What are cleft lip and cleft palate?
Cleft lip and cleft palate are birth abnormalities of the mouth and lip. Approximately 1 in 600 babies born in the US has some type of cleft. American Indian and Asian populations have the highest incidence of clefts. These abnormalities occur less frequently among African Americans. Cleft lip and cleft palate occur early in pregnancy when the sides of the lip and the roof of the mouth do not fuse together as they should. A child can have cleft lip, cleft palate, or both. Cleft lip and cleft palate together are more common in boys. It is also important to know that most babies born with a cleft are otherwise healthy with no other birth abnormalities.
Cleft palate occurs when the roof of the mouth does not completely close, leaving an opening that can extend into the nasal cavity. The cleft may involve either side of the palate. It can extend from the front of the mouth (hard palate) to the throat (soft palate). Often the cleft will also include the lip. Cleft palate is not as noticeable as cleft lip because it is inside the mouth. It may be the only abnormality in the child, or it may be associated with cleft lip or other syndromes. In some cases, other family members have also had a cleft palate at birth.
Cleft lip is an abnormality in which the lip does not completely form during fetal development. The degree of the cleft lip can vary greatly, from mild (notching of the lip) to severe (large opening from the lip up through the nose). As a parent, it may be stressful to adjust to the obvious abnormality of the face, as it can be very noticeable.
There are different names given to the cleft lip according to its location and how much of the lip is involved. A cleft on one side of the lip that does not extend into the nose is called unilateral incomplete. A cleft on one side of the lip that extends into the nose is called unilateral complete. A cleft that involves both sides of the lip and extends into and involves the nose is called bilateral complete.
Cleft lip and cleft palate may occur together in an infant, or separately. The degree of the abnormality of both cleft lip and cleft palate can vary greatly. The most common early problem associated with these abnormalities is feeding your baby.
What causes cleft lip and cleft palate?
The exact cause of cleft lip and cleft palate is not completely understood. Cleft lip and/or cleft palate are caused by multiple genes inherited from both parents, as well as environmental factors that scientists do not yet fully understand. When a combination of genes and environmental factors cause a condition, the inheritance is called "multifactorial" (many factors contribute to the cause). Because genes are involved, the chance for a cleft lip and/or cleft palate to happen again in a family is increased, depending on how many people in the family have a cleft lip and/or cleft palate. If parents without clefts have a baby with a cleft, the chance for them to have another baby with a cleft ranges from 2 to 8 percent. If a parent has a cleft, but no children have a cleft, the chance to have a baby with a cleft is 4 to 6 percent. If a parent and a child have a cleft, the chance is even greater for a future child to be born with a cleft. Genetic consultation is suggested.
What are the symptoms of cleft lip and cleft palate?
The symptoms of these abnormalities are visible during the first examination by your infant's physician. Although the degree of the abnormality can vary, upon inspection of the mouth and lips, the abnormality can be noted, as there is an incomplete closure of either the lip, roof of the mouth, or both.
What are the challenges associated with cleft lip and cleft palate?
Beyond the cosmetic abnormality, there are other possible complications that may be associated with cleft lip and cleft palate, including the following:
- Feeding difficulties
Feeding difficulties occur more with cleft palate abnormalities. The infant may be unable to suck properly because the roof of the mouth is not formed completely.
- Ear infections and hearing loss
Ear infections are often due to a dysfunction of the tube that connects the middle ear and the throat. Recurrent infections can then lead to hearing loss.
- Speech and language problems
- It is common for children who are born with cleft palate to have speech problems at some time in their lives. Before the palate is repaired, there is no separation between the nasal cavity and the mouth. This makes it difficult for the child to learn how to make many speech sounds since the child cannot build up pressure in the mouth and there is less tissue on the roof of the mouth for the tongue to touch. The delay in speech sound acquisition may negatively affect language development. Your child's speech and language skills should be monitored throughout your child's development by a speech language pathologist.
Due to the opening of the roof of the mouth and the lip, muscle function may be decreased, which can lead to a delay in speech or abnormal speech. Referral to a speech therapist should be discussed with your child's physician.
