Oral-facial clefts are birth defects in which the tissues of the mouth or lip don't form properly during fetal development. In the United States, clefts occur in 1 in 700 to 1,000 births, making it one of the most common major birth defects. Clefts occur more often in kids of Asian, Latino, or Native American descent.
The good news is that both cleft lip and cleft palate are treatable. Most kids born with these can have reconstructive surgery within the first 12 to 18 months of life to correct the defect and significantly improve facial appearance.
About Oral Clefting
Oral clefting occurs when the tissues of the lip and/or palate of a fetus don't grow together early in pregnancy. Children with clefts often don't have enough tissue in their mouths, and the tissue they do have isn't fused together properly to form the roof of their mouths.
A cleft lip appears as a narrow opening or gap in the skin of the upper lip that extends all the way to the base of the nose. A cleft palate is an opening between the roof of the mouth and the nasal cavity. Some kids have clefts that extend through both the front and rear part of the palates, while others have only partial clefting.
The three common kinds of clefts are:
- cleft lip without a cleft palate
- cleft palate without a cleft lip
- cleft lip and cleft palate together
In addition, clefts can occur on one side of the mouth (unilateral clefting) or on both sides of the mouth (bilateral clefting).
More boys than girls have a cleft lip, while more girls have cleft palate without a cleft lip.
Because clefting causes specific visible symptoms, it's easy to diagnose. It can be detected through a prenatal ultrasound. If the clefting has not been detected prior to the baby's birth, it's identified immediately afterward.
Causes
Doctors don't know exactly why a baby develops cleft lip or cleft palate, but believe it may be a combination of genetic (inherited) and environmental factors (such as certain drugs, illnesses, and the use of alcohol or tobacco while a woman is pregnant).
The risk may be higher for kids whose sibling or parents have a cleft or who have a history of clefting in their families. Both mothers and fathers can pass on a gene or genes that can contribute to the development of cleft palate or cleft lip.
Complications
Kids with a cleft lip or palate tend to be more susceptible to middle ear fluid collections, hearing loss, and speech defects. Dental problems — such as missing, extra, malformed, or displaced teeth, and cavities — also are common in kids born with cleft palate.
Many children with clefts are especially vulnerable to ear infections because their eustachian tubes don't drain fluid properly from the middle ear into the throat. Fluid accumulates, pressure builds in the ears, and infection may set in. For this reason, they may have special tubes surgically inserted into their ears at the time of the first reconstructive surgery.
Feeding can be another complication for an infant with a cleft lip or palate. A cleft lip can make it more difficult for a child to suck on a nipple, while a cleft palate may cause formula or breast milk to be accidentally taken up into the nasal cavity.
Special nipples and other devices can help make feeding easier; you'll get information on how to use the specialized feeding equipment and where to buy it before you take your baby home from the hospital. In some cases, a child with a cleft lip or palate may need to wear a prosthetic palate called an obturator to aid in proper eating.
If your baby has problems with feeding, your doctor can offer other suggestions or feeding aids.
Treatment
Thanks to medical advancements, reconstructive surgery can repair cleft lips and palates and, in severe cases, plastic surgery can address specific appearance-related concerns.
A child with oral clefting will see a variety of specialists who will work as a team to treat the condition. Treatment usually begins in the first few months of life, depending on the health of the infant and the extent of the cleft.
Members of the cleft lip and palate treatment team usually include:
- a geneticist
- a plastic surgeon
- an ear, nose, and throat physician (otolaryngologist)
- an oral surgeon
- an orthodontist
- a dentist
- a speech pathologist (often called a speech therapist)
- an audiologist
- a nurse coordinator
- a social worker and/or psychologist
The specialists will evaluate your child's progress regularly, and monitor hearing, speech, nutrition, teeth, and emotional state. They'll share their recommendations with you, and can forward their evaluation to your child's school and any speech therapists that your child may be working with.
In addition to treating the cleft, the specialists will work with your child on any issues related to feeding, social problems, speech, and your approach to the condition. They'll provide feedback and recommendations to help you through the phases of your child's growth and treatment.
Surgery for Oral Clefting
Surgery is usually performed during the first 3 to 6 months to repair cleft lip and between 9 and 14 months to repair the cleft palate. Both types of surgery are performed in the hospital under general anesthesia.
Cleft lip often requires only one reconstructive surgery, especially if the cleft is unilateral. The surgeon will make an incision on each side of the cleft from the lip to the nostril. The two sides of the lip are then sutured together. Bilateral cleft lips may be repaired in two surgeries, about a month apart, which usually requires a short hospital stay.
Cleft palate surgery involves drawing tissue from either side of the mouth to rebuild the palate. It requires 2 or 3 nights in the hospital, with the first night spent in the intensive care unit (ICU). The initial surgery is intended to create a functional palate, reduce the chances that fluid will develop in the middle ears, and help the teeth and facial bones develop properly. In addition, this functional palate will help speech development and feeding abilities.
