If your child has been diagnosed with Jeune's Syndrome or related chest wall conditions, you are most likely filled with countless questions, concerns and likely every other kind of emotion. The goal of this section is to give you a general overview about the condition as well as a candid Q&A session with our Jeune's head surgeon, Dr. Alistair Phillips. Once you have reviewed this section, use the link at the top of this page to visit the Medical Tutorial section of our site to find more about Jeune's related chest wall conditions, as well as our surgical and non-surgical approach to treatment.
Jeune's Syndrome is a form of congenital dwarfism causing children to have a deformity of their chest wall. The chest cage is extremely small and doesn't have enough room for proper breathing. The ribs are broad, short and irregularly joined between the cartilage and sternum, or breastbone. This produces a bell-shaped, inflexible chest cavity, which doesn't grow well in time. Without treatment, children with severe cases of Jeune's will slowly, and progressively, run out of room to breathe.
A child with Jeune's may have symptoms as early as a newborn, or as late as 4-5 years of age. Depending on the progression of the disease, respiratory symptoms can range from mild to severe. A mild symptom includes a slightly rapid breathing rate where a more severe form can include recurrent pneumonia. The most severe form may cause the inability to breathe without help from a breathing machine, or ventilator.
A child with Jeune's Syndrome is typically small in overall size and may have liver or kidney disease in addition. Children who have more mild symptoms often respond well to treatment that is aimed at ongoing medical support of their lungs.
Depending on the child's particular symptoms, treatment may include prompt antibiotics for infection, proper mobilization of secretions, supplemental oxygen, BiPap, non-invasive ventilator (or breathing) support or other forms of respiratory therapy. For more severe cases, Nationwide Children's Hospital has devised a method for expanding the chest cavity (see photo) that has created hope for many children who previously had few options.
Nationwide Children's cares for one of the largest groups of Jeune's Syndrome patients in the world. For those patients who need it, we have developed a procedure that expands the thoracic cavity. This surgery is explained in detail in the Our Approach section of our Medical Tutorial. This procedure appears to hold out hope of longevity and improved quality of life in several children.