Congenital 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 congenital 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.

Different 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 measuring 10 degrees or greater 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 function can be affected.

What are the three main types of scoliosis?

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

• congenital scoliosis: Congenital scoliosis is a rare form of scoliosis, affecting just one out of 10,000 persons. The condition developed because your child’s spine didn’t form correctly in utero. Some vertebrae (vertebral segments) of the spine formed abnormally (failure of formation), didn’t separate correctly (failure of segmentation) or there was some combination of both.
  
  Because the spine forms at the same time as certain other organ systems in the first six weeks of pregnancy, more than half of congenital scoliosis-affected children have one or more other improperly formed systems.
  
  This means that your child’s genitourinary, cardiac, auditory, nervous (neurological, neural) or renal system(s) may also have formed incorrectly or incompletely. Additional problems of the spine or spinal cord can also be associated with congenital scoliosis.

• neuromuscular scoliosis: the second-most common form of scoliosis, associated with disorders of the nerve or muscular systems like cerebral palsy, spina bifida, myopathy, muscular dystrophy or spinal cord injury.
   
• idiopathic scoliosis: the most common form of scoliosis, most often seen in adolescent and pre-adolescent girls. “Idiopathic” simply means that there is no definite cause. Most cases don’t need intervention.

What characterizes congenital scoliosis?

Some characteristics of congenital scoliosis include:

• occurs in children whose spinal vertebrae in utero formed abnormally (failure of formation), did not separate correctly (failure of segmentation), or there was a combination of both
   
• condition is present at birth (congenital)
   
• the least common form of scoliosis—affecting just one in 10,000 people
   
• greatly variable in severity—from mild to life-threatening
   
• sometimes detected in infants and toddlers, sometimes not until adolescent growth spurt—and frequently never noted or noted incidentally
   
• in general, but not always, the younger the child is when the curve appears, the more severe the curve tends to be—and the more aggressive its progression (worsening) tends to be
   
• not usually painful in children
   
• other organ system abnormalities associated with congenital scoliosis in half of patients
  • genitourinary and renal
  • congenital heart defects
  • spinal cord abnormalities
  • auditory
  • rib abnormalities (can limit chest cavity and lung growth)
  • limb abnormalities (such as radial aplasia, Sprengel’s deformity)
  • nervous system (neurological, neural)
     
• curves can progress most aggressively (fast) during a child’s two main growth spurts
  • at one to three years
  • pre-adolescent
     
• many congenital curves progress (worsen)
  • the type and severity of the curve help predict the risk of the curve progressing as a child grows
     
• some curve patterns progress slowly, some very aggressively (especially in younger children)
   
• bracing doesn’t usually correct or arrest congenital curves
  • bracing can help correct compensatory curves that develop to offset congenital curves
     
• physical therapy and exercises usually not effective alone
   
• surgery usually required for progressive curves
  • many surgical approaches—surgeons customize for each child’s unique case
     
• surgical goals are to:
  • reach the end of the child’s growth with a reasonably straight, balanced spine and balanced torso
  • allow the spine to grow normally as much possible (greater torso length than if he hadn’t been treated)
  • prevent compromise of lung function by creating room for chest cavity and lungs to grow (in young children)
  • prevent future back pain
  • prevent damage to nervous system
     
• children treated for congenital scoliosis must be followed until fully grown

What are the signs and symptoms of congenital scoliosis?

Congenital scoliosis curves can sometimes be visible in infants and toddlers. But often, they’re detected in small children only if there’s been another reason to x-ray the child’s chest or back. Another possible clue to congenital scoliosis can be a urinary, renal, auditory or neural (nervous system) condition, since these can be associated with congenital scoliosis.

Signs of associated conditions that might indicate congenital scoliosis can include:

• hairy patch or dimples on back
• rib hump or small bump on back
• pain or spasticity in lower extremities
• stiff or short neck or spine
• abnormally formed hand, arm or foot
• difficulty hearing
• genitourinary or renal anomalies or defects
• other musculoskeletal anomalies
• chromosomal abnormalities

Often, congenital scoliosis may become apparent during or just before the adolescent growth spurt. Common signs and symptoms may include:

• head not centered with the rest of the body
• uneven hip heights or positions
• uneven shoulder blade heights or positions
• uneven arm lengths
• when bending forward, the left and right sides of the back are asymmetrical

How do you diagnose congenital 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 any accompanying conditions. Your Children’s multi-disciplinary care team will evaluate the structure and function of his lungs, chest, kidneys, bladder, feet and hands, hearing and neurologic function.

