Two-year-old Ariana Martin had a rough start to life. A fraternal twin, she was born without four ribs on her left side, and with several health problems , including severe scoliosis. With an under-developed chest cavity, Ariana faced severe lung disease and difficulty breathing.
"It was very stressful knowing that we would have a long and difficult road ahead of us," recalls Ariana's mother, Nicolette Pompa. "I wondered if she would be able to tie her shoes, ride a bike - just the normal, regular things kids do."
AdvertisementUntil recently, there were few options available to help Ariana - and the tens-of-thousands of children like her born with a deformity of the chest wall or spine - to grow normally into adulthood.
But now a surgically-implantable device at the University of Michigan C.S. Mott Children's Hospital is bringing new hope to these children and their families.
Called VEPTR, for Vertical Expandable Prosthetic Titanium Rib, this implantable device is designed to mechanically stabilize and lengthen certain deformities of the spine and torso, allowing normal lung development and offering correction for some spinal conditions. Mott Hospital currently is the only hospital in Michigan to offer the VEPTR device.
"The options to spread the ribs and correct the spine to help children like Ariana were once quite limited within the realm of pediatric surgery," says Frances A. Farley, M.D., chief, Pediatric Orthopaedic Surgery at Mott Hospital. "Now with VEPTR, we're able to create room in the torso to allow the lungs to grow, and we're even able to control the scoliosis, or curvature of the spine."
VEPTR gives the rib cage room to grow when the child's breastbone fails to support normal breathing and lung growth. During surgery, the device - a curved metal rod - is attached perpendicular to the patient's natural ribs and lumbar vertebra.
Most often used for Thoracic Insufficiency Syndrome, a condition in which the thorax is unable to support normal respiration or lung growth, VEPTR can be implanted in children as young as three to six months old, or even immediately following birth if the child is experiencing severe breathing problems, says Farley.
As the patient grows, the device is expanded or replaced about every six months. With each expansion, the device is lengthened about a centimeter. Once the spine fuses, typically between the ages of 10 and 12, the VEPTR is removed and the child may then have surgery to correct any spinal problems. The use of the device prior to this surgery actually makes the procedure more "straight forward," say Farley.
About nine months ago, Ariana underwent surgery to implant the VEPTR device. The procedure kept her in the hospital for about a week and in intensive care at Mott for a few days.
Since then, Ariana has had the device lengthened, and Farley is very pleased with her progress.
"We have increased the size of Ariana's chest cavity, and she's able to run and play, and her scoliosis has stabilized," says Farley, associate professor, Department of Orthopaedic Surgery at the U-M Medical School. "Our hope is that the VEPTR will allow her spine to grow and stay as straight as possible, and that her chest cavity will continue to grow to allow for normal lung function."
Ariana's mother also is optimistic about the future of her daughter's health and development.
"She's happy and very playful with her twin sister. I think Ariana can do anything now. I have no doubt in her whatsoever," says Pompa. "She has a lot of surgeries to face, and I think she's going to go heads up with all of them, and that she's going to do just wonderful."
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