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Cleft lip, cleft palate, or both is a congenital (birth) defect, which is present at birth. It will happen in North America approximately one in every 650 to 700 births and is probably the most common of congenital birth defects. It occurs in males and females and occurs in all races. All clefts are different. They can occur at one or more different sites on the face, such as the palate, the lips, or the alveolus (gums). Clefts can be different sizes and some are bigger than others.

CLEFT LIP

A cleft lip is a developmental defect that occurs in the womb in the fourth to sixth week. It occurs when areas of the upper lip do not join together resulting in a cleft of the lip between the mouth and the nose. A cleft lip can occur in many different forms; from a small notch in the lip to a larger cleft, which completely separates the lip and extends into the nose and mouth. A unilateral cleft is one, which occurs on either the right or the left side. A bilateral cleft is one, which occurs on both the right and the left sides

CLEFT PALATE

A cleft palate is a birth defect that occurs in the eighth to twelfth week in the womb. If the structures that form the roof of the mouth and the nose do not come together, the result is an opening between the mouth and the nose. As with cleft lip, the separation can occur on either the right or left sides, or both. There are two parts to the roof of the mouth; the hard palate, and the soft palate. The hard palate is the bony, nonmoving part of the roof of the mouth behind the teeth. It provides a separation between the front portion of the mouth and the nose. Behind the hard palate is the soft palate. It is made up of muscles that move during swallowing and speech to separate the nose from the mouth. A cleft can occur only in the soft palate, or extend forward to include all of the hard palate was well. However most clefts of the palate involve both the soft and hard palates.

WHY DID IT HAPPEN?

No one knows the exact reason that clefts of the oral structures occur and no research has shown any specific cause. The most common theory is that the majority of clefts are due to a genetic predisposition, that when mixed with certain environmental factors, create the malformation. We know that clefting cannot be caused by events occurring in middle or late pregnancy, and there are no specific actions by parents that cause the condition. So therefore you, as a parent, do not need to feel responsible. Most babies born with a cleft are otherwise normal children with no other serious birth defects.

You may be concerned about the risk of having another child with a cleft condition. Future brothers and sisters of a child with a cleft run a greater risk than that of the general population. For most families the risk ranges from two percent to four percent, depending on the extent of the condition in your first child. The more extensive the cleft, the greater the chance that future siblings will be affected.

During your child's evaluation, the team will discuss your family history and the likelihood that you could have another child with cleft lip or palate. This counseling will help you make family planning decisions. If appropriate, you also will be seen by a geneticist, a physician who specializes in heredity.

Surgical repair of cleft lip and palate is most often a staged procedure, with some surgeries being performed when your child is quite young, and other surgeries being performed when your child is older. The following sections will explain the timing of surgery, why surgery needs to be performed, and the anticipated results. The members of the Cleft Palate-Craniofacial Team at the John W. Keys Speech and Hearing Centers work together closely, taking a flexible approach to the timing of cleft repair, in order to optimize your child’s speech development and facial growth. The maxillofacial surgeon on the team will be happy to explain the surgeries that your child will need and the optimum timing.

PRE-SURGICAL TREATMENT OF CLEFT LIP

Cleft lip and cleft lip nasal deformities can be modulated with presurgical oral appliances that can improve the post-surgical lip and nasal appearance. By using an appliance to mold the cleft lip, upper jaw, alveolus, and nose prior to surgery, we can achieve better results. This may lessen the extent of surgery necessary and the number of subsequent procedures.

INITIAL SURGERY

The first surgery that your baby will have is to close the cleft of the lip, repair the nose, and to rebuild the muscles around the mouth and nose. This is done after the baby has had time to grow and get somewhat stronger. Cleft lip repair is generally performed when the baby is 10 weeks old. The actual surgery takes about an hour and a half and your child will generally stay in the hospital overnight. Parents are encouraged to spend the night in the same room as their child. The surgical team will provide care for your child while in the hospital and will explain how to care for the surgical repair at home.

CLEFT PALATE CLOSURE

Closure of the cleft palate generally is performed between the ages of 12 and 15 months. The delayed sequence of this surgery will coincide with the initiation of speech development in the child. An intact palate is crucial to formation of many important speech sounds. By closing the cleft before the baby begins to talk, learning to produce those speech sounds can proceed in a more normal fashion.

The surgical procedure normally takes about an hour and a half. The tissues of the hard palate are advanced to close the gap between the mouth and the nose, and the muscles of the soft palate are reconstructed to meet at the midline.

