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A look at how one hospital is tackling cleft lip, cleft palate, and other craniofacial conditions.
The misconception that children born with cleft lip, cleft palate, or both need only a single surgery to address their cleft can leave patients with a lifetime of physical and emotional challenges. Pediatricians who encounter children with these facial differences should encourage parents and caregivers to pursue a focused, long-term, multidisciplinary approach to treating and managing cleft lip and/or palate because all affected children benefit.1
Cleft lip and palate is more than a plastic surgery issue, according to Karla A Haynes, RN, MPH, MS, CPNP, pediatric nurse practitioner, at Children's Hospital Los Angeles Craniofacial and Cleft Center, California, one of the country’s largest multidisciplinary centers for children born with congenital facial differences, such as cleft lip, cleft palate, and other craniofacial conditions.
“The best practice is a multidisciplinary evaluation, which can be an issue because, sometimes, people who aren’t very familiar with this patient population might see craniofacial abnormalities as a plastic surgery problem. They may not understand that this is a chronic condition, and that there is a need for ongoing monitoring and care throughout childhood,” Haynes says.
Occurring in about 1 in 600 live births in the United States, clefts involving the lip and/or palate are among the most common congenital anomalies affecting the craniofacial region.1,2
Oral facial clefts vary in incidence according to gender and ethnic background. Clefting is known to occur more in males, and Asians and Native Americans have the highest birth prevalence, with 1 in 500 births. The lowest prevalence is in people of African descent.2
Cleft palate and cleft lip often occur together. About two-thirds of those affected have clefts of the lip and palate.2 Cleft lip alone is more likely to occur than cleft palate alone, but when it does occur, cleft palate is more common among females.
Because many of the syndromes associated with clefting are inherited in an autosomal dominant pattern, each offspring of an affected person has up to a 50% chance of inheriting the syndrome.2 Recurrence of isolated cleft is low, however-around 2% to 6%. The risk increases as the number of affected persons in a family grows.
The lip normally fuses by 35 days in utero.3 The lip’s failure to fuse can impair subsequent closure of the palatal shelves, which typically close in the 8th to 9th week.
Although the cause of cleft lip sequence typically is a mystery, clefts occur in syndromic or isolated categories.2 Skeletal, craniofacial, eye, and other anomalies generally occur with syndromic clefts. Cleft-associated syndromes can originate from intrauterine exposures to alcohol, isotretinoin, cigarette smoke, and more, as well as result from genetic disorders.
Children with clefting are at higher risk for abnormal tooth development, as well as mild ocular hypertelorism, hypernasal speech, speech delay, recurrent otitis media, and hearing loss.3
Typically, community obstetrics providers will see a fetal cleft lip on ultrasound (the cleft palate generally is not so visible), according to Haynes.
Cleft lip not detected before birth should be seen in the immediate postnatal period. Cleft palate might be diagnosed later, during a routine newborn intraoral examination, especially if the newborn has a milder variant, such as bifid uvula or submucosal clefting. Pediatricians and others who diagnose cleft palate should conduct a systemic examination because every sixth newborn that has cleft palate is at higher risk for other malformations, including congenital heart disease or urinary tract anomalies.1
To differentiate between an isolated or syndromic cleft, the pediatrician should consult with a craniofacial team and geneticist, and take a careful family history of clefts and features of clefting syndromes, including skin disorders and speech abnormalities. 2 The pediatrician should also ask about prenatal exposures. In addition, assessments for vision and hearing should be done as early as possible.
Because cleft palate prevents an infant from effectively sucking, these babies have trouble with breastfeeding. Pediatricians can recommend that mothers pump their breast milk and give it to their babies using a special nipple designed to facilitate feeding infants with clefting.
The good news is breastfeeding can be successful in babies with isolated cleft lip only.2 However, parents should know that these babies tend to take in more air while feeding and might require more frequent burping. With palatal openings or dysfunction, nasal regurgitation also can occur.
Pediatricians should diligently monitor these children’s growth and more, as children with palatal clefts are at significantly greater risk for eustachian tube dysfunction, recurrent otitis media, and conductive hearing loss.2
According to Haynes, that feeding and growth can be significantly impaired and babies can really struggle to gain weight, with a small percentage requiring admission for failure to thrive until families master the feeding techniques. This can be challenging, she says.
Children's Hospital Los Angeles Craniofacial and Cleft Center features 13 subspecialty disciplines on its craniofacial team. The full list of specialty providers is plastic surgery, nurse practitioners, ears/nose/throat, audiology, speech pathology, genetics, dentistry, orthodontics, psychology, social work, pediatrics, pulmonology, and a registered dietician.
Nurse practitioners coordinate care at the center, and the surgical care is directed by a plastic surgeon who relies heavily on the input of the team members, Haynes says.
“Our patients all have their own community pediatricians that do their usual well-child health surveillance and sick visits,” Haynes said. “The pediatricians on our craniofacial team monitor patients . . . looking at signs of obstructive sleep apnea or gastroesophageal reflux or some of the things that kids with cleft lip and cleft palate or other craniofacial diagnoses are more likely to have.”
The care of these patients often begins in utero, according to Haynes. “My nurse practitioner colleagues and I also do prenatal counseling in conjunction with a . . . program at our hospital called the Institute for Maternal-Fetal Health,” she says.
