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Neuromuscular Scoliosis

Neuromuscular Scoliosis

Learn more about the Spine Program

What is neuromuscular scoliosis?

Neuromuscular scoliosis is a sideways curvature of the spine that is often caused by muscular, nerve or neurological disorders, such as cerebral palsy and spina bifida. Instead of a straight line down the middle of the back, a child with neuromuscular scoliosis has a spine that looks more like a letter “C.” The spine also may be rotated or twisted, pulling the ribs along with it to form a multidimensional curve. 

Neuromuscular scoliosis is the second most common form of scoliosis, next to idiopathic scoliosis. It is more severe in patients whose disease prevents them from walking.

Neuromuscular scoliosis can continue to worsen well into adulthood. As the spinal curve progresses, it may cause problems with walking, sitting, balance and breathing, and could possibly lead to thoracic insufficiency syndrome, a serious spine and chest-wall deformity.

Causes of neuromuscular scoliosis

Neuromuscular scoliosis is caused by poor muscle control, weakness or paralysis, and neurological (nerve) problems associated with underlying conditions such as:

Signs and symptoms of neuromuscular scoliosis

Common symptoms of neuromuscular scoliosis include:

  • Deteriorating ability to sit
  • Change in your child’s overall position (e.g., leaning toward one side or in an uneven posture)
  • Increasing need to use arms for seating support
  • Uneven shoulder heights, shoulder blade heights, hip heights and/or buttocks

Testing and diagnosis of neuromuscular scoliosis

At Children’s Hospital of Philadelphia (CHOP), our orthopedic physicians will complete a full medical and family history, perform a physical examination and visual inspection of your child’s spine, and review any diagnostic tests to determine the nature, extent and effects of your child’s scoliosis and underlying neuromuscular disease.

A team of clinical experts will evaluate your child’s nutritional status, lung and heart function, joints and extremities, as well as balance and ability to walk.

X-rays are the primary diagnostic tool for all forms of scoliosis to show abnormal spinal bones and the precise angles of curvature.

Other tests used in evaluation and treatment of neuromuscular scoliosis may include:

  • EOS imaging is a low-dose, 3D imaging system that scans your child while sitting upright in a chair with support or standing position, enabling improved diagnosis due to weight-bearing positioning. An EOS scan allows us to see how the joints interact with the rest of their musculoskeletal system, especially the spine, hips and legs. EOS uses a fraction of the radiation dose that a general X-ray uses, which is especially beneficial for patients with scoliosis that may require frequent imaging. CHOP was the first institution in the U.S. to introduce “EOS micro-dose” for use in scoliosis follow up. Micro-dose uses one-third of the standard EOS radiation dose. This imaging can be performed on patients aged 5 years and older.
  • Magnetic resonance imaging (MRI) uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body. An MRI does not expose your child to radiation.
  • Computed tomography (CT) scan uses a combination of X-rays and computer technology to produce cross-sectional images (“slices”) of the body including bones, muscles and organs. CT scans are helpful in detecting ribcage problems.
  • Pulmonary function tests, which measure how well your child's lungs take in and release air, and how well they move gases (such as oxygen) from the atmosphere into your child's body.

Treatments

At CHOP, we practice collaborative, family-centered care. Our multidisciplinary team includes world-renowned orthopedic physicians and surgeons, nurse practitioners, physician assistants, pediatric nurses, physical therapists, neurologists, rehabilitation specialists, physical and occupational therapists, social workers and others who specialize in pediatric patients. Together, we partner with you to treat your child with neuromuscular scoliosis.

Every spine condition is different, so treatment is determined on a case-by-case basis. In planning your child’s individual treatment, our team of specialists will consider the type and severity of your child’s neuromuscular scoliosis, as well as their age and stage of growth.

Nonsurgical options for neuromuscular scoliosis

Some children with neuromuscular scoliosis do well without surgery; and their quality of life may be improved with nonsurgical treatment options, including:

  • Back bracing, to both support the spine and prevent the curve from progressing
  • Wheelchair modification, to better support the spine
  • Physical therapy

Members of your child’s healthcare team can also offer suggestions on how best to adapt your child’s everyday environment to improve their mobility.

Surgical options for neuromuscular scoliosis

There are several surgical options to improve or correct neuromuscular scoliosis. Doctors will evaluate your child and determine which surgery is right for them based on the degree of your child’s spinal curve, your child’s age, stage of growth, long-term health needs and other considerations. 

Because neuromuscular scoliosis is often associated with other underlying conditions — such as spinal muscular atrophy — your child’s care team will work closely with specialists from neurology, pulmonology, general surgery, gastroenterology and others to treat your child’s overall health.

