SPLIT SPINAL CORD MALFORMATION

Split spinal cord malformation (SSCM) is a rare form of spinal dysraphism in which a mother gives birth to a baby with this splitting, or duplicated spinal cord. It may be characterized by complete or incomplete division of the spinal cord, resulting in two ‘hemicords.

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Split Spinal Cord Malformation

Split Spinal Cord Malformation, also known as Diastematomyelia, is a rare congenital condition that affects the spinal cord. The condition describes a spinal cord with two halves — separated by a bony or fibrous septum. This can cause a range of neurological symptoms, which may vary depending on the severity and location of the split.

Causes and Types of Split Spinal Cord Malformation

split spinal cord malformation The causes of SSCM are not fully understood, but there are several theories as to why it occurs. One theory suggests that SSCM is caused by a disruption in the development of the neural tube. This tube forms the brain and spinal cord during fetal development. Some believe the disruption could form from genetic mutations, environmental factors, or a combination of both. Another theory proposes that SSCM occurs because of abnormal vascular development. Therefore, leading to the formation of an abnormal blood vessel that compresses and splits the spinal cord.

Researchers have identified several genetic mutations that may increase the risk of SSCM. These include mutations in the genes that regulate neural tube development. Additionally, environmental factors such as maternal infections, exposure to certain toxins, and nutritional deficiencies have been linked to an increased risk of SSCM. It is important to note that SSCM is a congenital condition, which means it is present from birth. However, symptoms may not become apparent until later in life, depending on the severity and location of the split. Overall, the causes of SSCM are complex and multifactorial. Further research needs to fully understand the underlying mechanisms of this condition.

Types

There are two main types of SSCM: Type I and Type II.

Type I SSCM, also known as Diastematomyelia, occurs when the spinal cord gets split into two separate halves by a bony or fibrous septum. This septum can cause pressure on the spinal cord and can lead to neurological symptoms.
Type II SSCM, also known as Diplomyelia, occurs when the spinal cord gets split into two separate halves, but with no septum between them. Instead, each half of the spinal cord becomes surrounded by its dural sac, which can cause pressure on the spinal cord and lead to neurological symptoms.

In addition to these two main types, there are several subtypes of SSCM that have been identified. The identity of the subtypes is based on the location and severity of the split. These subtypes include dorsal, ventral, and lateral split malformations, as well as complete and incomplete forms of the condition.

QUESTIONS AND ANSWERS

What is Diastematomyelia, and how did my child develop this condition?

Diastematomyelia is a congenital disorder characterized by the presence of a cleft or split in the spinal cord. This split can be associated with a bony spur or septum. The exact cause of Diastematomyelia is not always clear, but it is believed to result from abnormal embryonic development during the early stages of pregnancy. Genetic and environmental factors may contribute. The condition is typically present from birth and may be detected during infancy or childhood.

What are the treatment options for Diastematomyelia, and what is the prognosis for my child?

The treatment for Diastematomyelia depends on the severity of the condition and the presence of associated abnormalities. In some cases, surgical intervention may be recommended to address the split in the spinal cord and correct any associated issues. The prognosis varies depending on factors such as the extent of the malformation and the success of surgical interventions.

With appropriate medical care and management, many individuals with Diastematomyelia can lead relatively normal lives. However, the outcome can vary, and the healthcare team will provide more specific information based on the child’s individual case.

What kind of follow-up care and support does my child need after treatment?

After treatment, regular follow-up appointments with healthcare providers are essential to monitor the child’s development, assess the success of surgical interventions, and address any emerging issues. Physical therapy and rehabilitation may be recommended to support the child’s mobility and overall function. The medical team will work closely with the parents to create an individualized care plan for their child. Ongoing communication with healthcare professionals is crucial to address any concerns and ensure the child’s ongoing well-being.

Parents should seek guidance and information directly from their child’s healthcare team, as the specifics of Diastematomyelia can vary widely among individuals. The medical professionals involved in the child’s care will provide personalized advice, treatment plans, and support based on the child’s unique circumstances.

Schedule an appointment and see a pediatric orthopedist quickly at Medical City Children’s Orthopedics and Spine Specialists.

How Common Is Split Spinal Cord Malformation?

Although the name sounds like something you’d only find in a med school textbook, split spinal cord malformation (SSCM) is, in fact, rather rare. Epidemiological studies show it’s found more often in females, with a ratio leaning about 1.5 females to every male diagnosed.

