It is important to remember that a given individual with WAGR syndrome may or may not have or develop the condition listed below.
|
| Scoliosis and WAGR Syndrome |
Some children and teens with WAGR syndrome also have a diagnosis of scoliosis (curvature of the spine). In one study, there were 7 cases of scoliosis in 54 patients with WAGR syndrome (1).
While the total number of children who have both of these conditions is unknown, information about current trends in scoliosis treatment and research – and how these may apply to individuals with
WAGR syndrome - may be helpful for both parents and physicians.
Recommended treatment for moderate to severe scoliosis is usually a body brace, worn up to 23 hours a day, 7 days a week, or spinal surgery. Unfortunately, there are some children with WAGR syndrome who, because of behavioral and/or cognitive issues, could not tolerate wearing a brace. These issues may also make the prospect of spinal surgery seem out of the question.
The alternative is not to treat the scoliosis at all, in which case the curvature of the spine may continue and increase, potentially resulting in pain, deformity, and eventually even compromised heart or lung function.
Families and physicians considering these dilemmas may wish to consider the following information:
Bracing: For over 50 years, various types of body braces have been used to treat moderate scoliosis. The goal of bracing is to stop the spinal curve from getting worse until the child reaches skeletal maturity and stops growing. Problems with bracing include the fact that some curves continue to progress despite the brace, and some curves progress not only after maturity, but throughout the rest of the person's life. Another problem is that some experts believe that bracing has not been adequately studied, and that there is little proof that this treatment truly alters the natural course of the disorder. In other words, it may be that some curves will stop progressing on their own, and other curves will continue – regardless of whether a brace is used.
Also, for typical children with scoliosis, brace treatment involves many additional issues, from poor compliance and parent/child relationship difficulties, to altered body image and lowered self-esteem. Researchers wonder whether the benefits of bracing are worth the significant problems that may result from it. A large multi-center study has currently underway, which hopes to answer these and other questions about brace treatment:
http://itsnt166.iowa.uiowa.edu/uns-archives/2006/july/071906scoliosis.html
Surgery: In many cases of severe or progressive scoliosis, surgical treatment is recommended. Spinal surgery is done to correct the curve as much as possible, and to fuse the vertebrae to prevent the curve from progressing further. Techniques for this type of surgery have improved dramatically over the years, but it is still a complex operation, and recovery from it can be a challenge for any patient.
If the person with scoliosis is unable to comprehend or to cooperate with restrictions on their movements after surgery, there is concern that they may cause significant injury to themselves. For this reason, parents may feel that they have no choice but to forego surgical treatment for their child.
However, there are options for safe surgical treatment of these patients. Orthopedic surgeons who treat infantile and juvenile forms of scoliosis are often familiar with techniques which work with very young patients. Some of these same techniques can be successfully applied to older patients who have significant cognitive or behavioral problems. These techniques include: a coordinated, team approach to care of the patient, scrupulous attention to post-operative pain control, and post-operative sedation and/or anti-anxiety medications as needed. Although post-operative bracing is not often indicated for most typical patients, it may be considered for some patients with special needs. Finally, in-home nursing care may be arranged after the patient is discharged, to provide continuing assistance with lifting/positioning as well as with administering appropriate medications.
Finally, parents and physicians should be aware of a landmark study completed in 2003:
“Health and Function of Patients with Untreated Idiopathic Scoliosis: A 50 year Natural History Study” (Weinstein, et al):
http://jama.ama-assn.org/cgi/content/full/289/5/559
“Findings from the longest-running study of its kind show that individuals with untreated late-onset idiopathic scoliosis (LIS) have life expectancies and health outcomes that are similar to the general population. The findings challenge a prevailing belief that this type of scoliosis, if left untreated, inevitably leads to severe disability.” http://www.uihealthcare.com/news/news/2003/02/10scoliosis.html
|
|