Cleft lip and palate: Team approach to treatment
A multidisciplinary center for children born with cleft lip, cleft palate, or other craniofacial conditions ensures that patients emerge with little evidence of deformity.
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.
Cleft lip and palate facts and figures
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.
What’s meant to happen in utero, doesn’t
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
Often detected in utero
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.