Cleft Lip and Palate

Clefts are relatively common, occurring in approximately 1 in 500 to 1 in 2,000 births, depending on the parents’ cultural background. For example, the incidence is lower in families coming from Africa but higher in families originating from Asia. Children can be born with only a cleft of the lip, just a cleft of the palate (roof of the mouth), or both a cleft lip and palate. Moreover, the cleft lip can be incomplete, complete, or bilateral (see Figure Cleft Types).

What causes a cleft?

Clefts of the lip and palate are among the most common birth defects seen in newborns. It’s important for families to know that a cleft does not occur because of anything a mother did or did not do during pregnancy.

Interestingly, nearly one‑third of babies born with a cleft have a relative on either parent’s side who is also affected. This suggests that genetics play a role in the development of cleft lip and palate. Although many genes have been linked to clefting, current evidence indicates that no single gene is responsible for most cases. Instead, clefts are thought to result from a combination of multiple factors acting at the same time, including the influence of many different genes along with additional contributing environmental impacts.

Numerous drugs (alcohol, cigarettes, some seizure medications, vitamin A derivatives) have also been shown to make clefts more likely but are not causing a cleft by themselves. There is also some evidence to suggest that taking prenatal vitamins before conception may lower the chance of having a baby with a cleft. It is most likely that many factors must come together for a cleft to occur. Once a family has a child with a cleft lip and palate, the chances of having a second child with a cleft is low, somewhere between 2% and 4%, depending upon if the cleft was unilateral or bilateral (which carries a slightly higher risk). Many families find it helpful to meet with a geneticist to further discuss these chances.

How are clefts treated?

The treatment a child with a cleft depends on whether the cleft involves just the lip, only the palate, or both together. For babies born with an isolated cleft palate, an evaluation with a geneticist is important to test for certain other possible conditions, such as: Pierre Robin Sequence, velocardiofacial, DiGeorge, Stickler, and others. There are many different ways to repair clefts, and surgeons will differ on when it is the best time to proceed and which technique they prefer. This section will begin by reviewing some of the more commonly recommended treatments, followed by an explanation of the approach we recommend for our patients treated at The Craniofacial Center in Dallas.

Step 1. Feeding and the initial evaluation.

Children born with a cleft involving the palate are unable to create enough suction to feed naturally and therefore will have difficulty getting enough milk with each feed to grow as they should. This inability to generate suction is the result of the opening along the roof of the mouth, which allows air from the nose to leak in while the baby is trying to create suction to swallow. Less suction means less volume, so the baby must work harder to get any milk at all, and whatever is swallowed is usually mixed with a lot of air that can cause colic. If mothers attempt to breast feed their baby, or use a regular bottle and nipple, they will find that after about 30 minutes their baby will get tired of trying to get enough milk and will fall asleep. Then, 60-90 minutes later their baby will wake up again, crying and hungry. The result of this cycle is that the baby does not gain enough weight to grow normally and the parents become exhausted.

Therefore, for children born with a cleft involving the palate a feeding specialist (typically a speech pathologist or occupational therapist) is called in to help. There are a few different ways to feed a baby with a cleft palate and sometimes it takes trying more than one way to find out which is best for each child. One feeding method is to enlarge the hole in an extra soft nipple; so when baby compresses the nipple, milk will stream out by gravity. It usually does not take very long for an infant to learn how to control the amount of milk (breast or formula) that will flow out of the bottle, by gravity. Another method is to feed the baby with a squeeze bottle called a Haberman feeder, where either the baby or the parent can squeeze a little milk into the mouth before each swallow. The two most used specialty bottles are the Medela SpecialNeeds feeder and Dr. Brown’s Specialty Feeding System. Whatever technique works the best, it is important that a baby gets enough milk with each feed to be able to grow normally.

2. Presurgical orthopedics.

This term refers to any technique that is used before surgery to move both sides of the cleft, to narrow or better position each side, with the goal of making the eventual repair easier. There are a number of different types of presurgical orthopedics that might be recommended. The simplest of these is a specialized elastic tape, which is stretched across the cheeks in order to slowly bring the two sides of the cleft lip closer together. Although this requires keeping tape across the upper lip, it can be surprising how just over the course of a few weeks, the two sides will come so much closer together. For babies born with bilateral clefts, this taping can help better position the central part of the lip, called the prolabium, which can stick out too far forward (see Figure, Prominent Prolabium)

Prominent Prolabium

Another type of presurgical orthopedics is called NAM, which is short for nasoalveolar molding. This process relies on a plastic splint that is put inside the baby’s mouth, with an arm coming off the top of that pushes up the nose from the inside. While many centers will not offer NAM, some remain enthusiastic about this technique. The often-cited issues with NAM are the increased burden of care. NAM requires weekly visits to an orthodontist, who needs to continually reshape the plate inside the mouth and adjust the posts pushing up the nose, as the baby grows bigger. More than half of all children will develop sores that cause parents to drop out of treatment. There are also associated costs of treatment. Lastly, newer studies suggest this technique has a negative impact on facial growth. It can also cause scars inside the nose that limit the ability to breathe through the nose.  Whether the potential benefits justify the downsides is a discussion that should be made with your surgeon.

