Know the Risks of Pectus Excavatum (Sunken Chest)

Pectus excavatum, or sunken chest, is the most common congenital chest wall abnormality in children. Although some may think of the abnormality as purely a cosmetic problem, the limited chest cavity space can displace the heart as well as limit lung capacity, says Dr. Mustafa Kabeer, a pediatric surgeon at CHOC Children’s. Fifteen percent of patients can experience arrhythmia (an abnormal heart rhythm) or mitral valve regurgitation, wherein the heart valves allow blood to leak back into the heart, as a result of the inward compression from the sternum.

pectus excavatum
Dr. Mustafa Kabeer, pediatric surgeon at CHOC Children’s

Surgery to expand the chest wall can eliminate many symptoms. The pediatric surgeons at CHOC perform the Nuss procedure to repair pectus excavatum.

The Nuss procedure is a newer, minimally invasive procedure that dramatically reduces the appearance of incisions and the time spent in the operating room. A small incision is made on either side of the chest, and a small camera is inserted for observation as the surgeon passes a thin, curved metal bar through the chest cavity below the sternum. When the bar is flipped, the sunken chest is instantaneously repaired. The bar remains in place for two years, and is periodically monitored by a pediatric surgeon.

pectus-excavatum-illustration
During the minimally invasive Nuss procedure, A small incision is made on either side of the chest, and a small camera is inserted for observation as the surgeon passes a thin, curved metal bar through the chest cavity below the sternum.

A historical approach to these surgeries involved a large incision across the chest, and up to six hours in the operating room as the chest wall was taken apart and then reconstructed, known as the Ravitch procedure. There can also be significantly greater blood loss with the Ravitch procedure, and virtually none with the Nuss procedure.

The pectus excavatum team at CHOC is comprised of experts in pediatric pulmonology, cardiology and surgery. The multidisciplinary team performs various tests to examine heart and lung function, before and after surgery. By using the latest techniques in minimally invasive surgery, along with recent improvements in pain management, patients are able to return home and get back to their daily activities sooner than ever before.

Although the cause is unknown, 40 percent of patients report a family history of pectus excavatum, and 40 percent of cases occur in tandem with scoliosis, says Kabeer.

Parents may notice an indentation in their child’s chest wall either when they are first born, or closer to puberty, when changes in the chest wall can become more pronounced.

“When pectus excavatum presents itself closer to puberty, it may be difficult for parents to notice, since teens or pre-teens are more clothed than they were as babies, so their parents might not see the change in their chest wall right away,” says Kabeer.

Warning signs tend to be subtle, but if your child experiences frequent shortness of breath, difficulty breathing during exercise, chest pain or light- headedness, or otherwise is not able to keep up with their peers, consult your child’s primary care physician, who may refer you to a pediatric surgeon or pectus excavatum specialist.

VIDEO: Get more answers to pectus excavatum questions

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Alicia’s Story: Repairing a Right-Sided Congenital Diaphragmatic Hernia

CHOC Surgical NICUAlicia was born at full term, beautiful with 10 fingers, 10 toes and a life-threatening defect buried inside her tiny chest.

She had a congenital diaphragmatic hernia (CDH), a condition where a hole in the diaphragm allows abdominal organs to move into the chest. And this case was especially serious.

Though less common, hernias on the body’s right side are more dangerous because the liver, a larger organ, can move into the chest cavity, impairing lung development, impeding blood vessel functionality and ultimately causing pulmonary hypertension, says Dr. Mustafa Kabeer, Alicia’s surgeon at CHOC Children’s.

Just 20 percent of CDH cases are right-sided, and about 40-50 percent of babies nationwide survive their treatment; conversely, more common left-sided hernias yield about an 80-90 percent survival rate, Dr. Kabeer says.

Prenatal meetings remain key

After the diagnosis, Alicia’s mother Marlen began meeting with Dr. Kabeer and other specialists to prepare for her baby’s birth and treatment afterward.

“It helped because we had the prenatal meeting,” Dr. Kabeer says. “That way, parents can connect a face to a particular job in the care of their baby. They are educated and know what to expect during treatment.”

Just hours after her birth, Alicia’s condition began dramatically deteriorating. A transfer to CHOC was necessary, and Marlen and her husband were warned that Alicia would not likely survive the 2-mile ambulance ride.

