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
Infants 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.
Cleft palates are openings in the palate, which is the roof of the mouth. The size and position of the opening varies. The cleft may only be in the hard palate, the bony portion of the roof of the mouth opening into the floor of the nose, or it may only occur 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.
Cleft lips can develop with or without cleft palates. Cleft palates may also occur without cleft lips.
Genetic profile
Cleft lip 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 increases with 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 also increase the incidence of clefting, such as phenytoin, sodium valproate, and methotrexate. The pregnant mother's nutrition may affect the incidence of clefting as well.
Demographics
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 two in 1,000. The incidence among caucasians is one in 1,000 newborns. African Americans have an incidence of 0.3 in 1,000 newborns.
Signs and symptoms
Babies born with a cleft lip will 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 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.
Infants with cleft lips and palates have feeding difficulties, which are more severe in those 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 which 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. After birth, cleft lip and palate are diagnosed by physical exam.
Treatment and management
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 patients 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 breast feed 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. Orthodontic treatment for cleft lip should begin 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 judgement 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 plate and center where the procedure is being performed. Depending on 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
Individuals 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.
PERIODICALS
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Christensen, Karr. "The 20th Century Danish Facial Cleft Population–Epidemiological and Genetic-Epidemiolical Studies." Cleft Palate–Craniofacial 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.
Denk, Michael J. "Topics in Pediatric Plastic Surgery." Pediatric Clinics of North America 45 (December 1998).
Litwak-Saleh, Kim. "Practical Points in the case of the Patient with post-Cleft lip repair." Journal of Post Anesthesia Nursing 8 (February 1993): 35-37.
Rohrich, Rod J. "Optimal Timing of Cleft Palate Closure." Plastic and Reconstructive Surgery 106 (August 2000): 413-421.
