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Pectus Perfected
For ten years Cody Chase didn't pay much attention to the unusual inward curve of his chest. After all, his two brothers, his dad and his uncle had the same condition. Called pectus excavatum, it is a chest wall deformity in which several ribs and the sternum (breastbone) grow abnormally, producing a caved-in appearance. The problem is present at birth, sometimes occurring by random chance and sometimesÑas in Cody's caseÑ with familial incidence, inherited from a parent. About 1 in 300-400 children are born with pectus excavatum, and nearly 80 percent are boys.
Cody's pectus excavatum was the most noticeable in the family. Years ago his parents discussed surgical treatment with a local surgeon and they were told that Cody was a little young for corrective surgery at that point. They were glad to have more time to make a decision, because the standard procedure for pectus repair was an invasive and extensive open surgery.
By the time Cody was ten the pectus seemed to be getting worse, and occasionally he was teased about it. He also seemed to be having difficulty running when he played sports, becoming winded and tiring more easily.
An Internet search led Cody's mom, Karen, to Dr. Hebra. She had been reading about the "Nuss procedure", a fairly new way to fix pectus excavatum with minimally invasive techniques developed especially for children. One of the most experienced surgeons in the country with this new procedure was André Hebra, M.D., a pediatric surgeon trained at the Children's Hospital of Philadelphia, currently the chief of pediatric surgery at the Medical University of South Carolina.
When Dr. Hebra examined Cody he confirmed that the pectus was severe enough to warrant surgical repair. The CT scan he ordered showed that Cody's heart and one of his lungs had been pushed up and over to the side as his ribcage and sternum were growing in such abnormal way. Without surgery, the problem could worsen when Cody began the rapid growth that comes with puberty.
Cody's parents gave him time to think all this over and decide if he felt ready for surgery. He decided it was a go. The operation would be done soon after Cody finished fourth grade.
Mighty MIRPE
"Cody was an ideal patient for minimally invasive pectus surgery," explains Dr. Hebra. "The severity of his pectus meant he was likely to have medical problems in the future if it were not corrected. He was old enough to understand the procedure and the care he would need in the week after surgery, but young enough that his cartilage and bones were still very malleable, making the operation much easier."
The traditional surgery to correct pectus excavatum involved making a large incision through the front chest wall and cutting through a good deal of muscle, cartilage and bone. The affected cartilage was removed and the sternum was fractured in order to allow for remodeling of the chest. Frequently the surgery took four to five hours and had significant blood loss, says Dr. Hebra, who directs the Minimally Invasive Pediatric Surgery Program at MUSC.
"The beauty of minimally invasive repair of pectus excavatum (MIRPE) is that it requires very small incisions on each side of the rib cage and it is not necessary to cut or fracture any bone or cartilage."
Other benefits of MIRPE include shorter operating time, minimal blood loss, fewer post-operative infections, minimal complications, outstanding cosmetic results, and an early return to full activity.
Cody's surgery, a pretty typical MIRPE procedure, took about 40 minutes. His pediatric anesthesiologist administered general anesthesia and also placed an epidural catheter in the space near Cody's thoracic vertebrae. The Pain Management team confirmed the observation that an epidural catheter was an excellent way to deliver pain relief medication in the first few days following surgery. Even with the minimally invasive procedure, there is some pain for several days due to the forceful displacement of the sternum and cartilage by the pectus bar.
Cody spent six nights on the Hospital's medical/surgical unit, and his mom stayed with him every night. "The first few days were rough going because of Cody's discomfort and limited mobility," Karen remembers.
What Cody remembers most from his stay is watching videos and trying to get enough sleep!
Cody's dad, Patrick, took charge of getting him up and walking the hallways to help speed his recovery. The day that Cody left the Hospital was the last day that he needed any type of pain medication. Progressive exercise was prescribed to help with Cody's recovery.
Cody was moving very well by the time he returned to school that August. His only recommended restriction was that he shouldn't participate in contact sports for the first 6-12 months after the surgery.
A Farewell to Bars
For most patients who undergo MIRPE surgery, the pectus bar stays in place for two years. Every few months Cody visited Dr. Hebra's office for a follow-up exam. When Cody and his classmates traveled to Tallahassee, FL, on a field trip, his teacher brought along a note explaining Cody's surgery--just in case the pectus bar set off the metal detector in the state Capitol! Fortunately, that wasn't a problem.
The only problem Cody encountered came after he turned twelve and began a growth spurt. The stainless steel sutures that attached the small stabilizer bar to his chest muscle eventually gave way. He noticed an occasional pain in his side, which always went away once the bar settled into a new position. He never had any additional problems and always felt great.
Last June, Dr. Hebra removed the bars in a brief outpatient surgery, and Cody was pleased at how quickly he was feeling fine. These days he is an active seventh grader who enjoys Little League Baseball and tae-Kwan-do classes and competitions (even full frontal combat!), not to mention playing, swimming, riding his bike, and playing with his friends and siblings.
"I think we all agree that surgery was a good choice for Cody," Karen says. "We've let our other boys know that they can think about whether they might want the same procedure, and when they might feel ready."
MIRPE Grows Up
Dr. Hebra is a co-investigator in a multicenter outcomes research study to follow the long-term results of patients who undergo MIRPE surgery. The procedure can now safely be done in children as young as seven years of age, and through the late teens. In fact, Dr. Hebra has received special permission to treat several adult pectus patients.
"We recently presented our experience to the Society of Thoracic Surgery with adult patients who were treated with this technique," he explains. "Our report, which was published in the American Surgeon journal, is one of the first published on this technique being applied to adult patients."
"The minimally invasive technique has revolutionized the treatment of pectus excavatum in children and adults. Since its first description in the surgical literature almost 10 years ago, the operation has had several technical refinements and is now considered highly effective in the treatment of this congenital chest wall malformation. The modern trend in surgery is to provide less invasive treatment options without compromising outcomes."
For more information on pectus surgery using the minimally invasive technique, visit emedicine.com.
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