NEUROMUSCULAR SCOLIOSIS

In children who require wheelchairs, spinal curves may cause changes like those mentioned above, as well as the following:

• Tilted pelvis
• difficulty in sitting and the need to use the hands and arms for support while seated
• Pressure sores due to the pelvic tilt and overall imbalance
• A change in one’s overall posture, such as slouching or leaning more to one side

Children with this condition may also develop other types of spinal curves,  Also, large curves in the upper back or large inward curves in the lower back may decrease the space in the chest for the lungs and lead to breathing problems.

Diagnosing Neuromuscular Scoliosis

To help the doctors at Southwest Scoliosis and Spine Institute determine the severity of this condition, spinal curves are closely monitored.  To assist us, we use our advanced EOS imaging system. This system allows doctors to determine the location of the curve on the spine.  Also, it measures the degree of curvature while delivering a very low dose of radiation.  Because patients with neuromuscular scoliosis are often unable to stand, the EOS imaging system provides another advantage.  Our EOS system allows us to obtain images of the patient in a seated position.

MRI scans may also be necessary, especially in patients who require surgery, because an MRI is able to provide clear images of the soft tissues and spinal cord.

Treatments

Treatment for Neuromuscular Scoliosis includes specialists in other medical fields.  All elements of care must follow best practices to ensure that every aspect of treatment provides the best outcome. Treatment for this condition depends largely on the type and severity of the spinal curve and the nature of any other conditions, as well as the patient’s age and the stage of the curve.

Nonsurgical Treatment

Although nonsurgical treatments will not prevent your child’s curve from getting worse, they may delay the progression and help improve function and quality of life.

Wheelchair Modification

This is the most common nonsurgical treatment for neuromuscular scoliosis. If the patient’s pelvis has shifted in a way that affects their balance, changing the wheelchair to improve posture can lead to improvements. Changes may include adding specially molded backings and adjusting the side positioners to improve balance while in a seated position.

Scoliosis Brace

For some patients who require wheelchairs, doctors recommend a molded plastic brace. The brace goes around the upper body — stabilizing the patient and permitting the patient to use their arms and hands. In patients who are able to walk, bracing is not recommended, as it can make them more likely to lose their balance while walking and fall – especially patients who have muscle weakness or an unsteady gait.

Comprehensive Treatment Approach

A more comprehensive approach to treating neuromuscular scoliosis involves integrating multiple treatment forms such as bracing and rehabilitation. However, it’s crucial to note that bracing is not always successful in severe cases of NMS. Scoliosis-specific exercises often need to be modified to address the unique characteristics of NMS.

Challenges and Limitations

Treating patients with NMS presents unique challenges because the underlying neuromuscular condition places additional limitations on the patient’s function, mobility, and overall health. Patients whose neuromuscular deficits have landed them in a wheelchair are particularly difficult to treat. Many of these conditions are unresponsive to bracing, and being immobile introduces additional complications like spinal rigidity.

Treatment Goals

The primary treatment goal for NMS patients is to effectively stall the progression of scoliosis and improve their quality of life. While there is no cure for scoliosis, addressing the underlying neuromuscular condition that contributed to its development can make a significant difference.

Quality of Life Improvement

Ultimately, as there is no way to cure or resolve these types of cases fully, treatment is aimed at slowing the curvature’s progression as much as possible, for as long as possible. This approach hopes to improve the patient’s quality of life by maintaining as much function and mobility as possible.

Wheelchair modification

This is the most common nonsurgical treatment for neuromuscular scoliosis. If the patient’s pelvis has shifted in a way that affects their balance, changing the wheelchair to improve posture can lead to improvements. Changes may include adding specially molded backings and adjusting the side positioners to improve balance while in a seated position.

Scoliosis brace

For some patients who require wheelchairs, doctors recommend a molded plastic brace.  The brace goes around the upper body — stabilizing the patient and permitting the patient to use their arms and hands. In patients who are able to walk, bracing is not recommended, as it can make them more likely to lose their balance while walking and fall – especially patients who have muscle weakness or an unsteady gait.

Surgical Treatment

The decision to proceed with surgery depends on several factors, such as the curve, the nature of the condition, the age of the patient, ambulatory status, and how much their condition affects their ability to function normally. The doctors will consider many other options, especially with patients who are non-communicative.

In severe cases of neuromuscular scoliosis (NMS), surgical treatment like spinal fusion is often recommended. Surgery carries significant risks and side effects and should be considered only after other noninvasive treatment methods have been attempted.

Neuromuscular Scoliosis Criteria for Surgery

Surgery is generally recommended for cases with:

• Extreme spinal curvatures of more than 50 degrees
• Rapidly progressing conditions
• Continued progression into adulthood
• Significant pain
• Difficulty sitting
• Related functional deficits such as heart and lung impairment

Complexity for NMS Patients

For NMS patients, the decision to opt for surgery is even more complex due to their underlying condition. This makes the surgical choice more intricate compared to other patients with scoliosis.