Most cases are identified in childhood, often thanks to those telltale signs such as tethered cord syndrome or other spinal anomalies like scoliosis and myelomeningocele. However, it’s not unheard of for SSCM to fly under the radar until adulthood, sometimes even popping up in seniors after decades without obvious symptoms.

The advent of early MRI screening truly changed the game, making it easier for doctors to spot this condition in younger patients, sometimes even before symptoms cause any trouble. When looking under the hood, specialists have noted that Type I SSCM is the main culprit, representing roughly two-thirds of all reported cases.

Early diagnosis is more than just a box to check—it’s key for timely intervention and the best possible outcome.

Symptoms of Split Spinal Cord Malformation

The symptoms of Split Spinal Cord Malformation (SSCM) can vary depending on the severity and location of the split. In some cases, the patient may exhibit no symptoms at all. However, in other cases, the condition can cause a range of neurological problems.

Common symptoms of SSCM may include:

Back pain: People with SSCM may experience chronic back pain due to pressure on the spinal cord.
Muscle weakness: Depending on the location of the split, people with SSCM may experience muscle weakness in the legs or arms.
Sensory changes: SSCM can cause sensory changes, such as numbness or tingling, in the affected areas of the body.
Bladder and bowel problems: SSCM can affect bladder and bowel function, leading to incontinence or difficulty with urination and bowel movements.
Scoliosis: Some people with SSCM may develop scoliosis, a curvature of the spine.
Foot deformities: In some cases, SSCM can cause foot deformities, such as clubfoot or other abnormalities.
Hydrocephalus: In rare cases, SSCM exists with hydrocephalus, a condition where there is an accumulation of fluid in the brain.

The severity of symptoms can vary between severe and none at all for individuals with SSCM. If you or a loved one is experiencing any of these symptoms, it is important to seek medical attention. Expert doctors like those at Medical City Children’s Orthopedics and Spine Specialists will test the patient to confirm SSCM and immediately devise a treatment plan.

Significantly, approximately 3 percent of individuals with progressive curvature may eventually experience severe complications such as worsening scoliosis, chronic back pain, or even nerve compression leading to numbness, weakness, and leg pain. This underscores the importance of prompt diagnosis and ongoing management.

Diagnosis of Split Spinal Cord Malformation

To diagnose Split Spinal Cord Malformation (SSCM), doctors may perform a variety of tests and exams. The diagnosis will evaluate the patient’s symptoms and determine the location and severity of the split in the spinal cord.

Some of the diagnostic tests that diagnose SSCM include:

Magnetic resonance imaging (MRI): This imaging test uses powerful magnets and radio waves to create detailed images of the spinal cord and surrounding tissues. An MRI can help identify the location and extent of the split in the spinal cord.
Computed tomography (CT) scan: A CT scan can provide detailed images of the bones and soft tissues of the spine. The scan can help identify any abnormalities or malformations.
Electromyography (EMG): This test measures the electrical activity of muscles. This test can help determine if there is any nerve damage or muscle weakness in the affected area.
Spinal tap (lumbar puncture): A spinal tap can help rule out other conditions, such as infection or inflammation, that may cause the patient’s symptoms.
Neurological exam: A doctor may perform a neurological exam to assess the patient’s reflexes, muscle strength, and sensation in the affected areas.

In some cases, doctors can diagnose SSCM during a prenatal ultrasound or other imaging studies before birth. However, in other cases, doctors cannot diagnose the condition until later in life when symptoms appear.

It is important to seek medical attention if your child has any symptoms of SSCM. An accurate diagnosis is critical for developing an effective treatment plan.

Surgical and Non-Surgical Treatments for Split Spinal Cord Malformation

Doctors often treat Split Spinal Cord Malformation using a mix of surgical and non-surgical methods. Treatment depends on severity and location. Physical therapy may improve strength and mobility. Pain relief options include medications or nerve blocks. Surgery may be required when patients develop significant neurological symptoms or complications like syringomyelia. The type of surgery depends on the split’s location and extent. Doctors also consider the patient’s general health.

Non-Surgical Treatments

Doctors often recommend conservative care when symptoms are mild and don’t affect daily life. Observation and regular checkups help monitor changes. Physical therapy strengthens muscles, improves flexibility, and enhances daily function. Braces may support spinal stability and correct deformities. Some patients benefit from pain medications or nerve blocks. Others may only need consistent monitoring and periodic imaging.

Surgery Considerations for Asymptomatic Patients

Families often ask if patients without symptoms should have surgery. There’s no universal answer. Some doctors recommend early surgery to prevent future issues. This often applies when doctors discover the condition before other spinal procedures. Others prefer monitoring until symptoms appear. Surgery carries risks, including spinal cord re-tethering from scar tissue. Doctors weigh benefits and risks for each case. They consider age, spinal anatomy, other health issues, and planned surgeries.

