Wyss/Campbell Center for Thoracic Insufficiency Syndrome

Thoracic Insufficiency Syndrome (TIS) encompasses a number of complex skeletal conditions that result in a small chest cavity, which can severely impair lung growth and general growth in children. Building from the development of a pioneering device — the vertical expandable prosthetic titanium rib (VEPTR) — to help expand the chest, the Wyss/Campbell Center for Thoracic Insufficiency Syndrome has increased CHOP's ability to treat more patients with TIS who have acute needs from all over the world.

As both patient referrals and surgical volume have risen, so has the need for more TIS research. This Frontier program focuses on innovating novel imaging techniques to better understand the mechanics of TIS so that scientists can develop new devices and refine surgical strategies to care for these children.

Here are some highlights of the Wyss/Campbell Center for Thoracic Insufficiency Syndrome's research efforts:

• The Center is working to redefine outcomes for the complex constellation of conditions in terms of pulmonary and health related quality of life outcomes. These efforts involve establishing metrics, refining care, defining outcome expectations, and improving care.
• The Center is collaborating with the University of Pennsylvania Department of Radiology Medical Imaging Processing Group and the Department of Bioengineering at Drexel University on dynamic lung MRI image analysis to develop a new metric to define thoracic performance.
• In conjunction with refinement of this new quantification technique, the Center launched the Virtual Growing Child project to help establish normative data for comparative analysis.
• Funding from the Frontier Program supported a new basic science research lab that is adapting an animal model to study the patho-biology of TIS and to facilitate development of new devices for treatment.

The Wyss/Campbell Center for Thoracic Insufficiency Syndrome received Frontier status in Fiscal Year 2017.