Thoracic insufficiency syndrome (TIS) is the inability of the thorax to support normal respiration or lung growth. This syndrome results from serious defects affecting the ribs or chest wall (i.e.severe scoliosis, rib fusion [which may accompany scoliosis]), and various hypoplastic thorax syndromes (i.e. Jeune’s Syndrome and Jarcho-Levin syndrome). Spine, chest, and lung growth are interdependent. While the coexistence of chest wall and spinal deformity is well documented, their effect on lung growth is not completely understood.
Progressive thoracic insufficiency syndrome includes respiratory insufficiency, loss of chest wall mobility, worsening three-dimensional thoracic deformity, and/or worsening pulmonary function tests. As a child grows, progressive thoracic deformity and rotation toward the concave side occurs with worsening respiratory compromise. This progression is often accompanied by a need for supplemental oxygen and can require mechanical ventilation. While spinal fusion is one approach to treatment, it may not be successful and also may limit growth (lengthening) of the spine.
The VEPTR device is a curved rod placed vertically in the chest that helps to shape the thoracic cavity. It is positioned either between ribs, or between the ribs and either the spine or the pelvis. The device is designed to be expanded every four to six months as growth occurs and also to be replaced if necessary. Some patients require multiple devices.
A VEPTR has received approval from the FDA under a humanitarian device exemption (HDE). The FDA review noted that the device is indicated for the treatment of TIS in skeletally immature patients. This review also indicated that the device should not be used in patients younger than six months.