The first step for patients who come to Children’s Hospital of Philadelphia’s Wyss/Campbell Center for Thoracic Insufficiency Syndrome (CTIS) is to undergo a comprehensive evaluation of their spine and chest wall deformity.

A thorough evaluation with our multidisciplinary team helps us develop an individualized treatment plan that will provide the best possible outcomes for each patient.

Before your visit

Before your child comes for their first appointment with our center, you will be contacted by Susanne Manzoni, BSN, RN, our program’s nurse manager. These experienced nurses will gather important information about your child’s medical history, any pertinent medical records and imaging, as well as demographic and insurance information, to begin creating a patient chart that our physician team will review before your child’s first visit.

We do our best to adapt the scheduling of evaluation and treatment of your child based on the distance your family must travel.

Initial TIS evaluation at CHOP

During your child's first appointment with our orthopedic staff, including Patrick J. Cahill, MD, Jason B. Anari, MD or John M. Flynn, MD, we will:

• Collect a more complete medical history of your child from your point of view
• Conduct a physical exam of your child
• Review X-rays and any other imaging previously performed of your child's spine and thoracic region with you

To make the best recommendation for each child, our team needs to examine the patient in-person. If your child is currently hospitalized elsewhere, our team will work with you to help start the transfer to Children’s Hospital of Philadelphia (CHOP) and obtain necessary insurance authorizations.

Further testing to understand your child’s unique case

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

Our nurse manager will help schedule these added tests and evaluations. Testing will span a few days and will be tailored to meet your child’s individual needs. Some children may need more consultations than others.

During these intensive days of testing, we will conduct:

• Lab tests to evaluate for any potential clotting issues.
• Pulmonary function tests, including a lung MRI.
• Radiology and imaging tests, including X-rays, computed tomography (CT) scans and magnetic resonance imaging (MRI) of your child’s chest and spine. Your child will visit with the Anesthesia Resource Center prior to imaging tests. If your child is anxious or may have trouble holding still for their tests, we’ll work together to plan for any sedation needs to help them stay calm and comfortable during the tests.
• Dynamic MRI — this test gives clinicians an inside view of your child's chest and allows us to see exactly how your child’s thoracic insufficiency syndrome is affecting air supply to their lungs.
• Genetic testing, if indicated.
• Consultative exams with pulmonary, general surgery, physical therapy, orthopedics and other departments or specialties as needed.

Recommendations after your child’s initial TIS evaluation

Once all the consultations, 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 on the different treatment approaches that may be available to your child, including:

• Vertical expandable prosthetic titanium rib (VEPTR) expansion thoracoplasty
• Traditional growing rods
• Magnetic (MAGEC) growing rods
• Any other treatment options

We will then meet with your family to discuss our recommendations and answer any questions you may have. If surgery is recommended and accepted by your family, we will move forward.

Evaluation and treatment timelines

The multidisciplinary team will work with you to coordinate your child's care so that your appointments are as convenient as possible for your family.

A typical evaluation and treatment plan will follow this basic structure:

• An initial consultation with Dr. Cahill, Dr. Anari or Dr. Flynn⁺ 
• Second visit: further testing⁺
• Third visit: pre-op, surgery and post-op
• Follow-up visits until your child reaches skeletal maturity

⁺ In many cases, we try to combine the first consultation and testing into one visit

International patients

If your family is traveling from another country for treatment at CHOP's Wyss/Campbell Center for Thoracic Insufficiency Syndrome, we will work with our partners in Global Patient Services (GPS) to help coordinate your trip. GPS will help your family manage all 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 GPS to get as much information about your child's condition — for example, medical records, X-rays and other imaging — before you arrive in the United States.

Plan for a several-week stay in Philadelphia for your first visit. This will allow us time for your first visit, as well as imaging and consultations needed to decide 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 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 GPS to ensure all your child's health needs are met in a coordinated fashion during follow-up visits.

Long-term follow-up for adult TIS patients

Even after your child reaches skeletal maturity and no longer needs routine adjustments to their VEPTR or growing rods, our team at the Wyss/Campbell Center for Thoracic Insufficiency Syndrome will continue to be a resource to your family.

All patients who have graduated from our program or were a patient of the late Robert M. Campbell Jr., our former Center director, will always have a medical home at Children’s Hospital of Philadelphia. Dr. Campbell’s knowledge, experience and compassion continue to live on within our team.

Our multidisciplinary team of clinicians from orthopedics, pulmonology and plastic surgery will help transition your child to adult healthcare and act as a safety net for any issues that arise down the road, such as broken or misaligned hardware, infections, progressive damage or other issues.

Outpatient and inpatient care are possible at CHOP for patients of any age diagnosed with thoracic insufficiency syndrome.