Thursday, July 25, 2013

Sumbing Lelangit (Cleft Palate)


Cleft palate is the most common birth defect that occurs. It happens in about 1 in 700 births. It is not a fatal problem. Most children born with clefts do well in developed countries. They may have difficulties with feeding initially, but with proper guidance, parents learn to feed their child with a cleft, and the child learns to compensate for the cleft during the first months after birth. Clefts are repaired within the first year of life, usually by a surgeon who has extensive experience and training with this disorder. The clefts are repaired in part to facilitate feeding, but most importantly to support normal speech. It is the problems with speech that I'd like to focus on.







Orofacial clefts are birth defects in which there is an opening in the lip and/or palate (roof of the mouth) that is caused by incomplete development during early fetal formation.


Cleft lip and cleft palate occur in about 1 or 2 of every 1,000 babies born in the United States each year, making it one of the most common major birth defects. 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. Most kids born with these can have surgery to repair these defects within the first 12-18 months of life.

About Oral Clefting
An orofacial cleft occurs when parts of the lip or palate do not completely fuse together during the first 3 months of pregnancy. A cleft lip may appear as a small notch in the edge of the lip only or extend into the nose. It may also extend into the gums.

A cleft palate may also vary in size, from a defect of the soft palate only to a complete cleft that extends through the hard palate. Because the lips and the palate develop separately, it is possible for a child to be born with a cleft lip only, cleft palate only, or both.



Most clefts can be categorized into three broad categories:

1.cleft lip without a cleft palate

2.cleft palate without a cleft lip

3.cleft lip and cleft palate together

A cleft can occur on one side of the mouth (unilateral clefting) or on both sides of the mouth (bilateral clefting).

Cleft lip with or without cleft palate is generally more common among boys; however, cleft palate occurring alone is more common in girls than boys.
For the most part, because a cleft lip is visible it is often easier to identify than a cleft palate alone. A cleft lip may be detected through prenatal ultrasound; however, diagnosing a cleft palate this way is more difficult and it might not be seen.

Even if a cleft condition is detected during pregnancy, the diagnosis and extent of cleft lip and palate is confirmed by physical examination after the birth of the child.

Causes
Sometimes a cleft occurs as part of a syndrome, meaning there are birth defects in other parts of the body, too. Other times, it's genetic and runs in families — the risk may be higher for children whose sibling(s) or parents have a cleft or who have a history of cleft in their families. In these situations, both mothers and fathers can pass on a gene or genes that can contribute to the development of cleft lip or cleft palate.

Sometimes a cleft may be associated with environmental factors such as a woman's use of certain medications, exposure to cigarette smoke, or lack of certain vitamins while pregnant. Most of the time, though, the cause isn't known.

Associated Problems
Cleft lip and palate can be associated with other problems, including feeding difficulties, middle ear fluid buildup and hearing loss, dental abnormalities, and speech difficulties.

Feeding Problems
Infants with a cleft lip alone usually have fewer problems feeding than those with a cleft palate. Feeding can be a big problem for a newborn baby with a cleft palate. Normally, the palate prevents food and liquids from entering the nose. The baby with an unrepaired cleft palate has difficulty sucking on a regular nipple and will usually require a special nipple and bottle along with proper positioning in order to feed. With these techniques, the caregiver will learn how to feed the baby before taking the baby home from the hospital. The child's doctor will carefully monitor the child's weight.

Middle Ear Fluid Buildup and Hearing Loss
Many children with cleft palate are prone to the buildup of fluid in the middle ear and/or ear infections caused by malfunction of the Eustachian tube. This fluid buildup behind the eardrum can cause hearing loss. For this reason, kids with cleft palate usually need small pressure equalization (PE) tubes placed in their eardrums to help them to drain the fluid and improve hearing. Kids with cleft palate should have their ears and hearing checked once or twice a year; more often if there is ear drainage or a child seems to be having difficulty hearing.