Surgical Treatment and Its Benefits

Doctors may recommend surgery if non-surgical methods fail or symptoms worsen. Some surgeries remove the septum dividing the spinal cord. Others fuse both spinal halves to improve function. Doctors base decisions on symptoms, location, and general health. After surgery, teams manage orthopedic issues like scoliosis or rigid ankles. They also treat neurogenic bladder problems to avoid complications. Urodynamic tests help assess bladder function and guide care. Surgery helps prevent symptom progression and improves quality of life. Regular follow-ups adjust treatment plans as needed.

Surgical Procedures

Surgeons select the best technique based on patient needs:
Detethering Surgery: Surgeons release spinal cord tension by removing abnormal attachments.
Spinal Fusion: This joins vertebrae to stabilize and align the spine. Surgeons may use rods or screws.
Tumor Excision: Surgeons remove tumors near the split to relieve pressure on the spinal cord.
Sometimes, surgeons remove the fibrous or bony septum. Other times, they fuse the spinal halves for better function.

Goals of Treatment

Doctors aim to relieve symptoms, stop neurological decline, and improve life quality. Each plan fits the patient’s specific condition. Factors include age, general health, and malformation type. Teams may use surgery, non-surgical care, or both.

Benefits of Surgical Treatment

Surgery offers important benefits. Detethering reduces spinal cord tension and prevents complications. Fusion adds stability and aligns the spine. Surgery may ease pain and increase independence. Many patients report better mobility and less discomfort. Some also feel more emotionally stable and independent. Early treatment improves both short- and long-term outcomes.

What to Expect from Surgery

Most patients improve or stabilize after surgery. Detethering and structural correction ease symptoms. Results vary depending on spinal complexity. Additional spinal issues may require more procedures later. A collaborative team approach ensures the best care plan. Doctors evaluate anatomy, risks, and the potential benefits of surgery.

Recovery

Surgery, like any medical procedure, carries potential risks and complications that patients and doctors must carefully consider. Some patients experience neurologic changes caused by scar tissue or re-tethering, which may require future surgical intervention. Doctors decide whether to operate based on individual risks and benefits, especially when the patient has no symptoms.

Recovery varies depending on the procedure and the patient’s response to treatment. Postoperative care includes pain management, physical therapy, and regular follow-up visits to monitor healing and progress. Physical therapy helps patients rebuild strength and regain functional movement after surgery. Recovery can last several weeks or months, depending on the condition’s complexity and the treatment used.

Doctors combine surgical and non-surgical treatments to create customized plans that support each patient’s unique needs and recovery goals.

After surgery, children must attend regular follow-up visits to track development and identify any new or ongoing medical concerns. These appointments help evaluate surgical outcomes and address issues as they arise. Physical therapy and rehabilitation often support mobility and functional improvements in children recovering from surgery. Physical therapists guide patients through strength-building and flexibility exercises to restore movement. The recovery timeline usually ranges from a few weeks to several months, depending on the child’s condition and progress.

The medical team collaborates with parents to design a personalized care plan tailored to the child’s specific needs. Open communication with healthcare providers ensures quick responses to concerns and supports the child’s ongoing well-being. Regular follow-ups allow for timely therapy adjustments and medical changes, helping to achieve the best possible outcome.

Possible Complications After Surgery for Split Spinal Cord Malformation

Like any surgical procedure, surgery for Split Spinal Cord Malformation carries potential risks and complications. While many patients experience improvement after their procedure, it is important to be aware of what can occur post-operatively.

Some possible complications include:

Infection or Meningitis: Any surgery involving the spine and nervous system can increase the risk of infections, including meningitis, which requires prompt medical attention.
Cerebrospinal Fluid (CSF) Leak: Occasionally, surgery can lead to leakage of the fluid surrounding the spinal cord, which might need further intervention to repair.
Neurological Changes: Some individuals may experience worsening of neurological symptoms in the immediate period after surgery, such as increased weakness, numbness, or changes in sensation.
Retethering of the Spinal Cord: Over time, scar tissue may form at the surgical site, which can cause the spinal cord to “retether” or reattach abnormally. This can lead to new or recurring neurological problems and may necessitate additional interventions.

Discussing these potential outcomes with your medical team can help set realistic expectations and allow for early recognition should complications arise. Regular follow-up appointments are key to monitoring progress and addressing any concerns quickly.

