What is thoracic insufficiency syndrome?

Thoracic insufficiency syndrome is a complex condition that involves chest wall deformities that affect normal breathing and lung growth. In most cases, children with thoracic insufficiency syndrome are also born with congenital spinal disorders, such as scoliosis.

Thoracic insufficiency syndrome is the inability of the thorax to support normal breathing or lung growth. The thorax is the part of your child’s body between the neck and abdomen that includes the spine, ribs and sternum (breastbone). In normal developing children, lung growth parallels chest and spine growth. In children with thoracic insufficiency syndrome, lung growth is limited by rib deformities and spinal curves.

As children with thoracic insufficiency syndrome 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.

An orthopaedic surgeon at Children’s Hospital of Philadelphia (CHOP) invented the vertical expandable prosthetic titanium rib (VEPTR) device, the first FDA-approved device for treatment of thoracic insufficiency syndrome.

Treatments for thoracic insufficiency syndrome

Our team considers all possible treatment options for children with thoracic insufficiency syndrome, but the most commonly used treatment is the vertical expandable prosthetic titanium rib (VEPTR), a device that expands a child’s chest, stabilizes spine curvatures, and is lengthened with periodic outpatient expansions to keep up with the growth of the child.

Our core team of pediatric orthopaedists, general surgeons, pulmonologists, advanced practice nurses and other experts provide complete care for children with thoracic insufficiency syndrome — from our unique multidisciplinary evaluation system to implant surgery, expansion surgeries and long-term supportive follow-up.

The Center for Thoracic Insufficiency Syndrome at 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 orthopaedic surgeons to expand the device at regular intervals in outpatient surgery.

Follow-up care

After the initial surgery to place the vertical expandable prosthetic titanium rib (VEPTR), your child will receive follow-up care in our center on a regular basis. We will monitor your child's progress and schedule additional surgeries about twice a year to expand the device to accommodate your child's growth.

Expansion surgeries

VEPTR expansion is a short surgical procedure involving an approximately 1-inch incision. Typically, children spend one night in the Hospital on a medical/surgical floor and are discharged the following day.

Final surgery and "graduation"

When your child's spine and chest have matured and the titanium rib has been fully expanded — at approximately 10 to 16 years old — your child will "graduate" and a final surgery will be scheduled.

During this procedure, the spine-supporting expandable titanium rib will be removed and a spinal fusion will be performed. In a spinal fusion, your child's abnormal curved spinal bones are realigned, fused together and metal implants may be inserted to further stabilize the spine. Spinal fusion is often needed to better support a child's spine and minimize the effects of scoliosis and other spine-curving disorders.

This final step in your child's journey generally requires several days of recovery in the Hospital. Our multidisciplinary team continues to monitor your child and assess other health issues that your child may have.

In rare cases, such as if your child has missing ribs; a VEPTR device may be left in place permanently to help your child retain chest wall form and function.