The Center for Thoracic Insufficiency Syndrome at The Children’s Hospital of Philadelphia is the first national multidisciplinary program devoted solely to the treatment and research of thoracic insufficiency syndrome.
For children with scoliosis or other curved spine disorders, growth in one or both lungs is restricted by the size and shape of the spine and/or rib cage. Sometimes the rib cage is excessively shortened or narrowed by a birth deformity and this can constrict both lungs.
Robert M. Campbell Jr., MD, director of the Center for Thoracic Insufficiency Syndrome at CHOP, was the first to identify thoracic insufficiency syndrome as a disease condition that required specialized care and treatment. He also invented the vertical expandable prosthetic titanium rib (VEPTR) device, the first FDA-approved device for treatment of thoracic insufficiency syndrome.
This revolutionary implant allows a surgeon to expand a child’s rib cage surgically to nurture lung growth and correct spinal deformity. Then, the gains in chest volume are continued by further expanding the device in outpatient surgery twice a year.
Thoracic insufficiency syndrome is the inability of the thorax to support normal breathing or lung growth. The thorax is the part of the body between the neck and abdomen that includes the spine, ribs and sternum (breastbone). Lung growth must parallel chest and spine growth.
This rare condition most commonly occurs when a baby is born with progressive scoliosis (spinal curvature) and severe rib fusion.
Other causes of thoracic insufficiency syndrome include:
Children with thoracic insufficiency syndrome cannot support normal breathing or lung growth. As they grow, their rib cage and spine do not keep pace. As a result, their chest wall becomes deformed (sunken) and the children may become dependent on nasal oxygen or ventilator support to breathe.
Conditions that may result in thoracic insufficiency syndrome fall into three general categories:
Thoracic insufficiency syndrome is diagnosed in very young children by a pediatric orthopedic surgeon or pediatric pulmonologist. The child usually will be less than 5 years old and always less than 10 years old.
At the Center for Thoracic Insufficiency Syndrome at The Children’s Hospital of Philadelphia, we fully evaluate pediatric patients with a combination of tests and consultations with our team of orthopedic surgeons, general surgeons, radiologists, pulmonologists, neurologists, geneticists, anesthesiologists, bioengineers, nurses and other clinical specialists.
All patients undergo pulmonary function tests and a unique test, the dynamic lung MRI, to reveal the movement of the lungs and the breathing muscles. The dynamic MRI allows clinicians to better understand each child’s thoracic insufficiency syndrome.
Each patient with thoracic insufficiency syndrome is discussed in detail at our Center’s bimonthly team meetings to define the child’s condition from each consultant’s area of expertise, and to arrive at a consensus of the best treatment options possible.
The Center for Thoracic Insufficiency Syndrome at The Children’s Hospital of Philadelphia is one of only a few institutions in the United States that offers an FDA-approved treatment for thoracic insufficiency syndrome.
The treatment involves use of a special titanium implant called a vertical expandable prosthetic titanium rib (VEPTR). In VEPTR surgery, the expandable titanium ribs are implanted into a child's back and chest, and anchored to the spine and ribs. The VEPTR was designed to allow orthopedic surgeons to expand the device at regular intervals in outpatient surgery.
The Center uses a unique multidisciplinary approach to provide comprehensive care for patients with rare scoliosis, fused or absent ribs, and hypoplastic thorax, complementing the longstanding early-onset scoliosis program at CHOP.
Through research into the natural history of thoracic insufficiency syndrome, projects to pioneer new imaging techniques for the advanced study of this complex disease, and new medical and surgical approaches to the disease, the Center team is working to develop the best total care approaches for children with thoracic insufficiency syndrome.
For more information about treatment at The Center for Thoracic Insufficiency Syndrome at CHOP, use the links below:
To schedule an appointment with the Center, call 215-590-3722 or contact us online.
Reviewed by: Robert M. Campbell Jr., MD
Date: January 2013