Tethered Spinal Cord Syndrome

What is Tethered Spinal Cord Syndrome (“TSCS”)?

Tethered spinal cord syndrome (TSCS) is a neurological condition that typically occurs in children where the spinal cord is abnormally attached or "tethered" to surrounding tissues. Normally, the spinal cord is free to move within the spinal canal as a child grows. However, in cases of tethered spinal cord syndrome, the spinal cord becomes anchored or pulled down, restricting its movement, as the child’s height grows.

What are the Types of TSCS in Children?

TSCS in children can be categorized into different types based on the underlying causes and anatomical features. Here are some of the types of TSCS that can occur in children:

Occult Tethered Cord Syndrome: In this type, there may not be any obvious external signs of tethering, but the spinal cord is still abnormally attached or restricted in its movement. This can be due to an abnormal filum terminale or other subtle structural issues.
Tethered Cord Syndrome due to Spina Bifida: Children with spina bifida, a congenital condition where the spine doesn't form properly, can develop TSCS as a result of the underlying spinal abnormalities.
Tethered Cord Syndrome due to Lipomas: Fatty tumors called lipomas can develop in or around the spinal cord. These tumors can cause tethering and compression of the spinal cord.
Postoperative Tethered Cord Syndrome: Some children can develop tethering of the spinal cord as a complication of spinal surgeries, particularly if surgery involved an intradural approach.
Tethered Cord Syndrome in Association with Spinal Boney Anomalies: TSCS can also be associated with scoliosis or abnormal bony developments, as seen in diastematomyelia (a condition where the spinal cord is split), and others.
Tethered Cord Syndrome with Caudal Regression Syndrome: Caudal regression syndrome involves underdevelopment of the lower spine and spinal cord. It can be associated with tethered cord syndrome.
Tethered Cord Syndrome with Anorectal Malformations: Some children with anorectal malformations may also have a tethered spinal cord as part of their condition.

What Might Cause TSCS in Children?

Tethered Spinal Cord Syndrome (TSCS) in children is typically caused by an abnormal attachment or fixation of the spinal cord, which prevents its normal movement within the spinal canal. Several underlying factors can contribute to the development of TSCS in children:

Tethered Filum Terminale: One of the most common causes of TSCS is an abnormality in the filum terminale, a thin thread-like structure that normally anchors the spinal cord within the spinal canal. In children with TSCS, the filum terminale may be abnormally thickened, elongated, or attached to surrounding tissues, restricting the movement of the spinal cord.
Spinal Cord Lipomas: Fatty tumors known as lipomas can develop in or around the spinal cord. These tumors can lead to tethering by exerting pressure on the spinal cord or attaching to it.
Spina Bifida: Children born with spina bifida, a congenital condition where there is an incomplete closure of the spinal neuronal structures, are at a higher risk of developing TSCS. The malformation associated with spina bifida can cause the spinal cord to become tethered.
Spinal Abnormalities: Other structural abnormalities of the spine, such as diastematomyelia (split spinal cord), scoliosis (curvature of the spine), or other congenital malformations, can contribute to TSCS.
Scar Tissue Formation: Previous spinal surgeries or traumatic injuries to the spinal cord can result in the formation of scar tissue, which may attach to the spinal cord and cause tethering.

What Symptoms Might TSCS Cause in Children?

Tethered Spinal Cord Syndrome (TSCS) in children can manifest with a wide range of neurological, orthopedic, and urological symptoms. The severity of symptoms can vary depending on the degree of tethering and the underlying cause. Some common symptoms of TSCS in children include:

Back Pain: Children may experience lower back pain, especially in the area of the spine where the tethering is occurring.
Leg Weakness or Numbness: TSCS can affect the nerve signals traveling along the spinal cord, leading to weakness, numbness, or tingling sensations in the legs.
Foot Deformities: Children with TSCS may develop deformities in the feet, such as clubfoot or other abnormalities in foot position.
Changes in Bladder and Bowel Function: TSCS can impact the nerves controlling the bladder and bowel, leading to issues such as urinary or fecal incontinence, retention, or other bladder and bowel control problems.
Orthopedic Problems: TSCS can cause abnormal alignment of the spine, leading to issues like worsening scoliosis (curvature of the spine) or other orthopedic problems.
Changes in Gait: Children may exhibit changes in their walking pattern (gait) due to the impact of TSCS on lower limb function.
Skin Abnormalities: Skin changes, such as dimples, hairy patches, or birthmarks near the spinal cord area, can sometimes be indicative of TSCS.
Sensory Changes: Children may experience changes in sensation, including altered perception of touch, pain, or temperature in the lower back, buttocks, or legs.
Neurological Symptoms: TSCS can lead to various neurological symptoms, including muscle weakness, spasms, and difficulty with fine motor skills.
Pain or Discomfort: Children with TSCS may experience pain, discomfort, or a feeling of "pulling" in the lower back or legs.
Tethered Cord Symptoms Following Growth Spurts: Symptoms of TSCS may become more pronounced during periods of rapid growth, as the spinal cord's tethering becomes more restrictive.

Tethered Spinal Cord Syndrome Causing Sensory Changes in a Child

Tethered Spinal Cord Syndrome Causing Back Pain in a Child

Tethered Spinal Cord Syndrome Causing Scoliosis in a Child

How is TSCS Treated in Children?

Once your child’s has been diagnosed with TSCS, I (as a pediatric neurosurgeon in New York) will work with you, your child, and other medical professionals (e.g., neurologists and orthopedic surgeons) at Children's Hospital at Montefiore (CHAM) to formulate a customized treatment strategy tailored to your child’s needs. This customized treatment typically involves surgical intervention to release the tethered spinal cord and alleviate the pressure on the affected nerves, but may utilize any one or a combination of the following interventions:

Surgery: Surgery is the primary treatment for TSCS when symptoms are significant or worsening. The goal of surgery is to untether the spinal cord by removing any tissue or structures causing the tethering. The surgical procedure may involve the following approaches:
- Spinal Cord Detethering: In more complex cases, a more extensive surgery may be required to address structural abnormalities of the spine and to release the tethered spinal Filum Terminale Release: If the filum terminale is the primary cause of tethering, the surgeon may perform a procedure to release or divide the filum terminale, allowing the spinal cord to move freely within the spinal canal.
- Tumor or Lipoma Removal: If a lipoma or other tumor is causing the tethering, surgical removal of the tumor may be necessary to relieve pressure on the spinal cord.
- Spinal Fusion or Stabilization: If there are associated spinal deformities (such as scoliosis), spinal fusion or stabilization may be performed to correct the deformity and prevent further issues.
- Neurological Monitoring: During surgery, neurophysiological monitoring may be used to monitor the function of the spinal cord and nerves in real time, helping to reduce the risk of neurological complications.
Postoperative Care and Rehabilitation: After surgery, children will require a period of recovery and rehabilitation. Physical therapy and other rehabilitative measures may be recommended to help restore strength, mobility, and function.
Long-Term Follow-Up: Children who have undergone surgery for TSCS will need regular follow-up appointments with their medical team to monitor their progress and address any potential complications or recurrent symptoms.

Dr. Behbahani operating on child with TSCS