Multiple: Cleft Lip and Palate

Definition
Description
Demographics
Causes and Symptoms
Diagnosis
Treatment
Prognosis
Prevention
The Future
For more information

Definition

Cleft lip and palate are birth defects that affect the shape and function of the upper lip and the roof of the mouth. A cleft is basically an abnormal split or division. A child may have either a cleft lip (CL), a cleft palate (CP), or both (CL/P).

Description

Cleft lip is a birth defect that affects only skin tissue rather than underlying bone or cartilage. It can be categorized as either partial (a small gap or indentation in the baby's upper lip) or complete (the split runs from the upper lip up into the nose). It can also be defined as either unilateral, occurring on only one side of the mouth, or bilateral, occurring on both sides of the mouth.

Cleft palate is a defect in which the two plates of bone that ordinarily join to form the hard palate in the front of the roof of the mouth fail to fuse completely during the baby's development before birth. Cleft palate may be either incomplete, a defect in which only the soft palate at the back of the roof of the mouth is split; or it may be complete, when the hard palate as well as the soft palate is divided.

In addition to complete and incomplete cleft palate, there is a third type of defect affecting the roof of the mouth known as a submucous cleft. This is a condition in which a cleft in either the hard or the soft palate is hidden by a layer of tissue. As a result, the cleft may not be discovered until some months after the baby's birth.

Demographics

In the United States, between one in 500 and one in 550 babies in the general population are born with cleft lip, cleft palate, or both. There are

considerable differences among racial and ethnic groups, however. The highest rates of these birth defects are among Native Americans and the lowest rates among African Americans:

• Native Americans: 3.74 per 1,000 babies are born with cleft lip or cleft lip and palate
• Asian Americans: 3.36 per 1,000
• Caucasians: 1.86 per 1,000
• Hispanics: 1.04 per 1,000
• African Americans: 0.93 per 1,000

The male/female ratio varies somewhat depending on the type of defect. Boys are almost twice as likely than girls to have either cleft lip or cleft lip and palate, while girls are slightly more likely than boys to have cleft palate.

Causes and Symptoms

Cleft lip and cleft palate are caused by the failure of two or more lobes of tissue failing to join correctly during the baby's development during pregnancy. There are five lobes of tissue involved in the formation of the baby's face and upper lip: one that grows downward from the top of the head; two that grow inward from the cheeks; and two that grow inward

just below the cheeks to form the chin and lower lip. If two or more of these five lobes of tissue fail to join completely, a cleft lip or even complete facial deformity may result. The palate is formed later than the upper lip; it is composed of two bony plates that grow together as the last step in joining the five lobes of tissue described earlier. The entire complex process of facial and oral formation can be interrupted by a number of genetic and environmental factors.

Some of the causes of cleft lip and palate that have been identified as of 2008 include:

• A gene mutation discovered in 2004 that increases the risk of cleft lip and palate by a factor of three.
• Genetic disorders involving chromosomal abnormalities, including Down syndrome, Edwards syndrome, and Patau syndrome.
• Nutritional deficiencies in the mother, particularly a lack of vitamin A.
• Exposure to lead, pesticides, and other environmental toxins.
• Use of certain prescription medications, particularly drugs given to control seizures.
• Smoking, alcohol abuse, or use of crack cocaine or heroin by the mother.

The symptoms of cleft lip and cleft palate may include feeding difficulties and failure to gain weight, problems learning to speak, a misshapen nose, crooked teeth, and recurrent ear infections, as well as the visible defects in the baby's face and mouth.

Diagnosis

Cleft lip and palate can be determined by the doctor at the time of the baby's birth by a careful physical examination of the newborn. Cleft lip can also be diagnosed before birth by ultrasound.

In addition to a physical examination of the baby, the doctor may recommend genetic testing to rule out a genetic cause of the defect or to evaluate the possibility that the parents will have another child with cleft lip or palate.

Treatment

Surgery is the primary treatment for cleft lip and palate. In most cases the baby will be evaluated and treated by a team of doctors and other health

care professionals in order to assess his or her need for speech therapy, dental surgery, and other treatments in addition to repair of the upper lip and palate. The treatment of cleft lip and palate is highly individualized.

