Treatment for BWS at the Paley Institute focuses on alleviating the orthopedic difficulties, namely correcting the limb length discrepancy (LLD) that results from hemi-hypertrophy. Treatment of the discrepancy is based on many factors and is completely individualized to each patient, based on their age and actual or predicted limb length discrepancy.
Treatment for limb length discrepancy can be done in two ways: lengthening of the short limb or shortening of the long limb.
Shortening of the longer limb is generally only considered in patients that are predicted to be quite tall; shortening of the longer limb will result in an overall loss of height as an adult, which is often an undesirable outcome. Shortening of the limb in a growing child is done by surgically closing the growth plate of the bone. It is a precisely timed procedure that is done based on remaining skeletal growth. It is a minimal outpatient procedure that has little to no recovery time.
The alternative to shortening is limb lengthening. Lengthening of the shorter limb can be done with either an external fixator or an internal lengthening device. The internal lengthening device we utilize here at the Paley Institute is the PRECICE intramedullary nail. During surgery the nail is inserted into the canal of the bone to be lengthened and the bone is osteotomized (surgically separated). The PRECICE uses an external remote control (ERC) device that lengthens the nail using a magnetic coupling system. Activation of the ERC causes the PRECICE nail to lengthen similar to an antenna extending. As the PRECICE separates the bone segments, new bone forms in the gap resulting in increased length. For more information on lengthening see Limb Lengthening Center. For more information on the PRECICE see The PRECICE.
We have had great success lengthening patients with BWS using the PRECICE nail. Patients and families are very pleased as to both the results and the limb lengthening process. Most patients with BWS will require only one lengthening to correct the discrepancy. The timing of the lengthening can vary and is based on the current and predicted LLD.
During the lengthening process the patient is expected to stay locally and attend daily physical therapy (PT) as well as follow up appointments every two weeks. It is important to receive daily PT and attend all follow up appointments to avoid developing complications from the lengthening. The successful outcome of limb lengthening is dependent on an entire team effort; everyone at the Paley Institute is dedicated to the sole goal of a safe and predictable outcome.