3. The lip repair.

The traditional timing for repairing a cleft lip has been sometime around 12 weeks of age. Parents may hear about the “rule of 10’s,” which means that the repair should wait until the baby is 10 weeks old, has a hemoglobin (blood level) that is over 10, and the baby weighs at least 10 pounds. One exception to this rule has been that some surgeons might recommend an even earlier surgery to perform a lip adhesion, which is done well before 12 weeks of age. This operation doesn’t actually repair the cleft lip, but it does bring the two sides of the lip together with the intention of making the final repair easier to do. Today, lip adhesions are not commonly recommended, although some surgeons might still make this recommendation.

Most craniofacial surgeons will now recommend a cleft lip repair closer to 4 months of age. At the end of the operation the lip is typically closed using either dissolving or permanent stitches, which must be taken out 5 days later. Babies often spend one night in the hospital before being discharged home, wearing arm restraints called “no-no’s.” These are cloth or plastic tubes that wrap around the elbows, which are intended to prevent babies from putting their fingers inside their mouths. Some doctors might also recommend babies not be fed using a nipple after the surgery, instead being feed by either a cup or a syringe. Rarely, surgeons will simultaneously close the back half of a cleft palate at the same time as doing the lip repair, to begin a two-staged closure.

4. The palate repair.

Most cleft surgeons recommend repairing both the hard and soft palate in one operation, usually sometime between 9 and 18 months old. Studies suggest that repairing the palate earlier may help with speech development, while other reports show that waiting a little longer can support better growth of the upper jaw. The thought is that giving the upper jaw more time to grow might reduce the chances of needing jaw surgery in teenage years. Because the research isn’t completely one‑sided, surgeons try to balance these factors and choose the timing that’s best for each child. Not only do surgeons vary on when to fix the palate, they also differ on how to repair it. While there are different techniques used to close a cleft palate, they all work in a similar way: the tissues on each side of the cleft are gently brought together so they meet in the middle. This means surgeons don’t need to take tissue from anywhere else in the body to fill the gap. After the operation is over, children will spend at least one night in the hospital before going home. After surgery, many children will again have to wear soft arm restraints, called “no‑no’s,” intended to keep their hands away from their mouth. Feeding instructions can vary from one surgeon to another, but most recommend avoiding nipples for a short period after the operation to protect the repair.

5. The Ears

Children born with a cleft palate are more likely to develop fluid behind the eardrums, a condition called serous otitis media. This occurs because the muscles that normally open the Eustachian tube are out of position, preventing the tube from normally opening. As a result, fluid can build up in the inner ear, making it hard for children to hear clearly. Many ear, nose, and throat specialists (ENTs) recommend placing ear tubes when children with a cleft palate develop fluid behind their eardrums, since this fluid can affect hearing. Cleft lip and palate teams often try to coordinate ear tube placement with other planned surgeries, such as a lip or palate repair, so children don’t need to go through separate anesthetics or additional procedures. Long-term hearing loss can occur in children with clefts, especially if they have experienced multiple untreated ear infections.

6. Speech

Children who have had a cleft palate repair can be more likely to experience speech challenges as they grow, simply because the palate plays an important role in how sounds are made. Many kids do very well, but some may need extra support from a speech‑language pathologist to help them develop clear speech. In addition, if babies who have not had tubes placed when needed, may experience speech delays.

One of the more common issues with speech after a cleft palate repair is called nasal air emission, or velopharyngeal incompetence (VPI). When we talk, the space between the back of the palate and the back of the throat opens and closes depending on the sound we’re trying to make. For most vowel sounds, this space stays open, and for many consonants, it needs to close tightly. Children born with a cleft palate may have a palate that’s shorter from front to back, which makes that open space larger and harder to close. Others may have a palate that doesn’t move as strongly. Either of these can make certain speech sounds more challenging. There’s some debate right now about whether speech therapy on its own can improve nasal air emission. Different specialists have seen different results, so opinions vary, but most believe therapy alone is not enough.