THE TEETH AND JAWS

Clefts, which extend through the alveolus (upper gums), can cause problems relating to the eruption of your child’s teeth. The oral and maxillofacial surgeon will work closely with the team’s orthodontist to coordinate the proper eruption and alignment of the teeth and jaws. Repair of an alveolar cleft often requires the placement of a bone graft and closure of the oral mucosa, usually between the ages of 5 and 11. This will help to properly support your child’s permanent teeth and aid in proper arch alignment. Traditionally bone has been harvested from the hip and is still used to this day. One of the new materials that may be used for bone grafting is Bone Morphogenic Protein. This protein is synthesized to induce humans to form bone in the area the protein is placed. By using this bone inducing protein kids can play normally after surgery because they have not had to undergo the painful hip surgery. Discuss the use of BMP with your Surgeon to see if it is right for you.

FUTURE SURGERIES

As your child grows, he may need more surgeries at different ages. It depends if problems arise and on the treatment plan that the Cleft Palate-Craniofacial Team at the John W. Keys Speech and Hearing Center has made for your child.

• Your child will be screened regularly by a speech/language pathologist to monitor his speech development. If air is escaping from your child’s nose (Velopharyngeal insufficiency), this may need to be surgically repaired.
• Before your child reaches school age, he may have a nasal tip reconstruction (repair of the end of the nose) and a revision of the lip scar.

During your child’s mid-teens, he will have the final repairs to the lip and/or palate and a last repair of scar tissue. He may also need surgery to advance the upper jaw. As the child continues to grow and develop and when skeletal maturity is reached careful attention will be directed to dental occlusion and to the position of the upper and lower jaws. In some patients corrective jaw surgery (orthognathic surgery) is recommended to recreate a more normal occlusal relationship. This may mean that the upper jaw may be brought forward, the lower jaw brought backward or both procedures performed. The orthodontist and the oral and maxillofacial surgeon prior to the performance of these operative procedures carry out careful preoperative planning.

The Speech/Language Pathologist is the specialist on the Cleft Palate-Craniofacial Team who evaluates speech, language, and the effects of soft palate malfunction on speech sound production (velopharyngeal insufficiency). Speech and language are evaluated according to a) the developmental status of the child and b) the presence or absence of soft palate malfunction in speech. In the event of soft palate malfunction, a recommendation is made for either therapeutic or surgical intervention to correct the problem. If and when surgery has taken place, the child's speech will be evaluated again to assess the results. If needed, recommendations for treatment, including speech therapy or additional surgery, will be made.

As your child begins to make intelligible sounds the "first words" will be noted, and the number of words and phrasing will be evaluated. Another thing listened for as speech develops is articulation. We will note the movement of the structures of the mouth to make speech sounds. The lower jaw, teeth, tongue, lips, hard palate, and soft palate (velum) all participate to make speech sounds. When soft palate movement is inadequate and speech inappropriately escapes into the nose, hypernasality and nasal air emission result. We will also carefully observe your child's speech adaptations to the structural changes made by the oral and maxillofacial surgeon and orthodontist.

TREATMENT GOAL

Our goal is age-normal speech by the time your child enters kindergarten. This is usually a reasonable goal, unless there are some conditions in addition to cleft palate that are affecting speech. Early identification and treatment of speech disorders is important if this goal is to be realized. Consistent Cleft Palate-Craniofacial Team follow-up of your child is essential to insure proper speech and language development.

ABOUT LANGUAGE

Language is the system of rules by which understanding and expression of meaning takes place. You might say that we learn language through a sort of "building blocks" approach: First children make general sounds, then they try to make sounds which approximate speech sounds, then they actually make speech sounds. After we put some of these sounds together to form a few words, we have the foundation to start learning the rules for making sentences, which will ultimately provide the foundation for learning written language. In children with clefts, the development of verbal language can be hindered because of mechanical and anatomical differences in the speech apparatus. If the children do not learn to make the same sounds as other children, it means they are starting with a different set of building blocks! Our job is to make sure that your child with a cleft is able to build the same linguistic skills as other children. You, your family, and other caretakers will also play important roles as models in helping this process along; because it is your voices to which your youngster will be closely listening as he or she learns to speak.

ABOUT HEARING

Obviously, hearing plays an important role in the development of speech and language and not surprisingly in the ability to read and write later in school. One aspect of soft palate malfunction in cleft palate is that the muscle which provides fresh air to the middle ear cavity doesn't work properly. As a result, youngsters with cleft palate are especially prone to middle ear infections which in turn affect hearing. For this reason, middle ear function and hearing status is monitored at every team visit. This condition is likely to persist off and on until the age of 10. Therefore, it is critical to speech and language development that we insure consistently good hearing.

LEARNING NORMAL SPEECH AND LANGUAGE

Children who do not receive adequate amounts of repetitive modeling may have difficulty developing language skills such as vocabulary, concepts, and grammar. Their speech may develop slowly, or they may not learn certain skills simply because they are not exposed to them. There are a few things that you can do to improve your abilities as a speech model for your child: Use short sentences when speaking to your child. Avoid over-doing "baby talk" because it does not provide the normal model speech and language examples that the child needs.

