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Cleft Lip and Palate

Cleft lip and palate

Definition

A cleft lip and/or palate is a birth defect (congenital) of the upper part of the mouth. A cleft lip creates an opening in the upper lip between the mouth and nose and a cleft palate occurs when the roof of the mouth has not joined completely.

Description

Cleft means split or separated. During the first months of pregnancy, separate areas of the facesuch as bony and muscular parts, mouth, and throat, develop individually and then join together. If some parts do not join properly the result is a cleft, the type and severity of which can vary. During the fifth through ninth weeks of pregnancy genetic and environmental factors are most likely to affect lip and palate development. Cleft palate occurs when the right and left segments of the palate fail to join properly. The back of the palate (toward the throat) is called the soft palate, and the front section (toward the mouth opening) is known as the hard palate. A cleft palate can range from just an opening at the back of the soft palate to a nearly complete separation of the roof of the mouth (soft and hard palate). In some cases, an infant with a cleft palate may also have a small lower jaw and have difficulty breathing. This condition is called Pierre Robin sequence.

Cleft lip occurs when the lip elements fail to come together during fetal development, thus creating an opening in the upper lip between the mouth and nose. The lip looks split. A cleft lip may be complete, meaning that there is complete separation in one or both sides of the lip extending up and into the nose, or it may be incomplete, in which case there is only a notch in the fleshy portion of the lip. The incomplete cleft lip results in less facial distortion because the connected parts of muscle and tissue have a stabilizing effect. In a complete cleft lip, the muscles pull away from the center of the face, resulting in distortion of the nose and mouth. A cleft on one side is called a unilateral cleft. If a cleft occurs on both sides, it is called a bilateral cleft.

Demographics

Over 5,000 infants are born each year in the United States with a cleft lip or palate (about one in every 700 births). Cleft lip without cleft palate is the third most common congenital malformation among newborns in the United States and is estimated to occur roughly twice as often in males than in females. Cleft palate without cleft lip is fifth most common, and it affects roughly twice as many girls as boys. Clefts may affect the left or right side of the mouth only (unilateral) or both sides (bilateral). Left-side clefts represent 70 percent of all unilateral clefts. In the United States, clefting seems to be at least in part related to ethnicity, occurring most often among Asians, Latinos, and Native Americans (one in 500), next most often among persons of European ethnicity (one in 700), and least often among persons of African ethnicity (one in 1,000).

Causes and symptoms

The causes of clefts are as of 2004 still poorly understood. Most scientists believe that clefting occurs as a result of a combination of genetic and environmental factors. In the United States and western Europe, researchers report that a family history of facial clefts is present in approximately 40 percent of all cases. The likelihood of a baby being born with a facial cleft increases if a first-degree relative (mother, father, or sibling) has a cleft. Mothers who abuse alcohol and drugs, lack vitamins (especially folic acid ) during the first weeks of pregnancy, or have diabetes are more likely to have a child with facial clefts.

Clefts may occur alone or with other abnormalities that may be hidden or obvious. Up to 13 percent of infants with cleft lip or palate have other birth defects. Some cases involve genetic syndromes that may result in specific problems for the infant and may have a high risk of affecting others in the family. For this reason, newborns with clefts should be thoroughly examined by a specialized physician soon after birth.

When to call the doctor

Families with a history of cleft lip or palate or any other syndrome or condition associated with clefting should discuss the chances of recurrence with a genetic counselor.

Diagnosis

Because clefting causes specific physical manifestations, it is easy to diagnose. Although some types of clefts can be detected during pregnancy by an ultrasound test, many are not discovered until birth.

Treatment

A cleft lip and/or palate can be repaired with corrective surgery, performed in a hospital under general anesthesia. Generally, within the first few days following birth of an infant with a facial cleft, a team is assembled to prepare a plan for treatment of the cleft. The treatment team usually includes representatives from several medical or psychological specialties, including pediatrics, plastic surgery, otolaryngology, orthodontia, prosthodontics, oral surgery, speech and language pathology, audiology, nursing, and psychology. It is common for one team member to coordinate service and communication between the team members and the family.

Surgical repair of a cleft lip is carried out at about three to four months of age. The whole emphasis in repairing the lip is on the muscle repair in order to mold the distorted front central section of the upper gum containing the four upper front teeth (premaxilla) back into its proper position. Cleft lip often requires only one reconstructive surgery, especially if the cleft is unilateral. The surgeon makes an incision on each side of the cleft from the lip to the nostril, drawing the two sides of the cleft together and suturing them together. Bilateral cleft lips are usually repaired in two surgeries, about a month apart. The first surgery is performed when a baby is between six and 10 weeks old and usually requires a one-night stay in the hospital.

Cleft palate can require several surgical procedures during the course of a child's first 18 years. The first surgery to repair the palate usually occurs when the infant is between six and 12 months old. It usually involves palatal lengthening and drawing tissue from either side of the mouth to rebuild the palate. The procedure usually requires two or three nights in the hospital, the first night in the intensive care unit. The purpose of this surgery is to create a functional palate, reduce the chances that fluid will develop in the middle ears, and help the proper development of the child's teeth and facial bones. In addition, the functional palate helps the 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 percent of children with a cleft palate require further surgical procedures 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.