By understanding these specific criteria and considerations, patients and their families can make a more informed decision about whether surgery is the right path forward.

Should your child require surgery to correct this condition, the expert surgeons and caregivers at our practice can provide the care and attention they deserve. Our expert physicians, Richard Hostin, MD, Devesh Ramnath, MD, Ishaq Syed, MD, Shyam Kishan, MD, and Kathryn Wiesman, MD have been treating children and adolescents for more than 12 years.

Spinal Fusion

This is the most common surgical procedure used to treat neuromuscular scoliosis. The surgeons use metal rods, screws, etc. to fuse together two or more vertebrae to stabilize the spine.  This procedure will also straighten out the curve.

In patients who are still quite young and growing rapidly, the treatment team may opt to use growing rods that facilitate changing every few months as the child grows. This allows the spine to continue growing while the rods stabilize it, but it is a temporary solution, and a spinal fusion procedure may become necessary later in life if the curve continues to progress.

What are The Risks and Complications Associated With Untreated Scoliosis?

Untreated scoliosis can lead to a number of significant risks and complications, impacting various aspects of health.

Neurological Symptoms

One of the primary concerns is the onset of neurological symptoms. These can range from nerve pain and zapping sensations to shooting pains, tingling, numbness, and muscle weakness. These symptoms occur because the curved spine can compress surrounding nerves. If this nerve compression continues without intervention, it may lead to permanent nerve damage, making it difficult, if not impossible, to restore proper nerve function.

Progressive Nature

Scoliosis is a progressive condition, meaning the curvature of the spine tends to worsen over time. As the curve increases, so do the uneven forces exerted on the body, exacerbating symptoms and complications. Larger curvatures can significantly impact the body’s overall balance and structural integrity.

Organ Complications

Severe forms of untreated scoliosis can lead to additional complications affecting vital organs. For instance:

• Cardiac Issues: As the curve worsens, it can distort the chest cavity, potentially impacting heart function.
• Lung Impairment: A distorted rib cage can compress the lungs, leading to reduced lung capacity and breathing difficulties.
• Digestive Problems: The abnormal spinal curvature can press on the stomach and intestines, causing digestive issues.

Management and Proactive Treatment

To minimize these risks and manage the progression of scoliosis effectively, early and proactive treatment is crucial. Addressing the condition in its early stages can help in managing symptoms, preventing deterioration, and avoiding severe complications.

By understanding these potential risks associated with untreated scoliosis, individuals can better appreciate the importance of timely medical intervention and continuous monitoring to ensure better health outcomes.

What Are the Side Effects of Spinal Fusion Surgery for Neuromuscular Scoliosis?

One of the most significant side effects of spinal fusion surgery is the loss of mobility. When vertebrae are fused, the resulting solid bone restricts movement in that specific area. While the procedure aims to halt the progression of the curvature, success is not guaranteed.

Patients with neuromuscular scoliosis (NMS) often experience more pronounced mobility loss. This is particularly true in non-ambulatory cases where the entire spine might be involved. The reduction in movement can lead to notable functional deficits, as the flexibility of the spine is drastically compromised.

Moreover, spinal fusion surgery is highly invasive and comes with numerous risks. Here are some common side effects and complications:

• Infection
• Excessive bleeding
• Neurological damage
• Hardware failure
• Adverse reaction to the hardware
• Persistent pain
• Continued curvature progression
• Psychological impact
• Requirement for additional surgeries

One major concern is the limited data on the long-term effects of spinal fusion—spanning 20 to 40 years. The hardware used in the procedure doesn’t last indefinitely, leading to the possibility of further surgeries as it deteriorates, a risk that increases with age.

Increased post-surgical pain is reported by many patients due to the stiffness in the fused section, affecting surrounding muscles and tissues. The psychological impact of a fused spine should not be underestimated. Knowing that a fused spine is more vulnerable to injury can lead to anxiety and reluctance to engage in activities they once enjoyed.

In summary, while spinal fusion can address scoliosis progression, the trade-offs in terms of mobility, pain, and quality of life are considerable, making it essential to weigh these factors carefully.

How Does Cerebral Palsy Relate to Neuromuscular Scoliosis?

Cerebral palsy (CP) primarily affects the body’s musculoskeletal system, influencing movement and balance. This condition stems from brain damage that typically occurs during fetal development. Key symptoms often emerge in infancy or early childhood.

Linking Cerebral Palsy to Neuromuscular Scoliosis

Muscle Control and Nerve Function:

• CP impairs the body’s muscle control and coordination.
• The disruption in nerve function affects the muscles surrounding the spine.

Balance and Posture:

• Children with CP struggle with maintaining balance.
• This imbalance places uneven stress on the spine.

Spinal Curvature:

• The inability to properly control spinal muscles can lead to an unnatural spinal curvature, known as neuromuscular scoliosis (NMS).

What Is Neuromuscular Scoliosis?

