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On the count of 3, these patients see their lives changed

Dawn Brazell | MUSC News Center | October 13, 2014


Pectus excavatum
Photos by Sarah Pack

 
Medical student Ashley Smith, left, holds the camera that goes inside the chest while Dr. André Hebra and Dr. Mary Jordan, medical resident, get ready to rotate a bar "on the count of 3" during a pectus excavatum surgery to correct a congenital deformity. 

It’s mid-morning in the operating room and pediatric surgeon André Hebra, M.D., pulls out a suction bell to use on a caved-in area of the chest of an 11-year-old boy. The suction cup is used to lift the boy’s chest enough so Hebra has room to insert a camera and place a strut through a small incision on the side of the chest.

Hebra uses a stainless steel bar as a brace to instantly remodel a congenital deformity that has worsened over the years causing a condition known as pectus excavatum or funnel chest.

pectus excavatum 
Dr. Andre Hebra adjusts the bar used in the pectus excavatum surgery while Dr. Mary Jordan, medical resident (middle), and medical student, Ashley Smith, watch. 

“We do all of our work through tiny incisions,” he said, of the minimally-invasive surgery for a condition that is more common in boys by a 4-to-1 ratio. The boy is undergoing a pectus repair for a condition that can cause breathing and heart complications as well as psychological distress. The condition occurs in an estimated 1 in 300 to 400 births.

Hebra, who is chief of surgery of the Medical University of South Carolina’s Children’s Hospital sizes up what bar he’ll be using and checks sizing, noting how to customize the remodeling to the patient’s unique anatomy. He takes the bar “back to the garage” to a table in the operating room where he has a specialized tool to adjust it so it’s a custom fit. Hebra wants the boy’s chest to look as natural and as perfect as possible.

Bent and molded, the bar is ready for insertion. Hebra, using camera-guided views slips the bar beneath the sternum, navigating past lungs and heart. The U-shaped bar has been notched in the middle for a better purchase point beneath the sternum once it gets turned. At this point, it rests upside down.

He asks resident Mary Jordan, M.D., if she’s ready.

 chest wall defomity
 Drs. André Hebra and Mary Jordan prepare to turn the bar during a pectus excavatum surgery.

Hebra starts the countdown. “1-2-3.” At 3, they rotate quickly in unison, like turning the crank halfway on a wheel. The bar and thus the chest pops up. Hebra eyes their work and smiles broadly.  “Bye, bye pectus. See, the sternum is nicely corrected.”

It’s this instant gratification that makes this surgery one of his most favorite operations, coupled with the technical challenges that it poses, he said. “I’ve been doing this surgery for so many years, I’ve learned a lot to perfect the techniques, and I’ve learned a lot from other surgeons and from my patients.”

Though the effect is immediate, the bars remain in patients generally for three years, remodeling the chest and preventing it from growing back in.  “You watch them go through it and see the outcome and how happy they are and what an impact it has. It makes you feel good about what you do. We put them through a pretty significant intervention, and you watch the impact it has on their life forever. It is very rewarding.”

The depressed sternum creates less space for the lungs and can displace the heart to the left side, which may lead to significant physical problems, he said. The psychological effects can be even worse. Most of his patients are teenagers in which this condition impacts self-esteem and confidence.

“We see in real life as we take these patients and correct the pectus how much they feel transformed and how much better they feel.  They build confidence and they actually perform better in sports and social activities. Their lives frequently are completely changed.”

Overcoming Misconceptions

The best window for treatment is between 8 to 12 years of age because the rib cage still is fairly soft and malleable, and recovery is faster. Most of the patients actually are older though, because the deformity tends to progress as they age. He has had a patient as old as 45, he said.

“The most common misconception is that a child will grow out of it,” Hebra said, adding that he has never seen it improve and that in 90 percent of cases it will worsen. The reality is that because it is a genetic defect of the growth of the ribs and sternum, as a patient is growing, particularly in puberty and adolescence, that accelerated growth is going to go in the wrong direction.

The initial technique to do pectus repairs was the Ravitch method, which involved a fairly large incision in the front of the chest and breaking the sternum. The newer Nuss procedure is minimally invasive, but it’s still a significant intervention. “You’re working around the heart, the lungs and the vascular structures. It is an operation that carries significant risks so that’s why it’s extremely important that you must have extensive experience in performing the procedure and having your own way of doing it. You have to eliminate the risks of complications commonly seen during the learning curve when someone is just starting to learn and do the operation.”

pectus surgery 
Medical student Ashley Smith (left) observes camera images as Dr. André Hebra performs a pectus excavatum surgery. 

Hebra makes sure his teenage patients know recovery means several days in the hospital. “We’re bending the bone so there is a lot of pain.” Most patients also receive a thoracic epidural to help with pain management and reduce side effects from oral pain medications. After a month patients generally are back to a normal lifestyle, though contact sports are discouraged until the bar is removed.

Having trained with pediatric surgeon Donald Nuss, M.D., who developed the Nuss procedure in the late 1980s, Hebra said MUSC stays in the forefront of new developments that can be offered. MUSC has made significant contributions in developing standards for how the procedure is done and how to best stabilize the bar. Today, Nuss and Hebra frequently travel abroad to teach and train surgeons on how to perform this operation.

Bar displacement has been a significant problem, happening in one in 10 patients and requiring a reoperation. Hebra developed techniques to prevent this from happening, including the addition of a third point of fixation that secures the bar at the top of the chest to address the issue. The chances of bar displacement now have dropped to less than 2 percent. He hasn’t had a case in the last 10 years where he has had to operate on a patient for bar displacement.

“We also are actively involved in teaching other surgeons all over the world in how to do this operation and how to have good outcomes because that’s really what’s it all about – to make sure this is being done safely all across the world so everyone can have a good outcome.”

Hebra, who has been doing the procedure for the past 15 years, said the operation has become more popular because there’s more information on the Internet and by word-of-mouth about the advantages of the procedure.  In 1999-2000 there were a few hundred cases done in the U.S., and in 2012 that number had jumped to 50,000 cases worldwide, he said. “The number of cases has grown exponentially. Dr. Nuss and I are part of an international chest-wall interest group so we we have the opportunity to meet once a year with an international group of surgeons that have an interest in pectus surgery and that group now has 370 members from all over the world.”

The international collaboration has led to more awareness of all the different ways that this condition, also known as funnel chest, presents and of the various options available for treatment. The group also has learned that the condition is common in Caucasians and Asian people. However, it is unusual in African-Americans.

Hebra said he’s glad awareness is growing.

“I think this is a wonderful thing because it allows children and young adults affected by it to get treated and get the help they need. It has an impact on both sexes as far as their self-esteem,” he said, adding that males tend to be affected more because they lack breast tissue, which can sometimes mask and “cover” the condition.

“It’s fun to watch the patients and their family when you say, ‘Have you seen your chest?’ after surgery. We’ll pull the cover down. The parents go, “Oh, wow” because it’s an immediate result. It’s instantaneous. It’s pretty impressive. It’s very, very rewarding to see the relief and happiness on their faces.”

 


 

 

 

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