But she made it, and quickly began treatment under a life-saving device called extracorporeal membrane oxygenation (ECMO) to help her compromised lungs. First pioneered at CHOC about 40 years ago, ECMO is a heart and lung bypass machine that can be used to rest a failing heart or lungs, providing complete support until the organs recover.

About a week later, Dr. Kabeer performed the procedure to repair the diaphragmatic hernia, all while Alicia remained on ECMO with substantially high risks of uncontrollable bleeding due to the blood thinners needed while on the treatment.

Alicia sailed through the surgery with little bleeding, but within the next day or so, Dr. Kabeer needed to perform two separate procedures to relieve pressure building inside her abdomen that compromised blood flow to the lower half of her body.

Two weeks old and five surgeries

Shortly after this surgery, Alicia began to bleed. During the next four days, she had ongoing bleeding and during the fourth day, lost about 1,500 mL of blood, or about six times her normal blood volume. Hospital staff kept her stable, and Alicia was taken off of ECMO. Two days later, Dr. Kabeer performed a final surgery to close her abdomen, which had been left open all of this time to decrease pressure.

“All of those surgeries were very high-risk surgeries,” Marlen says. “There was a very high chance she wouldn’t make it, but she did perfectly.”

Throughout the entire process, Dr. Kabeer communicated with Marlen and Omar about the risks of the surgeries. And like every other time Alicia’s parents were cautioned about her survival, the tiny infant fought back.

Baby Alicia today.
Baby Alicia a few months after her surgeries.

“Even though it’s a difficult subject, and a complicated, emotional and anxiety-provoking issue, we want parents to understand the problem their child is facing and that we’re trying to help them and their baby overcome it,” Dr. Kabeer says. “That connection and rapport are very important and it all stems from honesty.”

“It involved a very transparent discussion,” Dr. Kabeer said. “I laid out for them all of the issues and all of my concerns, and made them see that we’re going to do our best and face these challenges together. I want to give parents reassurance and security to know that not only are they in a good place, but they’re with staff who are well trained.”

Coordinated care in the Surgical NICU

Between her five surgeries and afterward, Alicia was closely monitored inside CHOC’s Surgical Neonatal Intensive Care Unit, a special part of the hospital’s main NICU dedicated to the care of babies who need surgery.

In the unit, the team cares for patients jointly, discussing the cases of children like Alicia as a group and forming a treatment plan that often calls for the expertise of other specialties at CHOC.

After several months in the Surgical NICU, Alicia went home with her family. Today, she is a happy, charismatic four-year-old.

alicia-today-beach
Today, Alicia is a thriving four-year-old.

“Alicia’s case reinforces the fact that babies are extremely resilient,” Dr. Kabeer says. “It’s amazing that she tolerated all of this. Every patient is unique and this is a perfect example of why we should give them every chance possible.”

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Former CHOC Patient is Cancer Free Thanks to Da Vinci Robotic Surgical System

Julia Shenkman is a healthy and accomplished 17-year-old. She has a second-degree black belt in Taekwondo, is a member of her high school’s science team, and is fourth in her senior class in academics. Julia has another distinction: She’s a former CHOC Children’s patient who underwent a surgical procedure that involved the use of the then-landmark Da Vinci robotic surgical system.

In late 2003, CHOC became the first pediatric hospital in California to begin offering robotic surgery for children. The Da Vinci robot was an improvement over minimally invasive laparoscopic surgery because it gave surgeons a better view inside the surgery site and allowed them to more precisely manipulate instruments, which translated to less pain, fewer complications and shorter recovery time for patients.

In January 2006, Julia, then 8, was whisked to the emergency department at St. Joseph Hospital for severe abdominal pain and frequent vomiting. Blood work and X-rays for appendicitis turned up negative. It wasn’t until Julia’s doctor at CHOC, Mustafa Kabeer, pediatric surgeon, ordered an ultrasound that the cause of her distress was found: a large mass in her right ovary. Using the Da Vinci system, Dr. Kabeer operated on Julia and successfully removed the tumor, which was malignant. After three months of chemotherapy, Julia recovered and remains cancer free.

