Early Onset Scoliosis Center

Scoliosis Patient Rides Again Thanks to Life Changing Surgery

Welcome
Making the Diagnosis
   ScoliScore™

Treatment Options
   Casting for Infantile and Juvenile Scoliosis
   Spinal Fusion (Not Recommended)
   VEPTR and Growing Rods
   Spinal Stapling
   Spinal Stapling and VEPTR or Growing Rods
   Casting

Early Onset Scoliosis Research
Quality of Life
Case Study

Welcome to the Early Onset Scoliosis Center

The Center for Early Onset Scoliosis is led by Michael Vitale, MD, chief of pediatric spine and scoliosis surgery at NewYork-Presbyterian Morgan Stanley Children's Hospital and the Ana Lucia Associate Professor of Clinical Pediatrics and Orthopaedic Surgery at Columbia University College of Physicians and Surgeons. More than 400 patients under the age of five are seen each year in the Center by Dr. Michael Vitale, Dr. David Roye and Dr. Benjamin Roye.

While more than 100,000 children in the U.S. are diagnosed with scoliosis each year, most patients are diagnosed between ages 10 and 15 with Adolescent Idiopathic Scoliosis. Scoliosis identified at birth or up to the age of eight is rare, and is categorized as Infantile Scoliosis, Juvenile Scoliosis or Early Onset Scoliosis. The cause of Infantile Scoliosis is unknown; however considerable medical research has led to the development of hypotheses that include intrauterine molding, postnatal positioning and genetic influences.  Click here for more in-depth description. Early Onset Scoliosis is often associated with other diagnoses including congenital chest wall deformities, neuromuscular disorders such as cerebral palsy, myelomeningeocele or muscle disease, or spinal pathologies such as a tumor. Early Onset Scoliosis is a rare occurrence, requiring special knowledge and experience to solve the complex issues surrounding this condition. 

Early Onset Scoliosis (EOS) includes children with Infantile Scoliosis (from birth to three years) and Juvenile Scoliosis (ages three to eight years). Those with the infantile form will generally fall into two groups: those whose curvature disappears with growth and those whose curvature becomes progressive. Infantile scoliosis occurs more often in boys than in girls. Juvenile scoliosis is defined as children identified with a ten to fifteen degree curvature up to the age of eight — these children must be closely observed, as progression of the curve is common.

There are many different causes of early onset scoliosis therefore it is critical to perform the appropriate examination and tests. In addition to close observation, a careful neurologic exam, a spinal MRI and a series of x-rays to review if other structural problems exist are needed. Other associated problems may dictate other examinations or consultations including cardiac, pulmonary or renal function testing.

Making the Diagnosis

ScoliScore™ Predicts Risk of Severe Scoliosis
ScoliScore™, a groundbreaking molecular test that helps predict the risk of spinal curve progression, uses a DNA sample from the patient’s saliva. Within about two weeks, a report is sent to the physician with a score indicating the child's likelihood of having scoliosis that will progress. The scores are grouped into low, moderate and severe categories.

The ScoliScore test is intended for patients:

• With a primary diagnosis of adolescent idiopathic scoliosis (AIS), the most common type of scoliosis
• Over the age of 9 who are deemed “skeletally immature”
• With a mild scoliotic curve (defined as <25°), or
• With a moderate scoliotic curve (defined as >25°, but less than 40°)

If the test shows a patient has a high risk for serious spinal curvature of 50 degrees or more, Dr. Vitale and his colleagues can intervene earlier than they would otherwise, such as by prescribing a back brace. And since less than 10 percent of teens with scoliosis progress to the point where spinal fusion surgery is necessary, the new test can also prevent unnecessary testing. Radiation exposure from diagnostic X-rays is associated with increased risk of problems with bone and breast tissue (girls are more likely than boys to have scoliosis).

An estimated 4 percent of children between the ages of 10 and 16 have AIS, making up 80 percent of all scoliosis cases. The ScoliScore provides doctors with information about the likelihood that an abnormal spine curve will get significantly worse or stay the same, which allows for earlier intervention and helps guide treatment.

ScoliScore is now available to a wider age range of children. Previous indications stated that to be eligible for the test, a patient must be between the ages of nine and 13. New indications now state that there is no age limit to receive the ScoliScore as long as the physician deems the patient “skeletally immature.” This new regulation allows children over the age of 13 who meet the inclusion criteria to benefit from this breakthrough testing.

ScoliScore feedback from patients and their parents has been extremely positive. Read patient testimonials here.

