Testing and diagnosis of congenital scoliosis and congenital vertebral anomalies

The term congenital scoliosis is a misleading one. “Congenital” means something that is present when the baby is born. So “congenital scoliosis” implies that there is always a spinal curve at birth in all patients with congenital scoliosis. In fact, if it’s only the bone malformations that are present at birth, you might not see any difference in your child’s spine. Scoliosis may not develop until later.

Scoliosis is usually not painful, so if the curvature is not detected at birth, it can go undiagnosed until there are obvious signs. This can occur as late as adolescence. Early detection of scoliosis is critical for the best outcomes for your child.

How is congenital scoliosis diagnosed?

To diagnose congenital scoliosis, your CHOP orthopedic team will conduct a thorough family and medical history, perform a physical examination of your child, and may use the following diagnostic tests.

EOS imaging – EOS is a low-dose, 3-D imaging system that scans your child standing up. An EOS scan shows us your child’s natural, weight-bearing posture and allows us to see how the joints interact with the rest of their musculoskeletal system, especially the spine, hips and legs.

X-rays – A primary diagnostic tool for scoliosis to show abnormal bones in the spine and the exact angles of their spinal curve.

Magnetic resonance imaging (MRI) – Also used in congenital scoliosis to detect problems within the bones and to evaluate the spinal cord. 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. CT scans are especially helpful in detecting ribcage problems.

Your child with congenital scoliosis may sometimes have other health issues, as their heart, kidneys, vertebra and spinal cord all form at the same time in utero. Your orthopedic surgeon will refer your child to cardiology, nephrology, and additional spine imaging. An ultrasound using high-frequency sound waves may be conducted. These studies and referrals are to evaluate your child’s organs to detect any differences.

If your orthopedic surgeons detect congenital scoliosis while your child is a baby, we may start treatment as early as six months of age. Early detection is critical for the best outcomes for your child. Learn more about the signs and symptoms of early-onset scoliosis. 

How is congenital scoliosis treated?

At Children’s Hospital of Philadelphia (CHOP), we generally recommend ongoing monitoring of congenital scoliosis by a pediatric orthopedic surgeon.

Nonsurgical treatment for congenital scoliosis

The first line of management for congenital scoliosis is observation. Congenital scoliosis is not typically braced. Your orthopedic surgeon may discuss bracing if the other non-congenital curves in the spine worsen or become progressive.

For children with curves between 25-40 degrees, the treatment we recommend depends on their individual circumstances.

Surgical options for congenital scoliosis

Surgery for congenital scoliosis may be recommended on an individualized basis, and for curves that are greater than 50 degrees and progressive (gradually worsening). 

Spine surgery can stop further progression and often improve congenital scoliosis. Surgery can also be a stressful experience for children and families. At CHOP, 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 surgery a top priority.

The most common surgical treatments for congenital scoliosis are spinal fusion or growth-friendly instrumentation. The right surgery for your child will depend on their age, skeletal maturity, and many medical considerations.

If your child has other health issues like heart, lung or kidney problems, our orthopedic team will work closely with experts and other specialists from our Cardiac Center and our divisions of pulmonology (lung doctors) and nephrology (kidney doctors) to determine the order in which medical issues should be addressed.

Growing rods

For young children with congenital scoliosis, spinal surgery to implant growing rods offers a dual benefit: correcting the spinal curve and allowing continued growth of the spine.

Growing rod surgery is done through the back. In most cases, the curve in your child’s back is spanned by one or two rods under their skin to avoid damaging the growth tissue of the spine. The rods are attached at two spots — above and below the curve. The growing rods will help guide future spinal growth and drive the spine straight.

On a regular interval, your child will return to CHOP to have the rods expanded to keep pace with their growth. In some cases, the growing rods may need to be replaced if they outgrow the maximum expansion level of the rods.

