Cleft Palate and Cleft Lip

In the United States, an estimated one in 700 babies each year – and possibly as many as one in 500 – are born with one or both of the birth defects known as cleft palate and cleft lip. Meaning “split” or “opening,” clefts are among the most common major birth defects.

Cleft lip and the combination of cleft lip and cleft palate occur twice as often in males as in females, while cleft palate alone occurs more often in females. Clefts occur most often in children of Asian, Latino or Native American descent. These deformities are known to occur less frequently in African Americans.

Cleft Palate

Cleft palate is an oral deformity affecting the hard palate (bony part of the roof of the mouth). Occasionally the soft palate, which is the soft, fleshy portion at the back of the roof of the mouth, is also affected. This deformity causes an opening from the inside of mouth into the nasal cavity. This opening might run from the front of the mouth (possibly including the lip area) to the throat and uvula (the dangling tissue that resembles a punching bag). Cleft palate occurs when the two plates of the roof of the mouth do not fuse together completely during gestation.

Cleft palate is usually not visible because it develops inside the mouth. However, cleft palate can also affect the development of the upper lip, in which case the cleft would be visible. The extent of cleft palate abnormalities can vary significantly.

Cleft Lip

Cleft lip is a visible facial/oral deformity characterized by the insufficient development of skin or lip tissue, resulting in a split or opening. Cleft lip can be mild, appearing as a simple notch or scar in the soft, pink tissue of the lip or the skin above the lip. Severe cases of cleft lip appear as a gap or opening in the lip that extends up into the nose.

Cleft lip deformities are categorized based on the location of the abnormality or defect and how much of the lip is involved.

Unilateral Incomplete: A cleft on one side of the mouth that does not extend into the nostril.

Unilateral Complete: A cleft on one side of the mouth that extends into the nostril.

Bilateral Complete: Clefts on both sides of the mouth, each of which extends into the nostril.

Microform Cleft: Considered a mild form of cleft lip, a microform cleft appears as a small, insignificant notch in the pink area of the lip, or a minor scar stretching up from the lip into the nose. Microform clefts may involve muscle tissue in the lip underneath the cleft that requires surgery, but other microform clefts may require no reconstruction.

Factors Causing Cleft Lip and Cleft Palate

The specific factors responsible for causing cleft lip and cleft palate are unknown, but are generally believed to be a combination of genetic and environmental influences. If you have a cleft, or have a family history of cleft lip or palate, your child has a greater chance of being born with the abnormality.

Environmental factors that might be implicated as possible causes include exposure during pregnancy to anti-seizure medications, acne medications, alcohol and/or tobacco, illegal drugs (eg, cocaine, heroin, crack cocaine), vitamin A derivatives and other chemicals/toxins.

Diagnosis of Cleft Lip and Cleft Palate

Following birth, cleft lip and cleft palate are visible and obvious, making the conditions easy to diagnose. A thorough examination of an infant’s mouth, nose and palate confirms that cleft lip and/or cleft palate exist. To rule out the presence of other possible birth defects, additional diagnostic tests may be performed.

During pregnancy, cleft lip and cleft palate can sometimes be confirmed using ultrasound.

Implications, Complications & Special Considerations

Cleft lip and cleft palate abnormalities are correctable birth defects. Most children born with these abnormalities can undergo corrective and plastic surgeries to restore proper function and appearance, making it possible for them to live healthy and productive lives.

Although the long-term prognosis for a baby with cleft lip or cleft palate who receives ongoing care, treatment and support is positive, there are physical, medical, social and developmental problems associated with cleft lip and cleft palate that parents and family members should be aware of.

Speech: Children born with cleft palate are likely to experience speech problems hindering development of their verbal ability. More than half of the children born with cleft palate will require speech therapy during childhood. Exactly how much and what type of speech therapy will vary.

Feeding: Babies with cleft palate can have a difficult time feeding because of the opening in the lip and/or palate. Food and liquids can pass through the opening from the mouth up into the nose and nasal cavity. There are certain baby bottles and nipples specially designed to help keep liquids flowing downward into the stomach. Babies with a cleft palate will likely feed better in an upright position. Infants with a cleft palate may also find that an obturator (prosthetic palate) enables them to eat properly and nutritiously until surgery can be performed.

Ear Infections/Hearing Loss: When a cleft palate is present, the Eustacian tubes (tubes that connect the ears to the throat) and external ear canals may not be positioned correctly. As a result, individuals with cleft palate are prone to middle ear infections (otitis media) caused by fluid build-up. Repeated ear infections could lead to hearing loss. For this reason, children with cleft palate usually require special tubes to be placed in their eardrums to facilitate fluid drainage. This minor surgery can be performed at the time of cleft palate repair or during a separate procedure.

Dental Problems: As a result of the abnormalities in the upper arch of the mouth, teeth may not erupt properly or may be missing completely. In such cases, artificial teeth and orthodontics (braces) are usually required. Routine oral hygiene, tooth brushing and flossing are still required to maintain healthy teeth and gums and prevent gum disease (periodontitis) and tooth decay. If the size and shape of a child’s mouth doesn’t permit the use of a regular toothbrush, a toothette (a soft sponge containing mouthwash) can be used. However, the use of mouthwash for infants or young children is not recommended.

Psychological Issues: Most children and adults with cleft lip and/or cleft palate do not experience major social or psychological problems. This is because surgeries to repair the abnormality and correct the visible defect have advanced significantly and can be performed at earlier ages. However, some people with cleft lip and/or cleft palate are dissatisfied with their facial appearance and therefore experience feelings of depression and anxiousness. Psychological support from the treatment team’s psychologist, family members, teachers and medical professionals can help with this and should be encouraged, starting at an early age.