Dental
AbnormalitiesChildren with a cleft lip and palate frequently have dental problems. These include small teeth, missing teeth, extra teeth (called supernumerary), or malpositioned teeth. They may have a defect in the gums or alveolar ridge (the bone that supports the teeth). Defects of the alveolar ridge can displace, tip, or rotate permanent teeth, or prevent permanent teeth from coming in properly.

Speech Difficulties
Kids with cleft lip have fewer speech problems than those with cleft palate. Approximately 15%-20% with cleft palate may have speech problems after repair of the cleft palate (palatoplasty). The most common is excess nasality or hypernasality. This happens because the palate that normally separates the nose from the mouth for most sounds does not close adequately. This condition makes it sound as if the person is talking through the nose.

Children with clefts also can have other types of speech problems unrelated to the cleft condition; for example, age-related errors such as saying "wed" instead of "red." Sometimes, the dental problems associated with the cleft will cause some sounds to be distorted, particularly "s," "sh," "ch," and "j" sounds.

A speech-language pathologist will carefully assess a child's speech and language skills. If your child does have a speech problem, the pathologist will identify the cause and recommend treatment.

Treatment
The complex needs of a child with cleft lip and cleft palate are best met by an interdisciplinary team of professionals from various specialities who work together. This is a standard of care that begins soon after the child's birth and continues to adulthood.

The members of the cleft lip and palate treatment team include:
  • geneticist
  • pediatricianplastic
  • surgeonear, nose, and throat physician (otolaryngologist)
  • oral surgeon
  • orthodontist
  • dentists peech-language
  • pathologist
  • audiologist
  • nurse
  • social worker
  • psychologist
  • team coordinator

The frequency of team visits will depend on the child's needs and can range from two to three times per year to once every 2 to 3 years. Which team members the child needs to see during a given visit will depend on his or her health needs, including psychosocial issues.

After each visit, a team report will be sent to the family and other professionals involved in the child's care. The team coordinator will help organize the visits with team members and other professionals.

Surgical Treatment for Cleft Lip and Cleft Palate
A cleft lip is usually repaired between the ages of 3 to 6 months. Some children who have very wide clefts of the lip may require a procedure such as lip adhesion or a device such as a molding plate to bring the parts closer together before the full lip repair. A child with a cleft lip that is repaired will have a scar on the lip under the nose. Surgery is performed in the hospital under general anesthesia.

A cleft palate is usually repaired between 9 and 12 months of age. By repairing the palate, the soft palate muscles from each side are connected to each other and the normal barrier between the mouth and nose created. Surgery for cleft palate is performed under general anesthesia and usually requires a 2-night stay in the hospital.

The goal of surgery is to create a palate that works well for speech. Some kids, however, will continue to sound nasal after cleft palate repair and some may become nasal due to natural growth changes or adenoid shrinkage.

In some cases, additional surgery may be needed to improve speech. This surgery is called a pharyngoplasty. It is often done when kids are in their early school years, but also can be done later.

As kids grow older, they might need additional surgeries, such as an alveolar bone graft, which is used to close the gap in the bone or gums near the front teeth. This provides stability for the permanent teeth and is usually done when kids are between 6 and 10 years old.

Other procedures might be options as kids get older. They may want to have their scars made less noticeable, improve the appearance of their nose and upper lip, or improve their bite with orthognathic surgery. These operations may improve speech and breathing, dental occlusion, and appearance. Your child's surgeon will talk with you about the timing and nature of these surgeries.

Dental and Orthodontic Treatment
The primary goal of dental care in kids with cleft lip and palate is to maintain healthy teeth and prevent cavities. Because of the various types of dental problems they may have, it is very important that they see their dentist regularly and keep their teeth clean by brushing and flossing regularly.

Orthodontic treatment is common in kids with cleft lip and palate and may begin as early as 6 years of age. Often orthodontic treatment involves various phases, typically starting with palatal expansion done to normalize the width of the palate. Later, braces are put on to place the teeth in their proper position. Your orthodontist will discuss timing of the phases of treatment with you.

Some kids with a cleft might be missing a permanent tooth, which can be replaced with a removable appliance or, in early adulthood, with a dental implant.

