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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 ().
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.
- 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.
- 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.
- 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.
- 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.
- 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
What causes a cleft?
Diagnosing clefts
<<More about clefts
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