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Jeune's Syndrome, or asphyxiating thoracic dystrophy is a congenital deformity of the chest wall, characterized by an extremely small chest cage that creates insufficient space exists to allow proper and efficient breathing. The chest cavity does not grow well with time. As a result, these children slowly and progressively run out of room to breathe, and therefore, asphyxiate. A child with Jeune's Syndrome may have symptoms that develop as newborns, or as late as four to five years of age, depending on the progression of the condition. Because the chest is very small in these patients, they have respiratory difficulty that can range from mild to severe. Mild symptoms include a slightly rapid breathing rate. Children with more severe symptoms may suffer from recurrent pneumonia, and children with the most severe form of Jeune's Syndrome may be unable to breathe without help from a breathing machine, or ventilator. A child with Jeune's Syndrome is typically small in overall stature, and may have liver or kidney disease.
Many children with Jeune's Syndrome are candidates for Lateral Thoracic Expansion. The largest experience we have had is with patients over two years of age, and these patients have all had positive clinical results. In many of the cases, we have been able to measure significant improvement in the ability to breathe. Subsequently, we have successfully done surgery on other children under the age of two, but we have required 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, but not eliminate, the use of a ventilator. Only two patients in our series did not respond well to treatment, and both of these children were under one year of age. However, review of their cases indicates that they both had significant lung disease before the operation, and they passed on from the progression of the lung disease, not from complications related to the Lateral Thoracic Expansion. The surgery did not harm them; they simply did not benefit from the operation.
We will typically expand one side of the chest cavity at a time, with each surgery separated by six months to one year. The surgery involves a collaborative effort between thoracic surgery and plastic surgery. Ribs four through nine are approached. After elevating the skin, tissue, and muscles overlying the chest wall, the ribs are separated from their underlying rib beds as well as the remainder of the chest wall. The ribs are divided in a staggered fashion. The underlying chest wall is also divided in a staggered fashion in the opposite direction from the rib divisions. The long ends of the fifth and sixth ribs are then separated and put together to expand the diameter of the chest. This is also done with the seventh and eighth ribs. The divided ribs allow for expansion of the chest cavity. The two expanded rib segments are held together with titanium plates to stabilize the enlargement. The plates act as an internal cast to maintain the expansion while the ribs heal.
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 have come to us on ventilators or from other hospitals can be returned within a week to their own hospitals, where they can complete their recoveries. We believe that any child with Jeune's Syndrome who is symptomatic can be considered a candidate for Lateral Thoracic Expansion if the lungs are reasonably normal. We expect health and developmental improvements with the use of this procedure, and we can return the patient promptly to his or her hometown hospital for recuperation and follow-up care. Only minimal follow-up care will need to be done at Nationwide Children's Hospital.
A portion of the surgery description was taken from the Winter 2001 issue of Pediatric Directions, "Creating Breathing Room: Nationwide Children's Surgical Approach to a Rare Disease", by J. Terrance Davis, MD, Robert L. Ruberg, MD, & Robert G. Castile, MD
We do not know whether or not re-expanding the chest is possible at this time. Over our experience to date, we have not needed to re-expand a patient's chest cavity.
Our operation involves creating new chest wall area entirely from the patient's own tissue in a way that ultimately heals and is not dependent on foreign material, once healing has occurred. Thus, it is what we would call "autologous" in its nature-that is entirely made up of the child's own body. We use small Titanium Plates only to act as internal casts- to keep the bone together until actual bone to bone healing occurs. New bone is formed from the rib bed released from the old rib, and thus more rib area is created.