Treatment Options*

Many children with EOS do not require treatment — close clinical follow is needed, but many do not progress. For those who do demonstrate progressive curves there are a number of options outlined below. For children who experience progression of scoliosis, early intervention is indicated to prevent chest wall deformity and to allow normal lung development.

The pediatric orthopaedic surgeons at Children’s Hospital are dedicated to advancing treatment options for Early Onset Scoliosis through research and clinical innovation. 

* Upon meeting, each child will be evaluated and appropriate treatment options will be discussed. Although early results are positive, the new treatments using VEPTR, Growing Rods and Stapling do not have an extensive history. Results may vary for each patient.  

Casting for Infantile and Juvenile Scoliosis
The very young child with scoliosis is often not a candidate for surgery and in fact may not need surgery. The EOS Center at Children’s Hospital has state of the art non-invasive treatment techniques available for these children. The patient has a cast applied to the trunk on a specially designed table that allows us to control and correct the curves. The cast is changed regularly until the curve is appropriately reduced.

Our infantile idiopathic casting program utilizes an Amil casting frame and follows the techniques of Dr. Mehta to utilize growth as a corrective force in the treatment of progressive infantile scoliosis. Further information about casting, including tips for care of the young child in a spinal cast, is available at www.infantilescoliosis.org.

Spinal Fusion is Not Recommended Treatment for Early-Onset Scoliosis in Young Children
Ground breaking research in 2007 by the Pediatric Orthopaedic Research Team at Children's Hospital revealed that spinal fusion, once a standard practice when treating Early Onset Scoliosis, prevents growth of the spine and thorax during a critical period of lung development. The study followed 27 patients who received spinal fusion, which permanently connects several vertebrae. After 10 years, their pulmonary function, measured by lung volume, and reported quality of life were significantly less than that of a healthy child.

"We recently presented evidence that spinal fusion in young children can lead to significant issues in quality of life and pulmonary function over the long term," says Dr. Vitale, who presented the findings at the International Conference on Early Onset Scoliosis in Montreal.

Surgeons at Children’s Hospital and other specialized centers, therefore, attempt to avoid spinal fusion in young children with early-onset scoliosis. Where spinal fusion has been shown to improve quality of life and life expectancy in adolescents who have achieved normal pulmonary capacity before the onset of scoliosis, this treatment has quite a different result in skeletally immature children. Spinal fusion irreversibly limits growth of the patient's spine, thorax and lungs and may result in progressive pulmonary insufficiency. 

In the webcast, "Avoiding Fusion in Early Onset Scoliosis: Growing Rods and the VEPTR™ (Vertical Expanding Prosthetic Titanium Ribs) Treatment Option for Children Suffering From Early-Onset Scoliosis,” Children’s Hospital surgeons discuss why spinal fusion should be avoided and the new techniques that are available to treat young children with Early Onset Scoliosis. These new options offer hope and significantly improve patients’ outcomes and quality of life.    

Avoiding spinal fusion is the goal, however, there may be times when it is the only treatment option. As mentioned, all options will be discussed when determining the most effective treatment plan specific to each patient.  

VEPTR™ and Growing Rods
Treating Early Onset Scoliosis with growing rods and/or VEPTR permits continued growth in the spine, maximizes space available for lungs and enhances pulmonary function. VEPTR (Vertical Expandable Prosthetic Titanium Rib) is the most advanced treatment option for children with Thoracic Insufficiency Syndrome (TIS). While the condition is rare (less than 4,000 children in the United States each year), children with TIS have severe deformities of the chest, spine and ribs that prevent normal breathing and lung development. VEPTR straightens the spine and opens a larger space for the lungs and other internal organs to grow by placing a titanium device between two ribs to push them apart.

In the post operative period, there is no casting or bracing necessary and regular activities are not limited.

VEPTR expands to allow for rib cage and lung growth as the patient grows. Children receive expansions, an outpatient surgical procedure, every four to six months.

“Before VEPTR, we had no way of dealing with the entire chest wall,” explains Dr. David P. Roye, Jr. “Straightening the spine without growing the ribcage was not enough. Now we can straighten the spine while we increase room in the rib cage for the lung.”

Learn more about VEPTR here.

Growing Rods are used in a similar fashion to VEPTR. Rods are attached to the spine and affixed to vertebrae at the top and the bottom. Growing rods are expanded over time using a mechanism that allows the lengthening to be performed in a simple outpatient surgery. The approach minimizes spinal deformity, allows spine growth and most importantly allows lung development to occur to preserve a normal life span for the patient.

Learn more about Growing Rods here.

