Neuromuscular scoliosis

At Children’s Hospital Boston, our Spinal Program team develops innovative treatments for scoliosis and other spine defects. We’re home to the world’s most extensive pediatric hospital research enterprise, and we partner with elite health care and biotech organizations around the globe. But as specialists in innovative, family-centered care, our physicians never forget that your child is precious, and not just a patient.

In dealing with your child’s neuromuscular scoliosis, you may want to know the basics about the spine and about the several forms of this spinal condition.

What is the spine?

Made up of many individual bones called vertebrae, the normal spine is joined together by muscles and ligaments. Flat, soft discs separate and cushion each vertebra from the next. Because the vertebrae are separate, the spine is flexible and can bend. Together the vertebrae, discs, muscles and ligaments make up the spine or vertebral column.

Various regions of the spine are named differently: The cervical spine refers to the neck, the thoracic spine to the chest, and the lumbar and sacral spines to the lower back.

What is scoliosis?

Although the spine has natural curves from front to back, it shouldn’t curve sideways very much. A side-to-side curve is called scoliosis, and may take the shape of an abnormal “S” (double curve) or a long “C” (single curve). The scoliotic spine is also rotated or twisted, pulling the ribs along with it to form a multi-dimensional curve. In serious cases, lung and heart function can be affected, as well as other organ systems.

What are the three main types of scoliosis?

Scoliosis occurs, and is treated, as three main types:

• neuromuscular scoliosis: the second-most common form of scoliosis, associated with disorders of the nerve or muscular systems like cerebral palsy, spina bifida, muscular dystrophy or spinal cord injury.
   
• congenital scoliosis: Congenital scoliosis results from a fetus’ abnormal spinal development in utero, such as a partial or missing formation or a lack of separation of the vertebrae.
   
• idiopathic scoliosis: the most common form of scoliosis, most commonly seen in adolescent and pre-adolescent girls. “Idiopathic” simply means that there is no definite cause. Most cases require no intervention.

What are some of the defining characteristics of neuromuscular scoliosis?

Some characteristics of neuromuscular scoliosis include:

• occurs in children with underlying neuromuscular disease
   
• usually a “C”-type curve
   
• onset much younger than many other forms of scoliosis
   
• more common and severe in non-ambulatory patients (those whose neuromuscular disease prevents them from walking)
   
• curve virtually always progressive; progression can continue into adulthood
   
• progresses (worsens) more during growth spurts
   
• progressing (worsening) curve can lead to collapsed torso and raised diaphragm, reducing lung space, impeding lung function and increasing risk of pneumonia; more severe effect if lung function is already compromised by weak muscles that control breathing (as in muscular dystrophy and high-level spinal cord paralysis)
   
• many organ systems involved due to underlying nerve or muscular disease, which can cause:
  • contractures of hips and knees
  • vision and hearing loss
  • lung function impairment
  • seizures
     
• bracing useful for functional support but rarely stops the curve
   
• commonly requires spinal fusion surgery in non-ambulatory, severe patients by adolescence, usually sooner than with idiopathic scoliosis
  • fused portion of the spine is longer than with idiopathic scoliosis—often extends to sacrum and pelvis
  • may require both anterior and posterior surgeries
    • some posterior elements may be missing from spine (as in spina bifida)
    • helps achieve a spine with torso in balance over pelvis (usually in non-ambulatory patients)

• spine surgery’s goals are to:
  • stabilize the curve and stop its progression
  • balance the spine and pelvis (usually in non-ambulatory patients)
  • regain the ability to sit upright (in children who have lost this ability)
  • improve/preserve lung function

What are the signs and symptoms of neuromuscular scoliosis?

You, your child’s doctor or his caregiver will most likely notice his spinal curve because of a deteriorating ability to sit or a change in his body’s overall position:

• he may begin leaning toward one side of his seat in an uneven seating posture
• he may increasingly need to use his arms for seating support

You may notice that his:

• shoulders are of uneven heights
• head isn’t centered with the rest of his body
• hips are of uneven heights or positions, or uneven buttocks
• shoulder blades are of uneven heights or positions
• arms hang beside his body unevenly
• left and right sides of his back appear different in height when he bends forward

How do you diagnose neuromuscular scoliosis?

