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cleft lip and palate

Alternative Names
cleft lip , cleft palate , harelip , oral cleft

Definition
Cleft lip is a birth defect that involves a split in the upper lip. Cleft palate is a birth defect that involves a split down the centre of the roof of the mouth, also known as the palate. These defects may occur by themselves, which is known as isolated cleft lip or isolated cleft palate. They may also occur together, which is called cleft lip with cleft palate.

What is going on in the body?
Cleft lip and cleft palate both develop while a baby is in the womb. Cleft lip occurs when grooves that are normally present in the face of a developing foetus don't smooth out as it grows. The baby is then born with a split or opening where the grooves were, called a cleft lip.

The roof of the mouth, on the other hand, develops from tissue on each side of the mouth. This tissue usually grows together and fuses at the middle of the roof of the mouth. If the tissue does not fuse normally, the baby is born with a split extending from front to back along the roof of the mouth. This is called a cleft palate.

What are the signs and symptoms of the condition?
Symptoms of cleft lip and cleft palate vary from person to person, depending on the extent of the defect. Cleft lip may show up only as a small notch in the border of the upper lip. It may also involve a complete split of the lip that extends into the floor of the nose. Cleft lip may involve one or both sides of the upper lip. Often, the bone that supports the upper teeth is involved to some degree. Extra, missing, or deformed teeth may also be part of cleft lip. Frequently, the outside of the nostril is somewhat flattened, too.

Cleft palate may involve only the uvula or it may involve the entire roof of the mouth. The uvula is the soft, fleshy mass that hangs down from the roof of the back of the mouth.

Cleft lip and palate may be associated with other birth defects. Cleft lip and palate are birth defects that are usually visible at birth. Some mild types of cleft palate may not be seen, but can be detected by nasal speech or other types of speech problems.

What are the causes and risks of the condition?
Cleft lip and palate are caused by environmental factors and genetics. For instance, people who have a cleft lip alone or cleft lip with cleft palate are more likely to have children with cleft lip. The actual risk depends on the severity of the split and family history. While people with cleft palate may also have affected children, the risk is not as high as it is for cleft lip.

Despite the genetic risk, most cases of cleft lip and palate occur randomly. Certain conditions during pregnancy, such as alcohol or drug use, some medications, or poor maternal instincts, are associated with higher than average risk for cleft lip and cleft palate.

Taken together, cleft lip or palate occurs in about 1 in 700 live births, with cleft lip being more common. Cleft lip is more common in boys than in girls.

What can be done to prevent the condition?
Since cleft lip and palate occur early in pregnancy, there is little that can be done to prevent it. Pregnant women may reduce the foetus' risk of developing cleft lip and palate by avoiding certain environmental factors, such as drugs used to treat seizures.

Genetic counselling may be useful for families in which there is cleft lip and palate.

How is the condition diagnosed?
Cleft lip and palate are visible at birth. They may also be detected during ultrasound, a special x-ray used to see the foetus inside the mother.

What are the long-term effects of the condition?
  • Infants with clefts may have trouble feeding.
  • Infants with cleft palate are at increased risk for chronic ear infections. This may persist even after the roof of the mouth has been repaired. Hearing loss from repeated ear infections may occur.
  • Speech defects also are common with cleft palate. These may also last after the roof of the mouth has been repaired.
  • Teeth may come in crooked or be poorly positioned.
  • After surgery to repair the cleft, cosmetic problems could persist. This could cause psychological problems, including depression and low self-esteem.
What are the risks to others?
Cleft lip and palate are not contagious. Children of affected people are at greater risk than others to inherit the genetic factors that cause cleft lip and palate. The actual size of the risk depends on the family history.

What are the treatments for the condition?
Feeding is the first priority after a baby is born. Because of the split in the roof of the mouth, it is hard for the baby to make enough suction to pull milk out of the breast. Therefore, breast-feeding is difficult. Special nipples and infant nursing bottles are useful in feeding babies with cleft lip and palate. These special nipples and bottles deliver formula or pumped breast milk to the back of the baby's mouth so it can be swallowed. Dental devices can also be used to temporarily seal the opening in the palate enough to allow sucking.

Surgical repair of cleft lip and palate are advised. Surgery is usually done in several stages and by several specialists. The first operation for cleft lip is usually done at about 2 months of age. By this time, most infants have gained enough weight to tolerate the surgery and have built up immunity against infection. The palate is usually repaired before the baby is 1 year old. This helps with normal speech development. The cosmetic outcome of the surgery depends on the extent of the defects and the skill of the surgeon.

What are the side effects of the treatments?
Regardless of how good the surgery to repair cleft lip and palate is, there may be scarring. Scars from the surgery may cause speech problems or emotional embarrassment. Side effects from the surgery and medications used during surgery are possible. All surgery carries a risk of bleeding, infections, and reactions to pain medication. If antibiotics are needed for infection, side effects, including stomach upset and allergic reaction, may occur.

What happens after treatment for the condition?
After surgery, especially in an infant, it is important that the area of the surgical repair not get infected. The baby's arms may have to be restrained with splints that do not allow the elbows to bend. This keeps the baby's hands away from his or her face. Special feedings and fluid diets may be needed for several weeks after the operation.

How is the condition monitored?
A person with cleft lip and palate should be monitored by a team of specialists. It is important for these experts to monitor this person's:
  • growth and development
  • need for and effects of surgery
  • speech development
  • alignment and condition of teeth
  • psychological adjustment
  • hearing
This person also may need help accessing community resources. People with cleft lip and palate may want genetic counselling before having children.

Author: Ronald J. Jorgenson, DDS, PhD, FACMG
Reviewer: HealthAnswers Australia Medical Review Panel
Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia
Last Updated: 1/10/2001
Contributors
Potential conflict of interest information for reviewers available on request


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