Nutritional concerns

Infants with cleft lip or cleft soft palate generally have few feeding problems. However, when the cleft involves the hard palate, the infant is usually not able to suck efficiently. For these infants, caregivers must experiment with various feeding techniques, such as special nipples or alternate feeding positions. The infant with a cleft should be held in a nearly sitting position during feeding to prevent the breast or formula milk from flowing back into the nose. In addition, the infant should be burped frequently, approximately every three or four minutes. The sucking reflex is strong in all infants and should be encouraged in infants with facial clefts even if the sucking is inefficient, since the reflex seems to help the later development of speech. It is important to keep the cleft clean and not to allow formula, mucus, or other matter to collect in the cleft.

Prognosis

Both cleft lip and cleft palate are treatable birth defects. Most children born with either or both of these conditions undergo reconstructive surgery while they are still infants to correct the defect and significantly improve facial appearance. With advances in surgical techniques and with more complete repair of facial clefts, about 80 percent of affected children have normal speech development by the time they enter school. Continuation of speech therapy results in continuous improvement for most common speech problems.

Prevention

While little is known about how to prevent clefts, researchers from the California Birth Defects Monitoring Program found that women considering pregnancy may be able to reduce the risk of facial clefts (and possibly other birth defects) in their offspring by taking a multivitamin containing folic acid for one month prior to becoming pregnant. Other studies have shown that fetuses with certain predisposing genes may be at increased risk for cleft palate if their mothers smoke. Because some types of medications (for example some drugs used to treat epilepsy) have been linked to increased risk of clefts, women who take medications for chronic illnesses should check with their doctors before they become pregnant.

Parental concerns

Parents of a newborn baby with a cleft lip or palate are often confused and afraid of the impact the defect will have on their child's life. These feelings can be alleviated by learning about the cleft and treatment options. They also must communicate what they are learning about clefts to family, siblings, and friends. It is important for people who come into contact with the child to realize that a cleft is not a wound, although it may give the impression that it is tender or sore. Parents can help others understand that the cleft does not hurt and that it will be repaired. To ensure normal psychological and speech development, parents should interact with their infant as they would with any newborn in the family and should encourage others to do the same.

KEY TERMS

Bilateral cleft lip A cleft that occurs on both sides of the lip.

Cleft An elongated opening or slit in an organ.

Complete cleft A cleft that extends through the entire affected mouth structure.

Congenital Present at birth.

Palatal lengthening (palatal pushback) A surgical procedure in which tissue from the front part of the mouth is moved back to lengthen it.

Palate The roof of the mouth.

Premaxilla The front central section of the upper gum, containing the four upper front teeth.

Unilateral cleft A cleft that occurs on only the right or left side of the lip.

As the child with a cleft grows and develops, he or she will certainly experience many good and bad reactions from adults and children. Other children may tease the child or use the term "harelip." It may be helpful for parents of a child with a facial cleft to meet with his classmates and teachers to explain the history of the term harelip. Although a facial cleft was once referred to as a harelip to reflect its similarity to the mouth of a rabbit, the term is considered insulting today. Educating adults and children about cleft lip and palate is the best way to relieve others' anxiety about the defect and lessen any negative psychological effects that bad reactions might have on the child.

See also Language development.

Resources

BOOKS

Bzoch, Kenneth R. Communicative Disorders Related to Cleft and Lip Palate. Austin, TX: PRO-ED Inc., 2004.

Golding-Kushner, Karen J. Therapy Techniques for Cleft Palate Speech and Related Disorders. San Diego, CA: Singular Publishing Group Inc., 2001.

Gruman-Trinkner, Carrie, and Blaise Winter. Your Cleft-Affected Child: The Complete Book of Information, Resources, and Hope. Berkeley, CA: Publishers Group West, 2001.

Wysznski, Diego I. Cleft Lip and Palate: From Origin to Treatment. Oxford, UK: Oxford University Press, 2002.

PERIODICALS

Caniklioglu, M. C. "Use of a nickel titanium palatal expander in cleft-palate cases." Journal of Clinical Orthodontics 38, no. 7 (July 2004): 37477.

Chapman, K. L. "Is presurgery and early postsurgery performance related to speech and language outcomes at 3 years of age for children with cleft palate?" Clinical Linguistics and Phonetics 18, no. 45 (June-August 2004): 23557.

Hermann, N. V., et al. "Early craniofacial morphology and growth in children with bilateral complete cleft lip and palate." Cleft Palate and Craniofacial Journal 41, no. 4 (July 2004): 10405.

Mulliken, J. B. "The changing faces of children with cleft lip and palate." New England Journal of Medicine 351, no. 8 (August 2004): 74547.

Smith, A. S., et al. "Prenatal diagnosis of cleft lip and cleft palate using MRI." American Journal of Roentgenology 183, no. 1 (July 2004): 22935.

Van Lierde, K. M., et al. "Vocal quality characteristics in children with cleft palate: a multiparameter approach." Journal of Voice 18, no. 3 (September 2004): 35462.

Whitehill, T. L., and C. H. Chau. "Single-word intelligibility in speakers with repaired cleft palate." Clinical Linguistics and Phonetics 18, no. 45 (June-August 2004): 34155.

ORGANIZATIONS

About Face USA. PO Box 969, Batavia, IL 605100969. Web site: <www.aboutfaceusa.org/default.htm>.

American Cleft Palate: Craniofacial Association (ACPCA)/Cleft Palate Foundation (CPF). 1504 East Franklin Street, Suite 102, Chapel Hill, NC 275142820. Web site: <www.cleftline.org>.

American Speech-Language-Hearing Association. 10801 Rockville Pike, Rockville, MD 20852. Web site: <www.asha.org>.