Speech Therapy
Expect to meet with the speech-language pathologist before your child's cleft palate surgery (about 7-9 months) to review the impact of the palate on speech and what to expect in speech development after surgery. An overview of treatment and ways to stimulate speech and language development will be discussed. After the cleft palate surgery, the speech-language pathologist will continue to closely monitor your child's speech and language and recommend therapy if needed.

Some kids with cleft palate will not require speech therapy after cleft palate repair. Others will have abnormal speech and will need intervention. After cleft palate repair, about 15-20% will have hypernasal speech and may require additional surgery or other forms of management to improve speech. Although surgery might improve nasality, it may not result in immediate correction of certain speech errors and speech therapy might be needed.

Some kids may require speech therapy for speech problems unrelated to the cleft. Whether or not your child will depends on the results of a detailed evaluation done by a certified speech-language pathologist.

Dealing With Emotional and Social Issues
Though they might encounter social, psychological, and educational challenges, kids with a cleft just want to be treated like everyone else.

Some kids struggle growing up with a cleft lip or cleft palate and might need help handling certain situations. The psychologists and social workers on the cleft palate team are available to guide you through these difficult times. The good news is that most kids with cleft lip or cleft palate grow up to be healthy, happy adults.

In the meantime, you can support your child with these tips:

  • Try not to focus on the cleft and don't allow it to define who your child is.
  • Create a warm, supportive, and accepting home environment where each person's individual worth is openly celebrated.
  • Encourage your child to develop friendships with people from diverse backgrounds. Lead by example.
  • Point out positive attributes in others that do not involve physical appearance.
  • Encourage independence by giving your child the freedom to make decisions and take appropriate risks, letting his or her accomplishments lead to a sense of personal value. Having opportunities to make decisions early on — like picking out which clothes to wear — gives kids confidence and the skills to make bigger decisions later.
  • Consider encouraging your child to present information about cleft lip and palate to his/her class with a special presentation that you arrange with the teacher. Perhaps your child would like you and/or a member from the cleft palate team to talk to the class. This can be especially effective with young children.
If your child is teased, talk about it and be a patient listener. Provide tools to confront the teasers by asking what your child would like to say and then practicing those statements. And it's important to keep the lines of communication open as your child approaches adolescence so that you can address his or her concerns about appearance.

If your child has difficulty with self-esteem or other psychosocial situations, contact a child psychologist or social worker for support and management. Together with the cleft palate team, you can help your child through tough times.





Others information:


Cleft Palate: Frequently asked questions


Our two-week old baby has Pierre Robin Sequence (PRS) with a soft cleft palate. Swallow study shows nasal reflux. No air problems, but she won't swallow anything. What we can do until cleft repair at one year of age?
Usually babies with this problem do learn to feed orally. However, some children with PRS have difficulty breathing while suckling and will require some additional help. It is generally true that an interdisciplinary team best manages children with clefts of any sort. If this family has yet to receive team care, I would advocate that as a first step towards answering this question.

My three-week-old son has a cleft palate and lip. We wanted to know if you would need the foreskin from his penis to correct his lip?
It would be highly unusual to repair a lip in that fashion.

How many surgeries is average to correct the palate and make it not-so-noticeable?
That varies from patient to patient. At the minimum, one surgery is needed to repair the lip and a separate surgery is needed to repair the palate. However, several surgeries are needed to make the lip appear as normal as possible. And sometimes additional surgeries involving the palate are needed to improve speech.

Does a cleft palate always result in a cleft lip?
No. About 50 percent of children with clefts have clefts that involve the lip and palate. Another 25 percent have clefts that involve only the lip and the remaining 25 percent have clefts that only affect the palate.

What can be done for the child's speech, from the beginning of infancy until school?
Most importantly early on is to treat the child as you would any other child, meaning talk to the child, expose the child to lots of good language. That is necessary for all of us to learn speech normally. Then, by the time your child is a year old, he or she will begin to produce their first words, and an evaluation by a speech pathologist is indicated. In fact, it is a good idea to see a speech pathologist earlier than that, such as during the months when the child is beginning to babble. Once that begins to happen, we can get an idea about whether the cleft is affecting the child's speech. It is important to note that only between 20 and 30 percent of children born with clefts of the palate have speech problems requiring secondary surgery after the palate is repaired. The remaining 70 to 80 percent develop speech normally.

