Treatment for Russell Silver Syndrome focuses on correcting the short stature and limb length discrepancy (LLD). Most patients with RSS have an LLD, typically around 3 cm.
Many RSS patients are first treated with human growth hormone (hGH) to correct their short stature and growth problems. The growth hormone can increase height, but it will not correct any LLD present.
To correct LLD, are two options: epiphysiodesis and limb lengthening. Epiphysiodesis is a guided growth procedure in which small metal plates (8-plates) are inserted into the growth plate of the long bone (usually the femur). This impedes growth of that bone. Epiphysiodesis can be used to correct LLD by inserting the 8-plates into the unaffected leg. This allows the shorter leg to “catch up” during growth. The major downside to epiphysiodesis in RSS patients is that the procedure results in reduced adult height. Since RSS patients are short to begin with, further decreased height is not typically desired.
The second option is a limb lengthening procedure. Dr. Paley uses the most advanced limb lengthening procedures. We use the PRECICE intramedullary nail to lengthen the femur and correct LLD in patients with RSS. The femur is osteotomized (cut), and the PRECICE is inserted inside the medullary canal of the bone. The PRECICE lengthens using an external remote control (ERC) that uses magnets to extend the PRECICE nail. The nail lengthens the femur by approximately one millimeter per day.
During the lengthening procedure, the patient will receive daily outpatient physical therapy. After the desired length is achieved, no more modifications are made to the PRECICE nail. Within a few years the nail is removed in a minor outpatient procedure.
For more information see Limb Lengthening Center
Dr. Paley has achieved excellent results using the PRECICE to equalize LLD in patients with RSS. Dr. Paley is the most experienced limb lengthening surgeon in the world and the PRECICE is the most technologically advanced lengthening device available today.