Timing of Surgery and Surgical Techniques

The timing of surgical intervention for Split Spinal Cord Malformation (SSCM) can significantly influence outcomes. Generally, earlier surgery—ideally before serious neurological symptoms develop—provides the best chances for preserving nerve function and preventing further complications. In fact, when SSCM is diagnosed at a young age or soon after symptoms appear, early treatment may help minimize long-term issues with mobility, sensation, or bladder and bowel function.

Surgical techniques continue to advance. Some procedures are now performed using minimally invasive methods, such as endoscopic surgery, which can reduce complications and often leads to a quicker recovery. During these surgeries, doctors may use intraoperative neurophysiological monitoring to keep a close eye on nerve function—a step that adds a layer of safety and reduces the risk of nerve damage.

Making decisions about the timing and method of surgery is a collaborative process between the patient, family, and a specialized medical team. Each plan is tailored, taking into account the patient’s age, overall health, and the specific characteristics of the spinal cord split.

Prevention Measures and Recovery Time for Split Spinal Cord Malformation

As Split Spinal Cord Malformation is a congenital condition, there are no specific preventive measures that can avoid the condition. The recovery time for Split Spinal Cord Malformation will depend on the severity of the condition and the type of treatment received. In cases where non-surgical treatments are effective, individuals may resume normal activities relatively quickly. However, in cases where surgery is required, recovery time takes longer and may involve a period of physical therapy to regain strength and mobility.

In general, individuals with Split Spinal Cord Malformation may require ongoing monitoring and management of their symptoms, as the condition can cause progressive neurological complications over time.

Why Choose the Doctors at Medical City Children’s Orthopedics and Spine Specialists

When caring for a child with split spinal cord malformation (SSCM), having a team of specialists makes a significant difference in both safety and outcomes. Since every child’s anatomy is unique and SSCM can present with a variety of symptoms and complications, a multidisciplinary team—typically comprised of pediatric neurosurgeons, orthopedic surgeons, anesthesiologists, radiologists, and rehabilitation specialists, works closely to tailor care to your child’s specific needs.

This collaborative approach ensures a thorough evaluation before any procedure. For instance, anesthetic plans are customized based on a child’s spinal cord anatomy to minimize risk, with alternatives like general anesthesia or peripheral nerve blocks considered when standard neuraxial techniques could be unsafe. Surgeons and radiologists review imaging together to fully understand the malformation, allowing for precision in surgery and careful planning to avoid preventable complications.

What factors should patients consider when choosing a treatment center for split spinal cord malformation?

Selecting the right treatment center for split spinal cord malformation is an important step in ensuring your child receives the best possible care. When weighing your options, there are several key factors to keep in mind:

Specialized Expertise: Look for centers with a dedicated team of pediatric neurological and orthopedic specialists, such as those commonly found at leading children’s hospitals or academic medical centers. Experience with complex spinal conditions, including Diastematomyelia and related disorders, is essential.
Comprehensive Care: The most effective centers offer a full range of diagnostic services, such as advanced imaging (MRI, CT scans), as well as a broad spectrum of surgical and non-surgical treatment options. This allows for a tailored approach based on your child’s specific needs.
Multidisciplinary Collaboration: Ideally, the care team should include neurologists, orthopedic surgeons, physical therapists, and other allied health professionals who work together to address all aspects of diagnosis, treatment, and rehabilitation.
Patient-Centered Philosophy: Excellent centers prioritize individualized care plans, taking the time to understand your child’s unique medical history, challenges, and personal goals.
Use of Advanced Technology: State-of-the-art surgical techniques and the latest technology can lead to more accurate diagnoses, safer surgeries, and improved recovery outcomes.

In addition to these factors, parents may also want to consider the center’s reputation, involvement in research, access to support services, and the willingness of staff to provide thorough explanations and ongoing support. Seeking care at established pediatric medical practices like Medical City Children’s Orthopedics and Spine Specialists can also provide peace of mind that your child is in capable hands.

Conclusion

Finally, our doctors at Medical City Children’s Orthopedics and Spine Specialists, with offices in Arlington, Dallas, Flower Mound, Frisco, and McKinney, TX, understand the importance of maintaining your child’s health. In addition, our experts and specialists have the training, knowledge, and experience required to take care of a split spinal cord malformation. If your child is born with this condition, don’t hesitate to call our office at 214-556-0590 to schedule an appointment at one of our five locations.

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Footnote:

National Organization of Rare Diseases: Split Spinal Cord Malformation

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