Cleft-Palate/Lip Surgery

THE CASE

"Nguyen Thi Bich, age 6, rarely smiled.  Conscious of her cleft lip and of the taunts that often accompanied her, she retreated to her house to escape the reality which awaits outside her door. Her parents worried about her future, a time when they would no longer be able to protect her. At a young age, Bich found it difficult to smile at the world. Now, thanks to the corrective surgery sponsored by SAP-VN, she smiles a lot more these days."

HOW YOU CAN HELP

Since the creation of the "A Smile for a Child" program in 1993, SAP-VN has financed more than 1,000 cleft-palate/lip surgeries throughout Vietnam. You can sponsor a child by donating $120. This is the total cost to provide corrective surgery and medical follow-up. It is an insignificant amount compared to the smile it will induce in a child such as Bich. Please join us and give "A Smile for A Child." If you have question(s) or would like to obtain further information about this program please email us at
info@sap-vn.org

CLEFT-PALATE/LIP SURGERY PROGRAM has sponsored 1,143 children since 1993.

Cleft Lip and Cleft Palate FAQ

What are cleft lip and cleft palate?

A cleft is a separation in a body structure. Clefts that occur in the oral-facial region often involve the lip, the roof of the mouth (hard palate) or the soft tissue in the back of the mouth (soft palate). Two major types of oral-facial clefts are cleft lip/palate and isolated cleft palate. Babies with cleft lip/palate have a cleft lip which usually is accompanied by cleft palate. In isolated cleft palate, the cleft palate occurs by itself, without cleft lip or other malformations. These two forms of oral-facial clefts are considered separate birth defects. Here we refer to each specific type of cleft by name, and use the term oral-facial cleft when the information applies to both.

There also are about 400 syndromes in which babies have some form of oral-facial cleft along with a wide variety of other birth defects. The diverse problems of these babies are not described here.

When do oral-facial clefts develop and how common are they?

These separations normally are present in early fetal development. The lip usually closes by 5 to 6 weeks after conception, and the palate by 10 weeks. The lip or the lip and palate together fail to close in approximately 1 in every 1,000 babies born. Cleft lip/palate occurs more often among Asians (about 1.7 per 1,000 births). Males are affected more frequently than females.

Cleft palate occurs alone less often, appearing in approximately 1 in 2,000 babies. Unlike the risk for cleft lip/palate, the risk for isolated cleft palate appears to be similar across all racial groups. Another difference from cleft lip/palate is that females are affected more often than males.

What Causes Cleft Lip/Palate?

The causes of cleft lip/palate are not well understood. Studies suggest that a number of genes, as well as environmental factors, such as drugs (including several different antiseizure drugs), infections, maternal illnesses, maternal smoking and alcohol use and, possibly, deficiency of the B vitamin folic acid may be involved.

Cleft lip/palate may occur alone or with other abnormalities that may be hidden or obvious. Up to 13 percent of babies with cleft lip/palate have other birth defects. Some cases involve genetic syndromes which may pose specific problems for the baby, and may have a high risk of affecting others in the family.

Are the Causes of Isolated Cleft Palate Different?

As with cleft lip/palate, the causes of isolated cleft palate are not well understood. A number of genes as well as environmental factors also may play a role. Antiseizure drugs do not appear to contribute to cleft palate, although maternal smoking and alcohol use and deficiency of folic acid may do so.

Cleft palate appears to be associated with genetic syndromes more frequently than with cleft lip/palate. Babies with what appears to be isolated cleft palate are more likely than babies with cleft lip/palate to have other birth defects, which shows the importance of a thorough examination soon after birth. Studies suggest that up to 50 percent of babies with isolated cleft palate may have other birth defects.

Are Oral-Facial Clefts More Common in Some Families?

If parents without either form of oral-facial clefts have a child with a cleft (and known causes, like syndromes, have been ruled out), the chance that a subsequent baby will have a cleft is 2 to 8 percent. These risk figures are the same for cleft lip/palate and isolated cleft palate. However, a second baby is at risk for only the same type of cleft that affected his or her sibling. If either parent has an oral-facial cleft, but no affected children, the risk of the same type of cleft in any pregnancy is about 4 to 6 percent. If more than one of the parents and/or children are affected, the risk for future offspring is greater.

How Do Oral-Facial Clefts Affect a Baby’s Face?

A cleft lip can range in severity from a simple notch in the upper lip to a complete opening in the lip extending into the floor of the nostril. The upper gum may be involved. Clefts can occur on one or both sides, and with or without involvement of the palate. More than 70 percent of babies with cleft lip also have cleft palate.

