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Published on: September 13, 2020
Thoracic Insufficiency Syndrome
Oscar Mayer1, Robert Campbell2, Patrick Cahill2
1Division of Pulmonology, The Children's Hospital of Philadelphia, 3510 Civic Center Boulevard, Colket 11309, Philadelphia, PA 19104.
Insights
Thoracic insufficiency syndrome (TIS) requires innovative treatments. The Vertical Expandable Prosthetic Titanium Rib (VEPTR) offers significant skeletal correction for chest wall and spine deformities, stabilizing the condition.
Area of Science:
- Pediatric Orthopedics
- Thoracic Surgery
- Biomedical Engineering
Background:
- Thoracic insufficiency syndrome (TIS) encompasses diverse disorders affecting the chest wall and spine.
- These conditions significantly impact respiratory capacity and development, often requiring early childhood intervention.
- Traditional treatments struggle to balance deformity correction with growth preservation.
Purpose of the Study:
- To evaluate the efficacy of the Vertical Expandable Prosthetic Titanium Rib (VEPTR) system in managing TIS.
- To assess the VEPTR system's ability to correct both spinal and chest wall deformities while preserving growth capacity.
- To analyze the functional outcomes, including quality of life and pulmonary function, following VEPTR implantation.
Main Methods:
- Surgical implantation of the VEPTR system to address complex thoracospinal deformities.
- Concurrent correction of associated chest wall and spinal abnormalities.
- Longitudinal assessment of skeletal alignment, pulmonary function tests, and quality of life metrics.
Main Results:
- The VEPTR system achieved dramatic skeletal correction in patients with TIS.
- Functional outcomes, such as quality of life and pulmonary function, showed stabilization rather than dramatic improvement.
- VEPTR implantation demonstrated consistent control over the progressive nature of TIS.
Conclusions:
- The VEPTR system provides significant skeletal correction for severe TIS.
- While functional gains may be modest, VEPTR effectively stabilizes progressive thoracospinal disorders.
- This approach represents a paradigm shift in managing complex pediatric chest wall and spinal deformities.
Abstract:
Thoracic insufficiency syndrome (TIS) is a broad grouping of disorders that have a substantial impact on the chest wall, spine, and in many situations, both. While the conditions are varied, they share a potentially substantial impact on respiratory capacity and development over time and a presentation and need for intervention that is often in early childhood. Addressing these conditions has required a new paradigm that involves both deformity correction and a preservation of growth capacity. While there are now a number of options to treat severe spinal deformity early in life, when the deformity causes or is caused by a chest wall deformity, the Vertical Expandable Prosthetic Titanium Rib(VEPTR) is able to support surgical correction of both. The skeletal correction is often quite dramatic, but the functional measurements of quality of life and pulmonary function often do not show as dramatic and improvement, but consistently show a stabilization indicating control of the progressive thoracospinal disorder that produced TIS.
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