Stanford Medicine Children’s Health is the first-in-nation to offer a treatment for small jaw other than surgery
When Brittany and Ben Proulx started planning their family, they both had one big worry – passing on Stickler syndrome, a genetic condition, to their kids. Stickler comes with a distinctive receding chin that can interfere with normal breathing and feeding, collectively called Pierre Robin sequence.
“It was always the first concern to pop in our minds. Our anxieties were realized when our second baby was born with Stickler syndrome, unable to breath on her own,” says Brittany Proulx.
The care team at another children’s hospital suspected the couple’s daughter Madelyn had severe Stickler syndrome prenatally, so a special team was ready to care for her at birth. She was immediately sent to the Neonatal Intensive Care Unit (NICU) and given a nasopharyngeal airway (NPA) – a thin tube placed in her nose to keep her blocked airway open – and a feeding tube. Her doctors recommended jaw elongation surgery, also called mandibular (lower jaw) distraction osteogenesis (MDO).
Seeking an alternative to jaw surgery
Jaw elongation (MDO) surgery requires at least two operations under general anesthesia several months apart. The first operation involves cutting the lower jawbone through the skin on both sides of the upper neck and implanting hardware that applies tension across the cut jawbone, making it slowly separate and allowing new bone to fill in the gap. The process continues until the lower jaw matches or is slightly forward of the upper jaw. Babies remain intubated for several days following the first surgery and pain is managed by narcotics. As the lower jaw bone gets longer, the tongue is pulled forward with the elongated bone, and the upper airway becomes open, letting babies breathe freely and feed better.
The second surgery is to remove all hardware after the jaw elongation is complete. For severe Stickler syndrome, MDO surgery or tracheostomy are the only treatments offered at nearly all children’s hospitals in the United States.
Because Brittany had previously watched several of her nieces undergo jaw distraction surgery for Stickler with multiple complications and repeated surgeries in subsequent years, she hoped for an alternative. Brittany shared her wishes with Madelyn’s treating otolaryngologist (ENT) who mentioned that Stanford was trying something new.
“When we heard that there was one doctor in the United States—just one—who was doing something different, and who happened to be 2500 miles from home, we knew we needed to explore it,” she says.
Relocating across the country to receive a novel treatment at Stanford Children’s
Doctors from the family’s hometown hospital reached out to Stanford Medicine Children’s Health, and connected them to HyeRan Choo, DDS, DMD, MS, Neonatal and Pediatric Craniofacial Airway Orthodontist and Medical Director of the Stanford Orthodontic Airway Plate (SOAP) Treatment Program. Dr. Choo had been exploring the use of orthodontics, specifically an OAP, to treat children born with micrognathia (small jaw) for years.
An OAP is a customized removable oral device (like an orthodontic retainer) that helps correct defects in a baby’s airway and lower jaw. Since the treatment concept was first introduced in Northern Ireland in 1967, the treatment has been offered only at a few centers outside the United States. Through research and over a decade of clinical experience of treating neonates with congenital facial deformities, Dr. Choo invented her OAP design and its novel implementation protocol named Stanford OAP (SOAP). Dr. Choo treated her first baby at Stanford Children’s in 2019. From there, The SOAP Treatment Program was born.
“Brittany reached out to me with photos of Madelyn and her medical records so we could conduct a preliminary multidisciplinary evaluation. She had a lot of really good questions, and five days later she transferred care to us,” Dr. Choo says.
While moving their family temporarily across country seemed beyond daunting, the Proulx’s didn’t hesitate. A medical plane for a NICU-to-NICU transfer was arranged within 48 hours. Fortunately, they could stay with family nearby Stanford Children’s.
“It was a whirlwind, but babies can get too old for the SOAP to work, so we hustled. We also felt that if we could serve as one more data point on treatments for Stickler syndrome and help advance that field of research, it would be a win-win. Madelyn could always still have the jaw surgery if the SOAP didn’t work, so we took a leap of faith,” Brittany says.
The family arrived on a Friday afternoon. They didn’t expect anything to happen until Monday, but Dr. Choo showed up to evaluate the baby and took an impression of the baby’s mouth on Friday evening. She built Madelyn’s custom SOAP over the weekend.
“Madelyn’s jaw was one of the smallest I’ve treated with the SOAP,” Dr. Choo says. “When a baby’s jawbone is small, it pulls the tongue over the airway making it hard to breath, sleep, or eat. With the SOAP holding the tongue out of the airway, babies usually are able to breathe on their own immediately. By promoting the tongue to move forward with bottle feeding exercises, the SOAP also stimulates the lower jaw to grow forward.”
Multiple specialists working together to help babies with small jaws
Evaluating a baby for the SOAP treatment involves an entire team of specialists, along with Dr. Choo. The SOAP evaluation team includes the world renowned pediatric Aerodigestive and Airway Reconstruction Center who provided airway management and airway surgical expertise to ensure the SOAP was the best treatment option for Madelyn. The evaluation team also includes experts from Neonatology, Genetics, Speech-Language Pathology, Critical Care, Pulmonary, Asthma & Sleep Medicine Center, Cleft & Craniofacial Center, and Pediatric Plastic Surgery at Stanford Children’s.
“For the SOAP treatment to be effective, we need to take a multi-specialty approach. The device is rigid, yet it has to deal with very dynamic and delicate soft tissues of the face and neck. The tongue, the throat, the voice box, esophagus, and the neck muscles are constantly moving during various functions such as crying, breathing, feeding, and swallowing, so we have to find a sweet spot for the device where it can work in harmony with the baby’s moving structures,” Dr. Choo says.