Doctors will use medical and family histories, physical exams and diagnostic tests to determine the nature, extent and effects of your child’s scoliosis and underlying neuromuscular disease. Your Children’s multi-disciplinary care team will evaluate his nutritional status, lung and heart function, functional level, joints and extremities (for contractures), balance and ambulatory (walking) level. Testing can include:

• x-rays
• magnetic resonance imaging (MRI)
• computerized tomography scan (CT or CAT scan)
• blood tests
• ultrasound (sonogram)
• bone scans
• bone density scans (dual-energy x-ray absorptiometry, DEXA, DXA)
• muscle biopsy
• pulmonary function tests
• Electromyogram (EMG) and nerve conduction studies

How do you treat neuromuscular scoliosis?

Most neuromuscular conditions are diagnosed early in a child’s life, and neuromuscular scoliosis may also manifest early. When it appears, your child’s team will use non-surgical treatments such as bracing, wheelchair modification, physical therapy and environmental adaptation to help your child adapt to his everyday environments, and to help improve his mobility.

While most non-ambulatory pre-adolescent children are usually treated with bracing to control (not correct) spinal curves while they grow, stabilization by spinal fusion surgery is the most common treatment for neuromuscular scoliosis by the time a child reaches adolescence. But treatment will vary according to the type and severity of a child’s neuromuscular condition.

What if the curve is severe in my very young child before he’s ready for spinal fusion?

A young child with a severe early-onset curve can be treated with growth-friendly (growth preserving) procedures such as growing rods and/or VEPTR. The metal rods inserted in these procedures can help control the curve until he’s ready for spinal fusion. The rods are made longer as the child’s spine grows.

What are the benefits of spinal fusion surgery?

A spinal fusion aims to:

• stabilize the curve and stop its progression
• balance the spine and pelvis (usually in non-ambulatory patients)
• regain the ability to sit upright (in children who have lost this ability)
• improve/preserve lung function

If my child needs surgery, what will happen beforehand?

Before surgery, your child’s surgical team will assess his:

• lung and heart function
• nutritional and protein status
• likelihood of post-operative feeding difficulties
• neurologic status and seizure medications
• urologic status
• bone health
• swallowing and regurgitation evaluation (for cerebral palsy patients)
• clotting and bleeding evaluation (for children subject to seizures, since some seizure medications can affect blood clotting)

Will there be complications after the surgery?

Because neuromuscular scoliosis is associated with other underlying neuromuscular conditions, the complication rate after surgery is higher than with some other types of scoliosis. Complications are common, anticipated and treatable. They can include:

• respiratory problems: Post-operative pneumonia is a risk for a child with a poor cough reflex or some paralysis of the chest (intercostal) muscles.
   
• wound problems: Skin breakdown and infection can occur.
   
• nutritional/feeding problems: A feeding tube may be necessary until the child can feed orally again.
   
• hip problems: Many children who have spinal fusion also have stiff or abnormal hips. Fusing the spine may make existing hip contractures more troublesome.
   
• intestinal obstruction (ileus): This can result from intestinal sluggishness (hypomotility).

Who will be on my child’s scoliosis treatment team at Children’s?

Your child’s scoliosis team at Children’s may include his doctor, an orthotist (a specialist who makes braces), a physical therapist and a nurse, who will guide you through the treatment process. Specialists, such as neurologists and physiatrists as well as social workers, speech/language specialists and others—will be part of the team as needed to address your child’s underlying neuromuscular condition.

As part of our family-centered approach, your child’s nurse will help with all your questions and appointments. The nurse can also:

• help you understand how best to care for your child, his brace, wheelchair, orthotics or other equipment
• design a schedule for you and him to follow
• help you meet others whose children have neuromuscular disease with scoliosis—in person and/or online

Will my child be OK?

The outlook for your child mostly depends on the nature, severity and effects of his neuromuscular disease, and less on his scoliosis.

With successful surgery and attentive post-operative care, your child can:

• return to the functional level he had attained before surgery
• have his spine solidly fused and in balance, reducing the deformity
• have the potential for improved lung function and decreased susceptibility to pneumonia
• find it easier to sit up; requirements for seating adaptation will usually be improved

Children’s Hospital Boston’s research into spinal problems—including neuromuscular scoliosis—means that we will provide your child with the most innovative care available.

FAQ

Q: What is scoliosis?

