SPLIT SPINAL CORD MALFORMATION

Epidemiological Research

Epidemiological research into split cord malformations indicates that this condition is quite rare. Studies have found a male-to-female ratio of 1:1.5, highlighting a slightly higher prevalence among females. While many individuals are diagnosed in childhood, often due to symptoms such as tethered cord syndrome or congenital malformations like scoliosis and myelomeningocele, this condition can also present later in life, even in the elderly.
The widespread use of early MRI imaging has increased the detection rate of these malformations at a younger age. Among the types of split cord malformations, Type I is more prevalent, comprising approximately two-thirds of reported cases. This data underscores the importance of early diagnosis and intervention to manage associated complications effectively.
Symptoms:
The symptoms of split spinal cord malformation can vary widely, and their severity depends on the extent of the malformation. Common symptoms include:

• Back Pain: Pain in the back or spine.
• Neurological Symptoms: Such as muscle weakness, numbness, or tingling in the lower extremities.
• Orthopedic Issues: Deformities of the spine, such as scoliosis or kyphosis.
• Bladder and Bowel Dysfunction: In some cases, problems with urinary or bowel control may occur.

Preventative Measures

When considering preventive measures for split cord malformations, folic acid supplementation emerges as a key strategy. Taking folic acid can lower the risk of myelomeningocele, a condition often linked with split cord malformations. Currently, aside from folic acid, there are no additional specific preventive measures known for these malformations.
Diagnosis:

Diagnosing Split Spinal Cord Malformation

Diagnosing split spinal cord malformation involves a comprehensive approach that combines the following techniques:

• Medical History: Physicians often conduct a detailed assessment of the patient’s symptoms and medical history.
• Physical Examination: A thorough examination can reveal neurological deficits or physical signs that suggest split cord malformations.
• Imaging Studies:
  • MRI: This is a primary screening tool to visualize the spinal cord, identify the presence and extent of any split cord malformation, and detect associated lesions such as a tethered or low-lying cord. It can also reveal non-adjacent abnormalities like syringohydromyelia and Chiari type 1 malformation.
  • CT Scans: CT myelography or 3-D spiral CT scans complement MRI by providing superior detail of the bony structures. They help classify split cord lesions into types I and II, which is critical for surgical planning. CT is particularly effective in delineating the size, shape, and configuration of bony septa and other vertebral abnormalities.
• Prenatal Ultrasound: Split cord malformations can be diagnosed prenatally, typically in the second and third trimesters, by identifying an extra echogenic focus in the midline of the spine.
• Electromyography (EMG) and Nerve Conduction Studies: These tests assess nerve function and help identify any abnormalities impacting muscle activity and sensory nerves.
• Urodynamic Studies: These are vital for evaluating bladder function, as many patients with split cord malformations experience neurologic symptoms like incontinence or recurrent urinary tract infections. Urodynamic testing can reveal neuropathic bladder changes that might not correlate with a standard neurological exam, guiding decisions on surgical intervention.
• Somatosensory Evoked Potentials: Although less commonly used, this test may monitor asymptomatic patients, providing early warnings of changes in nerve conduction before clinical symptoms appear.

This holistic diagnostic approach ensures a thorough understanding of the condition, guiding effective treatment planning.

Treatment Methods:

The treatment of split spinal cord malformation is typically individualized based on the severity of symptoms and associated issues. Treatment may include:

• Observation: In cases where symptoms appear mild and do not cause significant impairment, doctors may implement a watch-and-wait approach.
• Physical Therapy: Exercises to improve muscle strength, flexibility, and overall function.
• Orthopedic Management: Doctors may recommend bracing or other orthopedic interventions to address spinal deformities.
• Surgery: In severe cases with significant symptoms or complications, doctors may recommend surgical intervention.

Treatment for SSCM depends on the severity of symptoms and associated complications. In some cases, conservative management, such as physical therapy, pain management, and monitoring for potential issues, can work well. However, doctors may recommend surgical intervention in cases where there is a risk of worsening neurological function or if there are associated abnormalities that require correction. The goal of surgery is to address the anatomical abnormalities and improve overall spinal cord function.

Surgical Considerations

For those facing surgery, the procedure typically involves untethering the spinal cord by removing bony or cartilaginous spurs and any tethering lesions. This includes resecting the midline bony structures and addressing other lesions like thickened filums or intradural lipomas, which can tether the spinal cord. The surgical approach aims not only to alleviate current symptoms but also to prevent future complications, such as re-tethering due to scar tissue.

Timing and Techniques

The timing of surgery can be crucial. Data suggests that outcomes are better when surgery is performed earlier, ideally before significant neurological symptoms develop. In some cases, endoscopic surgery is considered to minimize complications and improve recovery times. Additionally, intraoperative neurophysiological monitoring is often advised during these complex procedures to prevent potential complications such as nerve damage.