Children's Craniofacial Association (CCA). 13140 Coit Road, Suite 307, Dallas, Texas 75240. Web site: <www.ccakids.org/default.htm>.

WEB SITES

"Cleft Lip and Palate Resource." Wide Smiles. Available online at <www.widesmiles.org> (accessed November 16, 2004).

March of Dimes Homepage. Available online at <www.modimes.org/> (accessed November 15, 2004).

Monique Laberge, Ph.D.

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"Cleft Lip and Palate." Gale Encyclopedia of Children's Health: Infancy through Adolescence. . Retrieved August 17, 2017 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/cleft-lip-and-palate-1

Cleft Lip Repair

Cleft lip repair

Definition

Cleft lip repair (cheiloplasty) is surgical procedure to correct a groove-like defect in the lip.


Purpose

A cleft lip does not join together (fuse) properly during embryonic development. Surgical repair corrects the defect, preventing future problems with breathing, speaking, and eating, and improving the person's physical appearance.


Demographics

Cleft lip is the second most common embryonic (congenital) deformity. (Club foot is the most common congenital deformity.) Cleft lip occurs in approximately one in 7501,000 live births. The highest incidence exists in North American Indians and Japanese (approximately one in 350 births). African Americans and Africans represent the lowest incidence of cleft lip deformity (approximately one in 1,500 births). There is a higher frequency of clefting in certain populations of Scandinavia and Middle European countries.

Cleft lip occurs more commonly in males, while cleft palate is more likely to occur in females. Cleft lip alone (without cleft palate) occurs in approximately 20% of cases across both genders. The majority of cases80%have both cleft lip and cleft palate. A unilateral cleft lip, commonly occurring on the left side, is more common than a bilateral cleft lip.


Potential causes

Most cases of cleft lip have no known cause. However, there is a strong genetic correlation. Other single gene defects that are associated with cleft lip include: Van der Woude syndrome, Opitz Syndrome, Aarskog syndrome, Fryns syndrome, Waardenburg syndrome, and Coffin-Siris syndrome. Approximately 5% of cleft conditions are associated with a genetic syndrome. Most of these syndromes do not include mental retardation.

Facial cleft has been implicated with maternal exposure to environmental causes, such as rubella or medications that can harm the developing embryo. These medications include steroids, antiseizure drugs, vitamin A, and oral anti-acne medications (such as Acutane) taken during the first three months of pregnancy. Cleft lip is also associated with fetal alcohol syndrome and maternal diabetes.

Risk of cleft lip increases with paternal age, especially over 30 years at the time of conception. Generally, the risk is higher when both parents are over 30 years of age. However, most cases seem to be isolated within the family with no obvious causation.

When the affected child has unilateral cleft lip and palate, the risk for subsequent children increases to 4.2%. Advances in high resolution ultrasonography (prenatal ultrasound exam) have made it possible to detect facial abnormalities in the developing embryo (in utero).


Description

Developmental anatomy

Important structures of the embryo's mouth form at four to seven weeks of gestation. Development during this period entails migration and fusion of mesenchymal cells with facial structures. If this migration and fusion is interrupted (usually by a combination of genetic and environmental factors), a cleft can develop along the lip. The type of clefting varies with the embryonic stage when its development occurred.

There are several types of cleft lip, ranging from a small groove on the border of the upper lip to a larger deformity that extends into the floor of the nostril and part of the maxilla (upper jawbone).

Unilateral cleft lip results from failure of the maxillary prominence on the affected side to fuse with medial nasal prominences. The result is called a persistant labial groove. The cells of the lip become stretched and the tissues in the persistent groove break down, resulting in a lip that is divided into medial (middle) and lateral (side) portions. In some cases, a bridge of tissue (simart band) joins together the two incomplete lip portions.

Bilateral cleft lip occurs in a fashion similar to the unilateral cleft. Patients with bilateral cleft lip may have varying degrees of deformity on each side of the defect. An anatomical structure (intermaxillary segment) projects to the front and hangs unattached. Defects associated with bilateral cleft lip are particularly problematic due to discontinuity of the muscle fibers of the orbicularis oris (primary muscle of the lip.) This deformity can result in closure of the mouth and pursing of the lip.

Classification

In addition to classification as unilateral or bilateral, cleft lips are further classified as complete or incomplete. A complete cleft involves the entire lip, and typically the alveolar arch. An incomplete cleft involves only part of the lip. The Iowa system (which also classifies cleft palate) classifies cleft lip in five groups:

  • Group Iclefts of the lip only
  • Group IIclefts of palate only
  • Group IIIclefts of lip, alveolus, and palate
  • Group IVclefts of lip and alveolus
  • Group Vmiscellaneous

Another widely accepted cleft lip classification is based on recommendations of the American Cleft Palate Association. This classification divides cleft lip into unilateral or bilateral (right, left or extent) in thirds(i.e., one-third, two-thirds, three-thirds), or median cleft lip, the extent of which is also measured in thirds.


Surgical procedure

Cleft lip repair can be initiated at any age, but optimal results occur when the first operation is performed between two and six months of age. Surgery is usually scheduled during the third month of life.

While the patient is under general anesthesia, the anatomical landmarks and incisions are carefully demarcated with methylene blue ink. An endotracheal tube prevents aspiration of blood. The surgical field is injected with a local anesthestic to provide further numbing and blood vessel constriction (to limit bleeding). Myringotomy (incisions in one or both eardrums) is performed, and myringotomy tubes are inserted to permit fluid drainage.