- Dental problems
As a result of the abnormalities, teeth may not erupt normally and orthodontic treatment is usually required.
Feeding my baby with cleft palate:
The most immediate concern for a baby with cleft palate is good nutrition. Sucking for children with a cleft palate is difficult because of the poorly formed roof of the mouth. Children with just a cleft lip (without a cleft palate) do not routinely have feeding difficulties and may direct breastfeed without problems. However if the palate is involved, direct breastfeeding exclusively is very difficult. The following are suggestions to help aid in the feeding of your infant:
- Hold your infant in an upright position to help keep the food from coming out of the nose.
- There are several cleft bottle options available. The cleft palate nurser by Mead Johnson is a soft squeeze bottle that helps with the delivery of milk. The following are some tips for using the bottle:
- Before putting the nipple in your baby's mouth, gently squeeze the bottle so you can tell how hard you need to squeeze to get an even flow.
- Squeeze the bottle as soon as you begin the feeding. Don't wait until your baby is tired to begin squeezing the bottle.
- Squeeze the bottle as firmly as baby can easily keep up with the flow of milk.
- If baby starts losing milk out of the mouth, then don't squeeze quite as hard.
- Do not squeeze milk into baby's mouth while resting. This could cause the baby to choke. It also disturbs the natural suck/swallow pattern.
- There are other cleft bottles available. Consult with the cleft Team regarding these options.
For more information on feeding an infant with a cleft palate, visit the Cleft Palate Foundation website and view the video Feeding Your Baby.
The team approach for managing and correcting cleft abnormalities:
There will be many people involved in management of a cleft abnormality for your child, because many different skills are needed to help with the problems that can occur with cleft abnormalities. The standard of care is a multidisciplinary team of specialists. The following are some of the members of the team:
- Plastic/craniofacial surgeon - a surgeon with specialized training in the diagnosis and treatment of skeletal abnormalities of the skull, facial bones, and soft tissue; will work closely with the orthodontists and other specialists to coordinate a surgical plan. Most craniofacial and plastic surgeons that repair clefts have received specialized training during a fellowship beyond basic plastic surgery residency training.
- Orthodontist - a dentist who evaluates the position and alignment of your child's teeth and coordinates a treatment plan with the surgeon and other specialists.
- Pediatric dentist - a dentist who evaluates and cares for your child's teeth.
- Speech and language specialist - a professional who will perform a comprehensive speech evaluation to assess communicative abilities and who will closely monitor your child throughout all developmental stages.
- Otolaryngologist (ear-nose-throat specialist) - a physician who will assist in the evaluation and management of ear infections and hearing loss that may be side effects of your child's cleft abnormality.
- Audiologist (hearing specialist) - a professional who will assist in the evaluation and management of hearing difficulties your child may have.
- Genetic counselor - a professional who reviews the medical and family history, as well as examines your child to help in diagnosis. A genetic counselor also counsels your family regarding risk for recurrence in future pregnancies.
- Nurse team coordinator - a registered nurse who combines experience in pediatric nursing with specialization in the care of your child and acts as liaison between your family and the cleft team.
- Social worker - a professional who provides guidance and counseling for your child and your family in dealing with the social and emotional aspects of a cleft abnormality and assists your family with community resources and referrals (i.e., support groups).
Treatment for cleft lip and cleft palate:
Treatment for these abnormalities includes surgery and a complete team approach to help with the multiple complications that can occur. Specific treatment will be determined by your child's physician based on:
- Your child's age, overall health, and medical history
- Specific qualities of your child's abnormality
- Your child's tolerance for specific medications, procedures, or therapies
- Involvement of other body parts or systems
- Your opinion or preference
Nasoalveolar molding (NAM) is a nonsurgical technique that has revolutionized the treatment of children with large clefts of the lip and palate. In the past, a large cleft required multiple surgeries during a child's growth years (birth to 18), which put the child at risk for psychological and social adjustment problems.