By increasing pulmonary capacity as well as straightening the spine, these treatments provide significant quality of life improvements and promote normal respiratory function.

The complex care required by children diagnosed with Early Onset Scoliosis has tended to limit their treatment to children’s hospitals offering a convergence of strong orthopaedic, pulmonary and ICU facilities. Children’s Hospital’s teams have significant experience in applying these techniques and are continuing to develop and test new techniques, and share in research, with the expectation of even better outcomes in the future.

Spinal Stapling
Children's Hospital is one of only a few hospitals in the country to offer spinal stapling, a new treatment alternative for scoliosis patients who have progressive scoliosis at a young age. Spinal stapling modulates growth, allowing correction of curves without fusion and without the necessity of multiple procedures.

"Stapling not only stops scoliosis from getting worse, but can even correct the curve. While stapling is very new," says Dr. Michael Vitale, "it promises to have a major effect on how we treat young people with scoliosis."

Spinal stapling is a two-hour minimally invasive surgery that involves implanting inch-long metallic staples across the growth plates of the spine. Made of a high-tech temperature-sensitive metal alloy, the staples are implanted using a camera called a thoracoscope with a very limited incision and minimal scar. The procedure is available to children with progressive moderate scoliosis (less than 30 degrees) who are still growing (girls up to age 14 and boys up to age 16).

Spinal Stapling and VEPTR or Growing Rods
For children with larger curves, the benefits of growing instrumentation — Growing Rods or VEPTR – can be combined with spinal stapling. This new hybrid technique is being applied for curves greater than 35 degrees and patients are showing tremendous outcomes. Children return to active vibrant lives just months after surgery. Adjustments are made every six to nine months during their growth periods and usually completed on an outpatient basis.

Early Onset Scoliosis Research

Physician-scientists of the Pediatric Orthopaedic Research Team at the Children’s Hospital conduct their own research to advance the care of children with musculoskeletal concerns, and are among an elite group of spine surgeons selected to participate in national research groups. The Chest Wall and Spinal Deformity Study Group is comprised of 20 members from across the country who are interested in defining new ways to treat severe deformities that affect both the spine an the rib cage. This group is collecting data on the outcomes of patients treated with VEPTR.

Also, members of the Spinal Deformity Study Group, a research committee of 50 surgeons from across North America, focus on multicenter studies of rare conditions such as Infantile Scoliosis and Early Onset Scoliosis.

Quality of Life

Studies to measure the impact of a child’s health on his life and his families’ life gather data in a number of domains including physical functioning, mental health and the impact of the child’s condition on the caregiver’s time and outlook. However, many of these studies are unable to accurately measure quality of life in Early Onset Scoliosis because of their compromised conditions. One of the major goals of the Pediatric Orthopaedic Research Group at Children’s Hospital is to develop new methodologies to measure the impact of Early Onset Scoliosis and the care of Early Onset Scoliosis with children whose quality of life we have not been able to measure before.

In a recent analysis, the Research Group found that patients with Thoracic Insufficiency Score (TIS) among the lowest in all pediatric populations in almost all domains when measuring quality of life. This is a significant benchmark that will allow effective measurement of the impact of the VEPTR treatment. Patients receiving VEPTR treatments return for adjustments every four to six months as they grow so that the device can be lengthened or replaced, until the chest cavity is large enough to support adult sized lung capacity. The Research Group will continue to collect data to understand the impact of this life-saving treatment.

Research

Below is a list of current and research conducted by the Pediatric Orthopaedic Research Group relating to Early Onset Scoliosis. Led by Dr. Michael Vitale, this group is engaged in multiple research projects each year and present their findings at national and international medical sympoisia. In addition to leading his own research team, Dr. Michael Vitale is a member of the Board of Directors for the Chest Wall and Spine Deformity Study Group, a research group dedicated to improving the quality of care and the outcome of treatment for patients and families dealing with chest wall and spine deformities. Dr. Vitale guides the Group's research direction and is an active participant in both retrospective and prospective studies. To see the 2010 annual report of the Chest Wall and Deformity Study Group, click here

Case Study

My 12-year-old son Jason was born 14 weeks premature. He had several complications due to his prematurity including scoliosis.

After a huge misstep with an orthopedic doctor when Jay was a baby, we luckily found Dr. Vitale. He has been Jay's doctor/surgeon for most of his life. I can't say enough about the level of care that Jay receives from Dr. Vitale and his staff. Read our story and others about families dealing with Early Onset Scoliosis at  www.vertebralstapling.com.

Lisa, Jason's Mom, 2/4/09