There are several operative techniques for cleft lip reconstruction. The Millard rotation advancement (R-A) technique is the most widely accepted form of repair. This method involves rotation of the entire philtral dimple (groove in the upper lip) and Cupid's bow (double curve of the upper lip). The scar falls along the new philtral column (central section of the upper lip), and is adjusted as required since the procedure allows for flexibility.

The Millard procedure begins with an incision on the edge of the cleft side of the philtrum, and the cutting continues upward, medially, and to the side. A second incision extends to the buccal sulcus (top part of the upper jaw). The length of this incision depends on the size of the gap to be closed. In this second incision, the surgeon frees soft tissue, which allows him or her to completely lift the lip from the underlying bone. This dissection should be tested to ensure free advancement toward the middle (inadequate dissection is the root cause of poor results). Nasal deformity can be dealt with by a procedure known as the McComb nasal tip plasty, which elevates the depressed nasal dome and rim. Cartilage from the cleft side is freed from the opposite side, and is positioned and reshaped using nylon sutures.

Advantages of the Millard rotation advancement technique (include:

  • It is the most common procedure (i.e., surgeons more familiar with it).
  • The technique is adaptable and flexible.
  • It permits construction of a normal-looking Cupid's bow.
  • A minimal amount of tissue is discarded.
  • The suture line is camouflaged.

The disadvantage of the Millard rotation advancement technique is the possible development of a vermilion notch (shortening of the entire lip in the vertical direction), resulting from contracture of the vertical scar.

Cupid's bow is a critical part of the repair, making it very important to accurately determine the high point of Cupid's bow on the lateral lip.


Diagnosis/Preparation

Facial clefting has a wide range of clinical presentations, ranging from a simple microform cleft to the complete bilateral cleft involving the lip, palate, and nose. A comprehensive physical examination is performed immediately after birth, and the defect is usually evident by visual inspection and examination of the facial structures.

Care must be taken to diagnose other physical problems associated with a genetic syndrome. Weight, nutrition, growth, and development should be assessed and closely monitored.

Presurgical tests include a variety of procedures, such as hemoglobin studies. It is important for the pateint's parents and physician to discuss the operation prior to surgery.


Aftercare

The postoperative focus is on ensuring proper nutrition, as well as lip care and monitoring the activity level. Breast milk or full-strength formula is encouraged immediately after surgery or shortly thereafter. Lip care for patients with sutures should include gentle cleansing of suture lines with cotton swabs and diluted hydrogen peroxide. Liberal application of topical antibiotic ointment several times a day for 10 days is recommended. There will be some scar contracture, redness, and firmness of the area for four to six weeks after surgery. Parents should gently massage the area, and avoid sunlight until the scar heals.

The patient's activities may be limited. Some surgeons use elbow immobilizers to minimize the risk of accidental injury to the lip. Immobilizers should be removed several times a day in a supervised setting, allowing the child to move the restricted limb(s).

Interaction between the orthodontist and surgeon as part of the treatment team begins in the neonatal period, and continues through the phases of mixed dentition.


Risks

There may be excessive scarring and contraction of the lips. Two types of scars, hypertrophic or keloid, may develop. Hypertrophic scars appear as raised and red areas that usually flatten, fade in color, and soften within a few months. Keloids form as a result of the accelerated growth of tissue in response to the surgery or trauma to the area. The keloid can cause itching and a burning sensation. Scratching must be avoided because it can lead to healing problems. Some patients require minimal revision surgery, but in most cases, the initial redness and contracture is part of the normal healing process.


Normal results

Ideal surgical results for cleft lip include symmetrically shaped nostrils, and lips that appear as natural as possible and have a functional muscle. Many characteristics of the natural lip can be achieved; however, the outcome ultimately depends on a number of factors, including the skill of the surgeon, accurate presurgery markings, alignment of bones within the affected area, uncomplicated healing of the initial repair, and the effect of normal growth on the repaired lip. Additional surgical correction to reconstruct nasal symmetry is sometimes necessary.


Morbidity and mortality rates

Generally, cleft lip repair is well-tolerated in healthy infants. There are no major health problems associated with this reconstructive surgery. Depending on the results, it may be necessary to perform additional operations to achieve desired functional and cosmetic outcomes.


Alternatives

There are no alternatives for this surgery. Obvious deformity and impairments of speech, hearing, eating, and breathing occur as a direct result of the malformation. These issues can not be corrected without surgery.


Resources

books

Aston, S.J., R.W. Beasley, C.H.M. Thorne, Grabb and Smith's Plastic Surgery 5th ed. Philadelphia, PA: Lippincott-Raven Publishers, 1997.

Bluestone, Charles. Pediatric Otolaryngology. Philadelphia, PA: Saunders, 2003.

Cohen, M. Mastery of Plastic and Reconstructive Surgery. Little, Brown & Company, Boston, MA: 1994.

Georgiade, N. Pediatric Plastic Surgery. C. V. Mosby, Co., St Louis, MO: 1984.

Millard, Ralph, D. Principlization of Plastic Surgery. Boston, MA: Little, Brown & Company, 1986.

Papel, Ira. Facial Plastic and Reconstructive Surgery 2nd ed. Thieme Medical Pub., 2002.

periodicals

Byrd, H.S. "Selected Readings in Plastic Surgery: Cleft Lip I: Primary Deformities." Baylor University Medical Center 8 (1997): 221.

