Chromosomal and Genetic Conditions
Jeune Syndrome Treatment
Few hospitals have experience with Jeune syndrome treatment because the condition is so rare. Seattle Children’s sees more children with this condition than many other hospitals because doctors from several states send patients with Jeune syndrome here for treatment.
Children with Jeune syndrome need special care from a variety of providers. We take a team approach that brings together experts in orthopedics, pulmonary medicine, general and thoracic surgery and nutrition. This helps us develop a comprehensive treatment plan.
Jeune Syndrome Treatment Options
Treatment for Jeune syndrome starts with helping children breathe. Babies with Jeune syndrome may need help from a breathing machine (ventilator) right away. This helps get oxygen in your baby’s lungs and carbon dioxide out.
Some children with Jeune syndrome may only need to be on a ventilator if a lung infection makes breathing more difficult. Children with lung infections may also need antibiotics, suctioning to remove mucus from airways or postural drainage. This means placing the child in a position that will help mucus drain.
Some children may need a feeding tube called a gastrostomy (pronounced gas-STRAH-stuh-mee). This provides a safe way to give your child nutrition until they can eat.
Surgery for Jeune Syndrome
Surgery to enlarge the rib cage may be an option for some children with Jeune syndrome who cannot breathe well. Our surgeons use several procedures to give the lungs room to grow. Which procedure is best depends on your child’s age.
One procedure uses the vertical expandable prosthetic titanium rib (VEPTR) to enlarge the chest. The VEPTR is inserted into the body and attached to your child’s ribs. Doctors usually expand the VEPTR every 6 months. This helps the child’s chest get bigger as they grow.
Doctors aren’t sure if VEPTR works by expanding the chest and giving the lungs more room to grow or by helping the diaphragm. We are studying VEPTR to understand exactly why it works and find ways to make it work better.
The VEPTR is best used in children who are at least 8 or 9 months old. Other procedures may be done for younger children.
To learn more about Jeune syndrome treatment at Seattle Children’s, call our General and Thoracic Surgery Department at 206-987-2794 x4.