Associated Conditions

Patients with SSCM often have other spinal issues, like scoliosis, which may require simultaneous management. Some experts advocate for addressing split cord malformations before scoliosis surgery to improve outcomes. For individuals with congenital scoliosis and SSCM, recent studies indicate that spinal deformity correction can be safely performed without additional neurologic interventions if the patient maintains stable neurological status.

Postoperative Management

After surgery, a multidisciplinary approach is essential. Management may include ongoing treatment for orthopedic deformities and neurogenic bladder issues to prevent long-term complications. Post-operative urodynamics can be useful for establishing baselines in bladder function. While surgery is often prophylactic, addressing these associated conditions is vital for overall patient health and quality of life.

In summary, while conservative management may suffice for some, surgical intervention remains a cornerstone of SSCM treatment, especially for those at risk of neurological deterioration. Each case requires careful evaluation to tailor the management strategy to the individual’s needs.

Surgical Procedures:

There are several surgical procedures aimed at addressing the complexities of split spinal cord malformation.

• Detethering Surgery: One common surgical procedure is detethering surgery, which aims to release any abnormal attachments of the spinal cord to surrounding tissues. This procedure is crucial when a tethered spinal cord is present, as it helps alleviate tension and prevent further complications.
• Spinal Fusion: Doctors use spinal fusion as another surgical option. This procedure involves joining two or more vertebrae together, stabilizing the spine, and preventing further misalignment. Spinal instrumentation, such as rods, screws, or other devices, will support and maintain spinal alignment. In cases of associated scoliosis or other spinal deformities, doctors may use spinal fusion to stabilize the spine.
• Tumor Excision: In cases where associated tumors appear present, doctors may use surgical excision to remove them. This not only addresses the tumor but also helps alleviate pressure on the spinal cord.

Comprehensive Surgical Considerations

Surgical management of split cord malformations involves more than just the procedures themselves. Decisions about surgery must consider patient-specific factors like age, neurological symptoms, and the presence of associated conditions. Early identification is particularly vital for high-risk individuals, such as those with external cutaneous manifestations or congenital spinal deformities.

Risks and Challenges:

• The decision to operate is a balance of the surgeon’s skill and the risk of complications such as meningitis, CSF leaks, and potential worsening of neurological symptoms immediately after surgery.
• Retethering due to scar tissue presents a long-term risk, potentially leading to late neurological deterioration.

Associated Conditions:

• Split cord malformations may coexist with other tethering lesions, such as thickened filums, dermoid, or intradural lipomas, which must be addressed to relieve spinal cord tension effectively.
• Myelomeningocele manque, an occult condition contributing to cord tethering, often necessitates repair.

Advanced Techniques and Monitoring

• Endoscopic Surgery: The use of endoscopic techniques can reduce complications and improve the benefit-to-risk ratio for surgical repair. These minimally invasive approaches may enhance recovery and outcomes.
• Intraoperative Monitoring: In high-risk surgeries, intraoperative neurophysiological monitoring is essential. It can prevent severe complications, such as paraplegia, by detecting changes in neurological function during the procedure.

Postoperative and Long-term Management

After surgery, patients often require ongoing management for orthopedic deformities, like rigid ankle deformities and scoliosis, which may continue to progress. Neurogenic bladder conditions also demand aggressive management to prevent renal complications, involving treatments like clean intermittent catheterization and anticholinergic medications.

A comprehensive, multidisciplinary approach is necessary both before and after surgery to ensure optimal outcomes. This includes close monitoring of the patient’s neurological and physiological status, with regular follow-ups to adjust care as needed.

Anesthesia Considerations for Patients with Spinal Cord Malformations

When administering anesthesia to patients with spinal cord malformations, several key considerations must be taken into account to ensure safety and efficacy:

Assessment of Vertebral Anatomy:

• Malformations can lead to abnormalities in the vertebral column.
• The presence of vertebral anomalies may impact the availability and integrity of the epidural space.

Challenges in Needle Placement:

• Anomalies can complicate access. In some cases, the epidural space might be absent or significantly altered.
• Careful imaging techniques, such as MRI or fluoroscopy, may be necessary to guide needle insertion and avoid complications.

Customized Anesthetic Approach:

• Each patient requires a tailored anesthetic plan based on their specific anatomy.
• Alternatives to neuraxial anesthesia, such as general anesthesia or peripheral nerve blocks, may be considered if direct access is problematic.