Surgical repair of the child's facial abnormalities is often carried out in the following order:

• Repair of a cleft lip. This repair is done between birth and three months of age so that the baby can feed as normally as possible as well as grow and gain weight. If the child has a bilateral cleft lip, the surgeon usually repairs one side first and the other side a few weeks later.
• Placement of ear tubes. These are placed around three months of age in babies with a cleft palate to help prevent ear infections.
• Repair of cleft palate. Done between nine and eighteen months of age.
• Orthodontic treatment. Teeth straightening usually begins when the child is around seven years of age and may continue through age eighteen.
• Cosmetic surgery to improve facial appearance. May be done when the child is fifteen through eighteen years of age.

Speech therapy is included in the child's educational program as needed.

Many children with cleft lip or palate also need some form of counseling or psychotherapy, particularly when they reach adolescence. Although preschoolers with a cleft lip or palate do not seem to feel worse about themselves than other children, by the early school years many children begin to withdraw socially or become fearful of being teased or rejected by classmates. In the teen years, many youngsters with speech problems or facial abnormalities related to cleft lip or palate become depressed because their peers usually rate appearance more highly than such personal qualities as humor or intelligence. Strong parental support can make an important difference in a child's ability to cope with social difficulties, as can counseling by a therapist.

Prognosis

Cleft lip and cleft palate are very treatable conditions. Most of the physical problems associated with cleft lip and palate, such as crooked teeth, speech difficulties, and an increased risk of ear infections can be treated surgically with a high degree of success. The psychological and social

difficulties of children with these birth defects, however, are less easy to resolve.

Prevention

Some causes of cleft lip and cleft palate cannot be entirely eliminated. Mothers can, however, lower the risk of having a child with these birth defects by quitting smoking, avoiding the use of alcohol and illegal drugs during pregnancy, and taking vitamin supplements during pregnancy.

The Future

One exciting new development in the field of cleft lip and palate repair is the possibility of operating on the baby before it is born. Advances in surgery that would allow correction of these facial and oral defects before birth would have many advantages. One benefit is that there would be less scarring; surgery performed on a fetus in the womb is known to leave less noticeable scars than surgery performed after birth. In addition, correction of cleft lip and palate before birth might lower the number of orthodontic and other procedures needed after birth.

SEE ALSO Clubfoot; Down syndrome; Ear infection; Edwards syndrome; Patau syndrome

WORDS TO KNOW

Congenital : Present at birth.

Hard palate : A thin bony plate located in the front portion of the roof of the mouth.

Prenatal : Before birth.

Soft palate : The soft tissue at the back of the roof of the mouth that does not contain bone.

For more information

BOOKS

Berkowitz, Samuel. The Cleft Palate Story. Thorofare, NJ: SLACK Incorporated,2006.

Cleft Palate Foundation. Cleft Lip and Palate: The Adult Patient. Chapel Hill,NC: The Foundation, 2000.

Judd, Sandra J., ed. Congenital Disorders Sourcebook: Basic Consumer Health Information. 2nd ed. Detroit, MI: Omnigraphics, 2007.

WEB SITES

American Academy of Family Physicians (AAFP). Cleft Lip and Cleft Palate. Available online at http://familydoctor.org/online/famdocen/home/children/parents/special/birth/034.html (updated July 2006; accessed June 18, 2008).

American Society of Plastic Surgeons. Cleft Lip or Palate. Available online at http://www.plasticsurgery.org/patients_consumers/procedures/CleftLipPalate.cfm?CFID=100476922&CFTOKEN=62793816 (updated 2008; accessed June 18, 2008). Includes diagrams of surgical repairs of cleft lip and palate.

March of Dimes. Cleft Lip and Cleft Palate. Available online at http://www.marchofdimes.com/professionals/14332_1210.asp (updated February 2007; accessed June 18, 2008).

Mayo Clinic. Cleft Lip and Cleft Palate. Available online at http://www.mayoclinic.com/health/cleft-palate/DS00738 (updated April 19, 2008; accessed June 17, 2008).

Nemours Foundation. Cleft Lip and Palate. Available online at http://kidshealth.org/parent/medical/ears/cleft_lip_palate.html (updated September 2005; accessed June 18, 2008).