Specialized otolaryngologists play a critical role in surgically repairing cleft lip and palate and caring for those born with the conditions throughout childhood, but the general public and referring physicians are not always aware of their significant contributions. “The field has largely been dominated by plastic surgeons who’ve done further training in craniofacial surgery,” said Laura Hetzler, MD, professor and vice chair of the department of otolaryngology–head and neck surgery at Louisiana State University in Baton Rouge and director of the of university’s residency program for the specialty.
What the general public and the cleft community often don’t know is that otolaryngologists who are trained in either craniofacial plastics or pediatric otolaryngology also perform corrective surgeries for the condition. These specialists are particularly well suited to this role due to their extensive training in head and neck anatomy and experience treating hearing, feeding, speech, and airway issues associated with cleft lip and palate.
Dr. Hetzler served on a 10-person task force on cleft and craniofacial surgery that was established in 2019 within the American Academy of Otolaryngology–Head and Neck Surgery (AAO–HNS) to address this and other related concerns. “The public is very well researched and we wanted to make sure we are recognized as an excellent combination of training for cleft lip and palate,” she said. “There is a fair amount of creativity and artistry that goes along with these surgeries, and marrying that form and function is something we do very well. No two cleft defects are alike, and no two surgeries are alike.”
After two years, the task force ultimately led to the formation of the Cleft and Craniofacial Surgery committee with a diverse mix of 20 pediatric otolaryngologists and facial plastic and reconstructive surgeons from across the country who perform cleft lip and palate surgery. In addition to increasing awareness, the committee’s focus includes expanding and improving research, education, and humanitarian efforts.
Both the task force and the committee were the result of a “seven-year dream” for Travis Tollefson, MD, MPH, director of facial plastic and reconstructive surgery at the University of California, Davis, and Steven Goudy, MD, MBA, director, division of pediatric otolaryngology and professor at Atlanta’s Emory University School of Medicine, who both believed in the importance of moving the field forward. “Steve and I have known each other since residency, but we took different paths,” said Dr. Tollefson. “He went into pediatric otolaryngology and I pursued facial plastic surgery. Even though we ended up in different specialties, we have cleft surgery and care in common.” The two otolaryngologists run interactive courses on the topic every other year, alternating coasts. “We wanted to create a bridge between pediatric otolaryngology and facial plastic surgeons to unify us and gather better information for research projects,” Dr. Tollefson said. “The task force was a way to get us all together at the same table versus just going to our separate meetings in our different specialties.”
Surgeons Receive Additional Cleft Training
After receiving their five years of training, otolaryngologists interested in these surgeries can continue on to fellowships in either pediatric otolaryngology or facial plastic surgery, both of which perform cleft lip and palate surgery and provide ongoing care up to adulthood and sometimes beyond. “[Cleft lip and palate surgery] is basically a subspecialty of the subspecialty,” said Brianne Barnett Roby, MD, an associate professor of otolaryngology at the University of Minnesota in Minneapolis and a pediatric otolaryngologist who serves on the new committee and specializes in facial plastic surgery. She added that helping reconstruct these patients’ congenital abnormalities is particularly compelling to clinicians who treat the condition. “It’s really hard for people who get training in cleft surgery not to be very passionate about it.”
During the COVID-19 pandemic, the task force met every two to three months over Zoom to define goals and create synergy among the specialties. They also formed a WhatsApp group of more than 85 head-and-neck cleft surgeons to provide support, enable discussion, and facilitate referrals for patients who move across the country. “Steve Goudy and I are confident that this young, diverse group of pediatric otolaryngologists and facial plastic surgeons on the committee can create measurable change in the care of patients with cleft lip and palate,” Dr. Tollefson said. “We were able to organically bring together a committee with equal gender distribution from different regions of the country with our intention to find the best people.”
In addition to Drs. Roby and Tollefson, members of the committee include Rajanya Shah Petersson, MD, MS, associate professor and, section director of pediatric oncology at Children’s Hospital of Richmond at Virginia Commonwealth University, and Krishna Patel, MD, PhD, director of facial plastic and reconstructive surgery, head and neck surgery at the Medical University of South Carolina in Charleston. All are eager to begin furthering the committee’s goals.