What type of specialists should be involved in treating a child with a cleft palate?
At a minimum, the cleft palate team should include a surgeon who specializes in cleft surgical management, a speech pathologist, and a dental professional. Those are the three core professionals that make up a cleft palate team. In addition to that, the cleft palate team may include a geneticist, a pediatrician, an audiologist and an orthodontist. Nurses, psychologists, and other professionals are sometimes involved as well.

Our son has not passed his hearing tests, and we wanted to know if the cleft palate could cause that?
Yes. It can. For the same reasons that some children with clefts have problems with speech, that same problem puts them at greater risk for hearing problems, and the issue has to do with middle ear infections. Children, even without clefts, are at greater risk for acquiring ear infections than are adults. Children with clefts are at yet a greater risk for ear infections. So ear health must be monitored very closely in children with clefts. That is why the ear, nose and throat doctor is an important member of the cleft palate team. That is also why the audiologist is an important member. The audiologist is responsible for measuring the child's ability to hear and the ear, nose and throat doctor or pediatrician can provide the necessary medication to treat ear infections.

What causes cleft palates?
We don't know. We believe that there are genetic causes in some children, we believe that there are environmental causes in other children, and in many children we suspect there is an interaction between genetics and environment. And by environment I mean the environment in which the baby develops in-utero, and the environment in which the mother lives. We know that exposure to alcohol abuse predisposes the baby in the womb to birth defects that may include cleft palate. That disorder is called fetal alcohol syndrome. We suspect there are other environmental issues, which may also interact with genetics to cause clefts. In children with Pierre Robin Sequence, clefts are a result of an underdeveloped mandible, which is the bone that makes up the chin. When the chin is too small, the tongue, as the baby develops, prevents the palate from fusing the way it should in-utero. So the cause of the cleft in that case is a mechanical one and has to do with the underdeveloped chin. Pierre Robin Sequence is a special case where clefts are concerned.

What is the normal recovery time after surgery?
Usually babies require several days of close monitoring after surgery. Recovery usually requires several weeks of special care. During those weeks, the first few days after surgery, movement of the child's hands may be restricted so they don't put their fingers in their mouths and disrupt the surgical repair.

Can you tell if a fetus will have a cleft palate?
That is a very interesting question. The technology is improving such that with ultrasound, it is sometimes possible to identify a cleft during the first few months of pregnancy. Most of the time, however, it is not possible to say for sure whether the baby has cleft or not. The smaller the cleft the harder it is to view with current ultrasound technology. The question that follows that one is if a cleft is observed in-utero can it be repaired before birth. The answer to that is no. Not at this time. There is research being pursued with animal studies that is working in that direction.

Can you tell if a fetus will have a cleft palate? What other ailments are they likely to suffer from, i.e. learning disabilities, etc?
Children with clefts are at a slightly increased risk for learning disabilities, in particular for having difficulty learning to read. Our colleague, Dr. Lyn Richman, has completed several research projects having to do with the learning abilities of children with clefts, and he expresses concern about the learning abilities of children with clefts. Having said that, it seems to be the case that the majority of children with clefts of the lip or palate develop normally unless there are other problems that occur along with the cleft. It is not uncommon for clefts to occur as part of a larger syndrome. Many of those syndromes include learning disabilities.

Is there currently any research being done regarding cleft palates?
Yes. There is research being done of many kinds. There is research that has to do with wound healing, genetic causes, and environmental causes of clefts, and research that has to do with the optimal means of treatment for cleft palates. The National Institutes of Health provide funds for a wide range of research projects in the area of clefts.

Who is responsible for "repairing" a cleft palate?
A specially trained cleft surgeon typically should repair a cleft palate. This may be a surgeon who is also trained as a plastic surgeon or pediatric otolaryngologist (ear, nose and throat surgeon) or other training prior to cleft and craniofacial training, including maxillofacial surgeons. More important than surgical specialty is the training and expertise and experience of the surgeon.