Julia with Dr. Mustafa Kabeer, CHOC Children's pediatric surgeon.
Julia with Dr. Mustafa Kabeer, CHOC Children’s pediatric surgeon.

Pediatric surgeons at CHOC have performed about 100 robot-assisted surgeries, says Dr. Kabeer, who before coming to CHOC helped implement the world’s first pediatric surgery robotics program at Children’s Hospital of Michigan in 2001. Recently, Dr. Kabeer started using the Da Vinci system to perform complex operations through one incision in the belly button, such as for removal of the gallbladder. The Da Vinci system at CHOC was made possible by a $1.5 million sponsorship from Costco Wholesale Corp.

“This type of technology helps us perform a wide variety of surgeries — some of them very complex,” Dr. Kabeer says. “It takes time, commitment and vision to start and maintain such a program, but this is one of the unique things about CHOC: We want to do this, and we are committed to doing it.

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CHOC’s Surgical NICU Offers Pioneering Coordinated Treatment

ICHOC Children's Surgical NICU nfants needing surgery require special attention, and a unique feature of CHOC’s Neonatal Intensive Care Unit (NICU) provides just that.

CHOC’s Surgical NICU, a dedicated space within the NICU, uses a comprehensive approach to care for these tiny patients. A coordinated treatment protocol – used in many adult intensive care units nationwide – has shown to result in fewer patient complications, better outcomes and faster discharges. And, CHOC’s Surgical NICU physicians and staff continue to research new ways to make the care even better.

“CHOC has one of the only two Surgical NICUs like it at any children’s hospital in the country,” says Dr. Mustafa H. Kabeer, who with Dr. Irfan Ahmad, a neonatologist, co-directs the Surgical NICU at CHOC.

“We have a room dedicated with eight beds and a second room with two beds that allow for surgeries to be done right there in the NICU. It’s a unique setting in which we have multi-disciplinary, coordinated care.”

“Babies in the NICU are very critical to start with and things can change minute by minute. When you add in the risks of surgery, it becomes more complex. We coordinate care through better communication with physicians and the nurses and the family. We involve all of these groups so everybody is on the same page. We have joint rounds with residents and fellows, and have initiatives on research and teaching, with monthly lectures on select topics. We are collecting data on outcomes and how the babies do,” says Dr. Kabeer.

Dr. Ahmad adds, “The experience has been so positive. We are all really happy with the care our patients are receiving. The surgeries and anesthesia have become safer.”

In a new effort, CHOC’s Surgical NICU team has begun a new research project to examine better pain control for babies following surgery. “This is going to be really big and is being planned by Dr. Kabeer from surgery, myself from neonatology, and Dr. Rebecca Sangster from anesthesia,” says Dr. Ahmad.

Other projects include investigating ways to decrease the occurrence of hypothermia, or low body temperatures, for babies having surgery. In addition, the Surgical NICU staff is maintaining a patient database that will help the staff design future quality improvement projects and clinical studies.

The Surgical NICU is special in other ways. Patient rounds there often include a dozen or more specialists who join the physician and nurses. “These rounds are very different than what goes on anywhere else. We try to educate the families about what this means and how unique this is,” says Dr.  Kabeer.

Babies in CHOC’s Surgical NICU receive consistent and coordinated care from a multidisciplinary healthcare team that includes neonatologists, nurses, surgeons, respiratory therapists, nutritionists and other specialists who may be needed. Parents and family members round out the team. The family joins the clinicians to discuss care plans, share information and make care decisions. Each patient receives a well-coordinated and consistent treatment plan.

Pediatric surgeons perform many types of surgeries in the Surgical NICU, including lung and thoracic surgeries, robotics surgeries, head and neck surgeries, repairs of umbilical and inguinal hernias, among a variety of different operations.

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Thank you, Parents and Families!

As I help CHOC Children’s celebrate its 50th anniversary, the overwhelming feeling for me and many I’ve met around the hospital is gratitude.

For me, I’m grateful for the care I received when I fell out of that tree in 1964 and the friends I’ve made ever since. So many patients I’ve met are thankful for the bright futures and milestones they’ve achieved thanks to CHOC’s care.

And CHOC’s physicians are no exception. They’re grateful for the trust that parents and families instill in them each and every day. In this video, CHOC physicians express their gratitude.