Speech is learned in the first few years of life. Normal speech depends not only on modeling, but also on good hearing, adequate mental functioning, and normal speech anatomy. Of all of these factors, the one you will personally have the most control over will be modeling. The Cleft Palate-Craniofacial Team will help to make normal speech mechanically possible, but they cannot do much about modeling. That is up to you and your family; and you should take special pride in that area as your child develops.

Practice for normal speech begins during the first year of life and continues over the next several years. Normal speech develops in a somewhat orderly fashion from sounds that are easiest to make (vowels) and simple consonants (p, b,) to sounds that require more coordination (s, r, th, st, and str).

Children will learn "object" words first and it is a good idea to name and talk about the objects in your child's environment. This includes the names of people, toys, food, eating utensils, and furniture. Take advantage of trips to the store or to relatives to name and talk about new objects. Between the first and second year, "action" and "description" words begin taking on importance. When your child begins to show interest in books, a good starting place is to talk about the pictures. Take turns naming the objects and talking about what is happening in the pictures.

NORMAL SPEECH SOUNDS

Normal speech consists of approximately forty-six sounds. Only three of these sounds emanate from the nose (nasal sounds), and approximately forty-three sounds emanate from the mouth (oral sounds). The nasal sounds are M,N, and NG. The remaining consonants and vowels are the oral sounds. Most of the consonants require pressure to be built up in the mouth. This pressure is then released (such as in the sounds P, T, or K) or it is constricted (such as in the sounds S, F, or TH.

ABNORMAL SPEECH

Hypernasality is the resonance that we hear when voice is allowed into the nose for those sounds that should not have nasal resonance.

Hyponasality is the resonance that we hear when voice is not allowed into the nose for the three nasal sounds. Glottal stops, pharyngeal fricatives, and posterior nasal fricatives are unusual sounds that are peculiar to children with cleft palate who still have speech escaping into the nose. These unusual articulation errors result when children try to stop sound from getting into the nose. These sounds are not otherwise heard in English, thus their unusual quality. A combination of hypernasality and these unusual articulation errors are sometimes referred to as cleft palate speech.

SPEECH ANATOMY

The oral sounds emanate from the mouth by closure of the velopharyngeal valve (that is, the soft palate and the pharyngeal muscles.

The hard palate is the bony roof of the front of the mouth. It separates the nasal and oral cavities. The soft palate (or velum) is the muscular part of the roof of the mouth in the back of the throat. The soft palate is like a muscular door, the pharyngeal wall is the doorjamb, and the nasopharynx is the doorway. In a condition of velopharyngeal competence (when these mechanisms are working properly), the velum opens the doorway for the nasal speech sounds and closes the doorway for the oral speech sounds. We refer to a condition of velopharyngeal incompetence when the soft palate does not successfully close off the nose for the oral sounds. The hypernasality and the nasal air escape often associated with clefts are usually the result of velopharyngeal incompetence.

SPEECH PROBLEMS

Speech problems are usually divided into two categories, hypernasality, and articulation problems. Either of these can make speech noticeably different from those of other children the same age and can make speech difficult to understand. Some of these speech problems may be caused by a structural defect that may require surgery, prosthodontics, or orthodontic treatment.

Some speech problems may be the result of poorly learned or incorrectly learned speech production. These will require speech therapy. Speech therapy may be provided by the speech therapist on the cleft palate team, at a clinic in your home community, or in school. Evaluation by the speech pathologist will determine the cause of any speech problems. In almost all cases, these problems can be managed through the combined efforts of the oral and maxillofacial surgeon, the orthodontist, and the speech pathologist.

BEFORE THE CLEFT PALATE IS CLOSED

Before a cleft palate is repaired, there is an opening between the oral and nasal cavities. Speech enters the nose through this opening. Although nasal sounds (m, n, and ng) sound normal, the oral speech sounds are distorted by too much nasal resonance and the inability to build up pressure in the mouth because it can be heard leaking out the nose. The child should be encouraged to use words that have nasal sounds such as "mamma." Unfortunately, the child will be unable to say "dada," very well because this word has oral pressure sounds. The cleft of the palate will have to be closed in order for your child to have normal speech.

AFTER THE CLEFT PALATE IS CLOSED

After the cleft is closed, you will be instructed about how to encourage your child to make sounds that will help the muscles in the soft palate begin to function. Most children have normal soft palate function after the cleft is closed. Some may not and will need a second surgery. The speech pathologist will evaluate your child's speech and soft palate function to make this determination.

