Children’s Now Offering Rare Rib Surgery
from Practice Update, Fall 2006
On July 13, The Children’s Hospital joined a short list of pediatric institutions across the country that performs a new type of rib surgery. The unique procedure treats children with Thoracic Insufficiency Syndrome (TIS), a disease marked by severe deformities of the chest, spine and ribs that prevent normal pulmonary development. Although TIS is relatively rare, 10 to 12 patients with the syndrome were seen at The Children’s Hospital last year. During the surgery, Mark Erickson, MD, Chair of the Department of orthopedics at The Children’s Hospital implants a Vertical Expandable Prosthetic Titanium Rib (VEPTR), a device recently approved by the FDA.
“Many of these children have a congenital fusion of the ribcage,” explains Dr. Erickson. “Almost 100 percent of kids with this disease have scoliosis or spine issues. These are complicated kids.” In some children, several ribs on one side fuse together. In others, all of the ribs fuse together. “The ribs can’t move, and the child’s lungs are trapped in a fixed space,” he said. This can cause the children with this disease to become oxygen-dependent or even die.
The implanted VEPTR device expands the ribs to help the lungs grow. Six to nine months later, Dr. Erickson will perform another outpatient surgery to expand the 3- to 8-inch device – which is curved like a rib – even more. The device is then expanded every six months to continue to allow the thorax to grow until the child reaches skeletal maturity. Although there is no set age for the procedure, the best candidates are younger than 5 years old but at least 16 to 18 months old.
“This treatment plan is a long-term commitment for the patients and their families,” Dr. Erickson explains, “You are buying into an eight- to ten-year process. You cannot do this surgery on a child who is too young because the bones are not fully developed. It takes a year or two to determine the extent of the problem.”
After surgery, patients typically stay in the hospital for 10 days – the first few days in the Pediatric Intensive Care Unit (PICU), then three to five days on the inpatient unit. It is important that the surgery be done in a children’s hospital. Children who have this surgery often need to see a variety of specialists, including pediatric pulmonologists, pediatric anesthesiologists, pediatric surgeons and child life specialists. “These patients have pre- and post-operative issues, and need the multidisciplinary care that only a children’s hospital can provide,” Erickson said. Following the procedure, patients are back to normal in a month or so and can participate in nearly all activities except contact sports.
“The VEPTR has shown great promise in children with these types of problems,” Dr. Erickson explains. “Instead of shortened lives or lives with curtailed activities, these kids will be able to live healthy, normal lives.”