Treatments for Cleft Lip and Cleft Palate

Children with cleft lip and/or cleft palate are treated over the course of 18 or more years. Treatment can involve a team of professionals beginning shortly after birth and continuing throughout adolescence. The treatment team includes medical, dental and other healthcare specialists who work together to address the many different and complicated needs specific to the individual.

The emphasis of treatment is focused on establishing normal function, speech, appearance and improved quality of life. Members of the cleft lip and/or cleft palate repair team typically include:

Oral/maxillofacial Surgeon: Performs surgeries involving the alignment of the upper jaw to enhance function and appearance, as well as for cleft palate repair and cleft lip repair

Plastic Surgeon: Performs necessary surgeries for cleft palate repair and/or cleft lip surgery, as well as procedures to correct abnormalities related to facial structures and soft tissues

Dentist/Pedodontist/Prosthodontist: These dentists provide routine oral hygiene, preventative and restorative care, as well as restorations and dental appliances to improve function for eating and speaking, as well as to enhance appearance

Orthodontist: Orthodontists straighten and reposition the teeth

Ear, Nose and Throat Doctor (Otolaryngologist): Monitors hearing and recommends therapies should hearing problems develop

Audiologist: Evaluates hearing problems that could affect interpersonal communication

Speech Pathologist/Therapist: Works with patients to develop proper speech and phonetic abilities

Nurse/Treatment Coordinator: Supervises patients’ ongoing health

Psychologist/Social Worker: Provides counseling and support services to the child and his/her family for dealing with the abnormality

Geneticist: Explains to the parents and adult patients the likelihood of them having more children with a cleft.

Cleft Lip Surgery

Cleft lip surgery is typically performed within two to three months after birth; however, treatment decisions are dependent upon each individual and the patient’s care team. If the cleft lip is unilateral (affecting one side), only one surgery may be necessary. There are a number of procedures that can be performed depending upon the extent of the cleft, the amount of lip and skin tissue present and what the treatment team determines will produce the best result.

Prior to cleft lip surgery, a special retainter called a nasoalveolar molding appliance (NAM) is worn in the baby’s mouth before the lip is repaired. This helps close the cleft gap inside the mouth, shape the nose and help the surgeon perform a better surgical repair of the cleft lip and nose. Taping across the cleft lip also helps bring the two sides of the upper lip together for easier surgical repair.

In general, incisions are made alongside the cleft and below the nostril to enable the two separated parts of the lips (either side of the cleft) to be joined together and sutured (stitched) so that the lip will appear normal. This includes joining the skin and muscles together.

If the cleft is bilateral (affecting both sides) and extensive, two surgeries may be necessary – one for each side, performed several weeks apart – to close the clefts. A short hospital stay is usually required for both unilateral and bilateral cleft repair.

Cleft Palate Repair

To help with feeding and nutrition, a cleft palate may be temporarily “closed” using an obturator until surgery can be performed. An obturator is a prosthetic (artificial) palate that fits the roof of the mouth to cover the opening. It enables an infant to eat properly.

Initial surgery for cleft palate repair is generally performed when the infant is between six and 18 months and is intended to create a functional palate that will enable the child to develop proper feeding and speech abilities. It typically involves making incisions on either side of the cleft (opening) in the roof of the mouth and then using soft tissue from each side of the mouth to close and/or rebuild the palate. Cleft palates are not typically closed by bringing the bones together because doing so may affect future facial growth. A short stay in the hospital is usually necessary.

Cleft palate repair is a more complex process, often requiring combinations of procedures as the child develops. These other surgeries may involve correcting defects involving the upper jaw and its alignment through an osteotomy, as well as the positioning of the teeth through orthognathic surgery.

Additionally, if the cleft palate extends into the front part of the upper arch, a bone grafting procedure may be required to provide bone for teeth to erupt. Correction of any upper jaw defects and the surgery required to do so may be postponed until the child reaches facial maturity in the late teen years. However, depending on the specific individual, earlier correction may be warranted in order to facilitate better speech therapy and overall function.

Cost of Cleft Lip and/or Cleft Palate Treatment

The cost of cleft lip surgery and/or cleft palate repair is estimated to run a minimum of $5,000. However, most children born with clefts require ongoing care that could involve multiple surgeries or different types of treatments, the costs for which could accumulate to hundreds of thousands of dollars.

More often than not, plastic and reconstructive surgeries associated with cleft lip and cleft palate are considered necessary treatments by medical insurance companies. Therefore, most or all of the costs associated with these procedures may be covered by health insurance. In cases that they are not, cleft advocacy and support groups such as the Cleft Palate Foundation can provide support and guidance detailing how to request coverage/review of the case.

Charitable Treatment

There are a number of charities dedicated to raising funds for cleft lip and cleft palate surgeries in under-developed countries throughout the world in order to improve the quality of life for children born with these birth defects. Such charities strive to keep the costs of providing these surgeries low (anywhere from $250 to $450 dollars).

In developing countries, individuals with unrepaired clefts cannot eat or speak properly, are kept from attending school or obtaining employment and may be subjected to further abuse and cruelty as a result of their birth defect. The biggest worldwide charity dedicated solely to cleft lip and cleft palate birth defects is Smile Train. The organization provides education and training to local doctors in developing countries about how to perform cleft lip and palate surgeries.

Other charitable organizations involved with cleft lip and cleft palate procedures include: Rose Charities, Operation Smile and Operation Blessing International.