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Cleft lip and palate surgery

By James Wright, MD, MPH, FRCSC

Cleft lip and cleft palate are among the most common birth defects. A cleft is a separation in the upper lip, the roof of the mouth, or both; approximately one baby in 700 is born with a cleft. At SickKids, our Cleft Lip and Palate program sees around 120 newborns per year and provides comprehensive follow-up care for 3500 children.

About cleft lip and palate

Early in fetal development, between the fifth and eighth weeks of pregnancy, different sections of embryonic tissue join to form parts of the face and mouth. Three of these sections connect at the philtrum, the little groove that runs from the upper lip to the nose. If for some reason two sections fail to join properly, the result is a cleft beside the philtrum, known as a cleft lip.

Slightly later in development, between the eighth and twelfth weeks of pregnancy, two other sections of tissue connect to form most of the palate, which separates the mouth from the nasal cavity. If there is a failure in this process, the result is a cleft palate.

Clefts in the lip may be small or large, ranging from a notch in the upper lip to a separation that extends all the way into the nose. Similarly, a cleft palate may be an obvious gap in the roof of the mouth or a smaller opening at the back of the soft palate. Clefts may also involve the upper jaw, gums, and teeth. Clefts may be unilateral, occurring on one side only, or bilateral, appearing on both sides.

We still do not know very much about why clefts happen. There is a genetic component: a parent who had a cleft is more likely to have a child with a cleft. Some clefts appear as part of a syndrome and are accompanied by other defects in the heart, genitourinary tract, or other parts of the body. However, most newborn babies with a cleft are otherwise normal and healthy.

Children with cleft lip and palate face a number of potential problems:

  • Babies with a cleft often have some trouble feeding. The structure of their mouth may make it difficult for them to suck, or milk or formula may come back up through the nose. An occupational therapist or other feeding specialist can help parents learn how to feed their baby effectively, either with the breast or with a specially designed bottle.
  • Children with a cleft often have trouble with the alignment of their teeth and jaws.
  • Cleft palate can cause fluid to build up in the middle ear, which in turn can cause infections or hearing loss.
  • Cleft palate can affect speech. Children with a cleft palate may have trouble producing certain sounds, or the sound quality of their voice may be affected.
  • Because they are visibly "different," children with a cleft may face stares, teasing, or social stigma, first from adults and later from other children.

Fortunately, these problems can be addressed with proper care. In nearly all cases, clefts can be successfully repaired with one or more surgical operations.

Treatment for cleft lip and palate

A newborn baby with a cleft should be assessed by a plastic surgeon within a week or two after birth. This will help the parents and the treatment team understand what needs to be done and decide on the course of treatment. The child should then be followed by a multidisciplinary care team until early adulthood, when all of the bones have finished growing.

The goals of treatment for a cleft are:

  • to improve mouth and nose function and speech
  • to improve appearance
  • to help the child develop normally, including making friends and doing as well as possible in school

The extent and timing of surgery to correct cleft lip or palate depend on the nature and extent of the cleft. A baby with a small cleft in the lip may only need one operation to repair it. By contrast, surgery for a full cleft lip and palate may include:

  • orthodontic treatment to narrow the cleft and align the gum ridges, where the teeth will eventually grow in, on either side of the cleft
  • lip repair at three to five months of age
  • palate repair at around one year of age to separate the nasal cavity from the mouth and help the child's speech
  • repairing the gum line with a bone graft taken from the child's hip when the adult teeth are coming in at about nine to 12 years of age
  • working to make the nose more symmetric when the child is a teenager
  • jaw surgery once the bones have finished growing; this is done when the upper jaw does not develop as much as the lower jaw, which can lead to an underbite and a flat or concave facial profile

The timing of these procedures will depend on the individual child's situation. Different teams may also take different approaches to treatment.

It is very important that the child is followed by an experienced, specialized multidisciplinary team until young adulthood. In addition to surgery, a child with a cleft may need treatment or support from many different health care professionals, including paediatricians, orthodontists, dentists, speech-language pathologists, audiologists, social workers, and more. A team approach helps ensure that everyone involved in the child's care is familiar with his history and can consult other team members when necessary.

Outcomes of cleft lip and palate surgery

Attempts at cleft lip repair date back to about 390 A.D. in China. Only in the last 50 years or so, however, have we made systematic attempts to assess the outcomes of surgery and find the best treatments.

For a variety of reasons, including difficulties finding a suitable control group and following up for a long enough period, studies of groups of children with clefts are rare. Outcomes are usually judged on an individual basis by the surgeon, the parents, and the child.

Although outcomes vary slightly, surgery for cleft lip and palate is usually very successful, and surgical complications are rare. At various points in the child's development, until the child has finished growing, it is important to look at his:

  • growth
  • teeth; children with a cleft may have gum problems, problems with tooth alignment, or missing, malformed, poorly positioned, or discoloured teeth, which need attention from a dentist or an orthodontist
  • speech; the child may need speech therapy after palate repair, or he may need a second operation if he is not developing normal speech
  • hearing; children with cleft palate may develop some hearing loss if they have infections of the middle ear
  • psychosocial development; some children have more trouble than others making friends or fitting in at school, regardless of how successful their surgery is

Prenatal counselling

With improvements in diagnostic imaging, many clefts are now diagnosed before birth. Obviously, the news comes as a shock to parents. However, it can also help parents become prepared and plan ahead for the baby's care, and may allow the treatment team to investigate other potential problems, which are more common in children with clefts. Studies suggest that most parents whose child was diagnosed with a cleft before birth are glad to have known ahead of time and would choose prenatal diagnosis again.

Plastic surgeons and other members of the craniofacial team offer prenatal counselling for parents of children with clefts. It is important for parents to have clear, accurate, and non-judgemental information and support to help them understand what to expect and decide how to proceed.

Fetal surgery for cleft lip and palate

Surgery for cleft lip and palate before birth has been explored. Fetal surgery can be life-saving, for example with certain heart and lung conditions. For cleft lip and palate, the main rationale for fetal surgery is that in theory it can improve the appearance of the resulting scar. However, the surgical results are not necessarily as good as they could be with the standard course of treatment, and fetal surgery can also result in spontaneous abortion. Surgeons at SickKids believe the procedure is unacceptably risky compared to the few benefits it might provide.

In conclusion

The birth of a child with a cleft is often a shock to parents. It is important that the child be assessed early and followed by a multidisciplinary treatment team. The prospects for treatment are good, and in most cases the child can go on to lead a normal, healthy, and happy life.

 

Dr. James G. Wright, MD, MPH, FRCSC, is Surgeon-in-Chief, Robert B. Salter Chair of Pediatric Surgical Research, and Senior Scientist, Population Health Sciences at The Hospital for Sick Children (SickKids). He is also a professor in the Departments of Surgery, Public Health Sciences, and Health Policy, Management, and Evaluation at the University of Toronto.

Surgeon's Corner columns by James G. Wright

1/21/2010

Johnson N, Sandy JR. Prenatal diagnosis of cleft lip and palate. Cleft Palate - Craniofacial Journal. 2003;40(2):186-189.

Strauss RP. Beyond easy answers: Prenatal diagnosis and counseling during pregnancy. Cleft Palate - Craniofacial Journal. 2002;39(2):164-168.





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