My 13 year old son was just diagnosed with scoliosis. He already has a curve of 39 degrees (top, to the right). His orthopedist is recommending 22/23-hour brace. Is there any treatment protocol for CdLS children with scoliosis? Is there any information on how well our children tolerate braces? I can't imagine my son keeping a brace on. How does one determine growth potential and consequently potential for further curvature? At 13 years old my son is just under 4 feet tall and weighs about 44 pounds. He looks about 7 years old. Do we treat his body as that of a 7 year old with 11 or more years of further growth or as a 13 year old with 5 or so years of continued growth? Or does it not matter?
In answer to your questions about scoliosis in CdLS and your son's management, first, scoliosis is not common in children with CdLS. There is no specific protocol for treatment and children are managed as any child would be. That means a brace for curves of about 25 to 50 degrees magnitude and surgery for curves greater than 50 degrees. Brace treatment is often not well tolerated by children with CdLS and even if it is, the brace may not stop progression of the curve. It can be successful and is worth trying, but brace treatment requires the cooperation of the child in terms of compliantly wearing the brace and tolerating the occasional discomfort and annoyance of the brace. If your son does tolerate the brace, the endpoint of treatment would most likely be the end of his growth and that would be determined by the cessation of his increase in height as well as some specific x-ray signs. The best estimate would be about 5 to10 years, but that may not be correct.
The treatment for a curve that is greater than about 50 degrees is a spine fusion operation, actually a very safe procedure that uses rods attached to the spine to correct the curve and bone graft to maintain the correction. Experience with this is very, very limited in CdLS, but the risks are very low and one would expect the results to be quite good. The hospital stay is about a week and no cast or brace is necessary after the surgery. Some feel that surgery may indeed be much more appropriate and "conservative" than years of difficulty dealing with a brace program.
In summary, I think that ideally the trial of a brace now is most appropriate. If it is not successful, then follow-up visits are in order. If the curve goes much beyond 50 degrees, surgery should be considered. I hope this information will be of some help.
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