Adèle Noyer’s parents saw the indentation in her chest even when their daughter was a baby. They weren’t completely surprised. Both her father and grandfather also had slight hollows in their chests. All three had a condition called pectus excavatum – commonly known as “sunken chest syndrome” – in which unusual growth of the rib cartilage pushes the breast bone inward.
“It's like if someone just sucked out the bone and the fat out of that one place,” said Adèle. “I just thought it was normal.”
But by third grade, she was having a hard time keeping up with friends on the playground and was out of breath faster. Her pediatrician referred the family to a pediatric surgeon at UCLA Health, who explained how surgery could eventually correct the issue.
In the meantime, the dip in her chest was measured every year. By seventh grade, it had contracted three inches. Adèle’s stamina had markedly decreased, and she struggled to complete two laps as a swimmer on the school’s team.
So in early March of this year, soon after a growth spurt tapered off, Adèle, now 13, underwent surgery at the UCLA Mattel Children's Hospital to repair her sunken chest. With two small incisions on either side, a titanium bar was inserted across her chest under her sternum.
“The bar is custom bent to the patient's shape, and the correction is immediate,” said her pediatric general surgeon Justin Wagner, MD. “But the remodeling of the chest shape is the process that takes a little bit longer. That's why we'll leave the bar in for three years to allow it to heal, scar and set. Then we'll take the bar out at a second procedure.”
Chest wall abnormalities are fairly common, affecting 1 in 300 to 1 in 500 people, with males affected three to four times as often as females. UCLA Health’s dedicated pectus program repairs a range of conditions in children to adults, from non-surgical interventions for mild defects to more severe cases in which the breastbone compresses the heart and lungs.
“Most people going through this have a fear that there's no way the chest can look normal,” said pediatric general surgeon Veronica “Ronnie” Sullins, MD. “The majority of them are not dangerous. But, they also change over time. The ideal time to evaluate patients is during adolescence when the chest wall is changing quickly, but we care for patients from childhood to older adults.”
Pectus pioneer
The long history of UCLA Health’s pectus program began in the 1960s with Eric Fonkalsrud, MD, an international authority on the management of chest wall deformities and a pioneer of pectus surgery. Dr. Wagner recalled the privilege of attending informal learning sessions over popcorn and soda in Dr. Fonkalsrud’s office.
“His teaching is so ingrained in pediatric surgery that it's hard to come across somebody who hasn't learned from him,” said Dr. Sullins, also an associate clinical professor of pediatric surgery at the David Geffen School of Medicine at UCLA. “I have no doubt that the people who taught me how to do pectus surgery probably learned from somebody who is connected with him.”
With each patient, surgeons first diagnose the pectus excavatum, which can range from a narrow cup shape to a wider bowl or plate shape. A physical exam as well as imaging, such as an x-ray or CT scan, can help assess the severity. A quantitative determination is made by measuring the space between the front of the chest and the spine in the most sunken portion and comparing that to the width of the chest at its widest.
“The question always comes up about whether this is a functional problem or an aesthetic one, and there's definitely a component of both,” said Dr. Wagner, also an assistant clinical professor of pediatric surgery at the David Geffen School of Medicine at UCLA. “The first consideration is the way a patient feels and the way it's affecting their lives. If a patient says to me that they're really unhappy, that they're unable to keep up with their peers and have trouble breathing, these are major contributors to the decision to have surgery.”
Sunken chest syndrome is most commonly repaired with the Nuss procedure, the minimally invasive insertion of the titanium bar. An important aspect of the approximately two-hour surgery and its aftermath is managing the patient’s pain. The surgeons have implemented and studied outcomes with intercostal nerve cryoablation as one method to minimize pain.
“We're correcting a defect that is formed over time,” said Dr. Sullins, “so suddenly moving the chest wall up, when it was down, can be kind of painful, especially with nerves coming around each rib. So we use a probe to freeze the nerves and make them numb for about three months.”
Most patients are discharged the next day after surgery. Cryoablation, along with Enhanced Recovery After Surgery (ERAS) protocols, have also greatly cut down on the need for opioids for pain relief.
“The first night at home, I woke up Adèle every three hours to give her ibuprofen, Tylenol and Gabapentin (a nerve medication), but none of the hard stuff,” recalled Linda Noyer, Adèle’s mother. “After three days, I gradually started to taper her off.”
Adèle said she could feel the titanium bar “like braces in my chest,” but eventually got used to it. She was back in school after a week.
There were specific instructions to follow for the three-month recovery.
“We want them up and walking around when they wake up from surgery,” said Christine Dichter, NP, with the surgery team. “They’re slow to move over that first week, but we really encourage it because it’s one of the best things they can do for their recovery. What we don't want is heavy lifting, lying on either side or stomach, or backpacks, because the bar could slip. We want that bar to really stay in place without movement.”
By her birthday in June, Adèle was back to all her regular physical activity. She built up her stamina – swimming laps was no longer a breathless challenge. “The surgery was really worth it,” she said. “I have no regrets.”
“Obviously her breathing and stamina are important,” said Noyer, “but to be a middle schooler and not worry about how she looks when wearing swimsuits or tank tops, has been great for her confidence as well.”
Adult patients
Most pediatricians are now aware enough of pectus excavatum to refer their patients to pediatric surgeons. But an older generation may not realize the need for surgery until the condition interferes with daily life.
Leigh Dannhauser, 32, had a two-inch divot in her chest for years. But she just thought it was “weird cleavage” and wasn’t self-conscious about how it looked.
But in 2021, her physical endurance markedly decreased. The UCLA Law student couldn’t complete her CrossFit workouts. Bike rides to the beach became difficult. She walked slower. During the summer in New York City, she avoided the subway because the stairs would leave her “embarrassingly short of breath.”
More worrying were the heart palpitations when lying down, and eventually, even when she was sitting up.
With a history of cardiac disease in Dannhauser’s family, cardiologist Megan Kamath, MD ran some tests. She then referred Dannhauser to her colleagues in thoracic surgery. Imaging showed that her sternum was compressing her heart and shoving it over into her lung.
“I wasn’t having actual palpitations,” said Dannhauser. “I was just feeling my heartbeat hitting the sternum. It's definitely scary to hear that something is actually wrong, but it was validating to me that it wasn’t because I’m out of shape or something in my head. We now had a path forward.”
Because the adult and pediatric surgeons work so closely together, Dannhauser was able to see both during her appointment. Dr. Sullins performed the Nuss procedure in late July 2022.
“When it’s a very focal defect, we sometimes need two bars to get a good correction across the entire length of the sternum,” explained Dr. Sullins. “Like a seesaw, if you put a bar where it's pointiest, then there might be some instability of that bar. We need to put in two bars so both of them can be stable.”
Dannhauser saw the smoothness of her chest immediately after surgery. The heart palpitations disappeared. Her first month of recovery was tough. If she moved the wrong way, it was very painful. It hurt to cough or sneeze. But she made it to the first day of her final year of law school – rolling backpack in tow – and lots of help from classmates, friends and family.
By the end of the year, she felt more normal, and was back in CrossFit the summer after graduating. She even climbed Mt. Fuji.
Next July, the two bars in Dannhauser’s chest will be removed.
“The surgery was worth it,” said Dannhauser. “I climbed an entire mountain in Japan when literally a year earlier, I couldn't climb the stairs out of the subway.”
