Treatment of Early Onset Scoliosis

Causes of EOS

Referring Patients

Observation
Observation is often recommended for children with early onset scoliosis. For young infants with no congenital malformations there is up to 80-90% resolution of scoliosis. It is still worthwhile to refer to a pediatric spine specialist, because there are certain radiographic and clinical features in infants that indicate high risk of progression. These children often can be successfully treated with serial spine casting.

Children with congenital scoliosis also are often observed when they are young. The risk of progression and rate of progression will vary based on type of congenital scoliosis. Referral to a pediatric spine surgeon is advised, as children will require unique care based on their individual deformity.

Other varieties of early onset scoliosis also may be managed with observation initially even if surgery is anticipated. This generally is done to allow for additional longitudinal growth and pulmonary development as long as possible prior to attempting to alter the natural history with casting or surgery.
Casting
Casting is increasingly being utilized for the treatment of early onset scoliosis. A cast is used to guide growth of the scoliosis into a straight spine, similar to how a crooked plant can be made to grow straight by tying it to a stake.

For some children, however, casting is used as a means to prevent or delay progression of scoliosis. This is especially true in cases of children with congenital spine and chest deformities, or scoliosis related to syndromes. Casting for infantile idiopathic scoliosis has variable success rates in the reported literature. Children who typically have better results with casting start with smaller deformities and at a younger age. If the scoliosis can be corrected to less than ten degrees most physicians will transition to a brace for a variable period of time and observe before declaring the scoliosis “cured”.

Recent data has also demonstrated the effectiveness of casting in delaying need for operative treatment up to 3 years even in children with congenital, syndromic, and high magnitude deformities

Application of a spine cast is done in a procedure center or operating room under anesthesia. Intubation is recommended due to the elevation in inspiratory pressures required for ventilation while the cast is being applied. No incision is made. Casts are typically changed every 2-4 months based on child’s age (longer interval as children get older). Some doctors place casts that go over the shoulder, some do not go over the shoulder.

Bracing
Bracing is also used for management of early onset scoliosis. There is less data on the effectiveness of bracing for early onset scoliosis compared to casting. Often bracing is used in conjunction with casting either during summer “breaks” or after your child’s spine has straightened with casting.
VEPTR™
VEPTR DeviceVEPTR™ stands for vertical expandable prosthetic titanium rib. It is a device that was developed to treat children with severe malformations of their chest and spine. It is an FDA approved device. VEPTR™ is now used for various spinal and chest deformities in young children.

The VEPTR™ was initially developed in San Antonio by Dr Robert Campbell and Dr Melvin Smith for children born with multiple fused ribs and spinal deformities related to syndromic conditions such as Jeune’s syndrome and Jarcho-Levin syndrome. Use of the VEPTR™ allowed these children who previously were at high risk for early demise due to restrictive lung disease to prolong their lives by allowing for thoracic volume growth and pulmonary development that was previously restricted due to their chest deformities.

Use of VEPTR™ has been expanded by surgeons to numerous other types of early onset scoliosis. Surgeons from the Children’s Spine Foundation are actively involved in research to evaluate the benefits and risks of VEPTR™ in various disorders. The VEPTR™ has been demonstrated to increase lung volumes and correct spinal deformity but does have a fairly high incidence of complications. These include infection, broken ribs, movement of implants, nerve injury, and prominence causing pain.

VEPTR™ relies on attachment sites on the ribs proximally and either distal ribs, spine or iliac wings distally based on the pathology being treated. Either hooks or pedicle screws can be used for spinal anchoring; hooks are usually used for anchoring on the iliac wings. Each site is attached to a rod and they telescope on each other to allow for expansions over time. If there are significant rib fusions, a thoracoplasty with separation of the ribs is also usually done at time of VEPTR™ insertion. The goal of VEPTR™ insertion is to correct spinal and chest deformity without fusing the spine. While children are growing VEPTR™ is generally expanded every 6-12 months in the operating room.
Growing Rods
Growing RodGrowing rods are placed along a child’s spine and connected to anchors on the spine. The term is a misnomer as the rods don’t “grow” but require surgery periodically to increase the construct length. The spine anchors can be hooks or pedicle screws and are placed above and below the curve. The anchored area at both ends is fused to provide strong support. The curved part of the spine remains unfused. Rods are attached on each side of the spine to the proximal and distal anchors. The rods connect either with a bracket that allows rod overlap or a central adaptor that connects the rods. Similar to the VEPTR™, growing rods help correct spine and chest deformity and need to be lengthened every 6-12 months in the operating room.