Does everyone with a cleft palate have to have surgery? What if they don't?
Many children in third world countries do not have the luxury of having surgery. They may grow up with unrepaired clefts. The implications include social isolation, speech problems, and feeding problems. In this country, we are fortunate to have the resources and expertise to provide cleft care for all babies born with cleft. So there is no reason for children in this country to grow up with an unrepaired cleft palate. The primary reason for repairing clefts includes appearance and speech development, as well as maximizing the health of the ears and facilitating eating. Submucous cleft palates may not need to be repaired and speech and feeding needs dictate the necessity of any surgery for these.

What kind of follow up is needed after surgery?
After surgery, it is really important for the family to follow up with the cleft surgeon to ensure that the healing process is progressing the way it should. There is always a risk of infection and breakdown of sutures at the surgical site, and so it is very important that the family stay in close contact with the surgeon post-operatively.

Our son has an exposed sinus passage that drains. Is there anything we can do to prevent the mucus from causing him to choke?
You need to see your doctor about that. Ear, nose and throat doctors specialize in problems involving the sinuses. You may ask your pediatrician whether a referral to an ear, nose and throat doctor is indicated.

Does a cleft palate have anything to do with lisps?
No. Lisps are usually caused by incorrect learning and not due to structural problems like cleft palate. Consequently, treatment of lisps is best accomplished by speech therapy.

Is there a high risk of infection after surgery?
No. There is not a high risk provided the family follows the at-home instructions provided by the surgeon and the nursing staff, and provided they follow up with the surgeon during the post-operative period.

What is a "hair lip"?
The correct spelling is harelip. That is in reference to the appearance of the lip of a rabbit. It usually carries a negative connotation so we do not encourage its use.

Is it difficult for children to eat and drink with a cleft palate?
Yes. But it can be managed, provided the parents are given appropriate training and provided appropriate bottles and nipples are used. The child with an unrepaired cleft palate can learn to feed by bottle. normally. Those problems are usually short-term since a cleft lip is usually repaired at four to six months of age and a cleft palate is usually repaired around 12 months of age, so feeding problems caused by the cleft should not last longer than this.

What does surgery entail?
Surgery involves moving tissue from the edges of the cleft into the cleft area. It is rare that tissue is needed from elsewhere in the body to close a cleft. Families who need to know more about cleft repair should be advised to talk with their surgeon.

Where can I find more information on cleft palate?
The American Cleft Palate Foundation provides a wide range of literature written by professionals for families about cleft palate and the problems associated with cleft palate. Families can call the Cleft Palate Foundation (CLAPAM). Many of the publications that are available through the Cleft Palate Foundation can be obtained through the website. They are all free of charge. I would encourage families and children with clefts to contact the Cleft Palate Foundation any time they have questions about cleft palate.

Why do clefts cause speech problems?
Clefts affect the soft palate, which is the posterior part of the roof of the mouth. If you move your tongue along the roof of your mouth from front to back, you will notice the roof of your mouth becomes soft as you move your tongue towards the back. The soft palate moves when we speak, to prevent air from escaping into your nose and causing a hypernasal quality. When that happens, it becomes difficult for the child to produce many of the speech sounds in English. Most of the speech sounds in English require that the space between your mouth and your nose behind the soft palate be completely closed. The only exceptions are the sounds M N and the sound at the end of ING. Those are the only three nasal sounds in English. All other sounds are oral sounds and require that the space between the nose and the mouth be closed by the soft palate. Children who have clefts are frequently unable to get complete closure of that space. As a result, they have abnormal speech. The treatment usually involves an extra surgical procedure to make that space smaller and more easily closed by the child with the cleft. Speech therapy also is needed to teach the child proper ways to produce the speech sounds that he or she may have learned incorrectly because of the cleft. All of this treatment for those children with clefts with speech problems should happen before the child starts first grade. So it is very important that the child be evaluated by a cleft palate team on a regular basis, meaning at least twice a year, from the time that the child is born until such time that all the problems associated with the cleft are resolved.