Collaborative Multidisciplinary Planning:

• Close collaboration with neurosurgeons, radiologists, and anesthesiologists is crucial.
• An interdisciplinary approach ensures comprehensive evaluation and reduces the risk of intraoperative complications.

By addressing these factors, practitioners can navigate the complexities associated with spinal cord malformations efficiently, thus minimizing risks for the patient.

Non-Surgical Treatment:

In cases where symptoms appear mild and do not cause significant impairment, our doctors will first take a conservative approach. This can involve close observation and monitoring of the condition. Physical therapy plays a pivotal role in managing split spinal cord malformation. Tailored exercises will improve muscle strength, flexibility, and overall functional ability. Orthopedic management, including the use of braces or other interventions, works well to address spinal deformities and provide support.

Benefits of Surgical Treatment:

Surgical treatment for split spinal cord malformation offers several benefits, including the potential for improved neurological function, stabilization of the spine, and prevention of further deformities. Detethering surgery can release tension in the spinal cord, reducing the risk of complications such as neurological deficits. In addition, spinal fusion and instrumentation provide structural support, contributing to enhanced spinal stability and alignment.

Additionally, surgical intervention may alleviate pain and improve the patient’s overall quality of life. Furthermore, the benefits extend beyond the physical realm, positively impacting psychological well-being and functional independence.

Typical Surgical Outcomes

Most patients undergoing surgery for split cord malformations experience significant improvement or stabilization in symptoms. This positive outcome is often the result of the spinal cord’s detethering and the correction of structural deformities. However, exceptions exist, particularly in cases with pre-existing vertebral column anomalies, which may necessitate further surgeries in the future.

Potential Complications and Considerations

While de-tethering can address initial concerns, some patients may experience later neurologic deterioration, often due to re-tethering caused by scar tissue. Fortunately, surgical correction can often effectively address these issues. The role of surgery in asymptomatic patients remains debated. Some experts advocate for early intervention upon diagnosis or before other corrective spinal surgeries, while others recommend monitoring and delaying surgery unless neurologic deterioration occurs.

Impact of Associated Conditions

Patients with split cord malformations and associated conditions like meningomyelocele may face more complex challenges. These individuals might experience worse outcomes compared to those without such conditions. Nonetheless, surgical intervention can stabilize the neurological status of both groups, underscoring the importance of tailored treatment plans.

This comprehensive approach ensures a balanced understanding of the potential outcomes and challenges associated with surgical intervention for split cord malformations, providing patients and caregivers with crucial insights for informed decision-making.

Recovery Period:

The recovery period following surgical treatment for split spinal cord malformation varies depending on the specific procedures performed and the individual patient’s response. In general, patients may experience a period of postoperative care, including pain management, physical therapy, and close monitoring for any signs of complications.

Physical therapy plays a crucial role in the recovery process. Moreover, therapists work closely with patients to gradually regain strength, flexibility, and functional abilities. The duration of the recovery period can range from weeks to months, and ongoing follow-up appointments with healthcare providers are essential to monitor progress and address any concerns.

Reasons to Choose the Southwest Scoliosis and Spine Institute:

Patients facing the complexities of split spinal cord malformation should seek treatment at the Southwest Scoliosis and Spine Institute. This specialized institute offers several advantages:

• Experienced Specialists: The institute is staffed with experienced spine specialists who possess expertise in the diagnosis and treatment of complex spinal conditions, including split spinal cord malformation.
• Comprehensive Diagnostic and Treatment Options: The institute provides a range of comprehensive diagnostic and treatment options, ensuring that each patient receives individualized care tailored to their unique needs.
• Multidisciplinary Approach: The Southwest Scoliosis and Spine Institute employs a multidisciplinary approach, involving collaboration among various healthcare professionals to address the diverse aspects of spinal conditions.
• Focus on Individualized Care: Patients benefit from a patient-centered approach, where treatment plans are customized to address their specific symptoms, concerns, and goals.
• Advanced Surgical Techniques: The institute maintains state-of-the-art technology, allowing for precision in surgical interventions and enhancing overall treatment outcomes.

Conclusion

In conclusion, the treatment of split spinal cord malformation necessitates a thorough understanding of its condition. While non-surgical treatments may suffice in milder cases, surgical interventions offer significant benefits in managing severe symptoms and preventing further complications. Therefore, the decision to choose surgical treatment is guided by individualized considerations, and the recovery period involves emphasizing physical therapy and postoperative care. For patients seeking specialized care for split spinal cord malformation, they should consider the Southwest Scoliosis and Spine Institute with offices in Dallas, Plano, and Frisco, Texas.  The reason is simple — we are a reputable and comprehensive center offering experienced specialists, individualized care, and advanced treatment options.

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National Library of Medicine: Characteristics of Split Spinal Cord Malformation