Neuromuscular scoliosis is a type of spinal curvature caused by conditions that affect the nerves and muscles, such as CP. The lack of muscular control leads to the spine developing abnormal curvatures.

Why It Matters

• Early Detection: Recognizing the link between CP and NMS can aid in early diagnosis and treatment.
• Comprehensive Care: Addressing both conditions concurrently can improve overall quality of life for affected children.

In conclusion, the impaired muscle control and balance challenges associated with cerebral palsy significantly contribute to the development of neuromuscular scoliosis. Understanding this relationship is crucial for timely and effective intervention.

The Link Between Muscular Dystrophy and Neuromuscular Scoliosis

Muscular dystrophy (MD) plays a significant role in the development of neuromuscular scoliosis. This condition encompasses a group of about 30 genetic diseases characterized by the progressive weakening and degeneration of skeletal muscles, which are crucial for movement and balance.

Key Points:

• Muscle Weakness: The core issue in MD is muscle weakness. When the muscles that support the spine become too weak, they can no longer maintain proper alignment. This loss of structural support often leads to scoliosis.
• Development Timing: Different forms of MD manifest at various stages of life. Some appear at birth, while others may develop during infancy or adolescence. The stage at which MD impacts the muscles can influence the progression and severity of scoliosis.
• Types of Muscular Dystrophy Involved: Several types of MD, such as Duchenne Muscular Dystrophy and Becker’s Muscular Dystrophy, are commonly associated with neuromuscular scoliosis. These types specifically target the muscle groups that help stabilize the spine.

Understanding the Severity

The severity of scoliosis in patients with MD can vary widely. Factors such as the type of MD, age of onset, and the overall health of the individual all play a role. However, the primary driver remains the inability of weakened muscles to support the skeletal structure effectively.

Impact on Daily Life

• Mobility Challenges: As scoliosis progresses, it can further impair mobility, making daily tasks more challenging and sometimes necessitating the use of mobility aids.
• Respiratory Issues: Severe spinal curvature can also impact lung function, as it may restrict the space available for proper lung expansion.
• Treatment Approaches: The management of neuromuscular scoliosis often involves a combination of physical therapy, bracing, and in some cases, surgical intervention. Addressing the muscle weakness through specialized therapies can also help slow the progression of scoliosis.

By understanding the connection between muscular dystrophy and neuromuscular scoliosis, patients, and caregivers can take proactive steps to manage both conditions more effectively.

What is Spina Bifida and How Does it Relate to Neuromuscular Scoliosis?

Spina bifida is a congenital condition that emerges during fetal development when the neural tube, which eventually forms the brain and spinal cord, fails to close completely. This incomplete closure results in a range of spinal malformations, affecting the spinal cord, its coverings, and sometimes the surrounding vertebrae.

Understanding the Relationship with Neuromuscular Scoliosis

Neuromuscular scoliosis is a type of scoliosis caused by neurological or muscular conditions that impair the body’s ability to maintain proper spinal alignment. Spina bifida falls under this category due to its direct impact on the spinal cord and neuromuscular system.

When spina bifida disrupts the normal development and function of the spinal cord, it can lead to imbalances in muscle control and strength. These imbalances often result in the spine curving abnormally, a hallmark of neuromuscular scoliosis.

Key Points to Consider:

• Developmental Origin: Spina bifida originates from a failure in the neural tube closure during early embryonic growth.
• Spinal Malformations: This condition can lead to multiple spinal anomalies, influencing both the physical structure and functionality of the spine.
• Neuromuscular Implications: The impaired spinal cord affects muscle coordination and strength, contributing to the development of scoliosis.

In summary, spina bifida is a significant cause of neuromuscular scoliosis due to its profound effects on the spinal cord and muscular system. Understanding this connection is essential for effective diagnosis and management of the associated spinal deformities.

If you Suffer from the Pain of Neuromuscular Scoliosis, Contact the Experts

The Southwest Scoliosis and Spine Institute is a premier Scoliosis Center with doctors who are among the best in the world at treating all types of scoliosis.  With offices in Dallas, Plano, and Frisco, Texas, the Southwest Scoliosis and Spine Institute specializes in Scoliosis.

The Southwest Scoliosis and Spine Institute maintains a team of highly experienced and skilled spine surgeons.  These doctors have received specialized training in the diagnosis and treatment of spinal conditions. Therefore, they use the latest techniques and technologies to ensure the best possible outcomes for their patients.

The Institute’s approach to spine treatment centers on the patient, meaning that the doctors and staff approach each patient differently. Moreover, they work closely with their patients to understand their individual needs and goals and develop a customized treatment plan that takes into account the patient’s unique situation, lifestyle, and preferences. The Institute also offers comprehensive follow-up care and support to its patients.  This includes physical therapy and rehabilitation, to help them recover quickly and fully from their surgery.

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American Academy of Orthopaedic Surgeons: Neuromuscular Scoliosis