VEPTR: past experience and the future of VEPTR principles

Abstract

This review summarizes the experience with the vertical expandable prosthetic titanium rib (VEPTR) device, which enables five new procedures to treat complex spine and chest wall abnormalities in pediatric patients, which cause thoracic insufficiency syndrome, the inability of the thorax to support normal respiration or lung growth. The literature on VEPTR was reviewed and discussed by the author, the inventor of the VEPTR. The central VEPTR treatment principle is to correct volume depletion deformity of the thorax, and maintain the correction until skeletal maturity, at which time procedures such as spinal fusion can be considered. For individual cases of complex deformity, VEPTR strategies can differ remarkably. The goal of VEPTR surgery is to pursue the surgical strategy that provides the largest, most symmetrical, most functional thorax that can grow as normally as possible. Assessment of these results is difficult, since natural history of VEPTR-treated diseases are not clearly known and no current imaging test can measure thoracic insufficiency syndrome, but dynamic lung MRI have promise for the future in better defining this potentially lethal condition. VEPTR and its principles of use have become an important first step toward improving the quality of life and longevity of children with thoracic insufficiency syndrome, but much work remains to advance both its design and its use.