Public perception is a challenge. People think that pediatric otolaryngologists only do ear tubes and tonsillectomies, and when families are doing their research, we might be missed as cleft surgeons. —Rajanya Shah Petersson, MD, MS
Stressing the importance of increasing awareness, Dr. Petersson said, “Public perception is a challenge. People think that pediatric otolaryngologists only do ear tubes and tonsillectomies, and when families are doing their research, we might be missed as cleft surgeons.”
Moving the Specialty Forward
One of the committee’s top goals is fostering more cleft research. The advantage of working together as a committee is that members can pool data from multiple institutions, giving them a much broader picture of which treatment protocols lead to the best outcomes. In addition to evaluating surgical outcomes, committee members also hope to assess prenatal risk factors and nutrition and feeding outcomes.
As an example of the type of research the committee would like to conduct more of on a larger scale, Dr. Roby pointed to one recent small, collaborative study published in which researchers found that the incidence of unanticipated intensive care unit admissions following cleft repair by head and neck surgeons was low (Otolaryngol Head Neck Surg. 2022. doi:10.1177/01945998221119730). Dr. Patel added that, while the field has become more focused on reporting clinical outcomes to guide proper surgical technique, “this data collection can be challenging because cleft surgeries occur at such a young age [and] results sometimes cannot be measured until the child is much older, so gathering enough data to measure outcomes may take two decades of your career.”
There is a fair amount of creativity and artistry that goes along with these surgeries, and marrying that form and function is something we do very well. —Laura Hetzler, MD
Being prepared to share any complications is critical for the specialty in terms of moving the field forward and learning from each other, all otolaryngologists interviewed agreed. “You may have 50 different teams that all have slightly different protocols,” Dr. Tollefson said. “The committee has an underlying goal of transparency of protocols so that we can reduce variability and create outcomes that are similar across the country.”
The committee also is developing cleft-related care milestones for otolaryngology residencies and fellowships, with the goal of ensuring that future surgeons are trained according to the same high standards. One major training advance that occurred about eight years ago was the development of 3-D–printed, silicone simulation models for the surgical repair of cleft lip and palate. “They simulate what you’ll encounter in surgery, which is so important because there is very little room for mistakes in these surgeries,” Dr. Patel said. “The more you can learn on a simulator before operating on a child is extremely beneficial.”
A Team of Specialists
Children with cleft lip and palate require multiple surgeries and medical specialists to help treat the condition. Cleft lip repair occurs around three months of age, followed by cleft palate repair at around nine to 12 months. Dr. Hetzler noted that it is not uncommon for these children to have approximately 10 surgeries by the time they are 18, but some undergo up to 30 surgeries when they join her team, depending on their specific condition. “The most important part of their care comes after we repair the cleft lip and palate in the first year of life,” she said. “That is when we focus on the social, functional, and developmental care of the child.”
As children grow, they undergo additional procedures addressing speech, orthodontics, bone grafting, and rhinoplasty. One of the most challenging complications is a fistula, which occurs when the primary surgical repair of the palate breaks down or fails to heal properly. It can cause major, long-term complications with mobility, speech, and nasal regurgitation—all areas that otolaryngologists are trained to manage.
In addition to head and neck surgeons and plastic surgeons, specialists who may be involved in children’s care teams include neurosurgeons, ophthalmologists, pediatric dentists and orthodontists, pediatricians, nurses, psychiatrists and psychologists, social workers, speech–language pathologists, and audiologists. There are nearly 200 multidisciplinary cleft teams around the country, said Dr. Tollefson. About 10% of them are run by otolaryngology cleft surgeons, Dr. Roby added.
Lack of Insurance Coverage
In the United States, insurance covers cleft lip and palate surgical repair, but important follow-up care, such as dental and orthodontia services, often is not uniformly covered. Children with cleft lip and palate can have malformed teeth that may be crowded, misplaced, rotated, and prone to cavities. Some states, such as Minnesota and California, have passed laws mandating dental coverage, but most states do not have any such legislation.