A: Scoliosis is a condition in which the spine, in addition to the normal front to back curvatures, has an abnormal side-to-side “S-” or “C”-shaped curvature. The spine is also rotated or twisted, pulling the ribs along with it. Sometimes, a child’s lung function can be compromised when the curvature is severe or starts early in life.

Q: What is neuromuscular scoliosis?

A: Neuromuscular scoliosis is the form that's associated with another, underlying condition involving the nerves and/or muscles, such as cerebral palsy, spina bifida, myopathy, muscular dystrophy and paralysis from spinal cord injury.

Q: If my child has neuromuscular scoliosis, will he be OK?

A: The outlook for your child greatly depends on the nature, severity and effects of his scoliosis and his underlying neuromuscular disease. With successful surgery and attentive post-operative care, your child can return to the functional level he had attained before surgery. His spine will be solidly fused and in balance—usually reducing the deformity and improving his breathing.

Q: How does Children’s treat neuromuscular scoliosis?

A: Most neuromuscular conditions are diagnosed early in a child’s life, and neuromuscular scoliosis may also present early. When it appears, your child’s team will use non-surgical treatments such as bracing, wheelchair modification, physical therapy and environmental adaptation to help your child adapt to his everyday environments, and to help improve his mobility.

While most non-ambulatory pre-adolescent children are usually treated with bracing to control (not correct) spinal curves while they grow, stabilization by spinal fusion surgery is the most common treatment for neuromuscular scoliosis by the time a child reaches adolescence. But treatment will vary according to the type and severity of a child’s neuromuscular condition.

Q: What are the signs and symptoms of neuromuscular scoliosis?

A: You, your child’s doctor or his caregiver will most likely notice his spinal curve because of a deteriorating ability to sit or a change in his body’s overall positioning.

Q: How is scoliosis usually diagnosed?

A: Doctors will use medical and family histories, physical exams and diagnostic tests to determine the nature and extent of your child’s scoliosis and underlying neuromuscular disease. Your Children’s multi-disciplinary care team will evaluate his nutritional status, lung and heart function, functional level, joints and extremities (for contractures), balance and ambulatory (walking) level. Testing can include:

• x-rays
• magnetic resonance imaging (MRI)
• computerized tomography scan (CT or CAT scan)
• blood tests
• ultrasound (sonogram)
• bone scans
• bone density scans (dual-energy x-ray absorptiometry, DEXA, DXA)
• muscle biopsy
• pulmonary function tests
• electromyogram (EMG) and nerve conduction studies

Q: What should I expect after my child has spinal fusion surgery for neuromuscular scoliosis?

A: After surgery, most patients go to the ICU (intensive care unit), where some may be put on a ventilator, and some may require a longer stay than others. Your child’s hospital stay will probably be between five and 10 days.

During your child’s hospital recuperation, his Children’s caregivers will closely monitor his lungs, fluids and nutrition. They will get him up and moving as soon as possible, and will extend his time upright a little more each day. His wheelchair will be modified for his improved sitting position.

Q: What's the long-term outlook for my child with neuromuscular scoliosis?

A: With successful surgery and attentive post-operative care, your child can:

• return to the functional level he had attained before surgery
• have his spine solidly fused and in balance, reducing the deformity
• have the potential for improved lung function and decreased susceptibility to pneumonia
• find it easier to sit up; requirements for seating adaptation will usually be minimized

His longer-term outlook greatly depends on the extent and severity of—and the prognosis for—his underlying neuromuscular condition.

Q: Will a straighter spine after surgery improve the function of my child’s organ systems?

A: A straighter spine can improve your child’s lung function and decrease his vulnerability to pneumonia. It can reduce deformity and help him with his sitting posture. And sometimes, a straighter spine will improve the organ function of a child with neuromuscular disease—but not always. (Rarely, a straighter spine can actually be detrimental to function.) However, not correcting his curve surgically will almost always result in breathing or positioning problems later on.

Q: What is Children’s experience treating neuromuscular scoliosis?

A: At Children’s Spinal Program, we’re known for our clinical innovations, research and leadership. We offer the most advanced diagnostics and treatments—several of which were pioneered and developed by Children’s researchers and clinicians.

Q: What are Children’s spine research and innovations?