Diagnostic testing can include:

• x-ray
• magnetic resonance imaging (MRI) (especially of the spinal cord)
• computerized tomography scan (CT or CAT scan)
• ultrasound (including spinal and renal ultrasound)
• pulmonary function tests
• chromosomal analysis

How do you treat congenital scoliosis?

Because congenital scoliosis may progress (worsen) with growth, your child must be followed until the end of his growth, with closest observation during periods of rapid growth. If his curve doesn’t progress, observation without treatment may be all that’s needed.

If your child’s curve is progressing, treatment will usually include one of several approaches to spinal fusion or other surgery by the time he reaches adolescence (earlier if a severe curve presents in a very young child).

If your child is developing a severe curve while very young, he can be treated with growth-friendly (growth preserving) surgical procedures such as growing rods and/or VEPTR. Metal rods inserted during 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.

For some localized curves, osteotomies (controlled cutting procedures) such as a hemi-vertebra wedge resection or a vertebral column resection may be appropriate. For older children with a curve involving a long section of spine, spinal fusion may be appropriate.

For more details, see Treatment & care.

Will there be complications after the surgery?

Complications are uncommon after spinal surgery for congenital scoliosis. There’s a small chance of infection or failure to heal, as well as a small chance of developing a new curvature above or below the fusion. And there is a risk of serious neurological injury (paralysis) that’s higher than with most other forms of scoliosis.

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. Neurologists (nervous system) cardiologists (heart), social workers and others can join the team as needed in addressing your child’s needs.

What will happen after surgery?

After surgery:

• If your child requires bracing or casting for support while his spine heals, the team will help him adjust to wearing his brace.
   
• 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 and his brace or other equipment
  • design a schedule for him to follow
  • help him plan his day-to-day activities
  • help you meet other parents of children with congenital scoliosis—in person and/or online
     
• Your child’s physical therapist will evaluate his posture, muscle strength and flexibility, and will design a home exercise program just for him.

Will my child be OK?

The outlook for your child greatly depends on the nature and severity of his congenital scoliosis, as well as any other accompanying conditions:

• Many children function perfectly normally.
   
• Commonly, the spine is shorter than normal, even if treatment isn’t needed.
   
• The spine is stiffer than normal.
  • The degree of stiffness is relative to how much of the spine is involved.
  • Fusion makes the spine stiffer.
  • For some children, this is visible and dictates restricted activities.
     
• For some children with early, severe curves, breathing may be affected.

With successful spine surgery and attentive post-operative care, he can have a spine that grows as normally as possible, with greater torso length than if he hadn’t been untreated.

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 very early in life.

Q: What is congenital scoliosis?

A: Congenital scoliosis is a rare form of scoliosis, affecting just one out of 10,000 people. The condition developed because your child’s spine didn’t form correctly in utero. Some vertebrae of his spine formed abnormally (failure of formation), didn’t separate correctly (failure of segmentation), or there was some combination of both.

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

A: The outlook for your child depends on the nature and severity of his scoliosis and any associated conditions. Some children function perfectly normally. Commonly, the spine is shorter than normal, even if treatment isn’t needed. The spine is stiffer than normal. For some children with early, severe curves, breathing may be affected.

Q: How does Children’s treat congenital scoliosis?

A: If mild and non-progressing, your child’s congenital scoliosis can be treated through simple observation and monitoring. If his curve progresses, treatment will usually include one of several approaches to spinal fusion or other surgery by the time he reaches adolescence (can be earlier if the curve is early and severe).

For more details, see Treatment & care.

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

A: Congenital scoliosis curves can sometimes be visible in infants and toddlers. But often, they’re detected in small children only if there’s been another reason to x-ray the child’s chest or back. Another possible clue to congenital scoliosis can be a genitourinary, renal, auditory or neural (nervous system) condition, since these can be associated with congenital scoliosis.

Signs of associated conditions that might indicate congenital scoliosis can include:

• hairy patch or dimples on back
• rib hump or small bump on back
• pain or spasticity in lower extremities
• stiff or short neck or spine
• abnormally formed hand, arm or foot
• difficulty hearing
• genitourinary or renal anomalies or defects
• other musculoskeletal anomalies
• chromosomal abnormalities

Often, congenital scoliosis may become apparent during or just before the adolescent growth spurt. Common signs and symptoms may include:

• head not centered with the rest of the body
• uneven hip heights or positions
• uneven shoulder blade heights or positions
• uneven arm lengths
• when bending forward, the left and right sides of the back are asymmetrical

Q: How is scoliosis usually diagnosed?