In addition, other cleft team members’ services are not always reimbursed. “Patients come in and see providers on the team, but if every provider bills separately, they may have separate co-pays, leading to out-of-pocket costs of up to $200,” Dr. Petersson said. “So, sometimes providers volunteer their time for the session, and we have not been able to fix that. The fear is that we’ll lose volunteers and people who are passionate about providing this care.”
In the dental arena, clinicians are hoping to soon see progress with the Ensuring Lasting Smiles Act (ELSA), bipartisan legislation that passed the U.S. House of Representatives in 2022. ELSA would require all private insurers to cover medically necessary procedures resulting from congenital abnormalities. “It shouldn’t matter where you live—it should be equal regardless of which state you’re in or if you’re in a city or a rural area,” Dr. Roby said.
Forging Meaningful Patient, Family Relationships
Because they typically see their patients about every six months until their 21st birthday, these specialists develop strong relationships with the children and their families. “All of the children are so meaningful to me,” Dr. Patel said. One child she operated on had been adopted outside the U.S. and had a lot of airway problems, including an inability to speak clearly or maintain air pressure in the back of her throat. Dr. Patel added that, after the child underwent a cleft-related speech surgery, “Her mom sent me a video of her blowing out her birthday candles. The girl’s instant expression of surprise and excitement made me so happy.”
Dr. Petersson recalls one particular incident when she operated on a 15-year-old boy in Ethiopia who hadn’t had his cleft repaired as a baby. He brought a tiny shard of a mirror so he could see what he looked like after the surgery. Because she normally operates on babies who can’t verbalize, the boy’s beaming reaction was particularly moving.
Dr. Tollefson also has had many incredibly rewarding patient experiences. One young woman, now in her first year of college, has done extremely well academically and started a Facebook group to help other children who were born with cleft abnormalities. “One day, I showed up in clinic and there was a huge box of stuffed animals of every shape and size that she had sewn cleft lips into,” he said. “She wanted children like her to have them so they could know that even though they’re different, it’s going to be okay. She doesn’t look at it as her problem, she looks at it as how she can help other kids going through it.”
One of Dr. Roby’s most memorable cleft surgery experiences occurred during a mission trip to Mexico, where she operated on a 3-year-old boy who lived in such a remote area that his family had to hitchhike for 12 hours to reach the hospital. He had a Tessier No. 4 cleft, a rare and challenging malformation that extends from the lip to the eye. Dr. Roby first repaired his eye, nose, and lip, then returned seven months later to repair the palate. “His mother was told there never would be enough money to help her child,” she said, adding that, in the United States, a parent would never be told that. “I have a video of her holding her son after the surgery and sobbing, saying through a translator, ‘You gave me life.’”
She added, “If I’m having the worst day ever, I can pull up that video and say, ‘I may not be having a great day, but I can occasionally help someone else have a great day.’”
Carrie Printz is a freelance medical writer based in Colorado.
Cleft Lip and Palate Incidence Remains Steady
Cleft lip and palate are the most common birth defects of craniofacial development, with up to 7,000 children in the United States born with the condition annually (Front Pediatr. 2013. doi:10.3389/fped.2013.00053). Cleft lips are more common, occurring in one in 500 to 600 babies born, whereas 1 in 1,000 live births have a cleft palate that is inside the mouth and not visible from the outside, according to Dr. Tollefson.
The incidence of cleft lip and palate has remained steady over the years. Certain factors are known to contribute to their development, such as smoking and taking the epilepsy medications topiramate and valproic acid during the first trimester of pregnancy. Rare syndromes such as Van der Woude syndrome also are associated with the condition. Many women may even be unaware that they are pregnant at the time clefts develop, which occurs at four to six weeks in utero for cleft lip and eight to 12 weeks for cleft palate, respectively, according to Dr. Hetzler.
While taking folic acid during pregnancy is recognized to help reduce risk, other causes and prevention methods of the condition are unknown. “There are fetal surgeries under investigation for spina bifida during which the surgeon and team operate while the fetus is in the womb and is still attached to the umbilical cord, but cleft lip and palate is not life threatening, and fetal surgery has significant risks,” Dr. Tollefson said. “Instead, advances will occur in fine tuning the protocols that are used to treat these kids.”