When children can’t make certain sounds because they’re unable to fully close the back of the palate, they often come up with other ways to ‘fake’ those sounds. These are called substitutions. If these patterns aren’t corrected early, they can become habits that are hard to change later. Many experienced speech‑language pathologists feel that one of the biggest benefits of speech therapy is helping children unlearn these habits and replace them with correct sound patterns. In most cases, it’s a good idea to try speech therapy before considering another surgery; but for children who aren’t making progress with therapy alone, there are additional procedures that can help improve speech. These include operations such as a posterior pharyngeal flap (a “P‑flap”), a sphincter pharyngoplasty, or a Furlow repair (also known as a 4‑flap palatoplasty). These surgeries are designed to help the palate close more effectively during speech, which can make certain sounds easier to produce.

When needed, these operations are more likely to result in normal speech when performed before ages 6 – 7. There are pros and cons to each of these procedures and surgeons must select the right procedure for each child. Finally, children with cleft palates are usually advised not to have their adenoids removed, because taking the adenoids out can create, or worsen, problems with closing off the back of the throat during speech. This can cause VPI and make speech sound more nasal.

7. Revisions of the lip and nose repairs.

As children get closer to school age, surgeons may recommend additional procedures to refine the appearance of the nose or upper lip. Whether these are needed depends on how the initial repair healed and how things look as the child grows. These operations are almost always performed as an outpatient and do not require overnight stays in the hospital. Some children may undergo 3 or 4 of these types of procedures during their growth years, depending on the surgeon.

8. Alveolar bone grafting.

Although babies born with cleft palates usually have their palate repaired around their first birthday, that first surgery typically doesn’t close the entire cleft. Most of the time, a small gap is left in the front, just under the upper lip, along the gum line. This is intentional. Research shows that leaving the cleft open in the gum line (called the alveolus) can allow the upper jaw to grow a little better than if it were closed too early. The hope is that this additional growth might lessen the chance that a child would subsequently require upper jaw surgery in teenage years. Most cleft teams recommend the alveolus be closed sometime between the ages of 6 and 8 years. Age older than 9 years is the single most significant predictor of bone graft failure. The ideal time to place this bone graft is usually made together with the cleft team orthodontist. Often, the orthodontist will place a palatal expander prior to this surgery to widen the arch of the upper jaw.

During the alveolar bone graft procedure (ABG), the gap in the gum line is filled with bone taken from the area near the child’s hip. The surgeon then covers this bone graft with gum tissue that’s moved over from the side. This new bone helps guide the eruption of a permanent tooth called the canine, it also strengthens the upper jaw, and it provides support for the base of the nose. Following this operation most children will spend a few nights in the hospital. Rarely, a surgeon might recommend placing a substance called BMP (bone morphogenic protein) in the cleft, instead of using the child’s own bone. This material encourages the body to fill the cleft with new bone and has the benefit of avoiding the need to take bone from another part of the child’s body (parents can’t donate bone to their children!). However, complications have been reported when BMP is used in growing children. Because of these concerns, the FDA has not approved its use for treating alveolar clefts in children and we do not recommend it.

8. Jaw Surgery.

Studies suggest more than half of all children born with a cleft lip and palate will require jaw surgery when they reach teenage years. Most often, this means moving the upper jaw forward (this operation is called a Le Fort I), and sometimes it might also be necessary to widen the upper jaw at the same time. This surgery brings the child multiple benefits, including moving the teeth into a normal relationship to improve the mechanics of chewing and significantly helping to normalize appearance. Advancing the upper jaw can also increase the clarity of speech, by making more room for normal tongue placement behind the upper teeth.

Before the jaw surgery can be performed, it can take an orthodontist somewhere between 18 months and two years to prepare the dental arches, so that they will fit together perfectly after the surgery is completed. For children whose midface sits further back than average, a technique called distraction may be recommended. This approach gradually moves the upper jaw forward and can achieve a significantly greater advancement than traditional jaw surgery alone.

How clefts are treated at the Craniofacial Center

Our foremost goal is to reduce the total number of operations that children with a cleft lip and palate typically undergo. We believe it is important for children to grow up seeing themselves as normal and not defined by their condition. One of the best ways to support this is by minimizing medical visits and surgical procedures whenever possible. As a guiding principle, less surgery is generally better than more. Below is a brief overview of the typical treatment plan at our center in Dallas.

1. Initial treatment.

We believe most babies born with a cleft palate will benefit from an initial evaluation with our feeding specialist to ensure that they are not having any problems growing and gaining weight. Once we are confident that babies are feeding well, nothing further is usually recommended until it is time to repair the lip. Although some centers recommend babies be started on nasoalveolar molding (NAM) right away, we do not recommend this for our patients.  We believe NAM excessively increases the burden of care, and the cost of treatment, by requiring weekly visits to an orthodontist who needs to continually reshape the NAM plate. This treatment also can produce irritating sores inside the mouth and nose, which later in life can impair nasal breathing. We are also aware of studies suggesting NAM hurts the growth of the face. More importantly, we are convinced NAM does not produce any improvements in appearance when children are older. We also do not offer cleft lip adhesion procedures, which we believe adds an additional operation that does result any measurable long-term benefits. However, children born with a bilateral cleft lip will often benefit from facial taping, and we show parents how to do this. Before any operation, babies may be referred for an evaluation by an ENT to see if ear tubes are needed, because we do not want children undergoing separate operations.