Keeping Madelyn’s airway safe was paramount, and the Aerodigestive and Airway Reconstruction team achieved this goal. They provided a comprehensive airway exam and also placed an endoscope (a thin flexible camera) in Madelyn’s nose to help Dr. Choo visualize the SOAP in action while Madelyn was crying and swallowing.
“We can show in real time where the SOAP sits in relationship to the tongue and larynx to help Dr. Choo determine how to best create the SOAP and how to adjust it as the baby grows,” says Douglas Sidell, MD, FACS.
Babies with small jaws that affect their airway often need experts from Stanford Children’s Voice and Swallow Program to help establish safe swallowing function. Madelyn was no exception.
“The SOAP is so simple, so common sense. And the risk is so limited. We thought Madelyn’s small jaw might be too severe for the SOAP to work, but what did we have to lose?” Brittany says.
Madelyn spent two weeks in the NICU at Stanford Children’s. Neonatologists monitored her response to the SOAP between Dr. Choo’s daily adjustments to ensure stable breathing, optimal weight gain, and sound sleeping on her back.
“This treatment is brand new in the United States, and we had to figure out many things together as we went along. Because it’s Stanford, figuring out ways to make care better is in our DNA. All of the specialty teams’ support gave me the confidence I needed to launch this program at our hospital,” Dr. Choo says.
Why Stanford Children’s for SOAP treatment
Stanford Children’s has what is needed to provide cutting-edge care because the hospital is focused on innovation with a culture of collaboration.
“At Stanford Children’s, we have multiple experts working together toward a shared goal,” says Karthik Balakrishnan, MD, MPH, surgeon-in-chief at Stanford Medicine Children’s Health. “We believe multidisciplinary care improves outcomes, speeds up diagnoses, reduces costs, and makes care safer and more efficient for patients.”
Doctors who conduct research and bring forward innovation are supported by hospital leadership and other teams who are always open to bringing new treatments to children.
“At Stanford Children’s our culture is to do the right thing for the patient, no matter what. We are driven by that,” Dr. Balakrishnan says. “We really feel the SOAP treatment adds great value for our patients.”
“Although MDO surgery is very successful, it is an operation that carries risks such as infection, excessive scarring on the skin, and damage to facial nerves, temporomandibular joints, and adult tooth buds. As a surgeon that performs MDO surgery, I welcome and support Dr. Choo’s new non-surgical treatment for infants with Pierre Robin sequence and Stickler Syndrome. I am excited that our unique multidisciplinary SOAP program is giving families other treatment options besides surgery,” says H. Peter Lorenz, MD, FACS, Chief of Pediatric Plastic Surgery.
Improvements after just a week in the hospital
Improvements happen fairly quickly and steadily with the SOAP. Toward the end of the first week, Madelyn started to bottle feed.
“When the SOAP is first placed you can see an immediate difference in how open the airway is and over time it is amazing to see children continually make real progress. These babies are getting excellent results and it’s wonderful to see,” Douglas Sidell, MD, FACS says. “With the SOAP, we see real changes in the structure of the facial skeleton, which required surgery in the past to make.”
The second week of Madelyn’s hospital stay was spent teaching her parents how to handle the SOAP on their own, including how to place it and clean it, and when and for how long it can be removed. Brittany and Ben mastered it quickly, and at the end of the week, they flew Madelyn back to Atlanta in a regular airplane, bringing her home for the very first time.
“Learning how to care for the SOAP on our own was intimidating at first,” Brittany says. “But after a few weeks at home it became a nonissue. She didn’t even cry when we put it in.”
Eating, breathing normally after six months
Madelyn wore the SOAP almost all of the time for six months. As her jaw grew forward, her airway naturally enlarged and her facial bone structure moved into balance.
“The SOAP treatment has been a godsend. My experience at Stanford Children’s gave me hope that pediatric medicine can be personalized treatment with doctors that empower parents as capable partners,” Brittany says. “It was truly healing through compassion with world class science at its finest.”
The family traveled back to Stanford Children’s every 4 to 6 weeks so Madelyn could receive regular checks from many experts and have her SOAP adjusted by Dr. Choo as she grew. She’s now home, eating and breathing naturally, and growing well. The couple is glad Madelyn was able to avoid surgery and other more invasive treatments, and the risks that accompany them.
“Fears of intubation and tracheostomy have been replaced by independent bottle feeds and 12-hour stretches of sleep. We are enjoying normal babyhood,” Brittany says. “We felt insane flying our newborn in an air ambulance across the country with nothing more than fingers crossed that this non-surgical option would pan out. But six months later, we can say that we would do it again in a heartbeat.”
100% success rate, even for severe cases of small jaw
Since implementing the SOAP treatment at Stanford Children’s in 2019, Dr. Choo has treated more than 25 babies with the support of multiple specialty teams. All babies who successfully passed the first week of the SOAP trial period were able to avoid jaw surgery or tracheostomy. Dr. Choo is grateful for Brittany and Ben for “being gutsy” and betting on the SOAP treatment and traveling across the country to give Stanford Children’s the chance to treat Madelyn.
“Our research data are very promising. When we evaluate these babies at the age of one year, their breathing and feeding are not different from those of any other healthy babies, which is hard to believe even for me,” Dr. Choo says. “One day, I want to see the SOAP become the first line treatment as the standard of care for all babies with Robin sequence across the nation and the world.”
Madelyn recently celebrated her first birthday, and all of the team members who cared for her at Stanford Children’s wish her a happy birthday and a happy life.
Learn more at https://oap.stanfordchildrens.org >
