Cleft Lip & Palate
Cleft lip and palate is the most common congenital anomaly (“birth defect”) of the head and face. When there is a cleft of the lip, there is a separation or a gap within the upper lip. This separation can vary from a subtle notch or groove to a wide gap. This separation can involve the roof of the mouth, or palate, also. Cleft Lip and Palate (CLP) and isolated Cleft Palate (CP) are two separate entities.
- Does the cleft cause my baby any pain?
- Are there different types of Cleft Palate?
- What are the different types of Cleft Lip?
- Who gets Cleft Lip and Palate?
- What causes these clefts?
- How will I feed my baby with a cleft?
- What are the main issues related to the cleft?
- Are there other problems that occur commonly with cleft lip and/or palate?
- What is the treatment for babies with clefts?
- What is done between the time my baby is born and the first surgery?
- What sorts of specialists will be involved in my baby’s care?
- Will we get to know our surgeon?
No, clefts are not painful for the infant. From the infant’s point of view, the cleft is “normal”. Facial movements, such as with crying or smiling, are not painful for infants with clefts.
Just as there are variations in the types of cleft lip, there are also different types of cleft palate. The palate forms the roof of the mouth, and extends from the gums just behind the lip to the uvula toward the back of the throat. Cleft palates may be Unilateral or Bilateral, depending on whether there is a gap on one side or both sides of the midline. The cleft may include just the front of the palate, just the back of the palate, or the entire palate.
When the cleft occurs on only one side of the upper lip, it is called a Unilateral Cleft Lip. When it occurs on both sides of the upper lip, it is called a Bilateral Cleft Lip. When the cleft results in a gap that goes all the way from the lip and mouth into the nose, it is called a Complete cleft. When the gap does not extend from the mouth all the way into the nose, it is called an Incomplete cleft. When the incomplete cleft is very minor (a groove or a notch), it is called a Microform cleft. Cleft lip and palate is more common on the left side, less common on the right, and least common bilaterally.
Clefts can occur in any newborn infant. Of all babies born with clefts, two-thirds have cleft lip and palate while one-third have isolated cleft palate. Clefts of the lip and palate together are more common in boys, while isolated clefts of the palate occur more commonly in girls. Clefts of the lip and palate are most common in Asians and least common in African-Americans. Clefts of the palate alone are equally common among all races.
Clefting is also more likely to occur in a newborn who has a family history of primary relatives (parents, siblings) with clefts.
There appear to be a number of different factors that are important in clefting. These include genetics, ethnic background, and certain environmental and chemical/drug exposures. Thus, clefting is considered a “multifactorial” problem. About one third of infants born with clefts have a family history of clefting. All families with cleft-affected children are encouraged to consult with a geneticist, who can define the risk of clefts in future children.
When the fetus is developing, the face forms by the fusion of five different facial processes. These processes are like the pieces of a jigsaw puzzle, that must fit together perfectly to create a complete face. When two of these processes fail to come together, for whatever reason, a cleft is formed.
It is extremely unlikely that the cleft is a result of anything that the parents may have done during the pregnancy. Many parents feel a sense of guilt that they may have somehow caused their baby’s cleft. However, in the vast majority of cases, that is not the case.
The feeding of infants with a cleft of the lip and/or palate is a critical issue for parents and health care providers to understand. These infants can have problems with failure to thrive if special care is not taken with their feeding. The basic problem is that infants with clefts involving the palate have difficulty generating suction with their mouths. Therefore, breast-feeding is difficult or impossible, as is sucking from a standard bottle and nipple. However, the infant can still receive the benefits of breast milk if the mother pumps milk that is then fed through a special type of nipple and bottle. These types of nipples and bottles work by a “squeeze” mechanism, so that the infant does not need to suck. There is almost never a need for feeding tubes to be placed.
Aside from nutrition, the three key issues related to clefting of the lip/palate are: 1) Speech; 2) hearing; and, 3) facial growth. The development of normal speech requires an intact palate with normal muscle function in the soft palate. Without timely repair of the palate (i.e. by 18 months of age), normal speech cannot develop. In addition, an intact palate with normal muscle function is necessary for proper drainage of the middle ear. This is why almost all infants with cleft palate will develop ear infections and require ear tubes to help drain the ears. Finally, some infants with clefts will have deficient growth of the upper jaw that may require surgical correction. The timing of and type of surgery performed to repair the lip and palate can also affect facial growth. Cleft treatment protocols must take all these considerations into account and balance the important concerns of developing normal speech while allowing normal facial growth.
Some children with a cleft will have another congenital anomaly or birth defect. The most commonly associated problems include cardiac abnormalities (heart problems), central nervous system (brain, spinal cord) problems, and club foot. It is important for the baby with a cleft to be carefully evaluated by the pediatrician and other specialists on the cleft treatment team to determine whether any associated problems exist. In some cases, the cleft is part of a syndrome, or collection of abnormalities that affect different areas of the body; these syndromes often have a genetic basis. Syndromes are more common in children with isolated cleft palate than in children with cleft lip-and-palate.
While every patient is treated individually, with treatment plans made specifically for him or her, some generalizations are possible. Babies with cleft lip and palate will have multiple surgeries during their infancy, childhood, and adolescence. Depending upon the severity of the case, these surgeries can include the initial repair of the lip and nose (in the first 6 months of life), the palate repair (by 12-18 months of age), and repair of the cleft in the gumline (approximately between 7 and 9 years of age). Each of these surgeries is done on an inpatient basis, with the child spending one or two nights in the hospital. Additional revision or “touch up” surgeries may be done on the lip and/or nose before the child starts school. In addition, some children need additional surgery on their palates to improve their speech. If the upper jaw does not grow properly (the patient has an “underbite”), a jaw surgery may be necessary during the mid-late teen years to move the upper jaw forward. Finally, a “finishing” rhinoplasty, or nasal surgery, may be done in the teen years.
If the infant has a cleft of the palate only, it is possible that only one surgery will be required, to repair the palate. However, further surgery is occasionally required for speech problems.
Infants are seen during the first week of life, and are followed closely as they grow. For infants with complete clefts of the lip and palate, some stretching and reshaping of the tissues of the lip and nose can be done before surgery. This can be done in various ways. We prefer to use either taping of the lip with a special technique, or a technique called nasoalveolar molding (NAM). NAM requires the placement of an intraoral plate, or retainer, and then the addition of an extension that goes up into the nostril(s) to gradually align the cleft segments of the palate and to shape the nose. The goal of any of these pre-surgical techniques is to stretch and align the tissues so that the surgical repair of the cleft is technically easier and, ultimately, creates a better appearance of the repaired lip and nose.
The optimal treatment of children with cleft lip/palate is achieved in a multidisciplinary setting. Children with cleft lip/palate have multiple issues, such as nutrition, speech, hearing, and facial growth that require specialized attention. A number of different health care providers from various specialties work together to treat these children. These providers include a plastic (craniofacial) surgeon, an orthodontist, a pediatric plastic surgery nurse, a speech pathologist, an otolaryngologist (ENT), a dietician, an audiologist, and an oral surgeon.
Because the treatment of children with clefts is an ongoing process that continues throughout childhood and adolescence, the children and their families become very familiar with their craniofacial surgeon, and often develop very close relationships with him or her.