang of a door, or in the frame of a chair—the balloon can no longer turn and flow freely with the breeze: Its range of motion is greatly reduced.
In a normal spinal canal:
- The spinal cord is covered by a tough membrane called the dura.
- A substance called cerebrospinal fluid, produced by the brain, flows over and around the spine and spinal cord, cushioning it from trauma.
- Before birth, the spinal cord grows along with the infant, gradually moving upward into the spinal canal.
- Around the time of birth, the spinal cord stops moving upward, but continues to hang freely in the fluid-filled “tube” of the spinal canal
- The cord bends and flexes with the child, moving up and down to accommodate sitting, standing, stretching and other motions.
When a child has tethered spinal cord syndrome, the spinal cord becomes affixed to the surround tissues for one of the following reasons:
- The spine, spinal cord and/or spinal canal did not develop properly (for example, because of spina bifida.
- The filum terminale, which connects the spinal cord to the backbone, thickens or hardens, pushing down on the cord.
- The child has undergone spinal surgery, resulting in scarring that weighs down the spinal cord.
- A tumor has formed on the spinal cord.
- A fatty piece of skin (called a dermoid or epidermoid) grows into the spinal cord, creating the same effect as a tumor although it is not cancerous.
As a result, the cord can’t flex or bend within the spinal canal.
Though the exact process is still not fully understood, neurological problems can develop in response to a tethered spinal cord. Experts believe that these problems are linked to abnormal stress and strain on the nerve roots at the lower end of the spinal cord; as they are taxed, the nerves themselves are damaged.
Research also indicates that the ongoing strain on the nerve roots may thin the nourishing, oxygen-rich blood that reaches the spinal cord nerves and their cells: Because the nerves are not getting the blood and oxygen they need, they are weak and more prone to injury.
As the condition progresses, a child with tethered spinal cord syndrome may eventually experience:
- weakness in the legs or feet
- problems controlling his bowels or bladder
- pain in his back or legs
- a curvature of the spine or scoliosis
|A unique Children’s program helps kids who are living with incontinence|
What causes tethered spinal cord syndrome?
Causes of congenital tethered cord
When a child has congenital tethered spinal cord syndrome—meaning he’s born with the condition—it’s due to a complicated biological process, beginning when the embryo is first growing in the mother’s uterus.
- The cells at the bottom of the embryo (caudal cell mass) play an important role in the development of the spinal cord and many other organs. These cells influence the development of the genital and rectal structures, bladder, lower kidneys, bones of the lower spine and the skin covering the lower back.
- As the embryo is forming, the cells in the caudal cell mass can experience some type of disruption or error.
- The error in these cells sets off a chain reaction of changes in the spinal cord and any or all of the other parts of the body influenced by these cells.
- This cause-and-effect process explains why some children with tethered spinal cord syndrome have unusual growths or markings on the skin of the lower back, while others may have abnormalities in the anus or genitals.
Certain congenital syndromes that affect a newborn's organ systems can also cause tethered cord. These syndromes include:
- VATER (vertebrae, anus, trachea, esophagus and radial and/or renal) syndrome, in which a baby is born with abnormalities in three or more of the above-referenced areas
- VACTERL (vertebrae, anus, cardiac, trachea, esophagus, radial and/or renal and limb) syndrome, in which a baby is born with anomalies in the heart and limbs as well as three or more organ systems affected by VATER syndrome. Learn how Children's treated one patient with VACTERL syndrome.
Causes of acquired tethered cord
Tethered spinal cord syndrome can also be an acquired condition, meaning it affects a child after her birth. An infant or older child may develop tethered cord because of:
- damage to the spine, spinal cord and/or spinal canal caused by another condition—most often, spina bifida
- past surgery on the spine that has caused scar tissue to form on the dura surrounding the spinal cord, weighing it down
- a condition called occult tight filum terminale, in which the long, thin bundle of nerve tissue joining the spinal cord to the backbone becomes thick or hardened and presses downward on the cord
- a tumor on the spinal cord
- a fatty mass of skin growing into the spinal cord
Signs and symptoms
What are the symptoms of tethered spinal cord syndrome?
Symptoms in newborns
Because the cells that form the spinal cord in utero also affect the development of many other structures in the lower body, a baby born with tethered spinal cord syndrome may have:
- an unusual dimple, raised lump, discoloration or patch of hair on the lower back
- abnormal anatomy of the genital organs
- abnormal anatomy of the anus
- one leg or foot that is noticeably longer or larger than the other
Symptoms in infants and older children
When a child develops tethered spinal cord syndrome later in infancy or childhood, warning signs may include:
- pain in the lower back
- weakness or numbness in the legs or feet
- problems standing or walking
- urinary incontinence
- fecal incontinence
Q: Will my child be OK?
A: Tethered spinal cord syndrome can affect different children in very different ways, depending on:
- the cause of the tethering
- how much of the surrounding tissues in the spine are affected
- whether any nerve damage has occurred before treatment
The good news is that tethered cord is a very treatable condition, especially when diagnosed and treated early in the child’s life. While surgery might not be able to restore neurological function that has already been lost, children with this disorder can go on to live full and healthy lives with proper care and follow-up.
Read more about children’s long-term outlook.
Q: Does Children’s offer testing for tethered spinal cord syndrome when a baby is still in the womb?
A: When a child’s tethered spinal cord is congenital (present at birth), prenatal ultrasound may be able to detect the disorder. Children’s provides prenatal ultrasonography through our Advanced Fetal Care Center.
Q: Can anything be done to ensure my child’s spine does not “re-tether” after surgery?
A: Unfortunately, while several techniques have been devised in the hopes of preventing or minimizing the risk of a child’s spinal cord becoming tethered again later in life, none have proven to be successful (and without associated complications) in long-term studies. Research continues into this important area.
Q: Will my child definitely need surgery?
A: Surgery is the only treatment that can actually “untether” the spinal cord, but the need for surgery will be based on careful discussion with a neurosurgeon.
Q: Can my child’s lost functions be restored through treatment?
A: Unfortunately, while surgery can untether the spinal cord, it cannot restore functions—including bowel and bladder control and sensation and mobility in the legs and feet—that may have already been impaired or lost. With rehabilitation and support, children with these complications can regain some function and go on to active and healthy adult lives.
Learn more about physical therapy and other support services at Children’s.
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Questions to ask your doctor
You and your family play an essential role in your child’s treatment for tethered spinal cord syndrome. It’s important that you share your observations and ideas with your child’s treating physician, and that you have all the information you need to fully understand the treatment team’s explanations and recommendations.
You’ve probably thought of many questions to ask about your child’s condition and prognosis. It’s often very helpful to jot down your thoughts and questions ahead of time and bring them with you, along with a notebook, to your child’s appointment. That way, you will have all of your questions in front of you when you meet with your child’s treating clinician and can make notes to take home with you. (If your child is old enough, you can encourage him or her to write down questions, too.)
Initial questions to ask your doctor might include:
- How did you arrive at this diagnosis?
- Are there any other conditions my child might have instead?
- What is the cause of my child's tethered spinal cord syndrome?
- Does my child require further testing or procedures?
- What is my child’s prognosis?
- What medications will you prescribe?
- What are the possible side effects of these medications?
- Will my child need surgery right away?
- What can you tell me about the surgical procedure you are recommending, and why are you recommending it?
- How should I talk to my child about this condition and the long-term outlook?
- How should I explain my child’s condition to others?
- Do I need to make any other changes to my child’s home and school routines?
- What other resources can you point me to for more information?