A summary of Part I re Cleft Palate Care
At Primary Children’s Hospital in Utah, pediatric surgeons are taking controversial risks with how they care for kids with cleft lip and palate. This is the conclusion of a ProPublica article of which the below is the second of three parts. I’m reprinting it in my blog because the article is disturbing. (I’ve added several new subheads in brackets below.)
It is interesting that the recent article was penned by Megan Rose a crime reporter who writes about topics I also write about. I’ve just published a true-crime book, Privileged Killers, and republished Cleft Heart, which details my various surgeries and outcomes.
[The basics about about cleft disorders]
‘With cleft lip and palate, often a split starts at the nostril, goes down the lip and through the upper jaw, and continues back through the roof of the mouth toward the throat. Sometimes the split is on both sides of the mouth. From the time a cleft is spotted on a sonogram during pregnancy, parents begin prepping for a series of surgeries to help their child with a noticeable facial difference. Each step of care throughout childhood builds upon the last, slowly marching the face toward the form it failed to take in the womb. A doctor’s misstep can veer the mouth off course or mean more surgery.
People unfamiliar with clefts, besides perhaps what they’ve seen on Operation Smile fundraising commercials, tend to think of the treatment as a one-and-done surgery to repair a small slit in the lip. And while that can be the case, for a lot of babies born with the condition, it’s much more involved. Often, a wide split starts at the nostril, goes down the lip, runs through the upper jaw and continues back through the roof of the mouth toward the throat. Sometimes there are splits on both sides of the mouth.
Cleft lip and palate together affect about 1 in 1,600 babies in the U.S.; around 2,500 are born with both each year. It is unclear why Utah has a higher rate. In general, the cause of a cleft is not well understood. According to the Centers for Disease Control and Prevention, there is some evidence that genetics can play a role, and some environmental factors, such as smoking during pregnancy or taking certain medications, can increase the chances of a cleft forming, which happens in the first trimester.
Illustration by Matt Twombly for ProPublica
With cleft lip and palate, often a split starts at the nostril, goes down the lip and through the upper jaw, and continues back through the roof of the mouth toward the throat. Sometimes the split is on both sides of the mouth [called bilateral clefts].
A cleft can affect a child’s teeth, position of the midface and nose, and ability to hear, breathe, swallow and speak. Many cleft patients need care from a range of specialists from their earliest days until adulthood. Successful cleft treatment repairs those issues, leaving patients’ faces with little evidence of their cleft issues by the time they are grown.
Like most well-known conditions, there is a treatment plan for cleft lip and palate that a majority of doctors follow, known generally as
The Standard [for Cleft Palate Care]
ProPublica spoke with more than a dozen cleft specialists nationwide, and all acknowledged the variability, subjectivity and debate in cleft care. Several pointed out there aren’t large randomized trials that have definitively provided answers, and care continues to evolve. Still, they all laid out the same general timeline of cleft treatment and the reasons for it.
Doctors usually repair the lip and nose around 3 to 6 months, close the palate around 9 to 18 months and then wait to do a bone graft until children are about 5 to 11 years old, though there is debate about when in that age range is best. Jaw surgery, if needed, typically waits until the child is done growing. Most patients will need rhinoplasty and years of orthodontia as well and, in some cases, additional surgeries to help with things like speech.
Richard Kirschner, chief of plastic and reconstructive surgery at Nationwide Children’s in Ohio and the editor of a widely used cleft textbook, said this timeline is based on evidence collected over decades in the care of thousands of patients.
[Pioneers in Cleft Palate Care]
To the doctors at Primary Children’s, the skepticism they’re facing is the plight of pioneers.
“I think it’s easy to keep doing things the same way in the way they’ve always been done,” said Agarwal, who noted that he doesn’t think there is a standard of care in cleft. “And, you know, that’s the answer for a lot of groups that they feel like they don’t want to try new things. And that’s OK. I mean, nothing’s wrong with that.”
