Your Child's Thoracic Insufficiency Syndrome Evaluation

The team at the Center for Thoracic Insufficiency Syndrome at CHOP can provide a comprehensive evaluation of your child’s spine and chest wall deformity. We do our best to adapt the scheduling of our evaluation and treatment based on the distance our patients travel.

Initial evaluation at CHOP

During your child's first appointment with our orthopedic staff, including Robert M. Campbell Jr., MDJohn M. Flynn, MD, or Wudbhav N. Sankar, MD, we will:

  • Collect complete medical history
  • Conduct a physical exam of your child 
  • Review X-rays of your child's spine and thoracic region

Further testing

If your child is a candidate for further extensive evaluation and you are interested in proceeding, we will schedule additional testing and consultations with the multidisciplinary team at CHOP.

These tests and consultations are generally scheduled within 4-6 weeks of your child's initial appointment and will span 2-3 full days.

We tailor evaluations to meet your child’s individual needs; therefore, some children may require more consultations than others.

During these intensive days of testing, we will conduct:

  • Lab tests to evaluate for potential clotting issues.
  • Pulmonary function tests. 
  • Radiology and imaging tests, including computed tomography (CT) scans of the chest and lumbar spine, and magnetic resonance imaging (MRI) of the chest and spine. Your child will visit with the Anesthesia Resource Center prior to imaging tests.
  • Dynamic MRI — this test gives clinicians an inside view of your child's chest and allows you to see exactly how your child’s thoracic insufficiency syndrome is affecting air supply to his/her lungs. Watch a dynamic MRI video » 
  • Consultative exams with pulmonary, general surgery and other departments or specialties as needed.

Recommendations

Once all of the consults, testing and imaging are completed, the multidisciplinary team at CHOP meets to discuss the best treatment for your child. Each specialist involved in your child's care will weigh in with their opinions.

Together, the team will debate the pros and cons of VEPTR expansion thoracoplasty, growing rods and any other treatment options.

Once a consensus is reached, the team will meet with your family to discuss the recommendations. If surgery is recommended and accepted by your family, we will move forward. 

VEPTR surgery

Before surgery, your child will have a number of preoperative visits and testing, including:

  • Pre-op visit with Dr. Campbell, Dr. Flynn or Dr. Sankar 
  • Pre-op visit with the Anesthesia Resource Center
  • Pre-op labs obtained 

For details about VEPTR, how the device works and typical results, see VEPTR treatment. 

After surgery, your child will likely have a 7-10 day inpatient hospital stay; 3-5 days of which will be in the pediatric intensive care unit. Every child's recovery after surgery varies. If you have questions, please talk to your child's healthcare team.

Follow-up care

After your child is discharged from the Hospital, he or she will need to return a week later so clinicians can inspect the wound and ensure it is healing properly.

In most cases, additional follow-up will then occur every 4-6 months until your child reaches skeletal maturity (between 10 and 16 years old).

Because your child is growing rapidly during these years, surgery will need to be done to expand the VEPTR device so your child's lungs can keep pace. These additional surgeries generally require one overnight stay in the Hospital and recovery time is far quicker than the initial implantation surgery.

For details about continuing care and long-term outcomes for children with thoracic insufficiency syndrome, see follow-up care. 

Evaluation and treatment timeline

If your family is traveling from further away, the multidisciplinary team will work with you to coordinate your child's care into fewer visits to the Hospital.

East Coast patients

If your family lives more than two hours away from CHOP, but you don't have to travel by air, your child's evaluation and treatment plan will likely follow this timeline:

  • An initial visit with Dr. Campbell, Dr. Flynn or Dr. Sankar (1 day)
  • Second visit: further testing (2-3 days)
  • Third visit: pre-op, surgery and post-op (about 2 weeks)
  • Follow-up (one overnight stay every 4-6 months until your child reaches skeletal maturity)

National patients

If your family needs to travel across the country to come to CHOP, your child's evaluation and treatment plan will typically follow this schedule:

  • An initial visit with Dr. Campbell, Dr. Flynn or Dr. Sankar, and further testing (about 1 week)
  • Second visit: pre-op, surgery and post-op (2-3 weeks)
  • Follow-up (one overnight stay every 4-6 months until your child reaches skeletal maturity)

International patients

If your family is traveling from another country for treatment at CHOP's Center for Thoracic Insufficiency Syndrome, we will work with our partners in International Patient Services (IPS) to help coordinate your trip. IPS will help your family manage all of the details of your trip including travel, visas, insurance, places to stay and will arrange interpreters to work with your family and the clinicians treating your child.

Our Center will work with IPS to get as much information about your child's condition — for example, records, X-rays and other imaging — before you arrive in the United States.

Plan for a 6-week stay in Philadelphia for your first visit. This will allow us time for your initial visit, as well as imaging and consults needed to determine if VEPTR surgery is the best course of treatment for your child. If clinicians and your family agree to proceed, your child will undergo preoperative testing, inpatient surgery and recovery at The Children's Hospital of Philadelphia.

Like other patients, international visitors will need to return for expansion surgery as your child grows. We will work with you and IPS to ensure all of your child's health needs are met in a coordinated fashion during follow-up visits. 

Reviewed by Robert M. Campbell, MD on February 25, 2014