Growing rods are FDA approved. In contrast to VEPTR™ which was developed for syndromic scoliosis and is now being used by surgeons for other diagnoses, growing rods were developed for idiopathic scoliosis and now is being used by surgeons for syndromic and congenital scoliosis. They have similar risks to VEPTR™ including infection, movement of implants, nerve injury, inadvertent spinal fusion, and prominence causing pain.
MAGEC™
Magnetic Growing RodMAGEC™ rods are a specific type of growing rods. They are implanted in a similar manner to VEPTRs and growing rods. Surgeons can decide to pair this rod with other implant systems that attach to the spine including pedicle screws, hooks, wires, and anchors from the VEPTR™ system. An innovative and unique feature of MAGEC™ rods are that they may be lengthened with the use of an external magnetic controller without the patient having to undergo an invasive procedure or even be placed under anesthesia. The surgeon or his or her staff perform the lengthening in the doctor’s office. Due to the relative ease of the lengthening experience, many surgeons adopt a schedule of implant lengthening that is more frequent than the every five to six months used in VEPTRs™ and growing rods. Risks of MAGEC™ rods include the risks associated with VEPTRs™ and growing rods in addition to the risk of failure of the mechanical aspects of the lengthening mechanism. The implant is made by Ellipse Technologies, Inc. of California.
Stapling
StaplingStapling of the spine is a new technique that has demonstrated effectiveness in specific patterns of early onset scoliosis. The best results with stapling are achieved in children aged 4-10 with idiopathic scoliosis and curve magnitudes less than 40 degrees. Stapling has not been effective in more severe scoliosis or scoliosis associated with syndromes or congenital scoliosis. Indications for stapling are similar to those for bracing in juveniles with scoliosis. Stapling acts like an “internal brace” for the spine and can help correct scoliosis through altering the growth pattern of the spine. A metal staple is inserted on one side of the curved spine to limit its growth. It can often be done thoracoscopically. Stapling is not approved by the FDA and is only offered at a limited number of hospitals.

Tethering
Tethering is similar to stapling and can alter growth of the spine in an attempt to straighten it. It is not as well studied as stapling. Tethering can often be done thoracoscopically. Very few centers have used tethers on humans and the only human reports are case studies, with variable success. There are numerous animal studies published and also actively being conducted to evaluate tethering in scoliosis. Tethering is not approved by the FDA.

Shilla
ShillaThe Shilla technique involves doing a short spinal fusion at the most curved portion (apex) of the spine using pedicle screws and rods. The rods are attached then at the top and bottom of the spine to pedicle screws without locking plugs that allow for continued growth of the spine. The rods “slide” through the screws at the proximal and distal ends. The Shilla technique remains experimental, with limited data about its effectiveness. Insertion of pedicle screws successfully and safely requires a high degree of technical skill and experience. Inadvertent fusion of the spine away from the apex is a risk. The benefit of the Shilla technique is that if successful it does not require repeat trips to the operating room for expansion as is necessary with both VEPTR™ and growing rods. Complications of the Shilla technique are similar to growing rods and VEPTR. Availability of the special screws and rods needed for Shilla technique are limited in the United States.

Alternative Techniques
There are many other proposed treatment methods for early onset scoliosis. These include chiropractic care, acupuncture, stretching, and massage. Various braces, medications, exercise programs that propose to treat early onset scoliosis are also easily found on the internet. All of these various methods have not been scientifically studied and have not been demonstrated to show effectiveness in treating scoliosis. They often require significant out of pocket financial investment. These alternative treatments may have a role in reduction of discomfort, but are not believed to alter the natural history of early onset scoliosis.
Definitive Fusion
In some cases, spinal fusion may be indicated for a child with early onset scoliosis. Long fusions, especially in the thoracic spine, have been largely abandoned in young children because of the adverse affect on long-term pulmonary function. Fusion of the thoracic spine will stop longitudinal growth of the chest. Research has demonstrated adverse affects on pulmonary function in adolescence for children who had extensive spinal fusion at a young age

In some cases, however, a short fusion may be appropriate to try and prevent more serious progression of scoliosis. Short fusion is also done as part of the Shilla technique and the growing rod technique.

Usually a short fusion of the spine is done when a child has a hemivertebra. Most surgeons are now comfortable removing a hemivertebra and doing a short segment spinal fusion to stabilize the spine from a posterior incision. In many cases this can completely straighten the spine and prevent need for future surgeries. Usually at most a brace will be used for postoperative immobilization, however, if the surgeon cannot get adequate spinal fixation a cast may be required for 3-6 months to hold alignment while the spine fuses.

Future Directions
There is a large amount of innovation occurring in the treatment of early onset scoliosis. One of the most promising developments is spinal implants that can be lengthened externally, without surgery. Experimental devices with this feature have been implanted in children in Europe and Asia. They are not available in the United States. The safety profile and effectiveness of these devices are not yet known.

Additionally, there is hope that in the future we will be able to correct scoliosis without needing surgery. There is a genetic test commercially available that helps predict risk of progression in scoliosis (ScoliScore), however, it is designed for adolescents and its use is not recommended for children with early onset scoliosis.

Causes of EOS

Referring Patients