Medical Tutorial

The foundation of Nationwide Children's Hospital's approach to treating severe cases of Jeune's Syndrome lies in the fact that the chest cavity does not allow proper breathing room and additional space must be created within the thoracic area. We became involved with Jeune's when we learned of a patient activist group in 1992 seeking a facility that would undertake surgical treatment of this rare disease. After thorough assessments and scrupulous research, we began to formulate a procedure that we believe would be effective.

Treatment for all Jeune's patients begins with a series of tests by our multidisciplinary team of Jeune's specialists. Less severe cases of Jeune's are typically then treated with various forms of medical support for the lungs. Severe cases normally require surgery. Our unique surgical procedure expands both the left and right sides of the chest, which eventually allows for the type of room needed for breathing. It is a two-stage procedure, which involves linking the ribs together with titanium struts, as well as creating more of a chest wall by sliding out the rib from the underlying rib bed (see photograph). Specific details of our surgical procedure are provided in The Surgery page of this section.

Since 1993, our thoracic surgery, pulmonary medicine and plastic surgery teams have combined their skills. They have performed 24 surgeries on 14 patients. Our results have been extremely successful in patients older than two years of age. Of seven patients who were two years or older, we have seen significant clinical improvement. Several cases have shown a measurable increase in lung capacity. Patients who present in the first year or two of life have a more severe form of the problem and may have underlying lung disease which cannot be helped with simple expansion of the chest wall. On the other hand, we have had success in this age group if the lungs are normal. A new technique in CT imaging has improved our ability to define the anatomy of the lungs and rib cage in infants, as well as provide useful pre-operative information.

We are careful to emphasize to all parents that while Nationwide Children's has had some of the largest experience to date treating Jeune's, this remains largely unknown territory. Our procedure however, has proven to do well and improve quality of life in appropriate children. We continue to offer the procedure to appropriate patients and add even more to the useful lifetime of these patients.