We offer a wealth of resources about how to prepare your child for surgery, what to expect during surgery and specific ways we make safety in spine surgery a top priority.

The most common spine surgeries for neuromuscular scoliosis include growing rods and spinal fusion.

Growing rods

For children who have many years of growth remaining, a growth-friendly treatment choice is preferred. In growing rod surgery, the curve in your child’s back is spanned by one or two rods next to the spine. The rods are attached to your child’s spine and vertebrae above and below the curve. The growing rods help guide new spinal growth and drive the spine straight.

Spinal fusion

If your child is in the final 2-3 years of growth or has finished growing, spinal fusion surgery may be recommended to correct the deformity as much as possible. This surgery permanently stabilizes your child’s spine. During spinal fusion surgery, the abnormal curved spinal bones are realigned and fused together. Metal implants may be inserted to correct the curve.

VEPTR

A third option, vertical expandable prosthetic titanium ribs (VEPTR), may be recommended for children with neuromuscular scoliosis that is also affecting their rib and lung development, causing thoracic insufficiency syndrome. Left untreated, thoracic insufficiency syndrome can be fatal.

In VEPTR surgery, the expandable titanium ribs are implanted into a child's back and chest and anchored to the spine, ribs or pelvis. The VEPTR helps to correct and stabilize the curved spine, allowing your child’s ribs, lungs and spine to grow and develop more fully.

Growing rods and VEPTR can stabilize and straighten the spine, as well as correct rib cage problems. Whichever device is implanted will need to be expanded or lengthened every six to twelve months as your child grows. Your child will need to return to the hospital for minor surgery to adjust the rods until skeletal maturity.

About the diagnosis

10 facts about scoliosis

A scoliosis diagnosis can leave parents with a lot of questions. Learn essential scoliosis facts parents and caregivers should know about this common, treatable condition.

Follow-up care for neuromuscular scoliosis

Post-op recovery

Most patients receiving any type of spinal surgery to treat neuromuscular scoliosis will spend between five and 10 days in the hospital, during which time caregivers will closely monitor the child’s lung function, fluid and nutrition levels.

Our care team will also encourage your child to move around as soon as possible and modify a wheelchair for your child to improve their seated position, if appropriate.

CHOP is committed to partnering with you to provide the most current comprehensive and specialized follow-up care possible for your child.

Patients who receive surgery for neuromuscular scoliosis at CHOP are followed closely for the first one to two years after surgery, then at regular intervals afterwards at one of our two hospitals in Philadelphia or King of Prussia, or at one of our CHOP Care Network locations.

We also recognize your child’s pediatrician as an important part of the care team and will provide regular updates on your child’s progress. If your child needs continued care into adulthood, we will help transition care to an adult orthopedic team.

Outlook for children with neuromuscular scoliosis

Outcomes for children treated for neuromuscular scoliosis are typically good. The outlook for your child depends more on the nature, severity and effects of their neuromuscular disease, and less on the scoliosis.

With successful spinal surgery and careful post-operative care, a straighter spine can improve your child's breathing, decrease their vulnerability to respiratory problems, and improve their ability to sit in a stable seating position to promote activities of daily living.

Why choose CHOP’s scoliosis experts?

CHOP is consistently ranked among the best in the nation for orthopedic care, according to U.S. News & World Report. Our Spine Program is one of the largest multidisciplinary programs in the world dedicated to the diagnosis and treatment of pediatric spine conditions. We have led the development and introduction of new treatments, from innovative bracing and exercise therapy to new surgical approaches.

We provide complete evaluation and treatment to thousands of babies, children and teens with spinal deformities and other conditions affecting the spine. Every spine condition is different, so treatment is decided on a case-by-case basis. We care for children with all types of scoliosis, from mild to the most severe.

We know it’s important to explore all non-surgical options when it comes to treatment for your child’s spinal condition. That’s why we focus on non-invasive treatment options first, whenever possible, including bracing, casting and special physical therapy programs. When surgery is necessary, we use cutting-edge navigation and imaging equipment in our operating rooms and follow enhanced safety protocols. And in the most severe cases, where the curvature of the spine causes breathing problems or restricts your child’s lung development, our Wyss/Campbell Center for Thoracic Insufficiency Syndrome is here to help.

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Why choose CHOP

By sharing our surgical knowledge, clinical experience and innovative research, our program offers a depth and breadth of experience treating spine conditions that is unparalleled in the region.

Resources to help

Spine Program Resources

We have created video, audio and web resources to help you find answers to your questions and feel confident with the care you are providing your child.

Patient stories

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