When determining if bringing your child to Nationwide Children's Hospital for assessment is the right choice, it is critical to review and understand our expertise in this area, and to then discuss it with your child's doctor.
Our current Jeune's patients fall generally into two categories: those requiring surgery and those who normally only need medical support of the lungs. Both groups of patients require routine visits to their Jeune's specialists following treatment.
The majority of our experience has been with children who are indeed candidates for the Lateral Thoracic Expansion surgery. The largest experience we have had with this extremely rare condition is with patients over two years of age, all who had positive clinical results after the surgery. In many of the cases, we have been able to measure significant improvement in the ability to breathe.
We have successfully completed surgery on other children under the age of two, after obtaining a normal CT scan to rule out any disease in the lung tissue or airways. In these patients under age two, we have been able to significantly reduce, and sometimes eliminate, the use of a ventilator. Our goal is to continue to very slowly and completely remove these children from the ventilator. However, there can be significant mental, emotional attachment to the ventilator, which can limit the recovery ability in chronic patients.
Our only two patients who did not respond well to surgery were both under one year of age. After reviewing their cases, they both had significant lung disease before surgery. They passed on from the progression of the lung and airway disease, not from complications related to the Lateral Thoracic Expansion. The surgery did not harm them; they simply did not benefit from the operation. Based on this experience, we normally do not recommend the procedure in infants with underlying lung disease.
In conclusion: We remain enthusiastic about the procedure for children two years of age or older with severe cases of Jeune's. We generally recommend a staged Thoracic Expansion Procedure for these patients. We recommend the procedure for infants under one year of age, only after determining that they have otherwise normal lungs.
Although Nationwide Children's Hospital has one of the largest groups of Jeune's Syndrome patients in the world, our approach will likely continue to evolve as we gain ever more experience. As time goes on we will continually evaluate the durability of the repairs and determine if it is necessary or feasible to make further expansions to either side of the ribcage.