A: 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 any accompanying conditions. Your Children’s multi-disciplinary care team will evaluate the structure and function of his lungs, chest, kidneys, bladder, feet and hands, hearing and neurologic function.

Diagnostic testing can include:

• x-ray
• magnetic resonance imaging (MRI) (especially of the spinal cord)
• computerized tomography scan (CT or CAT scan)
• ultrasound (including spinal and renal ultrasound)
• pulmonary function tests
• chromosomal analysis

Q: What should I expect after my child has spine surgery for scoliosis?

A: After your child’s spine surgery, his Children’s pediatric orthopedist and physical therapist can advise you on at-home therapies for him, as well as any home therapy equipment you might need.

Q: What are the post-surgery and longer-term outlooks for a child with congenital scoliosis?

A: The outlook for your child greatly depends on the nature and severity of his congenital scoliosis, as well as any other accompanying conditions:

• Many children function perfectly normally.
   
• Commonly, the spine is shorter than normal, even if treatment isn’t needed.
   
• The spine is stiffer than normal.
  • The degree of stiffness is relative to how much of the spine is involved.
  • Fusion makes the spine stiffer.
  • For some children, this is visible and dictates restricted activities.
     
• For some children with early, severe curves, breathing may be affected.

With successful spine surgery and attentive post-operative care, he can have a spine that grows as normally as possible, with greater torso length than if he hadn’t been untreated.

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

A: At Children’s Spinal Program, we’re known for our clinical innovations, research and leadership. We provide 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: Our 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.

Learn more about Children’s spine research initiatives.

Causes

Congenital scoliosis developed because your child’s spine didn’t form correctly in utero. Some of the vertebrae (vertebral segments) in his spine formed abnormally (failure of formation), didn’t separate correctly (failure of segmentation) or there was some combination of both.

Signs and symptoms

The curve may be apparent in infants or toddlers, but may first become apparent in adolescence. In young children, the curve is sometimes detected incidentally by chest or back x-ray for other reasons.

Signs of associated conditions that might indicate congenital scoliosis can include:

• hairy patch or dimples on back
• rib hump or small bump on back
• pain or spasticity in lower extremities
• stiff or short neck or spine
• abnormally formed hand, arm or foot
• difficulty hearing
• genitourinary or renal anomalies or defects
• other musculoskeletal anomalies
• chromosomal abnormalities

Often, congenital scoliosis may become apparent during or just before the adolescent growth spurt. Common signs and symptoms may include:

• head not centered with the rest of the body
• uneven hip heights or positions
• uneven shoulder blade heights or positions
• uneven arm lengths
• when bending forward, the left and right sides of the back are asymmetrical

When to seek medical advice

Consult your doctor if you notice that your child’s:

• 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

Also, consult your doctor is your child:

• has problems with his genitourinary tract
• has breathing difficulty
• has rapid or irregular heartbeat
• has an irregularly shaped hand, arm or foot

Questions to ask your doctor

If your child is diagnosed with congenital 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

Congenital scoliosis is not thought to be an inherited disease and doesn't tend to run in families. Research studies continue, but there’s currently no reliable way to predict who will develop the condition.

Complications

Complications are uncommon after spinal surgery for congenital scoliosis. There’s a small chance of infection or failure to heal, as well as a small chance of developing a new curvature above or below the fusion. And there is a risk of serious neurological injury (paralysis) that’s higher than with most other forms of scoliosis.

Long-term outlook

The outlook for your child greatly depends on the nature and severity of his congenital scoliosis, as well as any other accompanying conditions:

• Many children function perfectly normally.
   
• Commonly, the spine is shorter than normal, even if treatment isn’t needed.
   
• The spine is stiffer than normal.
  • The degree of stiffness is relative to how much of the spine is involved.
  • Fusion makes the spine stiffer.
  • For some children, this is visible and dictates restricted activities.
     
• For some children with early, severe curves, breathing may be affected.

With successful spine surgery and attentive post-operative care, he can have a spine that grows as normally as possible, with greater torso length than if he hadn’t been untreated.

