Cleft Lip and Palate

Cleft lip and palate deformities originate in the second month of gestation when the front and sides of the face and the palatine shelves fuse imperfectly. They fall into four categories: clefts of the lip (unilateral or bilateral); clefts of the palate (along the midline); unilateral clefts of the lip, alveolus (gum pad), and palate (twice as common on the left side as the right); and bilateral clefts of the lip, alveolus, and palate. Another cleft disorder, Pierre Robin syndrome, occurs when micrognathia and glossoptosis coexist with cleft palate.

Cultural Tip Cleft lip and cleft palate are most common in Asians and Native Americans and least common in blacks. Cleft lip with or without cleft palate is more common in males. Cleft palate alone is more common in females.

Because the palate is essential to speech, structural changes - even in a repaired cleft - can permanently affect speech patterns. Children with cleft palates commonly experience hearing difficulties because of middle ear damage or infection. Early treatment preserves speech and language formation and swallowing.

Causes

Researchers believe that most cases of cleft lip and cleft palate are caused by a unique interaction of genetic and environmental factors, although a definite cause may never be discovered for every baby.

• Genetic factors. Either the mother or father can pass on genes that cause clefting, either as an isolated defect or as part of a syndrome that includes clefting as one of its signs. In some cases, babies inherit a gene that makes them more likely to develop a cleft and then an environmental trigger causes the cleft to occur.
• Environmental factors. Fetal exposure to cigarette smoke, alcohol, certain medications, illicit drugs and certain viruses have been linked to the development of a cleft. Researchers believe that some clefts may be caused by nutritional deficiencies in the mother, particularly a lack of folic acid.

Signs and Symptoms

Inspection findings range from a simple notch to a complete cleft that extends from the lip through the floor of the nostril on either side of the midline. A cleft palate may be partial or complete; if complete, inspection may show involvement of the soft palate, the bones of the maxilla, and the alveolus on one or both sides of the premaxilla.

In a double cleft, the most severe of all cleft deformities, inspection may disclose a cleft that runs from the soft palate forward to either side of the nose, separating the maxilla and the premaxilla into free-moving segments. The tongue and other muscles can displace these segments, enlarging the cleft.

Diagnostic tests

No specific tests exist for cleft lip and palate.

Treatment

Cleft deformities must be treated with a combination of speech therapy and surgery. The timing of surgery varies. Some plastic surgeons repair cleft lips within a few days of life to make feeding the baby easier. Many surgeons delay lip repairs for 8 to 10 weeks (sometimes as long as 6 to 8 months) to allow time for maternal bonding and, most important, to rule to associated congenital anomalies.

Cleft palate repair is usually completed by the 12th to 18th month. Some surgeons repair cleft palates in two steps, repairing the soft palate between ages 6 and 1 8 months and the hard palate as late as age 5 years. In any case, surgery is performed only after the infant is gaining weight and is infection-free.

Surgery must be coupled with speech therapy. Because the palate is essential to speech formation, structural changes, even in a repaired cleft, can permanently affect speech patterns. To compound the problem, children with cleft palates often have hearing difficulties because of middle ear damage or infections.

Prevention

• Genetic counseling. Your doctor will advise you on what genetic testing can tell you, such as how much a history of clefting in both your families increases your risk, as well as how blood or DNA samples can test for chromosomal syndromes.
• Prenatal vitamins. Some researchers believe that lack of folic acid or certain vitamins may be related to some cases of cleft lip and cleft palate. If you're planning to get pregnant soon, begin taking a prenatal multivitamin now.