The need for more operations depends on the skill of the surgeon as well as the severity of the cleft, its shape, and the thickness of available tissue that can be used to create the palate. Some kids require more surgeries to help improve their speech.
Additional surgeries may also improve the appearance of the lip and nose, close openings between the mouth and nose, help breathing, and stabilize and realign the jaw. Later surgeries are usually scheduled at least 6 months apart to allow time to heal and to reduce the chances of serious scarring.
It's wise to meet regularly with the plastic surgeon to determine what's most appropriate in your child's case. Final repairs of the scars left by the initial surgery may not be performed until adolescence, when facial structure is more fully developed. Surgery is designed to aid in normalizing function and cosmetic appearance so that kids will have as few difficulties as possible.
Dental Care and Orthodontia
Children with oral clefting often undergo dental and orthodontic treatment to help align the teeth and take care of any gaps caused by the cleft.
Routine dental care may get lost in the midst of these major procedures, but healthy teeth are critical for kids with clefting because they're needed for proper speech.
Kids with oral clefting generally need the same dental care as other kids — regular brushing supplemented with flossing once the 6-year molars come in. Depending on the shape of the mouth and teeth, your dentist may recommend a toothette (a soft sponge that contains mouthwash) rather than a toothbrush. As your child grows, you may be able to switch to a soft children's toothbrush. The key is to make sure that your child brushes regularly and well.
Children with cleft palate often have an alveolar ridge defect. The alveolar ridge is the bony upper gum that contains teeth, and defects can:
- displace, tip, or rotate permanent teeth
- prevent permanent teeth from appearing
- prevent the alveolar ridge from forming
These problems can be fixed by grafting bone matter onto the alveolus, which allows the placement of the teeth to be corrected orthodontically.
Orthodontic treatment usually involves a number of phases. The first phase, which starts as the permanent teeth come in, is called an orthopalatal expansion. The upper dental arch is rounded out and the width of the upper jaw is increased. A device called an expander is placed inside the mouth. The widening of the jaw may be followed by a bone graft in the alveolus.
Your orthodontist may wait until all the permanent teeth come in before starting the second phase, which may involve removing extra teeth, adding dental implants if teeth are missing, or applying braces to straighten teeth.
In about 25% of kids with a unilateral cleft lip and palate, the upper jaw growth does not keep up with the lower jaw growth. If this occurs, these kids (as teenagers or young adults) may need orthognathic surgery to align the teeth and help the upper jaw develop.
For these kids, phase-two orthodontics may include an operation called an osteotomy on the upper jaw that moves the upper jaw both forward and down. This usually requires another bone graft for stability.
Speech Therapy
Kids with oral clefting may have trouble speaking — the clefting can make the voice nasal and difficult to understand. Some will find that surgery fixes the problem completely.
Catching speech problems early can be a key part of solving them. A child with a cleft should see a speech therapist between the ages of 18 months and 2 years. Many speech therapists like to talk with parents at least once during the child's first 6 months to provide an overview of the treatment and suggest specific language- and speech-stimulation games to play with the baby.
After the initial surgery is completed, the speech pathologist will see the child for a complete assessment. The therapist will evaluate developing communication skills by assessing the number of sounds the child makes and the actual words he or she tries to use, and by observing interaction and play behavior.
This analysis helps determine what, if any, speech exercises a child needs and if further surgery is needed. The speech pathologist often will continue to work with the child through additional surgeries. Many kids will work with a speech therapist throughout their grade-school years.
Dealing With Emotional and Social Issues
Society often focuses on appearances, and this can make childhood — and, especially, the teen years — hard for someone with a physical difference. Because kids with oral clefting have a prominent facial difference, they might experience painful teasing that can damage self-esteem. Part of the cleft palate and lip treatment team includes psychiatric and emotional support personnel.
Ways that you can support your child include:
- Try not to focus on the cleft and don't allow it to define who your child is.
- Create a warm, supportive, ands accepting home environment, where each person's individual worth is openly celebrated.
- Encourage your child to develop friendships with people from diverse backgrounds. The best way to do this is to lead by example and to be open to all people yourself.
- Point out positive attributes in others that do not involve physical appearance.
- Encourage autonomy by giving your child the freedom to make decisions and take appropriate risks, letting his or her accomplishments lead to a sense of personal value. Having opportunities to make decisions early on — like picking out what clothes to wear — lets kids gain confidence and the ability to make bigger decisions down the road.
Consider encouraging your child to present information about clefting to his or her class with a special presentation that you arrange with the teacher. Or perhaps your child would like you to talk to the class. This can be especially effective with young children.
If your child does experience teasing, encourage discussions about it and be a patient listener. Provide tools to confront the teasers by asking what your child would like to say and then practicing those statements. And it's important to keep the lines of communication open as your child approaches adolescence so that you can address his or her concerns about appearance.
If your child seems to have ongoing self-esteem problems, contact a child psychologist or social worker for support and information. Together with the members of the treatment team, you can help your child through tough times.
Reviewed by: Louis E. Bartoshesky, MD, MPH
Date reviewed: October 2008