2. The lip repair.

There have been some relatively new studies that have raised questions about the effects of anesthesia on the developing brain (see the SmartTots website). Based on these concerns, we now delay the primary lip repair until over 6 months of age. Unlike many other teams, at our Center this operation is done as a day surgery, meaning that there is no need to spend the night in the hospital. During surgery the outer skin layer of the upper lip is closed with a medical grade glue, instead of using stitches (see Figures below). We have published this special technique for closing clefts and remain convinced that this type of closure results in a much better scar than with standard skin stitches (see reference #33). During the lip repair, the nose is also re-balanced using a technique that minimizes internal scarring. After the operation we allow babies to use a nipple, exactly the same way they were being fed before the operation. We also do not require that babies wear “no-no’s,” which we believe only frustrate children, without adding any benefit whatsoever.

3. Fixing the palate and speech.

We recommend repairing the palate sometime between 11 and 15 months of age. During this operation both the hard and soft palate are closed at the same time. Afterward, no restrictions are made on how the baby eats and we also do not use “no-no’s,” but children are watched overnight in the hospital. At our center, this is typically the only night that many families will end up spending in the hospital related to their child’s cleft care. Following the palate repair, children are followed by our team speech pathologist (and dentist, if necessary) to monitor the quality of their speech. Speech therapy is often recommended leading up to grade school. Most children do not require additional operations on the palate; if they do (about 20% end up needing additional palatal surgery for speech), we are most likely to recommend a 4-flap palatoplasty.  We believe this specific operation is the least likely to produce later problems with sleep apnea. However, we recognize that every operation needs to be tailored to the unique needs of each child.

4. Additional operations on the lip and nose.

While many centers routinely recommend “touch up” operations on the lip and nose, we have noticed that most children do not give their lip scars a second thought until they are older. So, following the philosophy: “ain’t broke, don’t fix,” any revisions of the nose or lip are left up entirely to the parents and children. We have noted that earlier operations are more likely to need to be repeated later, and that the longer you wait to make a revision, the less likely that future operations will need to be performed. So, by waiting until families believe their children are ready for such surgery, it is possible to eliminate operations, reducing this total number over a child’s lifetime.

5. Alveolar bone grafting.

At our center, we recommend only using the child’s own bone for filling in the alveolar cleft (along the upper gums). We work together with our team orthodontist to determine the ideal timing for this operation; however, generally it is best to do this well before age 9. At the end of the operation, a very thin, soft tube called a pain pump is placed into the donor site where the bone is taken (usually the right iliac crest, near the hip). For the next five days, a small, steady amount of numbing medicine is delivered directly to this area, which significantly reduces soreness. This method has been so effective that children no longer need to stay in the hospital overnight and can instead return home the same day as the surgery. We know that children feel more comfortable and less anxious when they can recover in their own beds, and this approach helps make that possible.

6. After the bone graft.

After children undergo alveolar bone grafting, many will not require any additional surgeries. We continue to follow them as they grow into their teenage years, and even into adulthood when needed, working closely with our team orthodontist and dentist. For families who live farther away, our orthodontist can coordinate with a local orthodontist to help guide the child’s ongoing care.

Although jaw surgery may be needed once growth is complete, any additional procedures on the nose, lip, or even for speech are only considered when children and their parents feel the benefits would be meaningful. We believe the best care is the kind that allows children to grow up seeing themselves as the normal individuals they are, which includes minimizing the total number of surgeries they experience. An added advantage of limiting operations is avoiding an “over‑operated” appearance. In many cases, fewer but well‑timed procedures lead to a more natural and pleasing result.

7. Final thoughts.

We encourage families to seek more than one opinion before choosing the team that will care for their child. Some may decide to travel farther from home in order to access more experienced providers. Surgeons vary not only in their surgical techniques, but also in their communication styles and overall approach to treatment. Meeting with more than one team helps families find the care team that feels like the best fit for them.

At The Craniofacial Center, our philosophy is to limit the number of operations a child may need and to reduce medical visits whenever possible, with the goal of helping children see themselves as the normal individuals they are.

Jeffrey A. Fearon, MD
cranio700@thecraniofacialcenter.com
972-566-6464