Oral-facial clefts are birth
defects in which the tissues of the mouth or lip don't form properly during
fetal development. In the United States, clefts occur in 1 in 700 to 1,000
births, making it the fourth most common major birth defect. Clefts occur more
often in children of Asian, Latino, or Native American descent.
The good news is that both cleft
lip and cleft palate are treatable birth defects. Most children born with
either or both of these conditions can have reconstructive surgery while
they're still infants to correct the defect and significantly improve facial
appearance.
An immediate problem after birth,
however, is feeding, but special nipples and even prostheses are
available to ensure that children with oral clefting receive adequate nutrition
until surgical treatment is provided.
For many parents, what's most
difficult is handling the fact that their child is different. Many parents feel
unreasonable guilt that somehow they caused the problem, as well as worry that
their child will be teased because of his appearance or because he has trouble
speaking. If your child has oral clefting, it's important that you inform
yourself about the defect and seek support from both the medical community and
other parents who have been through this experience. Support can help you focus
on your child's potential, rather than on the cleft.
The fact is, cleft treatments
available today are excellent. And for those with more pronounced physical
differences, plastic surgery can significantly improve specific problems that
may be a concern for you and your child.
Oral clefting occurs when the
tissues of the lip and/or palate of a fetus don't grow together early in
pregnancy. Children with clefts often don't have enough tissue in their mouths,
and the tissue they do have isn't fused together properly to form the roof of
their mouths.
A cleft lip appears as a narrow
opening or gap in the skin of the upper lip, all the way to the base of the
nose. A cleft palate is an opening between the roof of the mouth and the nasal
cavity. The front part of the palate is bony and is called the anterior or
hard palate, and the rear or posterior palate is called the soft palate.
Some children have clefts that extend through both the hard and soft palates,
whereas others have only partial clefting.
No one knows exactly what causes
clefting, although it's believed to be a combination of genetic and
environmental factors. "Everybody has some genetic possibility of having a
cleft," says Louis Bartoshesky, MD, a clinical geneticist for Christiana
Care Health System in Newark, Delaware. Dr. Bartoshesky is part of a cleft
palate team that specializes in treating children born with the condition.
The risk may be higher for
children whose sibling or parents have a cleft or who have a history of
clefting in their families.
Clefts can be broken down into
different categories:
In addition, clefts can be unilateral
(occurring on one side of the mouth) or bilateral (occurring on both
sides of the mouth).
Twice as many boys as girls have a
cleft lip, both with and without a cleft palate. However, twice as many girls
as boys have cleft palate without a cleft lip.
Because clefting causes specific
physical manifestations, it's easy to diagnose. The increasing use of prenatal
ultrasound means that some parents know before a child is born that a cleft
exists. If the clefting has not been detected in an ultrasound prior to the
baby's birth, it's identified immediately afterward.
Complications related to cleft lip
and palate include increased susceptibility to colds, hearing loss,
speech defects, a larger than average number of dental cavities, and missing,
extra, malformed, or displaced teeth.
Many children with clefts are
especially vulnerable to otitis media because their Eustachian tubes
don't drain fluid properly from the middle ear into the throat. Fluid accumulates,
pressure builds in the ears, and infection may set in. For this reason, many
children with clefts have myringotomy tubes surgically inserted into
their ears at the time of their first reconstructive surgery.
Another complication for infants
with clefts is feeding. A cleft lip can make it more difficult for your child
to suck on a nipple. In addition, a cleft palate may cause formula or
breastmilk to be accidentally taken up into the nasal cavity. Special nipples
and other devices can help make feeding easier; you will probably receive some
instructions in how to use them and where to buy them before you take your baby
home from the hospital. And in some cases, until they have reconstructive
surgery, children with cleft palates may need to wear a prosthetic palate
called an obturator to help them eat properly.
Many parents find that it takes
much longer to feed their baby, and the anxiety this creates can make feeding
difficult for both parents and baby. If you're experiencing problems, call the
coordinator of your cleft palate team - he or she can offer specific
suggestions to help you and your baby.
The good news is, there have been
many medical advancements in the treatment of children with oral clefting.
Reconstructive surgery can repair cleft lips and palates, and in severe cases,
plastic surgery can address specific appearance-related concerns for you and
your child.
Children with the condition
(particularly those who have cleft palate) will need to see a variety of
specialists who will work together as a team to treat your child's cleft.
Treatment usually begins in infancy and often continues through early
adulthood, and although different experts will focus on your child's different
needs at different stages, the entire team of experts will follow your child
through multiple surgeries and growth and development.
Members of a cleft lip and palate
team usually include:
The team specialists will evaluate
your child's progress regularly, and the entire team will typically meet
together annually to examine your child and discuss his case. In doing so,
"we try and look medically at the child as a whole child," Dr.
Bartoshesky explains. This means examining your child's hearing, speech,
nutrition, teeth, and emotional state.
During the team visit, each member
will evaluate your child. The entire team then meets to discuss your child's
treatment, sharing their findings and making recommendations. Copies of the
progress notes and recommendations are then forwarded to you, your child's
primary care provider, and perhaps to your child's school (especially if your
child is receiving speech therapy in school).
Dr. Bartoshesky and Phyllis
Thomas, RN, MS, the cleft palate program coordinator for Delaware, have these
additional tips for parents whose child is working with a cleft team:
In addition to treating your
child's cleft, the cleft palate team monitors feeding problems, reactions to
your child's appearance, how you encourage your child to talk, and your
attitude about your child's future. They'll provide feedback and
recommendations to help you through the phases of your child's growth and
treatment.
Surgery is usually performed
during the first year of life to repair both a cleft lip and cleft palate. Both
types of surgery are performed in the hospital under general anesthesia.