Cleft palate (whether alone or part of cleft lip/palate) can involve the soft palate only or extend forward through the hard palate. One or both sides of the palate can be affected.

Can Oral-Facial Clefts Be Repaired?

Surgery often is used to correct cleft lip/palate and isolated cleft palate. The timing and type of surgery depend upon a number of factors, including the preference of the surgeon, the health of the baby and the nature of the cleft. Most surgeons agree that cleft lip should, in most cases, be repaired by about three months of age. Cleft palate repair generally is timed to restore the partition between the nose and mouth as early as possible (often between 9 and 18 months). Additional surgical procedures often are needed as the child grows.

What Special Problems are Associated with Oral-Facial Clefts?

There may be problems with feeding, ear disease and speech development, as well as dental problems. They may differ between children with cleft lip/palate versus those with isolated cleft palate; the problems also will differ depending upon the specific cleft and its severity.

What About Feeding?

Babies with cleft lip usually do not have much trouble feeding, although those with cleft lip accompanied by cleft palate as well as those with isolated cleft palate sometimes do. A cleft in the roof of the mouth makes it difficult for the baby to suck forcefully enough to draw milk through a nipple. Some babies also have problems with gagging, choking, or milk coming out through the nose while feeding. To make feeding easier, some doctors recommend using a small plastic plate called an obturator that fits into the roof of the mouth and blocks the opening while feeding.

It is possible to breastfeed some infants with cleft palate, although this requires extra patience and modification of techniques. Breastfeeding is more likely to be successful in babies with less severe clefts.

What About Ear Problems?

Babies with cleft palate are especially susceptible to middle ear disease. The cleft can contribute to a buildup of fluid in the middle ear which can cause mild to moderate hearing loss. If the fluid becomes infected, the baby can develop fever and an earache.

If treated properly in infancy and childhood, the hearing loss need not be permanent. If not properly managed, speech development may be affected and hearing loss may become permanent.

How Is Speech Affected by Clefts?

Children with cleft lip generally have normal or near-normal speech. Some children with cleft palate (isolated or as part of cleft lip/palate) may develop speech a little more slowly than other children. Their words may sound nasal and they may have difficulty producing some consonant sounds. However, after cleft palate repair, most children eventually catch up and develop normal speech, although some will require speech therapy or additional surgery.

What About Dental Problems?

Children whose cleft lip/palate extends into the upper gums (which contain the teeth) have special dental problems. Some primary and permanent teeth may be missing, abnormally shaped, or out of position. Some children with isolated cleft palate also are missing teeth.

Can Oral-Facial Clefts Be Prevented?

While little is known about how to prevent oral-facial clefts, studies suggest that taking multivitamins containing folic acid before conception and during the first two months of pregnancy may help prevent cleft lip/palate and isolated cleft palate.

Other studies have shown that fetuses with certain predisposing genes may be at increased risk for isolated cleft palate if their mothers smoke.

Similarly, one study found that fetuses with a predisposing gene may be at increased risk of cleft lip/palate if their mothers drink during pregnancy. Women who are planning pregnancy or who are pregnant should avoid alcohol, which also can cause a number of mental and physical birth defects. Because some types of medications (such as some drugs used to treat epilepsy) have been linked to increased risk of cleft lip/palate, women who take medications for chronic illnesses should check with their doctors before they become pregnant. (They should not, however, discontinue their medication without discussion with their physician.) In some cases, the doctor may recommend stopping the medication or switching to a medication that is safer during pregnancy. All pregnant women should use only medications prescribed by a physician who knows of the pregnancy, and get early and regular prenatal care, beginning with a pre-pregnancy visit. Families with a history of cleft lip/palate, isolated cleft palate, or any other condition of which clefting is a part, may wish to discuss the chances of recurrence with a genetic counselor.

References

American Society of Plastic Surgeons. Cleft lip and palate surgery. Arlington Heights, IL, March 2001.

Children’s Hospital of Philadelphia. Your child’s health: cleft lip-cleft palate. Philadelphia, March 2001.

Cleft Plate Foundation. Cleft Palate Foundation publications. Chapel Hill, NC, March 2001.

Romitti, P.A., et al. Candidate genes for nonsyndromatic cleft lip and palate and maternal cigarette smoking and alcohol consumption: evaluations of genotype-environment interactions from a population-based case-control study of orofacial clefts. Teratology, volume 59, number 1, January 1999, pages 39-50.

Suzuki, K., et al. Mutations of PVRL1, encoding a cell-cell adhesion molecule/herpesvirus receptor, in cleft lip/palate-ectodermal dysplasia. Nature Genetics, volume 25, August 2000, pages 427-429.