A: Children’s Orthopedic Clinical Effectiveness Research Center (CERC) helps coordinate research and clinical trials to improve the quality of life for children with musculoskeletal disorders, such as scoliosis. CERC physicians are pursuing several areas of basic and clinical research based at Children’s and the Harvard Orthopaedics Biomechanics Laboratory.

For details see Children’s spine Research & Innovations.

Causes

Neuromuscular scoliosis is associated with an underlying neuromuscular condition such as cerebral palsy, muscular dystrophy, spina bifida, myopathy or paralysis from spinal cord injury.

Signs and symptoms

You, your child’s doctor or his caregiver will most likely notice his spinal curve because of a deteriorating ability to sit or a change in his body’s overall position:

• he may begin leaning toward one side of his seat in an uneven seating posture
• he may increasingly need to use his arms for seating support

You may notice that his:

• shoulders are of uneven heights
• head isn’t centered with the rest of his body
• hips are of uneven heights or positions, or uneven buttocks
• shoulder blades are of uneven heights or positions
• arms hang beside his body unevenly
• left and right sides of his back appear different in height when he bends forward

When to seek medical advice

Because your child has an underlying neuromuscular disease, your doctor and caregivers will be evaluating him frequently. His scoliosis will usually become apparent as the result of changes in his sitting ability and/or body position. Consult your child’s doctor if you notice that he is:

• leaning toward one side of his seat in an uneven seating posture
• increasingly needing to use his arms for seating support

Also consult the doctor is you notice that his:

• shoulders are of uneven heights
• head isn’t centered with the rest of his body
• hips are of uneven heights or positions
• shoulder blades are of uneven heights or positions
• arms hang beside his body unevenly
• left and right sides of his back appear different in height when he bends forward

Questions to ask your doctor

If your child is diagnosed with neuromuscular scoliosis, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you. Lots of parents find it helpful to jot down questions as they arise—that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.

Some of the questions you may want to ask include:

• What is happening to my child, and why?
• Are other tests needed to diagnose my child?
• What actions might you take after you reach a diagnosis?
• What will happen with growth over time?
• Will there be restrictions on my child’s activities?
• Will there be long-term effects?
• What can we do at home?

Who’s at risk

Children most at risk for developing neuromuscular scoliosis include:

• non-ambulatory children with quadriplegic cerebral palsy
• children who have spina bifida with a high level of paralysis
• children with spinal cord paralysis before age 10 years                                                           

Complications

Because neuromuscular scoliosis is associated with other underlying neuromuscular conditions, the complication rate after surgery is higher than with some other types of scoliosis. The complications are common, anticipated and treatable. They can include:

• respiratory problems
• wound problems
• nutritional/feeding problems
• hip problems
• intestinal obstruction (ileus)

Long-term outlook

With successful surgery and attentive post-operative care, your child can:

• return to the functional level he had attained before surgery
• have his spine solidly fused and in balance, reducing the deformity
• have the potential for improved lung function and decreased susceptibility to pneumonia
• find it easier to sit up; requirements for seating adaptation will usually be minimized

His long-term outlook greatly depends on the extent and severity of—and the prognosis for—his underlying neuromuscular condition.

For teens

Besides the typical issues any teenager faces—from social acceptance to body changes and more—if you have a neuromuscular disease that includes scoliosis, it’s true that you’ll also have to deal with medical appointments, feeling different and assuming a big personal responsibility for maintaining your health. You may also wonder why you need surgery.

It’s important for you to know that surgery for your scoliosis will fuse your spine to correct the curve and prevent further curvatures. You’ll be able to sit more upright in your wheelchair. You’ll also probably breathe better and be less susceptible to respiratory problems.

Even knowing the benefits of surgery and treatments, this can be a tough time for you. If you feel overwhelmed, depressed or anxious during this important time in your transition to adulthood, speak to your doctor or counselor to get help.

What to expect after your child’s surgery

After surgery, most patients go to the ICU (intensive care unit), where some may be put on a ventilator, and some may require a longer stay than others. Your child’s hospital stay will probably be between five and 10 days. Your child’s caregivers will closely monitor his lungs, fluids and nutrition. They'll get him up and moving as soon as possible, and will extend his time upright a little more each day. His wheelchair will be modified for his improved sitting position.

Prevention

Neuromuscular scoliosis is associated with your child’s underlying neuromuscular disease. Nothing you or your child did caused it, and there’s nothing you could have done to prevent it.

