About Jeune's Syndrome Surgery
In order to determine if surgery is the best treatment for a Jeune's patient, thorough assessments are made by each specialist with our Jeune's team. This team consists of thoracic surgeons, radiologists, pulmonologists and plastic surgeons. Some of the most important tests performed are specialized CT scans that provide a clear, useful picture of the child's lungs and rib cage. Dr. Robert Castile, of our Pulmonology Division, and Drs. Frederick Long and Brent Adler, in Radiology, have developed a new technique for CT scans that controls the breathing and degree of lung inflation during imaging. This has improved our ability to define the anatomy of the lungs and rib cage in infants, as well as provide useful pre-operative and post-operative information.
If the tests determine that the deformity of the chest wall does exist, but symptoms of Jeune's are mild or not present at all, we normally will not recommend taking on the risk of an operation. While there is indeed significant smallness of the chest, since the patients themselves are small in stature, they may be able to live a relatively normal life in spite of the deformity. In that case, ongoing treatment is simply aimed at medical support of their lungs: prompt antibiotics for infection, proper mobilization of secretions, supplemental oxygen if necessary, BiPap or other forms of respiratory support. There is no medical treatment of the underlying deformity. A child with a less severe case of Jeune's will have routine visits with his or her Jeune's specialist, even when no symptoms are present.
Patients, whose tests reveal symptoms of Jeune's, combined with reasonably normal lungs, are considered for Lateral Thoracic Expansion. Dr. Phillips and his team of thoracic and plastic surgeons will perform the first of two surgeries. Each surgery expands one side of the chest cavity and allows for expansion of both the left and right sides, thus doubling the total enlargement possibility. The surgeries are separated by six months to one year.
The procedure begins with ribs 4 through 9 (see diagram), where the skin, tissue and muscles on top of the chest wall are lifted. The ribs are separated from their underlying rib beds and remaining chest wall and are then divided in a staggered position. The underlying chest wall is also divided and staggered in the opposite direction from the rib divisions. The long ends of ribs 5 and 6, as well as ribs 7 and 8, are then pulled apart and put together to expand the chest length. The divided ribs allow for expansion of the chest cavity. The two expanded rib segments are held together with titanium plates, which stabilize the enlargement. The plates act as an internal cast to maintain the expansion while the ribs heal. The rib bed, which has then been exposed, will then make more rib-like boney tissue. The hopeful result of each surgery is to actually create more chest wall that can regenerate more rib.
The average hospital stay for a patient, who is not on a breathing machine prior to surgery and without any complications from the surgery, is approximately one week. Patients who arrive at Children's from other hospitals on ventilators can be returned within a week to their own hospitals where they can complete their recoveries.
Since our procedure is still relatively new and Jeune's is a rare condition, long-term outlook for patients remains unclear. At this point, the surgery has proven successful in older children. Our longest follow up is about 10 years and so far no patient has required re-expansion. We have great hope that rib expansion of the chest cavity will indeed result in expansion of the underlying lung. We would like to emphasize to all parents that there is much more learning, discovering and improving yet to come.
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