Salomon, J. and P.C. Hobar. "Selected Readings in Plastic Surgery: Cleft Lip II: Secondary Deformities." Baylor University Medical Center 2, no.22 (1997).

organizations

American Board of Plastic Surgery,Inc. Seven Penn Center, Suite 400, 1635 Market Street, Philadelphia, PA 19103-2204. (215) 587-9322. <http://www.abplsurg.org>.

International Birth Defects Information Systems. <http://ibisbirthdefects.org/start/index.htm>.

The International Craniofacial Institute. <http://www.craniofacial.net/additional_services/additional_services.asp>.

other

Wide Smiles. <http://www.communicationdisorder.net/Cleft Palate.html>.


Laith Farid Gulli, M.D.,M.S. Robert Ramirez, B.S. Randall J. Blazic, M.D.,D.D.S. Bilal Nasser, ,M.D., M.S.

WHO PERFORMS THE PROCEDURE AND WHERE IS IT PERFORMED?


A plastic and reconstructive surgeon performs the procedure in a hospital equipped with a surgical department. Plastic surgeons typically have five years of training in general surgery (or orthopedic surgical training), and two additional years of specialized plastic and reconstructive surgery training. A plastic surgeon should be certified by the American Board of Plastic and Reconstructive Surgery.

Many plastic surgeons who operate on cleft lip (or palate) patients have received additional surgical training in pediatric plastic surgery. However, completion of a surgical training program does not guarantee clinical expertise, and parents should seek surgeons who having both training and experience.

QUESTIONS TO ASK THE DOCTOR


  • How many cleft palate surgeries has the surgeon performed?
  • How is nutrition managed after surgery?
  • What lip care will be required after surgery?
  • How much activity will be allowed, and will the patient be immobilized following the surgery?
  • How much follow-up is required?
  • What cosmetic and functional results are expected?
  • How long is the surgical procedure?
  • What follow-up reconstructive surgery is anticipated?

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Cleft Lip and Palate

Cleft Lip and Palate

Definition

A cleft is a birth defect that occurs when the tissues of the lip and/or palate of the fetus do not fuse very early in pregnancy. A cleft lip, sometimes referred to as a harelip, is an opening in the upper lip that can extend into the base of the nostril. A cleft palate is an opening in the roof of the mouth.

Description

Babies born with cleft lips will have an opening involving the upper lip. The length of the opening ranges from a small notch, to a cleft that extends into the base of the nostril. Cleft lips may involve one or both sides of the lip.

Babies born with cleft palates have openings in the palate, which is the roof of the mouth. The size and position of the opening varies. The cleft may be only in the hard palate, the bony portion of the roof of the mouth, opening into the floor of the nose. It may be only in the soft palate, the soft portion of the roof of the mouth. The cleft palate may involve both the hard and soft palate and may occur on both sides of the center of the palate.

Babies may have cleft lips with or without cleft palates. Cleft palates may also occur without cleft lips.

The incidence of cleft lip and palate not associated with a syndrome is one in 700 newborns. Native Americans have an incidence of 3.6 in 1,000 newborns. The incidence among Japanese newborns is 2.1 in 1,000. The incidence among whites is one in 1,000 newborns. African Americans have an incidence of 0.3 in 1,000 newborns.

Causes and symptoms

Cleft lips and palates not associated with a syndrome are caused by a combination of genetic and environmental factors. Inheritance caused by such a combination is called multifactorial. The embryo inherits genes that increase the risk for cleft lip and/or palate. When an embryo with such genes is exposed to certain environmental factors the embryo develops a cleft.

The risk of a baby being born with a cleft lip or palate increases with the number of affected relatives and the number of relatives that have more severe clefts.

Environmental factors that increase the risk of cleft lip and palate include cigarette and alcohol use during pregnancy. Some drugs, such as phenytoin, sodium valproate, and methotrexate, also increase the incidence of clefting. The pregnant mother's nutrition may affect the incidence of clefting as well.

Babies born with a cleft lip will be seen to have an elongated opening in the upper lip. The size of this opening may range from a small notch in the upper lip to an opening that extends into the base of the nostril. The cleft lip may be below the right or left nostril or below both nostrils.

Babies born with a cleft palate will be seen to have an opening into the roof of the mouth. The size and position of the cleft varies and it may involve only the hard palate, or only the soft palate and may occur on both sides of the center of the palate.

In some cases the cleft palate will be covered with the normal lining of the mouth and can only be felt by the examiner.

Babies with cleft lips and palates have feeding difficulties, which are more severe in babies with cleft palates. The difficulty in feeding is due to the baby being unable to achieve complete suction. In the case of clefts of the hard palate, liquids enter the nose from the mouth through the opening in the hard palate.

A cleft palate also affects a child's speech, since the palate is necessary for speech formation. The child's speech pattern may still be affected despite surgical repair.

Ear infections are more common in babies born with cleft palates. The infections occur because the muscles of the palate do not open the Eustachian tubes that drain the middle ear. This allows fluid to collect and increases the risk of infection and hearing loss.

Teeth may also erupt misaligned.

Diagnosis

Cleft lip and palate can be diagnosed before birth by ultrasound. Magnetic resonance imaging (MRI) offers more accuracy for detecting cleft lip and palate, as it is a more detailed imaging method. It is particularly helpful in showing soft palate defects. In 2004, researchers reported discovery of a gene test for isolated cleft lip and cleft palate to help predict if parents who have one child with the isolated form of cleft lip or palate were likely to have a second child with the same defect. After birth, cleft lip and palate are diagnosed by physical exam.