Now, within a few weeks of birth, babies can be fitted with a painless, custom-made molding plate that looks like a dental retainer and that gently directs the growth of gums, lip and nostrils. This is possible due to the high levels of maternal estrogen present for several months after birth. Special elastic tape may be used on the lips with or without the appliance.
Because the plate causes no pain, babies adjust to its presence in a few days and can wear it 24 hours a day, seven days a week, even while feeding. The orthodontist adjusts the molding plate every week or two, depending on the progress.
By lessening the severity of the cleft lip or palate before surgery, NAM helps surgeons get a better result from the first surgery, which means fewer surgeries later in childhood.
For most infants with cleft lip alone, the abnormality can be repaired within the first several months of life (usually when the baby is 10 to 12 pounds). This will be decided by your child's surgeon. The timing of lip repair for infants undergoing NAM treatment will be determined by the orthodontist and the surgeon based on the position of the gums and nose. It is usually done at 4-6 months of age. The goal of this surgery is to fix the separation of the lip. Sometimes, a second operation is needed.
Cleft palate repairs are usually done between the ages of 10 to 18 months. This is a more complicated surgery and is done when the baby is bigger and better able to tolerate the surgery. The exact timing of the surgery will be decided by your child's physician. The goal of this surgery is to fix the roof of the mouth so that your child can eat and learn to talk normally. Sometimes, a second operation is needed.
Bone grafting the dental ridge of the upper jaw is a procedure that may be performed on patients with cleft lips and palates. By taking small amounts of bone from elsewhere on the body, such as the hip, surgeons can reconstruct the area of the cleft near the teeth. The bone graft allows for the formation of a continuous upper gum, supporting the teeth, lip, and nose, and improving facial symmetry and stability. This procedure is often performed before the age of 10. Once the bone graft is in place, missing teeth can be replaced by moving adjacent teeth, using a dental bridge, or implanting dental metallic bone
Surgery for cleft lip and cleft palate:
At your first visit with the plastic surgeon, he will discuss with you the details of the surgery, risks, complications, recovery time, and outcome. At this time, your child's surgeon will answer any questions you may have.
Cleft Lip Surgery
After the surgery for cleft lip, your child may be irritable. Your child's physician will prescribe medications to help with this. Your child will wear padded restraints on his/her elbows to prevent him/her from rubbing at the stitches and surgery site.
Stitches will either dissolve on their own or will be removed in approximately five to seven days. Specific instructions will be given to you regarding how to feed your child after the surgery. The scar will gradually fade, but it will never completely disappear. Surgeries are designed to attempt to hide scars in natural ridges and shadows so that they are less noticeable.
Your child will have an intravenous catheter (IV) to provide fluids until he/she is able to drink by mouth.
Your child's upper lip and nose will have stitches where the cleft lip was repaired. It is normal to have swelling, bruising, and blood around these stitches.
Cleft Palate Surgery
The surgery for cleft palate is usually more involved and can cause more discomfort and pain for the child than cleft lip surgery. Your child's physician will order pain medicine to help with this. As a result of the pain and the location of the surgery, your child may not eat and drink as usual. An intravenous (IV) catheter will be used to help give your child fluids until he/she can drink adequately.
Your child will have stitches on the palate where the cleft was repaired. The stitches will dissolve after several weeks and they do not have to be taken out by the physician.
There may be some bloody drainage coming from the nose and mouth that will lessen over the first day.
There will be some swelling at the surgery site, which will diminish substantially in a week.
Your child may be in the hospital for one to three days, depending on your child's physician's recommendation.
A small amount of water should be offered after every liquid meal to cleanse the incision. You can continue to rinse this area gently with water several times a day, if necessary.
Diet after surgery:
Your child will be placed on a blenderizedt diet for 2 weeks after surgery. For older infants and children, age-appropriate soft foods may include strained baby foods, melted popsicles, yogurt, and runny mashed potatoes. Note: your child should not use a straw, bottle or pacifier, as they could damage the surgical repair.
Follow-up with your child's surgeon and the cleft team is very important. This will be discussed with you. Your child's physician will also be an important part of the child's overall health management after the surgery.