Cleft lip and cleft palate — an opening in the lip or palate — may occur separately or in combination. These deformities originate in the 2nd month of pregnancy, when the front and sides of the face and the palatine shelves fuse imperfectly. Cleft deformities usually occur unilaterally or bilaterally, rarely midline. Only the lip may be involved, or the defect may extend into the upper jaw or nasal cavity.
Cleft lip and cleft palate occur in twice as many males as females; isolated cleft palate is more common in females.
Cleft lip or palate can occur as part of another chromosomal or Mendelian abnormality (more than 150 have been identified); however, exposure to teratogens during fetal development or a combination of genetic and environmental factors may also produce these defects.
Cleft lip with or without cleft palate occurs in about 1 in 1,000 births among Whites; the incidence is higher among Asians (1.7 in 1,000) and Native Americans (more than 3.6 in 1,000) but lower among Blacks (1 in 2,500). A positive family history is a risk factor for cleft defects.
Congenital defects of the face usually occur in the upper lip. They range from a simple notch to a complete cleft from the lip edge through the floor of the nostril, on either side of the midline, but rarely along the midline itself.
A cleft palate may be partial or complete. A complete cleft includes the soft palate, the bones of the maxilla, and the alveolus on one or both sides of the premaxilla. A double cleft runs from the soft palate forward to either side of the nose, separating the maxilla and premaxilla into freely moving segments. In Pierre Robin syndrome, the tongue is displaced due to micrognathia. The tongue prevents the palate from fusing. Glossoptosis coexists with cleft palate.
Isolated cleft palate is more commonly associated with other congenital defects than isolated cleft lip or cleft lip and cleft palate.
A typical clinical picture confirms the diagnosis. Cleft lip with or without cleft palate is obvious at birth; occasionally, more severe defects may be seen with diagnostic prenatal ultrasonography. Isolated cleft palate may not be detected until a mouth examination is done or until feeding difficulties develop. (See Variations of cleft lip and cleft palate, page 202.)
Treatment consists of surgical correction, but the timing of surgery varies. Some plastic surgeons repair cleft lips within the first few days of life to make feeding the baby easier. However, many surgeons delay lip repairs for 8 to 10 weeks (sometimes as long as 6 to 8 months) to allow time for maternal bonding and, most important, to rule out associated congenital anomalies.
Cleft palate repair is usually completed by the 12th to 18th month. Still other surgeons repair cleft palates in two steps, repairing the soft palate between ages 6 and 18 months and the hard palate as late as age 5 years. In any case, surgery is performed only after the infant is gaining weight and is infection-free.
Surgery must be coupled with speech therapy. Because the palate is essential to speech formation, structural changes, even in a repaired cleft, can permanently affect speech patterns. To compound the problem, many children with cleft palates have hearing difficulties because of middle ear damage or infections.
Clinical tip Research has indicated that ingestion of 0.4 mg of folic acid daily before conception decreases the risk of isolated cleft lip or palate by up to 25%. Therefore, all women of childbearing age should be encouraged to take a daily multivitamin containing folic acid until menopause or until they’re no longer fertile.
❑ Although most infants with a cleft palate can sleep on their backs without difficulty, an infant with Pierre Robin syndrome risks having the tongue fall back and obstruct the airway if placed in this position.
❑ Make sure the infant receives adequate nutrition to ensure normal growth and development. Experiment with feeding devices. A baby with a cleft palate has an excellent appetite but often has trouble feeding because of air leaks around the cleft and nasal regurgitation.
❑ Teach the mother how best to feed her infant. Advise her to hold the infant in a near-sitting position, with the flow directed to the side or back of the baby’s tongue. Tell her to burp the baby frequently because the cleft palate will cause him to swallow a lot of air. If the underside of the nasal septum becomes ulcerated and the child refuses to suck because of the pain, instruct the mother to give the mucosa time to heal. Tell her to gently clean the palatal cleft with a cotton-tipped applicator dipped in half-strength hydrogen peroxide or water after each feeding.
❑ Encourage the mother of a baby with cleft lip to breast-feed if the cleft doesn’t prevent effective sucking. Breast-feeding an infant with a cleft palate or one who has just had corrective surgery usually isn’t possible. (Postoperatively, the infant can’t suck for up to 6 weeks.) However, if the mother desires, suggest that she use a breast pump to express breast milk and then feed it to her baby from a bottle.
❑ After surgery, record intake and output and maintain good nutrition. To prevent atelectasis and pneumonia, the doctor may gently suction the nasopharynx (this may be necessary before surgery, too).
❑ Elbow restraints allow the baby to move his hands while keeping them away from his mouth. When necessary, use an infant seat to keep the child in a comfortable sitting position.
❑ Help the parents deal with their feelings about the child’s deformity. Start by telling them about it and showing them their baby as soon as possible. Because society places undue importance on physical appearance, many parents feel shock, disappointment, and guilt when they see the child. Help them by being calm and providing positive information.
❑ Direct the parents’ attention to their child’s assets. Stress the fact that surgical repairs can be made. Include the parents in the care and feeding of the child right from the start to encourage normal bonding. Provide the instructions, emotional support, and reassurance that the parents will need to take proper care of the child at home.
❑ Refer the parents to a social worker who can guide them to community resources if needed and to a genetic counseling service to determine the recurrence risk.
Review other book chapters online related to Aase Smith syndrome:
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Handbook of Diseases Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2003 ISBN: 1-58255-266-5 
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