Treatment

If cleft lip and/or palate are diagnosed by ultrasound before birth, further testing may be required to diagnose associated abnormalities if present. Referral to a cleft team is essential. A cleft team consists of specialists in the management of babies with clefts and includes surgeons as well as nurses and speech therapists. Members of the team inform the parents of all aspects of management. Feeding methods are also discussed, since feeding is the first problem that must be dealt with. It may be possible to breastfeed a baby born with only a cleft lip, but babies born with cleft palates usually have more problems with feeding and frequently require special bottles and teats. A palatal obturator is a device that fits into the roof of the mouth, thus blocking the cleft opening and allowing easier suckling.

Surgery to repair cleft lips is sometimes performed after orthodontic treatment to narrow the gap in the upper lip. The orthodontic treatment can involve acrylic splints with or without screws or may involve the use of adhesive tape placed across the gap in the lip. The orthodontic treatment for cleft lip should be started within the first three weeks of life and continue until the cleft lip is repaired.

The timing of surgical cleft lip repair depends on the judgment of the surgeon who will perform the operation. The procedure is usually performed between one and three months of age. The goals of the operation are to close the gap in the upper lip, place scars in the natural skin curves and to repair muscle so that the lip appears normal during movement. The closure is done in the three layers (skin, muscle, and mucosa) that line the inside of the lip. At the time of the procedure, if the nose is shaped abnormally due to the cleft lip, it is also corrected. Sometimes further surgery may be needed on the lip and/or nose to refine the result.

The goals of the surgeon repairing a cleft palate are normal speech, normal facial growth, and hearing for the affected infant. The repair of the cleft palate is usually performed between three and 18 months of age. The timing may extend beyond this and varies with the type of cleft palate and center where the procedure is being performed. Depending of the type of cleft palate, more than one operation may be needed to close the cleft and improve speech.

Nonsurgical treatment of a cleft palate is available for patients who are at high risk for surgery and consists of a prosthetic appliance worn to block the opening in the palate.

Babies born with cleft palates are vulnerable to ear infections. Their Eustachian tubes do not effectively drain fluid from the middle ear so fluid accumulates and infection sets in. This may lead to hearing loss. These children require drainage tubes to be inserted to prevent fluid accumulation.

Babies born with clefts usually require orthodontic treatment between 13 and 18 years of age. They also require speech therapy.

Prognosis

Babies born with cleft lip and palate have a good prognosis, and approximately 80% will develop normal speech. There is no known means of preventing clefting. Good prenatal care is essential and avoiding harmful substances appear to reduce the risk.

Resources

PERIODICALS

Bender, Patricia L. "Genetics of Cleft Lip and Palate." Journal of Pediatric Nursing 15 (August 2000): 242-249.

Christensen, Karr. "The 20th Century Danish Facial Cleft PopulationEpidemiological and GeneticEpidemiolical Studies." Cleft PalateCraniofacial Journal 36 (March 1999): 96-104.

Chung, Kevin C. "Maternal Cigarette Smoking during Pregnancy and the Risk of Having a Child with Cleft Lip/Palate." Plastic and Reconstructive Surgery 105 (February 2000): 458-491.

Cockell, Anna. "Prenatal Diagnosis and Management of Orofacial Clefts." Prenatal Diagnosis 20 (February 2000): 149-151.

"MRI More Accurate for Detecting Prenatal Cleft Lip and Palate than Sonography." Medical Devices & Surgical Technology Week July 25, 2004: 160.

"Researchers Report New Gene Test for Isolated Cleft Lip and Palate." Science Letter September 28, 2004: 518.

Rohrich, Rod J. "Optimal Timing of Cleft Palate Closure." Plastic and Reconstructive Surgery 106 (August): 413-421.

ORGANIZATIONS

Cleft Palate Foundation. (800) 24-CLEFT. http://www.cleftline.org.

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"Cleft Lip and Palate." Gale Encyclopedia of Medicine, 3rd ed.. . Encyclopedia.com. 17 Aug. 2017 <http://www.encyclopedia.com>.

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"Cleft Lip and Palate." Gale Encyclopedia of Medicine, 3rd ed.. . Retrieved August 17, 2017 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/cleft-lip-and-palate-0

cleft lip and palate

cleft lip and palate are congenital deformities of variable severity. The face begins to develop around the fourth week of life in the womb, from five different parts of the developing fetal tissue — a central one and two on each side. These embryonic cellular components normally migrate and fuse to give rise to the nose, the sides and centre of the upper lip, and the palate and the lower jaw. Any failure of fusion of these parts can result in a cleft. The severity may vary from a barely detectable groove or ridge with a gap in the underlying muscles, to a complete cleft on each side of the lip associated with clefts extending back through the gums and the palate. Although genetic factors are implicated, the causes of such deformities are considered to be multi-factorial. Cleft lip and palate (CL/P) and cleft palate alone (CP) are different entities with varying incidence, epidemiology, and genetics.

Clefts of the lip, with or without clefts of the palate, occur in 1 in 750 live births, as compared with 1 in 2000 for clefts of the palate alone. CL/P is commoner in boys and CP alone is commoner in girls. There are racial variations in incidence of CL/P, with a decreasing order of frequency in Orientals, Europeans, and Africans, but there is no heterogeneity in the incidence of CP.

The problems associated with clefts of the lip and palate are both aesthetic and functional.