Dr. Dana Johns in an exam room at Primary
Faizi Siddiqi, who helped start the university’s cleft team in 2003, stopped practicing last year and died from cancer in January at age 58. ProPublica was unable to reach him for comment before his death. Duane Yamashiro, an orthodontist who was the medical director of the cleft program for almost 20 years, declined through a spokesperson to comment. So did the most senior plastic surgeon currently on the team, Barbu Gociman. All three are named on the state complaint. Johns and the rest of cleft team doctors are not named on the complaint, and the hospital and the University of Utah declined to comment on it.
[Official Comments re Primary Hospital’s Program]
“We stand firmly behind our surgical approaches and believe they deliver the best outcomes for our patients, although, through an extensive review of our program we realize there are opportunities to improve the way we communicate with our patients and their families,” [University of Utah Health spokesperson] Wilets said in a written statement. “The safety and care of our patients is the most important thing to us.”
“We apologize for any distress to any patient family who feels we didn’t meet their expectations and did not feel comfortable addressing their concerns directly with us,” Wilets wrote.
The hospital has around 700 to 800 cleft patients at any given time, and Wilets said its cleft outcomes are in line with or better than other centers’.
Parent Concerns
In responding to concerns that patients’ families told ProPublica about, Jess Gomez, a hospital spokesperson, wrote, “We have no record of prior complaints from the families you’ve identified regarding their care, and we are saddened to learn of the concerns you’re sharing with us.” He added that the patients were cared for by Siddiqi, so the hospital is unable to respond to specifics about treatment discussions.
Innovating in surgery is a gray area. Unlike drug development, which has strict rules, surgeons are allowed wide leeway to try new things. “It’s a little bit of the Wild West,” said Dr. Jonathan Marron, director of clinical ethics at Harvard, and there isn’t agreement on what the right balance is between supporting innovation and protecting patients.
Doctors have an ethical obligation to make sure their patients are aware of how their protocol differs and what the rationale is for not doing the standard. “Informed consent, in my mind, is an important aspect of this,” he said.
As more and more doctors stick to a standard, the less acceptable a deviation from that standard practice is, Marron said. But figuring out where that line is can be difficult.
Regulation
Innovating in surgery is not an area that’s robustly regulated. Last year, Utah lawmakers made it easier for doctors to practice outside the standard by loosening the rules that had restricted it, though the law still requires that patients are informed in writing and consent.
The hospital and University of Utah didn’t respond to questions about whether patients are informed in writing about any deviations from the standard of care. Since ProPublica began reporting, the hospital has updated its written informed consent form regarding families’ options, Wilets said, and it now “more explicitly” tells patients that there is no standard protocol for cleft patients and different institutions have different protocols.
Regulation and the UK’s Experience
In other parts of the world, cleft lip and palate care has undergone changes based on government oversight. For example, in the United Kingdom there used to be many cleft centers with wide variations in success. Then the government audited outcomes, looking at factors such as how well patients were able to speak and how well their jaws aligned. Some centers achieved much better results with fewer surgeries. The government consolidated care among fewer centers to improve results and consistency. There isn’t any similar oversight in the United States.
“I think the important thing to understand here is that there’s not a whole lot of regulation in terms of who is looking after clinical outcomes,” Kirschner said. “And so it’s really [left] up to parents to do their research.”
An Outside Doctor [looks at Cleft Palate Care]
When Griner questioned the parents of the former Primary Children’s patients, he said, none of them understood that the treatment their child had received wasn’t the norm. The parents cycled through shock, anger and grief in his office so often that eventually he could tell by their faces what they’d say next, he said. The remorse stage was the hardest to hear, with parents blaming themselves for not asking more questions.
The parents, dogged by inchoate worries, had come to him seeking a second opinion once Griner and his partners opened their practice, he and some patients said.
Griner, who remembers going door to door to fundraise for Primary Children’s as a child, said he’d always believed that the hospital’s general excellence included the cleft team. He had wanted to work there. But Griner says that after he chose a fellowship position in Texas instead of with the University of Utah, relations soured.
Later, after seeing the unit’s handiwork firsthand in his practice, he took his concerns to the hospital’s administration. During 2018 and 2019, he said, he had three conversations with different levels of leadership, including the hospital’s then-CEO, about the poor cleft outcomes he and others were seeing. Each time, he said, his concerns weren’t taken seriously.
