Cleft Lip / Palate

What is a cleft lip?

A cleft lip is a notch or gap in the tissue that forms the lip.

If you look under your nose and above your mouth, you should notice a faint line or bulge of tissue under each nostril leading to the lip. These lines are known as philtrum lines. Each line can be a location of a cleft lip. Most of the time (80%) cleft lips are located on only one side (unilateral), more commonly on the left. The rest of cleft lips (20%) are located on both sides (bilateral).

The cleft can range in size from just involving a small part of the upper lip (vermilion border), to involving the entire lip and up to the nose.

What is the treatment for a cleft lip?

For an isolated cleft lip, in other words, the cleft occurs alone without a cleft palate or any other abnormality, the cleft is repaired surgically. This repair is usually performed between 2 and 10 weeks of age, depending on the size and health of the infant. The goal of this surgery is primarily to close the cleft, so the infant can suck normally. A second surgery, if needed, is performed as the child ages to correct any cosmetic facial or other abnormalities. A plastic and reconstructive surgeon usually performs this surgery.

A cleft palate occurring without a cleft lip will need cleft palate surgery (PALATOPLASTY). The pediatric ear, nose and throat specialist (otolaryngologist) is uniquely suited to evaluate a child with cleft palate. Because of their extensive training in the anatomy of the head and neck, especially involving the oral cavity and throat, we can evaluate your child for feeding and breathing complications of cleft palate and outline for you the most appropriate management, including timing and methods of repair.

What is a cleft palate?

If you lift your tongue to the roof of your mouth right behind your two upper teeth, you will feel your hard palate. If you continue to move your tongue backward, you should notice where the hard tissue becomes softer, or turns into the soft palate (velopharynx). Moving further back, you notice that the roof of the mouth ends as a small "bag" of tissue called the uvula. You can see your uvula if you open your mouth and look in a mirror (the little "punching bag" in the middle.) All of this is considered your palate.
A cleft palate is an abnormal opening in the palate. It results from the failure of the roof of the mouth to completely close during the development of the fetus during the first four to eight weeks of pregnancy.

A cleft palate can range in length from a split in the uvula (bifid uvula) to a cleft or gap completely through the soft and hard palates. The cleft most often exposes the underside of the nose, but occasionally the cleft will be "hidden" if the lining of the mouth is smooth and unbroken (submucous cleft).

How common is a cleft palate?

The incidence of cleft lip and/or cleft palate is 1 in 750 live births. This incidence is slightly affected by the race and/or sex of the child. An isolated cleft palate (occurring without a cleft lip) appears in about a quarter of these, with an increased incidence in females. The remainder either involves an isolated cleft lip (25%) or both cleft lips and palates (50%).

What causes a cleft palate?

Most of the time the exact cause is a combination of many factors. Some of the conditions that may result in a cleft lip and/or palate are:

1) Environmental factors - It is important to remember that a cleft lip and palate occur by 8 weeks of pregnancy, usually before most women know they are pregnant. That said, factors that may increase the likelihood of clefting include substances the mother ingested or was exposed to early on in the pregnancy. The position of the fetus or amniotic bands (strings of tissue in the uterus) may also contribute to clefting.

2) Genetic (inherited) clefts - Families with parents or other relatives with cleft lip and/or palate may have an increased likelihood of producing children with clefting.

It is recommended that the parents of any child born with a cleft undergo genetic testing so that they can make informed decisions about future children. To give a brief overview, if relatives and both parents of a child are normal, but the child is born with an isolated cleft palate, the chance of the next baby having a cleft palate is 2%. If one of the family's relatives is affected, the chance increases to 7%. If your baby has a cleft palate along with other congenital problems, the chance that the next baby will have a cleft palate drops again to 2%.

3) Cleft syndromes - A syndrome is an abnormality in inherited material (genes on chromosomes) that result in malformations or deformities that form a recognizable pattern. Most clefts ARE NOT part of a syndrome. However, if a child is born with a cleft, it is important to evaluate the child to make sure he/she does not have other deformities that would be part of a syndrome.

How is a cleft palate identified?

With the development of ultrasounds and their use during pregnancy, a cleft palate can often be detected before the baby is born. Most clefts are easily picked up during the initial exam of the newborn. Occasionally, very small clefts may not be identified until some cleft palate related conditions are noticed, such as ear problems or regurgitation of liquids into the nose.

How is a cleft palate treated?

There are two aspects of cleft palate treatment:

1) Repair of the cleft itself - this refers to the stages of surgical cleft palate repair or PALATOPLASTY. The initial stage usually takes place between 6 months to 1 year of life.

2) Treatment of common conditions that are associated with cleft palate

• Breathing Problems - With a cleft palate, the tongue has a tendency to fall back into the mouth and block the airway. In some children, the jaw bone (mandible) is also small which makes breathing even more difficult. This is treated by helping to keep the airway open with positioning of the child or insertion of various devices designed to help breathing.
  
  
• Feeding problems - In an infant with cleft palate, feeding may be difficult because the food does not follow the normal swallowing pathway into the back of the throat and, therefore, can confuse the child. There may also be some difficulty in using a bottle or breast-feeding. The abnormal roof of the mouth does not allow normal pressure or suction to be applied to the nipple, which results in inadequate sucking.
  
  Treatment consists of using specially formed bottles and nipples that help aid in the feeding of children with clefts. Trial and error with different methods and patience are also important. An occupational therapist may be of help if feeding continues to be a problem.
  
  
• Increased incidence of ear infections (OTITIS MEDIA) - Fluid in the middle ear (effusion) and repeated ear infections are present in at least 50% of children with a cleft palate. This is a result of the abnormal insertion of the muscles to the tube that drains the middle ear (Eustachian tube), which results in problems opening up the tube to equalize pressure and to help drain any fluid if present.
  
  Even after cleft palate repair, close follow-up of the patient's ears will be necessary into adolescence due to the high likelihood of continued middle ear problems.
  
  Please see OTITIS MEDIA and TYMPANOSTOMY TUBE PLACEMENT for more information on this condition.
  
  
• Hearing Loss - This is related to the frequency of ear infections. However, with proper treatment of the ear infections, and periodic hearing evaluations, the incidence of hearing loss should be significantly reduced.
  
  
• Speech problems - A patient with a cleft palate also has abnormal insertion of muscles into the back of the throat. As these muscles are involved with the production of normal speech, speech problems can result.
  
  Treatment of speech difficulties is handled both surgically and with speech therapy, using a team approach. As ear nose and throat specialists, we have extensive training in the reconstruction of muscles that are involved with speech. Because we also work closely with speech therapists, we can utilize the team approach to ensure appropriate development of speech and, if necessary, use specialized diagnostic tools to evaluate and treat any speech problems.
  
  
• Dental Problems - Many times children with clefts have teeth in unusual positions or need temporary prosthetic devices to aid with feeding, speech, etc. while the palate is undergoing reconstruction.
  
  This long-term treatment consists of the referral to a pediatric dentist, and/or an orthodontist, who helps to achieve a normal dental arch and teeth positioning, and a prosthodontist to help with specific dental appliances.
  
  
• Other Issues - These can include specific needs of the patient or the entire family regarding emotional issues, financial hardships, or other concerns.
  
  There are a variety of other professionals available to help provide the best possible care for the patient, as well as the family as a whole. It is our goal to provide your child with the best care possible.

If at any time during the evaluation and treatment of your child you have any questions or concerns, please do not hesitate to address them directly to us.