Cleft Lip and Palate

Cleft lip and palate can be quite disfiguring, in addition to creating a number of potentially serious difficulties with feeding, hearing and speech. Fortunately, however, cleft lip and palate can be surgically repaired with excellent results, although the process is sometimes lengthy and complicated. Sophisticated therapeutic techniques also can help an affected child learn to eat and speak normally.

Most children's hospitals use a team approach in treating cleft lip and palate—that is, a primary pediatrician coordinates the work of several different specialists, including surgeons, dentists, speech and hearing therapists and mental health professionals.

HOW DO CLEFT LIP AND PALATE DEVELOP?
About the fourth week of gestation, the tissues of the face begin to form. During this time, the primary palate, which eventually forms the upper lip and gums, and the secondary palate, which eventually forms the roof and back of the mouth, start to grow.

During the fifth and sixth weeks of gestation, clefts are present in the primary palate on each side of the upper lip; these clefts run into the openings that will become the nostrils. The clefts normally fuse by the seventh week, creating the facial area between the nostrils and mouth. A cleft lip results when these clefts in the primary palate fail to fuse.

The cleft in the secondary palate, running from the back of the mouth to the bottom of the uvula, which is the rounded pieced of tissue that hangs down at the back of the palate, normally fuses between the tenth and twelfth weeks of fetal development. A cleft palate results when the secondary palate does not fuse.

Cleft lips and palates vary greatly in size, shape, and degree. A cleft lip may be confined to the upper lip, extend to the nostrils, or be complicated by defects in the palate. Cleft palates may involve both the hard and soft palates, the soft palate alone, or just the uvula.

WHAT CAUSES CLEFT LIP AND PALATE?
In many cases, a tendency to develop a cleft lip or palate in inherited. For the malformation to occur, however, it is believed that unidentified environmental triggers must also be present. The genetic influence seems to operate more strongly in producing clefts of the lip than of the palate. Despite the evidence of genetic influence, though, eight out of ten cases of cleft lip and palate occur in children who do not have relatives with similar malformations.

In a small percentage of cases, clefts appear with other defects, all of which can be traced to a single gene. A hereditary disorder of this sort is referred to as a cleft syndrome; more than 300 such syndromes have been identified.

WHAT TREATMENTS ARE AVAILABLE?
The ultimate treatment goal is to close lip and palate clefts through surgery, leaving as little scarring as possible. Several separate operations performed at different stages of development are often needed to meet this goal. Surgical repairs of the lip and nose are generally scheduled during the first two to ten weeks of life. A subsequent operation is sometimes necessary at four to five years to correct the shape of the nose and lip for medical as well as cosmetic reasons.

Repair of the palate is usually performed between six months and two years of age. The goal is to fuse the cleaved segments to allow normal development of the upper jaw, as well as to promote normal speech and breathing.

CARING FOR A CHILD WITH CLEFT LIP AND PALATE
The day-to-day care of a child with a cleft lip and/or palate poses numerous challenges. They include:

• Feeding Problems.
The opening in the baby's mouth or lip prevents normal sucking. Specially designed nipples permit infants with cleft lip and palate to get adequate nutrition and develop the jaw muscles used in sucking. Custom-made prosthetic palates can also help babies nurse and take bottles until surgical repair is possible. Even with the help of these devices, though, feedings may take longer and require more patience on the part of the parent.

• Ear Infections.
Children with cleft palates are particularly susceptible to infections of the middle ear. Repeated ear infections at an early age can lead to hearing loss, so careful monitoring and prompt treatment are imperative. During regularly scheduled checkups, the pediatrician will look for developing infections and prescribe antibiotic treatment at the first sign of trouble.

• Abnormal Tooth Development.
A cleft involving the gums usually affects the growth and development of adult teeth. Some permanent teeth may be positioned incorrectly or simply fail to grow. Therefore, dental specialists are involved in caring for children with cleft lip and palate both before and after surgical correction. In many cases, dental plates are needed to replace missing teeth during childhood, and oral surgery and orthodontia are often necessary to reposition misaligned teeth.

• Speech And Language Difficulties.
In normal speech, muscles in the soft palate allow the speaker to direct the passage of air through the mouth or the nasal cavity. A child with a cleft palate may be unable to close off the nasal cavity completely. When the child makes vowel sounds, air escapes through the nose, giving speech a nasal sound. The same loss of air can weaken a variety of consonant sounds.

Speech therapy can effectively counter these difficulties. The child may also be fitted with a prosthetic palate that will simplify word formation.