A spokesperson for Primary Children’s said the former CEO and another administrator couldn’t recall any such conversations, and the third, a director of surgical services at the time, said he recalled only casual conversations between colleagues. “If a concern were adequately raised by Dr. Griner, the concern would have been investigated and necessary action would have been taken,” Gomez said in an email.
As Griner’s new cleft team settled in, their worries mounted. Once a month, the cleft team would meet with patients to collectively discuss next steps. After a former Primary Children’s patient would leave one of those meetings for the first time, the doctors said they would look at each other, shaking their heads: “Can you believe that?”
Dr. Griner checks Robbie McFerson re Cleft Palate Care
Primary Children’s undertakes two surgeries to repair the palate instead of one, something most doctors in the United States have stopped doing. The hospital is unusual in that it uses a prosthetic device to cover the palate hole, which requires more time under anesthesia when swapped out for a new one. The hospital also often surgically expands the palate of young patients when a retainer-like device would typically be used instead.
The timing of cleft palate care.
To Griner and his team, the timing of two major procedures — bone grafting and jaw surgeries — were worrying enough on their own, but along with other ways Primary Children’s is an outlier, they were concerned that patients were getting aberrant care at many stages.
Finally, in 2022, a group of doctors — many of whom compete with Primary Children’s — raised the issues with the state. Ten doctors’ names appear on the complaint, though one told ProPublica that although he agreed with many of the concerns, he wasn’t aware he’d been included on it.
(One of the plastic surgeons who signed the complaint, Rodney Schmelzer, has a complicated history with Primary Children’s. He lost his privileges there in 2017 and has two ongoing lawsuits against the hospital, one of which also names Yamashiro and Siddiqi. The hospital and the doctors have denied the allegations.)
Utah’s Department of Commerce, which oversees the Division of Professional Licensing, said it could not confirm nor deny an investigation.
The complaint alleges that Primary Children’s doctors “routinely exploit Cleft Patients and their families by … subjecting [them] to excessive numbers of surgeries outside the standard of care” without being honest about the risks, benefits or alternatives, which the doctors say violates medical ethics and Utah law.
Major Jaw Surgeries
Paige Holland started looking for a second opinion when Primary Children’s said her 7-year-old son would next need major jaw surgery to correct the underbite that commonly afflicts cleft patients.
The operation, called a LeFort distraction, sounded medieval to Holland: Doctors would cut part of his upper jaw from his skull and screw a rigid metal device, called a halo, to the outside of his head. Wires would attach his upper jaw to the device, and every day for weeks Holland would turn screws to tighten the wires and slowly pull her son’s jaw bone forward and allow more bone to grow. He’d wear the halo for months.
The LeFort Halo Procedure
Doctors make cuts along the upper jaw so it can be repositioned.Jaw surgeries aren’t unusual for cleft patients, but the age Primary Children’s often does them makes the hospital an outlier. Holland’s son would have been as young as 9 at the time of the surgery, according to his Primary Children’s medical records reviewed by ProPublica.
The American Cleft Palate Craniofacial Association guidelines state that “whenever possible, [jaw] surgery should be delayed until physical maturation is essentially completed.’’ Cleft doctors at six leading hospitals said they don’t perform jaw surgeries until children stop growing. For girls, that’s about 14 to 16 years old; for boys, it’s closer to 18.
Until recently, Primary Children’s website told families to expect possible jaw surgery between 9 and 10 years old. But it has since changed the age range to between 9 and 15-plus. Some other cleft centers also perform early jaw surgeries, and Kirschner told ProPublica he thinks that surgeries performed before maturity fall within the standard of care, though he personally waits for patients to be grown.
[The average Primary Halo surgery: 11+ years]
Primary Children’s data from 2020 showed that the average age of LeFort halo surgery there was 11.4 years old, according to a paper published by the hospital’s cleft team that analyzed nearly 60 cleft patients who had halos over a three-year period. The age of the patients ranged from 8 to 16 years old.
Cleft doctors ProPublica spoke with said they perform jaw surgery before maturity in only a tiny proportion of cases, usually only if medically necessary. Dr. Roberto Flores, head of the program at NYU, estimated the number at no more than 5%.
