Tethered spinal cords: Understanding, Diagnosis, and Treatment

Our Team acts for a number of clients who have had a delay in diagnosing a tethered spinal cord (sometimes known as spina bifida occulta or a type of spinal dysraphism).

For many of these clients, there were signs that should have given rise to concern when they were first born or shortly after their birth. Those signs include a birthmark, a dimple, a tiny hole in the skin, a hairy patch or a combination of these over the affected area of the spine (often the sacrum).

Spina Bifida Occulta
Spina bifida occulta is a neural tube defect that occurs during the early stages of fetal development when the neural tube fails to close completely. It is a congenital (born with) condition where the spinal vertebrae do not fully close around the spinal cord, but the spinal cord itself is not exposed.

In many cases, there may be a small gap or opening in one or more vertebrae, without any noticeable symptoms or spinal cord abnormalities.

In spina bifida occulta, the spinal cord and the nerves typically remain inside the spinal canal, which distinguishes it from the more severe forms of spina bifida, such as myelomeningocele, where the spinal cord and surrounding structures protrude through an opening in the spine.

The term “occulta” means hidden, and in this condition, the spinal defect is often covered by a layer of skin. This is why many individuals with spina bifida occulta may not even be aware that they have the condition. It’s often discovered incidentally when undergoing imaging tests for unrelated medical issues.

Tethered spinal cord
A tethered spinal cord is different to spina bifida occulta but is often described as spina bifida occulta.

A tethered spinal cord is a serious condition. It occurs when the spinal cord is abnormally attached to the surrounding tissues, limiting its movement within the spinal canal. When the spinal cord is tethered, the movement of the cord is restricted and it experiences tension and stress, which can impede its proper functioning.

A tethered spinal cord can lead to a range of neurological symptoms and complications, making early diagnosis and treatment crucial. In some cases, clinical negligence can result in delayed or incorrect management of a tethered cord, leading to devastating consequences for patients.

The spinal cord can also attach due to various other reasons such as spinal deformities, scar tissue from previous surgeries, or spinal cord injuries.

Symptoms
The symptoms of a tethered spinal cord or spina bifida occulta can vary from person to person but often both include:

  • Back pain: Chronic and localised back pain is a common early symptom.
  • Neurological deficits: Patients may experience weakness, numbness, or tingling in their lower extremities
  • Bladder and bowel problems: leading to incontinence and difficulty with urination and defecation
  • Orthopaedic issues: Scoliosis and other spinal deformities may develop over time

In tethered spinal cords, the symptoms will progress if the cord is not untethered. For example, a person may have problems with incontinence, but they will not have a neurogenic bladder. If left untreated, worsening symptoms as progressive conditions can develop.

Diagnosis
Where there are skin markers on the sacrum (for example a birthmark, tuft of hair, or dimple) which are noticed at or shortly after birth, this is an indicator of a possible issue with the spinal cord. There should be a suspicion by clinicians to investigate.

A new-born baby should have a scan of their spine to make sure there is no problem. This is often an ultrasound scan because the spinal cord can be seen because the vertebrae don’t fuse until a few months after birth. If the scan takes place a bit later, when a baby is older than around 4 months of age, the vertebrae may have fused and it will often need to be an MRI to be able to see the spinal cord.

If a scan takes place and a tethered cord is identified, paediatricians should tell the parents and put in place regular reviews with the family, refer the baby to a urologist (to keep an eye on their bladder) and to a neurosurgeon so they can consider whether and when surgery to untether the cord may need to take place.

When a tethered spinal cord is not diagnosed early, it is often when symptoms develop that imaging of the spine takes place.

Early diagnosis and treatment are essential to prevent further complications and improve the patient’s quality of life.

Treatment options
When someone has developed symptoms because of their spinal cord being tethered, surgical treatment cannot reverse any damage that has been caused, it can only prevent further deterioration.
Treatment options include:

  • Surgical intervention: Surgical untethering of the cord may be considered to address the specific problem. Surgery will not stop the symptoms of the tethered cord but will often (but not always) stop them from getting worse and it will prevent any further symptoms developing.
  • Bladder or bowel dysfunction: In cases where bladder or bowel dysfunction occurs, a healthcare provider may recommend therapies or medications to manage these symptoms
  • Therapies: If an individual experiences neurological symptoms like weakness or tingling in the legs, physiotherapy or exercises to strengthen the muscles and improve mobility may be prescribed. Occupational therapy can help to them to access activities of daily living
  • Monitoring: Regular check-ups and monitoring are essential to detect and manage any potential issues that may develop over time
  • Pain management: Pain relief or physiotherapy may be recommended to help with pain or discomfort.

There are some types of tethered cord that are more complicated to address surgically, for example, if the spinal cord is tethered into a lipoma (fatty lump). In these cases, the advice is often to wait and watch for further deterioration because the surgery has higher risks than in other cases. Cords that are tethered because they are low lying, tight or thick are easier to untether and neurosurgery to untether the cord is usually recommended immediately after further investigations and a surgical ‘work up’.

There are occasions where a sacral dimple or a tiny hole in the sacrum has a tract (a channel) which leads direct to the spinal cord. In these cases, it is really important to operate quickly because they can be channel for infection to get into the spinal cord which can travel up the spine causing abscesses or into the brain resulting in a risk of meningitis.

Clinical negligence in tethered spinal cord/ spina bifida occulta cases
This can occur when healthcare professionals fail to meet the standard of care expected in their field. It can happen in several ways:

  • Misdiagnosis or delayed diagnosis: Failure to recognise the symptoms of a tethered spinal cord or spina bifida occulta or thinking they relate to other conditions can result in a delay in diagnosis, allowing the symptoms to worsen
  • Incorrect treatment: Administering inappropriate treatments or surgeries that do not address the tethered spinal cord can exacerbate the condition.
  • Surgical errors: During surgery to release the tethered cord, errors can occur, leading to complications such as infection or further damage to the spinal cord.

Consequences of Clinical Negligence
A delay in diagnosis or misdiagnosis can lead to severe complications that can be devasting and life changing. Patients may experience a significant impact on their quality of life, including worsening neurological deficits, increased pain, and permanent disabilities.

Investigating whether mistakes were made, that could have been avoided, can provide answers.

We know that money can never make things as they were or should have been, but compensation can provide the equipment, housing, care and support and therapies to help those with tethered cords live the best life they can.

We work closely with Shine (Spina Bifida & Hydrocephalus Charity), to support their members who think that something may have gone wrong with their care.

Shine also have helpful information on their website about tethered cords

For further information about Claims arising from Spina Bifida or hydrocephalus please click here 

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