Cleft Lip and Palate:

Where to Get Treatment:

There are well over 100 cleft lip and palate teams in North America, as listed by the American Cleft Palate - Craniofacial Association (www.acpa-cpf.org). In most urban areas, families will have more than one team available to them. One good way to begin is to check for teams in your area, and plan on getting at least two opinions. Not only are there many different possible surgical repairs that may differ from doctor to doctor, but physicians differ on communication skills and treatment styles. Visiting more than one team will help families chose a team that is the “right fit” for them.

Treatment Basics:

The treatment that your child will need depends upon whether or not your child has an isolated cleft lip, an isolated cleft palate, or both. There are a number of different ways to surgically repair a cleft, and surgeons will vary as to which technique they prefer, as well as, the timing for the repair. This site will try to cover some of the basic treatments and controversies. This review is intended to help you to formulate questions for your doctor.

  1. Feeding. Children born with a cleft palate (with or without a cleft lip) cannot create suction and will have difficulty getting enough breast milk (or formula). This problem with feeding comes from the cleft of the palate, which allows air to come through the nose when the baby tries to suck. So, instead of milk coming into the mouth, the baby gets a lot of air. If parents try to feed their baby (born with a cleft palate) by breast feeding, or using a standard nipple and bottle, they will find that after about 30 minutes their baby will get tired of trying to get enough milk and will fall asleep. Then, 60-90 minutes later, their baby will wake up again crying and hungry. The result of this cycle is that the parents become exhausted, and the baby does not gain weight. Therefore, right after a baby is born with a cleft palate, a feeding specialist (typically a speech pathologist, or occupational therapist) is called in to help. There are two basic things that can be done to help a baby feed: the first is to enlarge the hole in the nipple, so that by using the tongue, the baby can control the amount of milk (breast or formula) coming from a bottle by gravity. The second is to use a squeeze bottle. Often, combinations of these two things are used in order to allow the baby to get enough milk to grow.
  2. Hearing. Children born with a cleft palate are more likely to develop fluid behind their eardrums (serous otitis), which may progress to ear infections. Often, babies will need tubes put in their ears to improve hearing (children who do not hear well develop speech delays). Cleft lip and palate teams should try to coordinate the placement of tubes, when needed, with the lip or palate repair in order to reduce the total number of operations a child will receive.
  3. Lip Repair. Some surgeons may recommend taping the lip together, in an attempt to narrow the width of the cleft before surgically closing it. Others believe that this taping has only a minimal effect, and is not worth the fuss. The timing for the lip repair is somewhat controversial. The traditional rule has been that the baby should be ten weeks old, and weigh at least ten pounds. Some surgeons recommend repairing a lip as early as one week of age, but most prefer repairing the lip at 12 weeks of age. One advantage of delaying the repair is that there is no evidence that the scar looks any better if repaired earlier, and anesthesia may be slightly safer in older, larger babies. However, there are many arguments pro and con performing an earlier repair.

    Some doctors will also recommend a "lip adhesion" instead of a repair. A lip adhesion basically involves sewing the edges of the cleft together at about 6-weeks of age, and then going back about 6 weeks later to perform the actual repair. The lip adhesion has the benefit of reducing the tension on the final repair, and potentially making the scar narrower. The downside of the lip adhesion is that it usually adds another operation. I do not recommend lip adhesions for my patients, because it does add an additional operation. Moreover, if the lip scar does end up being slightly wider (because of a very wide cleft), it can be improved at one of the other required operations (like the palate repair, or subsequent nose surgery, etc.).

    The actual surgical technique used to correct a cleft lip is fairly standard; most surgeons perform what is called a "rotation advancement" repair. This repair recruits skin and tissue from the sides, in order to vertically lengthen the lip, while bringing the edges together. There are now three ways of closing the skin at the end of the lip repair: regular stitches, dissolving stitches and tissue glue. Regular stitches usually do not leave a wider scar, but they must be taken out 3 to 5 days after being placed, in order to prevent bad scarring (most surgeons will take the baby back to the operating room in order to remove the stitches under anesthesia). Dissolving stitches have the advantage of not needing to be removed; but they do leave a slightly wider scar, so many surgeons prefer not to use them. For my patients, I prefer to use a "sutureless" skin repair. Actually, the cleft is repaired using multiple deep layers (below the surface of the skin) of dissolving sutures; the skin is then closed by temporarily using stitches until a, recently developed, glue for skin is applied (see photo sequence). In my opinion, this technique produces the best looking cleft lip scars of all the available techniques for closure. Tissue glue also eliminates the need for a second anesthetic to take out stitches (or, holding the baby down in order to get the stitches out).