Cleft lip often requires only one
reconstructive surgery, especially if the cleft is unilateral. The surgeon will
make an incision on each side of the cleft from the lip to the nostril. He or
she will then draw the two sides of the cleft together and suture them
together.
Bilateral cleft lips may be
repaired in two surgeries, about a month apart. The first surgery is performed
when a baby is between 6 and 10 weeks old and usually requires a one-night stay
in the hospital.
Cleft palate can require multiple
surgical procedures during the course of your child's first 18 years. The first
surgery to repair the palate usually takes place when the baby is between 6 and
12 months old. This surgery involves drawing tissue from either side of the
mouth to rebuild the palate and requires two or three nights in the hospital,
the first night in the intensive care unit. The initial surgery is
intended to create a functional palate, reduce the chances that fluid will
develop in the middle ears, and aid in the proper development of your child's
teeth and facial bones. In addition, this functional palate will help your
child's speech development and feeding abilities.
In both types of surgery, the
necessity for more operations depends on the skill of the surgeon as well as
the severity of the cleft, its shape, and the thickness of available tissue
that can be used to create the palate. About 20% of children with a cleft
palate require further surgeries to help improve their speech. Additional
surgeries may also improve the appearance of the lip and nose, close openings
between the mouth and nose, help breathing, and stabilize and realign the jaw.
Not all children need additional
surgery, though; you should meet regularly with your child's plastic surgeon to
determine what's most appropriate in your child's case. Subsequent surgeries
are usually scheduled at least 6 months apart to allow a child time to heal and
to reduce the chances of serious scarring.
Final repairs of the scars left by
the initial surgery will probably not be performed until adolescence,
when facial structure is more fully developed.
Children with clefts usually have
extensive surgery in their mouths and often undergo dental and orthodontic
treatment. Routine dental care may get lost in the midst of these major
procedures, but healthy teeth are critical for a child with clefting because
they're needed for proper speech.
Children with oral clefting need
the same dental care as other children - regular brushing supplemented with
flossing once the child's 6-year molars come in. Depending on the shape of your
child's mouth and teeth, your child's dentist may recommend a toothette rather
than a toothbrush for cleaning her teeth. A toothette is a soft,
mouthwash-containing sponge on a handle that's used to swab teeth. As your
child grows, you can switch to a soft children's toothbrush. The key is to make
sure that your child brushes regularly and well.
Children with cleft palate often
have an alveolar ridge defect. The alveolus is the bony upper gum
that contains teeth, and defects can:
These problems can be fixed by
grafting bone matter onto the alveolus, which allows the placement of your
child's teeth to be corrected orthodontically.
Orthodontic treatment usually
involves two phases, with the first phase beginning around age 7 or 8. The
first phase is intended to round out the upper dental arch and to increase the
width of the upper jaw - it's called an orthopalatal expansion and
involves the placement of a device called an expander inside the child's mouth.
The widening of the jaw is followed by a bone graft in the alveolus. After
that, your child's orthodontist will wait until the remainder of your child's
permanent teeth come in before beginning the second phase.
The second phase consists of the
removal of extra teeth, the insertion of dental implants if teeth are missing,
and the straightening of teeth using braces. About 25% of children with a
unilateral cleft lip and palate will require orthognathic surgery to
advance upper jaw development and align the teeth. The reason for this is that
upper jaw growth in some children who have clefts does not keep up with lower
jaw growth. For these children, phase-two orthodontics may include an operation
called an osteotomy on the upper jaw that moves the upper jaw both
forward and down. This usually requires another bone graft for stability.
Children with clefts may have
trouble speaking - their voices are often nasal and don't carry very well. They
may also be difficult to understand. Not all children have this problem, and
some will find that surgery fixes the problem completely.
However, according to Dr.
Bartoshesky, "early identification of speech problems is key in solving
them." He recommends that a speech therapist see a child between the ages
of 18 months and 2 years. And many speech therapists like to talk with parents
at least once during the first 6 months of the child's life to give them an
overview of the treatment and suggest specific language- and speech-stimulation
games to play with their child.
Shortly after the initial surgery
is completed, the speech pathologist will see your child for a complete
assessment. The therapist will look at your child's communication development,
including pre-speech and pre-language development, the number of sounds he's
making, his interaction and play behavior, and the actual words that
he's trying to use.
This analysis helps determine what,
if any, speech exercises your child needs and if he needs further surgery. The
speech pathologist will often continue to work with your child as he has
additional surgery. Many children who have clefts continue to work with a
speech therapist throughout their grade-school years.
Children with oral clefting have a
visible facial difference, which can lead to low self-esteem and painful teasing
from other children. You can help your child by:
When your child is young, you
might also consider encouraging your child to present information to his class
during show-and-tell or a special presentation that you arrange with his
teacher. Or perhaps your child would like you to come in with him and talk to
the class. This can be especially effective with young children.
If your child does experience
teasing, listen to him. Talk with your child about why he's being teased and
how it makes him feel. Give your child the tools to confront his teasers by
asking your child what he'd like to say and then practicing those statements
with him.
If your child seems to have
ongoing self-esteem problems, consult with a child psychologist or
social worker for support and information. Together with the members of your
child's team, you can help your child through his treatment.
Also, it's important to keep the
lines of communication with your teen open so that you can address any concerns
he may have about his appearance. Most children with clefts, if they want or
need it, are candidates for plastic surgery to revise the appearance of their
nose and lips around ages 14 to 18.
Our society often focuses on
people's appearances, and this can make the teen years especially difficult for
someone with a physical difference. Let your child know that you value him for
who he is - and be a supportive listener when he faces difficulties with his
peers.
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Northeast Indiana Pediatric Specialists, PC |
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Dr. Michael Dick & Dr. Todd Dillon nips@med-web.com |