A nurse practitioner and the institute’s perinatologist will meet with pregnant mothers who are referred to the institute because of a possible cleft on ultrasound. “[We] will do another anatomy scan with, in our case, the craniofacial nurse practitioner standing right beside the perinatologist, so we’re looking at the images together. And based on what the 2 providers see, the parents are counseled on the diagnosis,” Haynes says. “About half, if not more, of our babies are identified before birth.”
There are other centers of excellence focused on children with craniofacial conditions, including Seattle Children’s Hospital in Washington state, Brigham and Women’s Hospital/Children’s Hospital in Boston, Massachusetts, and Children’s Hospital of Philadelphia, Pensylvania, according to Haynes.
At the very least, teams charged with the care of children with cleft lip and palate should include speech-language pathology, surgery, and orthodontics specialists. The core team should have ready access to psychology, social work, audiology, genetics, general and pediatric dentistry, otolaryngology, and pediatrics/primary care professionals. The team should also be able to refer to a neurosurgeon, an ophthalmologist, a radiologist, and a geneticist, according to the American Cleft Palate-Craniofacial Association.4
Pediatric patients with cleft lip and palate require numerous surgeries throughout their childhood and into early adulthood to correct aesthetic and functional issues associated with their diagnoses. In 1 sample of patients, the average number of surgical procedures was 8.6.5
“For all of the facial structures and sociologic processes that are affected by craniofacial [conditions], we use different providers on our team that are looking out for all of these areas that are at risk,” Haynes says. “Sometimes, a lack of continuity of care is a problem. But definitely pediatricians should know that if there is a child who is born with a cleft, they need to be with ongoing team care.”
There are different stages in the surgery. When they’re done depends a lot on the phase of dentition, according to Haynes. She describes a potential timeline of treatment for children with cleft lip and palate: “The very first surgery that’s done is usually an initial cleft lip repair, in which the lip is repaired and the external nose, as well. That’s usually done anywhere between 1 and 3 months of age. Sometimes it can be a little later,” she says. “The second surgery is to repair the palate, which of course is the roof of the mouth, but it’s also the floor of the nose. So, it does address that second nasal component, and that’s usually done sometime around 1 year of age, ideally.”
The next surgery that many of these children need is an alveolar bone graft, which is done to repair the dental arch of the maxilla where the cleft has gone through the gums. “That can be done in several ways,” says Haynes, “but the children generally need to be in mixed dentition before they can do the orthodontics that allow them to have that surgery. That surgery literally can’t be done before they’re 7, 8 or 9 years of age.”
There are some optional surgeries, including scar revisions. “For many of our kids, once they reach skeletal maturity, they may need an orthognathic jaw procedure because one of the things that happens with kids with cleft lip and cleft palate is they often end up with a pretty significant underbite,” Haynes says. “Some of this is correctable through orthodontics, but for many kids the only way is to do a surgery that involves moving the bones in the face. And that can’t be done until kids are in their later teenaged years.”
Children with cleft lip and palate have a poor quality of life.6 In addition, fragmented or uncoordinated care of these children leads to poor outcomes in speech and hearing.7
Haynes says the children that get their care at Children’s Hospital Los Angeles emerge with little if any evidence of their cleft deformities.
“When the proper stepwise approach is taken, children have surgeries that address each anatomic issue to ultimately result in a child with good intelligible speech, normal hearing, a stable maxilla, proper occlusion of the jaws, and symmetric facial features,” Haynes says. “For families and providers both, one of the biggest, most compelling questions that remains is ‘Why does clefting happen?’ We may be getting closer to having a better understanding of the origin of the ‘isolated’ clefts because of the relative availability of exome sequencing. We would also like to know more about the role environmental factors, such as maternal diabetes, play.”
Haynes says Children’s Hospital Los Angeles is conducting research on the incidence of postpartum depression in mothers of infants born with cleft, and investigating safe infant sleep for infants born with Pierre Robin sequence.
1. Tighe D, Petrick L, Cobourne MT, Rabe H. Cleft lip and palate: effects on neonatal care. NeoReviews. 2011;12(6):e315.
2. Abbott MA. Cleft lip and palate. Pediatr Rev. 2014 May;35(5):177-181.
3. Jones KL, Jones MC, Del Campo M. Face defects as major feature: cleft lip sequence. In: Smith's Recognizable Patterns of Human Malformation. 7th ed. Philadelphia, PA: Saunders; 2013:316-317.
4. American Cleft Palate-Craniofacial Association. Standards for cleft palate and craniofacial teams. Available at: http://www.acpa-cpf.org/team_care/standards/. Revised April 13, 2016. Accessed May 11, 2016.
5. McIntyre JK, Sethi H, Schönbrunner A, Proudfoot J, Jones M, Gosman A. Number of surgical procedures for patients with cleft lip and palate from birth to 21 years old at a single children's hospital. Ann Plast Surg. 2016;76 suppl 3:S205-S208.
6. Zhou M, Liu ZX, Wang PL, Liu C. Quality of life and its influential factors of children and adolescents with congenital cleft lip and palate. [Article in Chinese.] Shanghai Kou Qiang Yi Xue. 2016;25(1):63-67.
7. Mohd Ibrahim H, Mohamed Yusoff FH, Ahmad K, Van Dort S. An exploratory study on speech and hearing outcomes in children with cleft lip and palate. Med J Malaysia. 2015;70(6):321-325.
Ms Hilton is a medical writer who has covered health and medicine for 25 years. She resides in Boca Raton, Florida. She has nothing to disclose in regard to affiliations with or financial interests in any organizations that may have an interest in any part of this article.