At Seattle Children’s Hospital, even in those extraordinary cases, the surgery would not happen until 12 to 14 years old, when most of the adult teeth are finished coming in, according to the hospital’s former chief of craniofacial surgery, Richard Hopper.
“I’ve been with the cleft team for 20 years, and I can pretty conclusively say we haven’t done a Lefort … on a patient younger than 12 years old, 11 at the earliest,” Hopper said last year before he took a job with Texas Children’s Hospital.
Earlier not Better?
“The earlier you do a jaw surgery, the more likely you’re going to have to repeat it later,” a cleft team surgeon at a top-rated pediatric hospital said. “Basically you’re committing kids to having an extra operation.”
The best practice, many doctors said, is to do one definitive jaw surgery close to adulthood.
“It’s a brutal enough operation. You wouldn’t want to have to do it twice,” a leader in the field said. Both doctors asked not to be identified out of concern it would jeopardize relationships within the small community of doctors who provide cleft care.
[Bullying and Cleft Palate Care]
Primary’s Johns said such critiques ignore the psychosocial issues children with cleft deal with. Bullying should be given more weight in treatment plans, she said, and repairing a child’s differences before high school has merit. Most of her surgeries wait till at least age 12, she said, and when she does them on younger patients, it’s for either psychosocial or other pronounced medical problems.
Moreover, patients who wait until maturity can sometimes require extensive operations involving both the upper and lower jaws, Johns said. Patients who’ve had the surgery young might require a second jaw surgery, she said, but it would involve only the upper jaw. The trade-off, she said, is worth it.
Rohit Khosla, surgical director of the cleft team at Stanford Medicine Children’s Health who trained Johns during her fellowship, reviewed all of the hospital’s protocols at her request. In general, Khosla told ProPublica that he thought the hospital’s methods were controversial but not problematic. He found several problems with the cleft team’s study designs and conclusions, but overall its “protocols are conscientiously thought out with [a] goal to provide a high level of care,” he said in a summary of his report.
The hospital would not share his full written review and asked Khosla to stop speaking with ProPublica once it was completed. Primary Children’s also set up a double-blinded review — neither the hospital nor reviewer knew whom the other was — to further assess its protocols, but the hospital said it would not make the report public when it was completed.
In looking at the hospital’s latest LeFort data, Khosla wrote that in the “last few years” the average age has increased to 14, “which I find more acceptable.”
Parent Concerns
On a Facebook page for Utah parents of children with cleft, numerous posts over the years speak to the hospital’s push to perform LeForts young. Some talk about how their children endured the surgery before 10 years old — “not going to lie the Halo is tough!” one mother wrote — and needed to repeat a jaw surgery as a teenager.
Holland said she thought there had to be another way to fix her son’s underbite and asked about braces. After Primary Children’s said there wasn’t, she said, she sought a second opinion with Griner, who sent him to an orthodontist instead. Holland’s son was one of at least seven former Primary Children’s patients whom Griner said he diverted from having surgery before maturity. None of them, in Griner’s opinion, had any medical necessity for the operation before maturity or reported bullying or other psychosocial issues. Schmelzer, who also signed the complaint against Primary Children’s, said his medical records showed similar numbers.
Griner recalled one startling case last summer in which Primary Children’s had lined up surgery for a 9-year-old child whose jaw was nearly normal. An independent plastic surgeon who specializes in cleft at a large academic institution reviewed the patient’s CT scan for ProPublica and confirmed Griner’s assessment that there was no need for surgery.
Holland’s son, now 13, has only a minor underbite after orthodontics and likely won’t need jaw surgery at all.
“He’s totally fine,” Holland said. “He was saved from a major surgery.” ‘
Any further thoughts re Cleft Lip & Palate Care
Please give me your thoughts in the Comment section below re cleft lip and palate care. Thanks!
My latest book, PRIVILEGED KILLERS, is a true story about a half-dozen Dark Triad people in my everyday life - narcissists, manipulators, and psychopaths. Three of 'em murdered people, and one came after my wife and me. Print and e-book versions of this (and CLEFT HEART) available at Amazon and elsewhere online. Also at your local bookstore.
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