Aesthetic problems — those related to appearance — are mainly in patients with clefts of the lip. These may be on one side of the central part or on both sides. Clefts of the lip are also associated with deformity of the nose and asymmetry of the underlying facial skeleton which contribute to the problems of abnormal facial appearance.

Functional problems are minor in unrepaired clefts of the lip except in the infant, when there may be difficulty in sucking due to inability to achieve an efficient lip seal, or later, when clarity of speech or whistling may be affected. Unrepaired clefts of the palate, especially the soft palate, cause significant functional problems associated with speech and swallowing. Eustachian tube malfunction in CP patients may result in middle ear infections and hearing loss.

The earliest repairs recorded in history were in China circa 390 ad; in Europe the first report was of surgical repair by a French dentist, le Monier, in 1764. Advances in surgical techniques, and especially anaesthesia, now allow anatomical correction with great precision in restoration of form and function. Traditional repair of the cleft lip is recommended at around the age of 3 months or at a weight of 10 lbs, and of the cleft palate at about 18 months — but various protocols and techniques exist. Current techniques are very successful but there is considerable controversy regarding the effects of surgery and possible adverse influences on facial growth and function.

Arup K. Ray


See also congenital abnormalities.

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"cleft lip and palate." The Oxford Companion to the Body. . Encyclopedia.com. 17 Aug. 2017 <http://www.encyclopedia.com>.

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"cleft lip and palate." The Oxford Companion to the Body. . Retrieved August 17, 2017 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/cleft-lip-and-palate

cleft palate

cleft palate, incomplete fusion of bones of the palate. The cleft may be confined to the soft palate at the back of the mouth; it may include the hard palate, or roof of the mouth; or it may extend through the gum and lip, producing a gap in the teeth and a cleft lip, which is cosmetically difficult to repair but is not disabling. The condition appears to be hereditary but not under the control of a single pair of genes. A cleft palate causes separation between the oral and nasal cavities. An infant cannot develop proper suction for drinking, and there is the danger of milk entering the nasal cavity and being aspirated into the lungs. Formula must be carefully placed at the back of the tongue for normal swallowing to take place. Ear infection may result from food or fluid passing from the nasal cavity to the middle ear by way of the Eustachian tubes. Proper speech articulation is difficult unless the cleft is surgically closed, with a prosthesis. The proper time for such an operation is in dispute; some authorities prefer early closure, before the cleft interferes with development of normal speech habits, while others prefer to wait for several years until facial growth has been completed. Dental, orthodontic, psychiatric, and speech therapy may be required.

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"cleft palate." The Columbia Encyclopedia, 6th ed.. . Encyclopedia.com. 17 Aug. 2017 <http://www.encyclopedia.com>.

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Cleft Palate

Cleft Palate

Mixed Feelings

What Is a Cleft Palate?

Why Are Babies Born with Cleft Palates and Cleft Lips?

What Happens to Children with Cleft Palate and Cleft Lip?

Resources

A cleft palate is a gap or split in the roof of the mouth (the palate). It occurs when the palate of a fetus does not develop properly during the first months of pregnancy.

KEYWORDS

for searching the Internet and other reference sources

Craniofacial syndromes

Otolaryngology

Reconstructive surgery

Mixed Feelings

Tonya and Phil were excited to hear their newborn sons first cry, but they were shocked when they saw him for the first time; baby Philips upper lip was split up the middle. The doctor told them that Philip had a cleft palate as well as a cleft lip, and that both are fairly common birth defects. The doctor reassured Philips parents that Philip was otherwise a very healthy babythe cleft lip and palate would cause Philip some problems, but none that could not be overcome. A plastic surgeon would repair Philips lip and palate, and a team of specialists would work on problems with his teeth, ears, and speech.

What Is a Cleft Palate?

Cleft means gap or split, and the palate is the roof of the mouth. A cleft palate occurs when the roof of the mouth in a fetus* does not develop properly during pregnancy, leaving a hole between the nose and the mouth.

* fetus
(FEE-tus) is the term for an unborn human offspring during the period after it is an embryo, from 9 weeks after fertilization, until childbirth.

The palate extends from the top teeth to the uvula (YOOV-u-la), which is the little piece of tissue that hangs in the back of the throat. There is a bony hard palate (which can be felt just behind the top teeth) and a muscular soft palate (just behind the hard palate). A fully formed palate is necessary to close off the nose and throat from the mouth. It keeps food from going up the nose, and it pushes food to the back of the throat when a person is swallowing. The palate also is important for speaking because it keeps air from going out of the nose instead of the mouth.

The mouth and nose of a fetus develop early during pregnancy: between 5 and 12 weeks. Three developing areas must fuse (close) together to form the face. The pieces that form the palate usually come together like a zipperwhen the growth process is disturbed for some reason, the zipper does not close all the way, leaving a cleft. Formation of the palate and the upper lip are separate processes, but they are linked, and many children born with a cleft palate also have a cleft lip. A cleft lip impairs sucking, speech, and appearance. The size and severity of a cleft depends on how much of the palate or lip has fused together. Clefts can consist of one split down the middle (a unilateral cleft) or two splits (a bilateral cleft).

Why Are Babies Born with Cleft Palates and Cleft Lips?

Cleft palates and lips probably are the result of a number of factors acting together. One in five cases is inherited, probably through the interaction of many different genes*. In most cases, however, clefts seem to be caused by environmental factors that the fetus is exposed to early in pregnancy. The German measles (rubella) virus, other infections, vitamin deficiencies, some medications, alcohol and drug use during pregnancy, all seem to increase the likelihood of a child being born with a cleft lip or palate. Prevention efforts are focused on teaching the public about these risk factors during pregnancy.

