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Pediatric Update

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Pediatric Update

 
Fall 2006

The Challenge of Cleft Lip/Palate

The nearly 7,000 babies born each year in the United States with an orofacial cleft will face issues beyond cosmetic appearance that encompass feeding, dentition, hearing and speech. Although the rehabilitative process can be lengthy, the coordinated efforts of an interdisciplinary team can greatly enhance the probability of successful outcomes for these children.

Clefts of the lip and/or palate result from incomplete development of the face or mouth, and occur in approximately 1 in 600 births. The resulting defect can range from a slight notch-like deformity to complete clefts of the lip and palate. In approximately 50 percent of cases, both the lip and palate are involved.Cleft lip and/or palate sometimes are associated with syndromic conditions, and diagnostic testing may be performed to rule out the presence of other physical or cognitive and developmental problems. However, the majority of clefts are non-syndromic, and most children will not experience any developmental delay.

Feeding is the most immediate challenge faced by parents of a newborn with a cleft. A baby with a cleft lip may have trouble making a good seal around the nipple to suck, and with a cleft palate the mouth cannot be closed off from the nose so little or no suction can be created. With patience and some adaptive techniques, breastfeeding an infant with a cleft lip may be possible, points out pediatrician Yoshio Setoguchi, M.D., co-coordinator of the UCLA Craniofacial Clinic. However, breastfeeding a baby with a bilateral cleft lip and palate is almost impossible. For mothers who are unable to or do not wish to breastfeed, there are specialized bottles and nipples available. Mothers also can be taught how to best position their baby during feeding—an upright or semi upright position is best—to minimize the amount of milk or formula that might flow through the open cleft into the nose or eustachian tubes. I

t is not always clear why a cleft occurs but the majority of non-syndromic clefts appear to be due to a combination of genetics and environmental factors that may include use of tobacco, drugs or alcohol during pregnancy. Nutrition during pregnancy also may affect the incidence of clefting, as may some medications, such as the anticonvulsants phenytoin and sodium valproate and the antimetabolite methotrexate. Folic acid supplementation prior to conception and during pregnancy has beenshowntoreduce the incidenceof clefting.

A cleft can be detected by ultrasound as early as the 16th week of pregnancy, but often it is not picked up on a routine exam, particularly if it is not expected. A Level II or “targeted” ultrasound that looks for specific markers of a suspected condition is more accurate in detecting a cleft, while a 3D ultrasound is the most accurate of all.

The risk of clefting increases with the number of affected relatives, particularly if some have syndromic forms. Clefting also is more prevalent among some racial and ethnic groups—1 in 500 births among Native American, Asian and Hispanic groups; 1 in 700 births among people from European roots; and 1 in 1,000 births among those of African ancestry. Only a geneticist can, after a thorough genetic study, give a family an accurate probability rate. Such an assessment is vital to establish the risk of recurrence in subsequent pregnancies, says Katrina M. Dipple, M.D., Ph.D., a pediatrician and geneticist and co-coordinator of the UCLA Craniofacial Clinic.

Team Approach Is Vital

Children with a cleft lip and palate need the expertise of an interdisciplinary medical team. In addition to requiring plastic surgery to repair the opening, interventions to correct issues with teeth, hearing and speech are necessary, as well as addressing children’s psychological development as they mature. At the UCLA Craniofacial Clinic, the team includes plastic surgeons, geneticists, pediatricians, neurosurgeons, otolaryngologists, orthodontists, oral surgeons, dentists, speech pathologists, audiologists and social workers. Other experts can be called in if required. “A coordinated approach is essential,” notes plastic surgeon James P. Bradley, M.D. “As a plastic surgeon, I can focus on reconstruction, but the other specialties are needed to address such problems as hearing or subtleties of speech. With a team approach, we cover all the bases.”

It is important to begin treatment as early as possible. A visit to the clinic in the first weeks after birth will provide a general pediatric evaluation with an emphasis on feeding, hearing, genetic counseling, dental evaluation and emotional support services for the family, as well as planning the course of treatment.

Hearing and speech problems are significant concerns and an otolaryngologist will evaluate the infant’s ears during the initial team visit. Because the nasopharynx, eustachian tubes and middle ears have an open connection to the back of the mouth, children with a cleft palate are susceptible to developing chronic middle ear fluid, which may or may not become infected but may nonetheless affect hearing, explains pediatric head and neck surgeon Nina L. Shapiro, M.D. If fluid and/or hearing loss occur, the surgeon often will place tympanostomy tubes when the cleft lip is repaired at around 3 months of age.

Prior to surgery to repair a cleft lip, some children require orthopaedic treatment to guide the growth of the mouth in the right direction and aid the correction process. This “molding” process usually starts within two weeks after birth and begins with a customfitted denture—the molding plate—that is placed into the baby’s mouth and causes the bones of the upper jaw at the cleft site to grow toward each other rather than farther apart. Once the gum line is in closer approximation, a nasal extension is added to lift the nose and nasal cartilages into place. This technique is especially important for children with bilateral clefts of the lip and palate.

Surgery to repair the cleft palate generally occurs when the child is about 1 year old and involves undermining mucoperiosteal flaps from the left and right sides of the palate and suturing them at the midline, and reconstructing the throat muscles. The most significant reason for repairing the palate is to preserve speech, explains plastic surgeon Henry K. Kawamoto Jr., M.D., D.D.S. “A child can have a hole in the roof of his mouth and still be able to eat OK—they adapt,” he says. “But with a cleft palate, they will never be able to communicate in terms of speech. Repairing the palate is, for that reason, very important.”

Speech Therapy Begins

Children generally begin speech therapy between the ages of 3 and 5 years. An additional surgery may be required between the ages of 7 and 10 to improve speech. During that period, the adenoids begin to involute—reduce in size—which is a natural process that occurs in all children. In children with cleft palate, however, the adenoids act as a sort of cushion that allows for a seal of the palate during speech production, Dr. Shapiro explains. For these children, articulation may worsen as the adenoids shrink, and they might sound as though they have an open, un-repaired cleft palate. An operation such as pharyngoplasty may be necessary to tighten the seal, or the child may require more intensive speech therapy.

Children with a cleft may have dental issues such as supernumerary, ectopic, rotated and missing teeth, and extensive orthodontics will be required. Generally, the first phase of treatment is at about age 7 to 9 years, explains UCLA orthodontist James F. Mulick, D.D.S., M.S.D. At that time, an appliance is used to widen the jaw and to improve the shape of the dental arch prior to placement of an alveolar bone graft, followed by secondphase treatment after eruption of the permanent canines/secondary molars. Additional orthodontic treatment may continue until young adulthood.

With a comprehensive and coordinated approach to care that includes close alliance with the community-based pediatrician as well as the family, the outlook for children born with a cleft is very positive.





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