For teens

Besides the typical issues all teenagers face—from social acceptance to body changes and more—if you have scoliosis and possibly another associated health problem, 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 help correct the curve and prevent further curvature. You’ll also probably grow somewhat taller than if you hadn’t been treated.

Even knowing the benefits of surgery and treatments, you might find this to be a tough time. 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 your child’s spinal surgery, his Children’s pediatric orthopedist and physical therapist can advise you on at-home therapies for him, as well as any home therapy equipment you might need.

Prevention

Your child’s congenital scoliosis developed because his spine didn’t form correctly in utero. Some vertebrae (vertebral segments) of the spine formed abnormally (failure of formation), didn’t separate correctly (failure of segmentation), or there was some combination of both. The condition isn’t hereditary, and there’s nothing you could have done to prevent it.

Congenital scoliosis glossary

• Adams forward bending test: a screening measure for assessing scoliosis
   
• 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 custom-made brace worn after spinal fusion surgery can protect your child’s spine while the fusion heals and becomes fully solid. Bracing is not an effective therapy for treating congenital scoliosis, but can be effective for controlling secondary (compensatory) curves that result from (compensate for) the congenital curve.
   
• 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
   
• compensatory: offsetting, counterbalancing
   
• 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
   
• convex hemi-epiphysiodesis: a procedure performed to stop abnormal growth on the convex side of the spine so that continued growth on the concave side can have a chance to correct the curve
   
• diagnosis, diagnostics: identifying disease or injury through examination, testing and observation
   
• excision: See resection.
   
• expansion thoracostomy (thoracotomy): a surgical procedure in which one or more separations are made between the ribs and the ribs are parted to make the chest larger. Sometimes, congenitally abnormal (fused) ribs are separated; other times, multiple cuts are made in the ribs, or the naturally occurring space between ribs is enlarged.
   
• genitourinary tract: the organ system of the reproductive organs and the urinary system, grouped together because of their proximity and common pathways
   
• hemivertebra, hemivertebrae: abnormal triangular- or trapezoidal-shaped vertebra(e), rather than rectangular-shaped as in normal vertebra(e) (See vertebra.)
   
• hemivertebra resection: a procedure in which the abnormally-shaped vertebrae are removed, and the vertebrae above and below the hemivertebrae are fused together. Instrumentation is often inserted to support the fusion, and the child will wear a cast while the spine heals. This procedure involves some risk to the nervous system.
   
• idiopathic scoliosis: the most common form of scoliosis, mainly affecting adolescent girls. “Idiopathic” simply means that there is no definite cause.
   
• instrumentation: metal rods, hooks, screws and wires implanted during spinal fusion surgery (and some non-fusion procedures) to correct the spinal curve and secure the spine in position while the fusion heals and becomes solid
   
• in situ: in place, at the (original) site
   
• in utero: in the womb
   
• musculoskeletal: relating to the muscles and skeleton
   
• neural (neurological): of, or relating to, the nervous system
   
• neuromuscular: affecting, or characteristic of, both neural (nerve) and muscular tissue
   
• neuromuscular scoliosis: the form of scoliosis that's 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 treatments 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
   
• osteotomy: controlled breaking or cutting and realigning of bone into correct position. A spinal osteotomy may be performed when there is significant rigid deformity of the spine.
   
• 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 and performed either simultaneously or in two stages
   
• progression, curve progression: worsening of a scoliosis curve
   
• radiograph, radiogram: x-ray
   
• resection (or excision): surgical removal of all or part of an organ, tissue or structure
   
• 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 defect 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: congenital (present at birth); neuromuscular (associated with neuromuscular diseases); and idiopathic (no definite cause).
   
• 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 (fusion). A fusion partially corrects a scoliosis curve, stabilizes the curve and stops its progression, as well as balancing the spine and pelvis.
   
• spine (spinal column, vertebral column): the series of moving vertebrae forming the axis of the skeleton and protecting the spinal cord
   
• thoracic insufficiency syndrome: the inability of the chest (thorax) to support normal breathing or lung growth. VEPTR surgery addresses this syndrome in children with congenital scoliosis and other spine and rib problems.
   
• torso: the body, excluding the head, neck and limbs; the trunk of the body
   
• 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 are the building blocks of the spinal column
   
• vertebral column resection: an osteotomy procedure that uses instrumentation to help restore spinal balance in children whose spinal deformity is particularly severe and rigid.