Children with clefts should be thought of as, with few exceptions, normal children who happen to have been born with a birth defect. Cleft palate is among the most treatable birth defects in this country. Families of children with clefts should be encouraged to learn as much as they can about clefts and then to teach those friends and family members that surround them about what they learned. This is that no one carries misconceptions about what a cleft palate is and what it means for the child. The best way for the family to acquire accurate information is to work closely with a multidisciplinary cleft palate team. You can identify a cleft palate team located near you by contacting the Cleft Palate Foundation, CLAPAM.



Speech Development Related to Cleft Palate

It is common for children born with cleft palate or with cleft lip and palate to have speech problems at some time in their lives.Over half of these children will need speech therapy, however, most will develop normal
speech by age 5. Typically, children with cleft lip only do not have speech problems related to clefting.

What speech problems might children with cleft palate have?
A child with cleft palate may have trouble with:

• Speech Sound Development
• Velopharyngeal Incompetence
• Hearing Loss

Speech Sound Development:
Children born with cleft palate may have a delay in the beginning of speech and development of speech sounds. In addition,children with cleft palate may produce speech errors that are directly related to clefting. These are called compensatory speech errors.   Generally, it is expected that children will start making sounds between 2 and 3 months of age and babble (such as bababa, dadada) between 6 and 10 months of age. Children typically start using real words (such as mama, dada) between 12 and 14 months of age.   The following is a general guideline for the age at which at which your child is expected to use sounds in words: By age :
2: m, n, p, b, h
3 – 4 years: t, d, k, g, w, y
4 – 5 years: f
5 – 7 years: s, z, v, sh, ch, th

Velopharyngeal Dysfunction (VPD)
During speech, the goal is to have good airflow through the mouth for all speech sounds except m, n and ng. To direct air through the mouth, the soft palate (back part of the roof of the mouth) lifts and moves towards the back of the throat. This movement closes the opening between the mouth and the nose. VPD happens when the opening between the mouth and nose is not closed properly and too much air leaks out of the nose during speech. All children who are born with cleft palate have velopharyngeal dysfunction resulting in hypernasality (too much airflow through the nose) until the cleft is repaired. After repair, some children still have velopharyngeal dysfunction. However, with additional intervention, most will have normal speech by age 5.

Hearing Loss:
Children with cleft palate are more likely to get fluid in the middle ear and infections. These may cause mild or moderate hearing loss. Because children learn to speak and understand language through hearing, it is very important that hearing concerns and ear health are closely watched. What can be done about speech
problems related to cleft palate? roblems related to cleft palate?

 • Speech therapy is an essential tool for children who have speech errors. The goal of therapy is to help a child learn to use his/her tongue and lips correctly. Therapy may also be an effective treatment option for mild hypernasality but it usually does not correct more serious velopharyngeal dysfunction. If speech therapy is recommended for your child, the Cleft Clinic speech pathologists will help you find speechservices close to home.
• “Speech Surgery”, such as a pharyngeal flap or sphincter pharyngoplasty, may be recommended to correct moderate to severe velopharyngeal dysfunction. A surgery is a big decision and not a “quick fix”. Your child may need speech therapy before and after the procedure. The Cleft Clinic will work closely with
you and your child to decide if surgical intervention may improve velopharyngeal closure for speech.

• Get regular ear and hearing exams even if you do not think that your child has a hearing loss. These exams are part of an annual Cleft Clinic visit but your child may require more care such as hearing aids, 6-month re-checks, and/or ear tubes. Good hearing is essential for good speech development.

How can I help my child develop good speech?

• Talk to your child and provide language- learning opportunities. Suggestions for home activities are given in the Patient/Parent Speech and Language Development handouts.

• Encourage your child to talk to you using his or her best speech.

• Work with your child’s speech therapist to get home activities that are directly related to your child’s therapy plan.

• Remember that to improve speech your child has to work on speech tasks. Blowing whistles or bubbles and sucking through straws will not improve velopharyngeal closure for speech.

• Schedule regular ear and hearing exams. If your child has hearing aids, make sure he or she is wearing them as often as possible.








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