Spinal fusion

After your child has stopped growing, or if growing rods were not considered a viable treatment option, we may recommend spinal fusion surgery to permanently stabilize your child’s spine. In this procedure, their spine is safely straightened and fused together with metal implants that are inserted to correct and hold the curvature.

Vertical expandable prosthetic titanium ribs (VEPTR)

In some cases, your child’s congenital scoliosis may also affect their ribs and lung development. If this is the case, your child may also be diagnosed with thoracic insufficiency syndrome, which means their lungs may not expand enough to receive enough oxygen for their body. Without treatment, thoracic insufficiency syndrome can be fatal.

An innovative surgical device called vertical expandable prosthetic titanium ribs (VEPTR) was invented and pioneered by the late Robert M. Campbell Jr., MD, once one of CHOP’s leading orthopedic surgeons. The VEPTR stabilizes your child’s curved spine, while expanding the space available for their lungs, allowing their ribs, spine and lungs to expand and grow.

Like growing rods, the VEPTR is surgically adjusted as your child grows and reaches skeletal maturity.

Safety in spine surgery

Surgery can dramatically improve the long-term outcomes for your child with early-onset scoliosis, but it can also be a stressful experience for you and your child. At CHOP, we offer a wealth of resources that can help you and your child prepare for surgery.

Additionally, we follow many best practices before, during and after surgery to decrease the risk of infection and increase positive outcomes. Our practices have been adopted by many other children’s hospitals around the world. Some safety protocols our orthopedic surgical team uses include:

• A strict antibiotic protocol before we operate
• The use of innovative navigation and imaging equipment during surgery, like StealthStation® and O-arm® Surgical Imaging
• Continuous monitoring of your child’s anesthesia during surgery, including specialized spinal cord monitoring
• Procedures after we operate (postoperative clinical pathways) to ensure quality standards, and rapid patient recovery

To learn more, read how we make safety in surgery a top priority.

Follow-up care for congenital scoliosis at CHOP

Here at CHOP, we are proud to offer ongoing support and services for your child and family. Our team is committed to partnering with you to provide the most current, comprehensive and specialized care for your child. 

After treatment – whether surgical or nonsurgical – your child’s care team will continue their follow-up care at our Main Campus or one of our CHOP Care Network locations. 

Types of follow-up care

• EOS micro-dose: We may use “EOS micro-dose” X-rays in your child’s scoliosis follow-up. CHOP was the first institution in the U.S. to introduce this method. Micro-dose X-ray technology uses one-third of the standard EOS radiation dose, reducing radiation exposure for children who may require frequent imaging.
• Bracing: If your child is being treated with a brace, they will be re-evaluated every six months until their condition is stable. This usually happens after your child reaches skeletal maturity. After their spine is stabilized, we recommend annual visits to an orthopedic surgery team.
• Growing rods and VEPTR: If your child received growing rods or VEPTR, an additional spinal fusion surgery may be necessary when they have finished growing. Annual visits to an orthopedic surgeon are recommended after spinal fusion.

Please know that we recognize your child’s pediatrician as an important part of their care team. We will give them regular updates on your child's progress. If your child needs continued care as they become an adult, we will also help transition their care to an adult orthopedic team.

What is the long-term outlook for children with congenital scoliosis?

We know that as a parent, a scoliosis diagnosis can feel overwhelming. But it’s important to know that this condition is highly treatable, and with proper care your child can go on to live a full, active life. Even if your child needs surgery, advancements in treatment have greatly improved outcomes, and can allow your child to return to their favorite activities, including things like sports, gymnastics and dance. With early detection and expert care, many children with congenital scoliosis can pursue any activity they choose without limitation. 

If your child has more complex scoliosis, or other nervous system conditions, successful spinal surgery and post-operative care can significantly improve their quality of life. With a straighter spine they may experience enhanced breathing, reduced respiratory risks, as well as better posture and mobility. Here at CHOP, our specialists will work closely with your family to do everything we can to give your child the opportunity to grow, thrive and engage in the activities they love.

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 determined 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 utilize 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.