Surgeons performed 1,538 surgical procedures during the study’s 14 years. Each child was expected to need many adjustments as a normal course of treatment. Those ratings reflect whether a child needs supplemental oxygen and other kinds of assistance to breathe.īecause the 257 patients treated were growing, the study device would periodically require expansion or replacement of its components. This study used “assisted ventilator rating” outcomes as an endpoint instead. The endpoints included increases in thoracic spinal height, hemithoracic height and width, and scoliosis correction.īecause the patients are young and sometimes developmentally delayed, standard pulmonary tests often can’t be done. The children served as their own control because there is no “typical” pattern on which to create a control group.Īll subjects in the study were children six months or older, up to the age of skeletal maturity, which is typically at 15–17 years of age. The 501(k) clearance was based upon a single-arm, nonrandomized, prospective multicenter trial done in two phases. Late last year, the agency gave the device 501(k) clearance, which means it can now be used more widely. The FDA originally approved Campbell’s VEPTR device in 2004 under its special humanitarian device exemption for conditions that affect small numbers of people. Other devices need to be replaced, and that often requires major surgery. Moreover, it can be adjusted through minor surgery as the child grows. The VEPTR is also unique because it treats the deformities of both the spine and thorax. Increasing life expectancyĪccording to an FDA staff report, VEPTR is the only treatment available that allows the chest cavity to be enlarged vertically, providing room for the lungs to grow and improving the function of the lungs and heart, thereby increasing the life expectancy of these terminally ill children. Because there is no standard presentation, any device for the condition either needs to be entirely customized or capable of being assembled, like parts of an erector set, to create a unique solution for each child. VEPTR is perhaps the most configurable device ever approved by the FDA. It consists of curved titanium rods that are surgically attached to the ribs, lumbar vertebra, or sacrum. The device was developed by Robert Campbell Jr., MD, a surgeon at Children’s Hospital of Philadelphia, who spent 25 years refining it. The Vertical Expandable Prosthetic Titanium Rib (VEPTR) addresses many of the deformities of the rib cage and spine. VEPTR device can be configured for rib-to-rib, rib-to-lumbar lamina, and rib-to-ilium (not pictured) attachment. In some cases, surgeons have resorted to splitting the breastbone-anything to allow the lung space to expand, not only for respiration but also growth of the child. Missing ribs have been replaced with artificial ribs of a fixed size or cadaver bone. The fused ribs needed to be split apart and kept apart with spacers. The rib cage in children with TIS has a variety of missing, fused, and deformed ribs. These disorders include constrictive chest wall syndrome progressive congenital, neurogenic, or idiopathic scoliosis without rib fusion and hypoplastic thorax syndrome, which includes Jeune’s syndrome, also called asphyxiating thoracic dysplasia Ellis-van Creveld syndrome, also called mesoectodermal dysplasia, and Jarcho-Levine syndrome, which has gone by nearly a dozen different names.Īs you might imagine, the deformities can come in a wide variety of forms because each of these conditions can affect the ribs and individual vertebrae in different ways.Įarly surgical interventions include fusion of the vertebrae to prevent twisting and turning and insertion of custom manufactured expandable instruments called “growing rods.” Other cases are associated with well-defined genetic disorders and disorders that are less well characterized from a genetic perspective but also cause deformities of the bones in the thorax. It may also be caused by resection of large tumors from the chest wall and surgical separation of conjoined twins. TIS may cause pulmonary failure because of decreased lung volume: The ribs fuse together, the thoracic spine twists and rotates, and the patient may slowly suffocate unless reconstruction is done to multiple ribs and the spine, and is repeated as the child grows. Thoracic insufficiency syndrome (TIS) is a collection of rare disorders of the thoracic skeletal system affecting about 4,000 newborns in the United States each year.
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