Your Child's Thoracic Insufficiency Syndrome Evaluation and Treatment Planning
At Children’s Hospital of Philadelphia’s Wyss/Campbell Center for Thoracic Insufficiency Syndrome, you child will undergo a comprehensive evaluation of their spine and chest wall deformity.
Before your child comes for their first appointment with our center, you will be contacted by either Mary Ovelman, BSN, RN, RRT, our CTIS nurse navigator, or 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 based on the distance our patients travel. (See our evaluation and treatment timeline below.)
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
In order to make the best recommendation for each child, our team needs to examine them in-person. If your child is currently hospitalized elsewhere, our team will work with you to help initiate the transfer to 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 navigator and nurse manager will help schedule these tests and evaluations within 4-6 weeks of your child’s initial appointment. Testing will span 2-3 days and will be tailored 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 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.
- Consultative exams with pulmonary, general surgery, physical therapy, orthopaedics and other departments or specialties as needed.
Recommendations after your child’s initial TIS evaluation
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 different treatments and treatment approaches, including: vertical expandable prosthetic titanium rib (VEPTR) expansion thoracoplasty, traditional growing rods, magnetic growing rods and any other treatment options.
Once the team reaches a consensus about the best treatment option or options for your child, we will 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.
VEPTR surgery for TIS
Before surgery, your child will have a number of preoperative visits and testing, including:
- Pre-op visit with Dr. Cahill, Dr. Anari or Dr. Flynn
- Pre-op visit with the Anesthesia Resource Center to plan for any anesthesia your child will need to prevent pain and keep them comfortable and safe during their surgery
- Pre-op labs obtained
We will communicate with all members of your child’s care team – before, during and after surgery – to ensure everyone is aware of your child’s care plan, any potential complications that may occur, and who to contact if anything unusual occurs.
After surgery, your child will likely have a 7- to 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.
For more details about VEPTR, how the device works, follow-up care, timing of expansion surgeries and typical results, see VEPTR treatment.
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 consultation with Dr. Cahill, Dr. Anari or Dr. Flynn+
- Second visit: further testing (2-3 days)+
- Third visit: pre-op, surgery and post-op (about 2 weeks)
- Follow-up (one overnight stay every 6-9 months until your child reaches skeletal maturity)
+ In many cases, we try to combine the initial consultation and testing into one visit
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. Cahill, Dr. Anari or Dr. Flynn, and further testing (about 1 week)
- Second visit: pre-op, surgery and post-op (2-3 weeks)
- Follow-up (one overnight stay every 6-9 months until your child reaches skeletal maturity)
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, 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 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 of your child's health needs are met in a coordinated fashion during follow-up visits.
