A Year Like No Other: The Wyss/Campbell Center for Thoracic Insufficiency Syndrome

Published on in Orthopaedics Update

Vertical Expandable Prosthetic Titanium Rib (VEPTR) patient The year 2019 was one of great strides for the Wyss/Campbell Center for Thoracic Insufficiency Syndrome. Newly named for our benefactor, Mr. Hansjorg Wyss, and for the inventor of the vertical expandable prosthetic titanium rib (VEPTR), the late Robert Campbell, MD, we took a large step forward toward further innovation for children with chest wall/pulmonary and spinal deformities under the leadership of Patrick J. Cahill, MD.

Thanks the generosity of Mr. Wyss, we have initiated multiple research projects and increased patient volumes, while striving to always improve patient outcomes and care.

Our approach to care: Attention to every detail

Our center cares for children from 34 states in the U.S. and nine countries around the world. Patients evaluated by our center come with numerous diagnoses, including but not limited to Jeune’s thoracic dystrophy, skeletal dysplasia, neuromuscular scoliosis, syndromic scoliosis and Jarcho-Levin syndrome.

For every diagnosis we see, the unique multidisciplinary composure of our center team allows for consistent, patient-centered care with clear communication across disciplines both in the outpatient clinic as well as inpatient hospitalizations. Our center team currently includes three pediatric orthopaedic surgeons specializing in spine and chest wall deformity in the growing child. These team members continuously collaborate with a pulmonologist, a general surgeon and a plastic surgeon, as well as physicians from neurosurgery, anesthesia, pulmonary hypertension, neurology and physical therapy.

Following clinical evaluation, the patient undergoes various imaging studies to help determine the best course of clinical care. These studies include a chest CT, a spine MRI and a dynamic lung MRI. The dynamic lung MRI is a radiology study unique to Children’s Hospital of Philadelphia. This imaging modality demonstrates the interaction between the chest wall and respiration, highlighting how the spine and chest wall deformity impact a child’s ability to exchange gas in the thoracic cavity.

Throughout each family’s journey, our nurse manager helps to coordinate all consultations and radiology studies so that the family can have as few interruptions to daily life as possible. Our insurance specialist navigates the complexities of out-of-state insurance plans and obtains authorizations needed to allow these patients to be treated out of state. Once consultations and imaging is complete, all our new cases are discussed in a monthly multidisciplinary clinical meeting and final recommendations are given to the family. 

Innovative research

In the laboratory

Vertical Expandable Prosthetic Titanium Rib (VEPTR) patient and doctor Our basic science lab focusing on the development of an animal model for thoracic insufficiency syndrome has taken off with the hiring of lab director Benjamin Sinder, MD. While in Philadelphia for less than six months, Dr. Sinder has overseen successful collaborations with the University of Pennsylvania School of Veterinary Medicine. Dr. Sinder’s team is also paving the way to understand how force is imparted on the spine both from the posterior and anterior sides through various surgical techniques.

With much unknown about magnetically controlled growing rods and spinal response to vertebral body tethering, our center is well positioned to further the field with innovation and the scientific method. Collaboration with Penn’s functional/metabolic imaging center and the medical image processing group highlight the unique approach we take to better understanding lung function and pathology in the growing child.

Clinical research highlights

Our clinical research team — which is composed of two research coordinators and an orthopaedic surgery resident — led successful projects off the ground from idea to publication while presenting the research at the annual ICEOS conference for early onset scoliosis in Atlanta this past November. Our center had three podium presentations as well as a poster, while the orthopaedic faculty led numerous sessions at the conference.

Given the unique ability to study lung function with the QdMRI at CHOP, our clinical team opened a prospective study on adolescent idiopathic scoliosis. This study will evaluate the effect of posterior spine fusion on thoracic performance to hopefully prove preservation and improvement of thoracic function following scoliotic correction. In the younger version of the same diagnosis — infant idiopathic scoliosis — we are conducting an ongoing genetics study in the hopes of identifying a single-gene foci responsible for the disease.

Advances in informatics, quality and safety

Beyond laboratory and clinical research, the informatics branch of our group, led by Lia McNeely, MA, MSN, CPNP, is optimizing how we manage and follow patients with the electronic medical record. In 2019, we launched the use of Healthy Planet, a live report for our entire patient population, both operative and nonoperative.

This report allows us to quickly access key features for each patient regarding their care, alerting providers for key milestones and dates in treatment as well as any historical issues to limit complications for future surgery. This has been a valuable tool to assist with coordinating and communicating care across the members of the team.

In a quality/safety/value initiative, the team rolled out a clinical pathway for parents and clinicians to access worldwide for Jeune’s thoracic dystrophy, the diagnosis Dr. Campbell reversed the mortality curve in with the VEPTR. This pathway facilitates transfer to CHOP for surgical treatment of this rare disease.

And finally, this year our center hosted the 6th annual FDA course, expanding it to a two-day event and inviting surgeons across all pediatric specialties at CHOP, including neurosurgery, cardiac surgery, cardiology and otolaryngology. One highlight from the event was a multidisciplinary discussion about pediatric device development between surgical innovators, the hospital technology transfer office and angel investors.

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