    (Above figures) A Cleft lip is brought together with a "rotation - advancement" repair. Sutures are then placed temporarily, tissue glue is applied, and the sutures are immediately removed. The appearance of the lip can be seen in the last picture (below right), two years later, after this one operation. This is only one example of a repair; scars will vary according to the width of the cleft and a child's genes for scarring.
  4. Palate repair. Clefts of the palate (roof of the mouth) can either be one sided, or bilateral (similar to clefts of the lip); and they may be either incomplete, or complete. An incomplete cleft usually just involves the back half of the palate. Complete clefts go from the back of the palate all the way forward through the gum line. A bilateral cleft means that the cleft involves both sides of the floor of the nose. Typically, if a baby has a cleft lip, the cleft of the palate will be unilateral and complete. Babies who are born with an incomplete cleft (which involves only the back half of the palate - the soft palate), and a small lower jaw, should see a geneticist to rule out other syndromes such as Pierre Robin Sequence, or Velocardiofacial syndrome (Pierre Robin Sequence with heart and other problems), among other potential syndromes. Adding to the confusion of cleft palates is the term submucous cleft. This is a cleft that cannot be seen from looking at the roof of the mouth. It involves the muscles of the palate underneath the mucous membranes of the roof of the mouth. Submucous clefts do not always need treatment but children need to be followed closely by a cleft lip and palate team speech pathologist until about 2nd or 3rd grade.

    The treatment of the palate may begin with a manipulation of the palate called "pre-surgical orthopedics." Some teams believe that better results are achieved by placing pins in the palate and literally cranking the split ends of the palate closer together. Other teams like to make splints for the roof of the mouth, which are constantly remolded, as the baby grows, to achieve the same thing more passively. Finally, some teams believe that these treatments are too much of an ordeal for the patient and family, and that the final results are just about as good without any of this preliminary treatment.

    The actual timing of the repair of the palate may vary from team to team. Some teams will recommend repairing the soft palate at the same time as the lip repair (about 12 weeks of age), and then will repair the hard palate between 9 and 18 months. Other teams prefer to repair the entire palate at the same time. Arguing in favor of the earlier repairs is that the sooner the palate is repaired, the better the child's eventual speech will be. However, the later the palate is repaired, the better the upper jaw grows. It is my opinion that a good balance between these two factors is to repair the entire palate at about 12 months of age, unless the child has an incomplete cleft palate, in which case the cleft is repaired around 6 months of age.

    There are also different surgical ways of closing the cleft of the palate, and there are many studies that attempt to investigate which technique produces the best speech, with the lowest complication rates. Unfortunately, there is no unanimous data pointing to one best repair, and many surgeons believe strongly that the repair they use is better than all the others. I suspect that, more important than which specific repair is used, is the experience that a surgeon has with a particular repair. Even though we cannot see the palatal repair, the repair of the palate may be more important than the lip. It takes a good palate repair in order to produce normal speech; and normal speech is essential, because many people judge others by the quality of their speech.

    It is important that any child, who has undergone a cleft palate repair, be closely followed by a trained speech pathologist, familiar with evaluating children with a cleft lip and palate. Somewhere around one out of every five children might require a secondary operation to improve speech. Once it has been determined that speech therapy cannot provide any further benefit, the sooner this secondary operation is performed, the better (so that a child does not have to unlearn how to speak, in order to speak better). There are a number of different operations that might be recommended. One is to re-cut the palate in order to try to lengthen it. Another is a called a spincteroplasty, in which muscles are cut from the sides of the throat and brought together at the back of the throat. The third option is called a posterior pharyngeal flap. In this operation, a flap of tissue is cut from the back of the throat and placed into the back of the palate. These various options, the pros and cons of each, are best discussed with your surgeon, who will recommend the one that he, or she, feels most comfortable with.

  5. Other Operations. For children born with a cleft of the lip and palate, there are additional operations that may be required. Most children with a cleft lip, even if the palate is not affected, require one or more operations to normalize the appearance of the nose. The first of these is often performed at the same time as the lip repair. Subsequent operations are typically scheduled before the child enters mainstream school, and during teenage years.

    For any child with a cleft that extends through the gum line (as with a complete cleft palate), bone must be placed into this cleft in order for the upper canine tooth to erupt. This operation is typically performed before, or around, 10-years of age. This bone usually comes from the hip, but some surgeons will use the skull as a donor site. It is not possible for parents to donate bone to their children. There is some evidence that if the cleft of the gums is repaired early in life, that the child will fill in bone on his or her own and not require a bone graft. Unfortunately, even if the cleft is repaired early, most children will not fill this area in, and will still require a bone graft later in life. Moreover, some surgeons worry that operating in this area early in life, may inhibit later growth (although there is not any solid evidence to show this). There is also some recent experimental evidence showing that it will be possible to place certain growth factors into the cleft and grow bone without the need to harvest it from somewhere else; although, this technique is not quite ready for routine use in children.

    One of the last operations a child might need is called a LeFort I. This operation brings the upper jaw further forward. Many children, especially those born with a bilateral cleft lip, will show deficient growth of the upper jaw (it does not grow far enough forward). In this operation, the jaw is cut, just above the teeth, from a cut made inside the mouth. In some severe cases, in which the jaw needs to be brought forward more than about half an inch, an external halo (sometimes referred to as a RED), or an internal distraction device might be used in order to help bring the upper jaw forward. The LeFort I is typically performed when growth is completed (15-years old in girls, and 16 to 17-years old in boys). This operation not only helps the teeth to fit together better, but it also may provide a significant improvement in the child's overall appearance (provided that the lower jaw is not simultaneously set back, which is something that I never recommend for my patients).

It is important to discuss the specifics of any proposed operation with your craniofacial surgeon, and ask any questions that come to mind, in order to make sure that you understand what is being done and why.

Jeffrey Fearon, MD Director, The Craniofacial Center Dallas, Texas U.S.A. 972-566-6464 cranio700@gmail.com

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