Successful secondary correction of this soft palate deficit often involves the combined skills of the oral and maxillofacial surgeon, orthodontist, and speech pathologist. The structure and function of the soft palate are evaluated by analysis of videofluoroscopy (motion x-ray pictures), special fiber-optic scopes, computerized speech instruments, and clinical judgments of hypernasality.. These studies determine which procedures are used to correct the deficit.

OUTLOOK

The outlook for good speech and language is bright. The combined efforts of caring specialists and cooperative families can help the child achieve normal speech and language - one of the primary goals of cleft palate team treatment.

The child with a cleft lip or palate may be observed or treated by the maxillofacial prosthodontist at several points along the road to adulthood. In some cases, the expertise of the maxillofacial prosthodontist will actually be called into play before the maxillofacial surgeon operates. For example, with some babies, it is necessary to align the gums of the upper jaw before the maxillofacial surgeon can operate on the cleft lip. In these cases, the maxillofacial prosthodontist makes a mold or impression of the upper jaw and designs an appliance to bring the upper tooth arch into alignment. Such appliances may involve headgear with straps that fit around the outside of the face and head, or they may be smaller appliances, pinned into the palate for several weeks before surgery. In either case, the maxillofacial prosthodontist works closely with the surgeon to mold the front bones of the upper jaw into a position that permits surgical correction of the cleft lip.

Most of us are aware of the role the orthodontist plays in straightening children's teeth through the use of "braces" and/or "retainers." In a broader sense, orthodontics is the specialty area of dentistry that deals with the growth and development of the face, jaws, and dental arches (teeth). Because clefts often affect parts of the mouth which play roles in the growth of teeth, the orthodontist plays a very special role on your cleft palate team.

The child with a cleft lip or palate may be observed or treated by the orthodontist at several points along the road to adulthood. As the child grows and the baby teeth erupt ("come in"), continuing orthodontic observation is needed, and some limited orthodontic treatment may be required. This may involve moving a few rotated teeth which are interfering with biting and chewing, or fitting the child with an appliance to cover a hole in the palate that interferes with speech. The contribution which the orthodontist can make to the speech-learning process is another indication of the advantages offered by the team approach to treating cleft lip and cleft palate.

For the most part, though, the bulk of orthodontic work with cleft conditions takes place after the permanent (adult) teeth erupt. Correcting rotated teeth and crossbite, and aligning the upper and lower teeth for adequate chewing and improved appearance are some of the goals of orthodontic treatment. Where there are missing teeth, orthodontic treatment can either close spaces or prepare the teeth for later placement of selected replacement teeth (fixed or removable). The appliances supporting these "replacement teeth" are made by the general dentist/prosthodontist, another member of the cleft palate team.

THE ORTHODONTIST'S ROLE AS TEAM CONSULTANT

As the growth and development specialist for the facial area, the orthodontist consults with the surgeon about the need and best time for certain surgical procedures.

In some children, the missing bone in the cleft of the upper dental arch requires replacement by a bone graft. The time to do this operation depends, in part, on the development of the permanent teeth in adjacent areas. At times, the bone graft is done before the eruption of a permanent tooth. This can allow the new tooth to "come in" through the new bone which provides support for the tooth. Otherwise, the tooth would eventually be removed due to the lack of bone support.

In the growing child with a cleft, the upper jaw does not always grow as much as the lower jaw. The resulting differences in the upper and lower dental arches can present problems in appearance, speech, or chewing. Sometimes, orthodontic treatment alone can correct the problem. In other cases, however, the child may require a combined orthodontic and surgical treatment for adequate alignment of the jaws and teeth. When required, the surgical procedure is usually done in the late teen-age years. In this orthognathic surgery, a deficient upper jaw can be moved forward, or a lower jaw that has overgrown the upper jaw can be set back appropriately.

In all of these instances, decisions about the timing and nature of surgery are based largely on the opinions of all consulting members of the cleft palate team during conferences. The treatment plan for your child is individualized according to his or her specific growth and development pattern.

TREATMENT RESULTS

The most common malformation of the face is cleft lip and palate. Clefting can affect about 1 in 660 children born in the United States. The clinic offers INTERDISCIPLINARY CARE for cleft patients through J.W.Keys Cleft and Craniofacial Team, including reconstructive maxillofacial surgery, pediatric dental evaluations, orthodontic and prosthetic care before and after surgery, speech pathology, audiology and pediatrics. There is also referral system in place otorhinolaryngology, genetics and neurosurgery. The goal of the team is to guide and direct parents in a manner that is consistent with the American Cleft Lip and Palate Association team guidelines. The clinic specialists work closely together, taking a flexible approach to the timing of cleft repair, to optimize the child's speech development and facial growth. Cleft lip repair is generally performed at 3-6 months of age, while cleft palate surgery take place at 12 to 15 months of age.