Neuromuscular scoliosis glossary

• Adams forward bending test: a screening measure for assessing scoliosis
   
• ambulatory: pertaining to walking, able to walk
   
• anterior fusion: spinal fusion surgery on the front of the spine approached from the side of the body; sometimes combined with posterior fusion, usually performed on the same day or in stages
   
• brace, bracing (spinal orthosis): A brace supports your child’s spine in a balanced position over the pelvis. A bracing program may help delay surgery.
   
• The Center for Families at Children’s: dedicated to helping families find the information, services and resources they need to understand their child’s medical condition and take part in their care
   
• Cobb angle: an angular measurement on x-ray to evaluate the severity and degree of scoliosis curves
   
• congenital scoliosis: a form of scoliosis resulting from abnormal in utero spinal development, such as a partial or missing formation or a lack of separation of the vertebrae
   
• contractures, joint contractures: joints with limited range of motion and that cannot be fully straightened or bent; frequent among patients who don’t walk; can be a complication in hips after surgery
   
• diagnosis: identifying disease or injury through examination, testing and observation
   
• ICU: intensive care unit
   
• idiopathic scoliosis: the most common form of scoliosis, mainly affecting adolescent girls. “Idiopathic” simply means that there is no definite cause. Nothing you or your child did caused the defect, and there’s nothing you could have done to prevent it.
   
• instrumentation: the metal rods, hooks, screws and wires implanted during spinal fusion surgery to correct the spinal curve and secure the spine in position while the fusion heals and becomes solid
   
• neuromuscular: affecting, or characteristic of, both neural (nerve) and muscular tissue
   
• neuromuscular scoliosis: scoliosis that is associated with disorders of the nerve or muscular systems like cerebral palsy, spina bifida, muscular dystrophy or spinal cord injury
   
• orthopedics: the medical specialty concerned with diagnosing, treating, rehabilitating and preventing disorders and injuries to the spine, skeletal system and associated muscles, joints and ligaments
   
• orthopedist, orthopedic surgeon: a physician specializing in surgical and non-surgical treatment of the spine, skeletal system and associated muscles, joins and ligaments
   
• orthotics: the science of designing and fitting of devices such as braces to treat orthopedic conditions
   
• physical therapy:a rehabilitative health specialty that uses therapeutic exercises and equipment to help patients improve or regain muscle strength, mobility and other physical capabilities
   
• posterior fusion: spinal fusion surgery approached from the back of the body; sometimes combined with anterior fusion, performed either simultaneously or in two stages
   
• progression, curve progression: worsening of a scoliosis curve
   
• scoliometer: a surface measurement device for evaluating the angle of torso rotation (ATR or scoliometer angle, which is not the ‘Cobb’ angle measured on x-ray)
   
• scoliosis: a spinal condition in which the spine, in addition to the normal front to back curvature, has an abnormal side-to-side “S-” or “C”-shaped curvature. The spine is also rotated or twisted, pulling the ribs along with it. Scoliosis occurs in three main types: neuromuscular (associated with neuromuscular diseases); congenital (present at birth); and idiopathic (no definite cause).
   
• seat supports, seating supports: supports for helping to sit upright (usually in a wheelchair) for children with insufficient balance for self-support. Seating supports help children sit upright, but they don’t correct curves.
   
• spinal cord: a nerve bundle within the vertebral column that extends down from the brain stem; it conducts signals in both directions between the brain and extremities, and  allows for bodily motion and sensation
   
• spinal fusion: usually a solid fusion (solidification) of the curved part of the spine, achieved by operating on the spine, adding bone chips and allowing the vertebral bones and bone chips to slowly heal together to form a solid mass of bone called a fusion. A fusion partially corrects a scoliosis curve, stabilizes the curve and stops its progression, as well as balancing the spine and pelvis (usually in non-ambulatory patients).
   
• spine (spinal column, vertebral column): the series of moving vertebrae forming the axis of the skeleton and protecting the spinal cord
   
• VEPTR™ (titanium rib) procedure: an operation that expands the chest and allows continued growth of the chest and spine. A curved metal rod fits the back of the chest and spine, helping the spine to become straighter and allowing the lungs to grow and fill with enough air to breathe. The device can be made longer as your child grows.
   
• vertebra, vertebrae: the individual spine bones that form the spinal column