* genes
are chemicals in the body that help determine a persons characteristics, such as hair or eye color. They are inherited from a persons parents and are contained in the chromosomes found in the cells of the body.

What Happens to Children with Cleft Palate and Cleft Lip?

Symptoms

An improperly formed palate and/or upper lip affects a childs physical and emotional health in many different ways:

  • A cleft lip is a highly visible disfigurement, and a cleft palate can cause abnormal growth of the face. Uncorrected, they can lead to serious self-esteem problems.
  • When a person speaks, sounds are made by directing air through the nose or the mouth. A complete upper lip also is required to make certain sounds. A cleft palate, however, lets air escape out the nose all of the time, resulting in unusual sounds.
  • A hole in the palate can allow food or liquid to come out of the nose. Usually, the palate acts as a barrier and prevents this problem.
  • Because a cleft palate allows liquid to invade the sinuses and ear tubes, children with cleft palates are prone to ear and sinus infections.
  • Many children with cleft palates and/or cleft lips have dental problems such as missing bone, missing or malformed teeth, and malocclusion (top and bottom teeth that do not fit together properly). Such problems interfere with chewing and cause facial disfigurement.
  • Some children with clefts also have congenital heart problems, growth disorders, or learning problems.

The U.S. and the World

  • Clefts are one of the most common birth defects in the world. Approximately 1 in 700 babies is born with a cleft palate and/or cleft lip.
  • More than 5,000 babies are born in the U.S. each year with these problems.
  • Among people with clefts, 50 percent have both a cleft palate and a cleft lip; 30 percent have only a cleft palate; and 20 percent have only a cleft lip.
  • Boys are twice as likely as girls to have a cleft lip or a cleft lip and palate, whereas girls are twice as likely as boys to have just the cleft palate.
  • People of Asian, European, and Native American ancestry are more prone to clefts than other ethnic groups.
  • Clefts are least likely to occur in people of African ancestry.

Operation Smile

Cleft palate repair requires multiple surgeries, and many people do not have access to or money for the medical care they need. In 1982, Dr. William P. Magee (a plastic surgeon) and his wife Kathleen (a nurse and social worker) founded Operation Smile to help make a difference.

Operation Smile and its volunteers provide free reconstructive (repair and rebuilding) surgery and other medical services for children in the United States and in 20 developing countries. Operation Smile volunteers have performed facial surgery on 50,000 children since 1982. In 1999, Operation Smiles World Journey of Hope 99 helped 5,000 children in 19 countries.

Treatment

Fixing Philips cleft lip and palate will require several surgeries. When he is about three months old, a plastic surgeon will repair his lip by stitching the edges together using flaps of skin from other parts of his mouth. Philip s cleft palate will be repaired when he is between six months and a year old. If Philip is prone to ear infections, the doctor will put tubes in his ears to help drain them of the fluid that leads to infection. These repairs are done as early as possible to prevent hearing and speech problems.

Philip also may require several cosmetic surgeries to adjust his facial features as he grows older. He may need dental work to encourage jawbone growth and to straighten his teeth. He will have his hearing checked frequently by a hearing specialist (an audiologist), and he will work regularly with a speech therapist to learn how to train his palate muscles to work properly.

Philip and his parents will have a lot to deal with over the next several years, but his prognosis is good. The doctor expects that when Philip enters first grade, he will be speaking well, and he will have barely a scar where his cleft lip once was.

See also

Alcoholism

Birth Defects

Genetic Diseases

German Measles (Rubella)

Growth Disorders

Heart Disease

Heart Murmur

Infection

Pregnancy, Complications of

Substance Abuse

Viral Infections

Resources

Books

Berkowitz, Samuel. The Cleft Palate Story: A Primer for Parents of Children with Cleft Lip and Palate. Chicago: Quintessence Press, 1994.

Charkins, Hope. Children with Facial Difference: A Parents Guide. Bethesda: Woodbine House, Inc., 1996.

Organizations

U.S. National Institute of Dental and Craniofacial Research, Building 31, Room 2C35, 31 Center Drive, MSC 2290, Bethesda, MD 20892-2290. http://www.nidcr.nih.gov

American Cleft Palate-Craniofacial Association, 104 South Estes Drive, Suite 204, Chapel Hill, NC 27514. Telephone 800-24-CLEFT or 800-242-5338 http://www.cleft.com

American Speech-Language-Hearing Association, 10801 Rockville Pike, Rockville, MD 20852. Telephone 800-638-8255 or 301-897-5700 (TTY) http://www.asha.org

Operation Smile, 6435 Tidewater Drive, Norfolk, VA 23509. (757) 321-7645. http://www.operationsmile.org

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cleft palate

cleft palate Congenital deformity in which there is an opening in the roof of the mouth, causing direct communication between the nasal and mouth cavities. It is often associated with harelip and makes normal speech difficult. Usual treatment includes surgical correction, followed by special dental care and speech therapy if necessary.

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cleft palate

cleft palate n. a fissure in the midline of the palate due to failure of the two sides to fuse in embryonic development. Only part of the palate may be affected, or the cleft may extend the full length with bilateral clefts at the front of the maxilla; it may be accompanied by a cleft lip and disturbance of tooth formation.

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cleft palate

cleft pal·ate • n. a congenital split in the roof of the mouth.

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