Cleft Palate and Cleft Lip Introduction Cleft lip and cleft palate are facial and oral

malformations that occur very early in pregnancy, while the baby is developing inside its mother. Clefting results when there is not enough tissue in the mouth or lip area, and the tissue that is available does not join together properly. A cleft lip is a physical split or separation of the two sides of the upper lip and appears as a narrow opening or gap in the skin of the upper lip. This separation often extends beyond the base of the nose and includes the bones of the upper jaw and/or upper gum. A cleft palate is a split or opening in the roof of the mouth. A cleft palate can involve the hard palate (the bony front portion of the roof of the mouth), and/or the soft palate (the soft back portion of the roof of the mouth). Cleft lip and cleft palate can occur on one or both sides of the mouth. Because the lip and the palate develop separately, it is possible to have a cleft lip without a cleft palate, a cleft palate without a cleft lip, or both a cleft lip and cleft palate together. Who Gets Cleft Lip and Cleft Palate? Cleft lip, with or without cleft palate, affects one in 700 babies annually, and is the fourth most common birth defect in the U.S. Clefts occur more often inchildren of Asian, Latino, or Native American descent. Compared with girls, twice as many boys have a cleft lip, both with and without a cleft palate. However, compared with boys, twice as many girls have cleft palate without a cleft lip. What Causes a Cleft Lip and Cleft Palate? In most cases, the cause of cleft lip and cleft palate is unknown. These conditions cannot be prevented. Most scientists believe clefts are due to a combination of genetic and environmental factors. There appears to be a greater chance of clefting in a newborn if a sibling, parent, or relative has had the problem. Another potential cause may be related to a medication a mother may have taken during her pregnancy. Some drugs may cause cleft lip and cleft palate. Among them: anti-seizure/anticonvulsant medications, acnemedications containing Accutane, and methotrexate, a drug commonly used for treating cancer, arthritis, and psoriasis. Cleft lip and cleft palate may also occur as a result of exposure to viruses or chemicals while the fetus is developing in the womb. In other situations, cleft lip and cleft palate may be part of another medical condition. What Problems Are Associated With Cleft Lip and/or Cleft Palate?

Eating problems. With a separation or opening in the palate, food and liquids can pass from the mouth back through the nose. Fortunately, specially designed baby bottles and nipples that help keep fluids flowing downward toward the stomach are available. Children with a cleft palate may need to wear a man-made palate to help them eat properly and ensure that they are receiving adequate nutrition until surgical treatment is provided.

Ear infections/hearing loss. Children with cleft palate are at increased risk of ear infections since they are more prone to fluid build-up in the middle ear. If left untreated, ear infections can cause hearing loss. To prevent this from happening, children with cleft palate usually need special tubes placed in the eardrums to aid fluid drainage, and their hearing needs to be checked once a year.

Speech problems. Children with cleft lip or cleft palate may also have trouble speaking. These children's voices don't carry well, the voice may take on a nasal sound, and the speech may be difficult to understand. Not all children have these problems and surgery may fix these problems entirely for some. For others, a special doctor, called speech pathologist, will work with the child to resolve speech difficulties.

Dental Problems. Children with clefts are more prone to a larger than average number of cavities and often have missing, extra, malformed, or displaced teeth requiring dental and orthodontic treatments. In addition, children with cleft palate often have an alveolar ridge defect. The alveolus is the bony upper gum that contains teeth. A defect in the alveolus can (1) displace, tip, or rotate permanent teeth, (2) prevent permanent teeth from appearing, and (3) prevent the alveolar ridge from forming. These problems can usually be repaired through oral surgery.

. Description 1. Cleft lip (cheiloschisis) is a congenital anomaly that occurs at a rate of 1 in 800 births. a. If the cleft does not affect the palate structure of the mouth it is referred to as cleft lip. b. Cleft lip is formed in the top of the lip as either a small gap or an indentation in the lip (partial or incomplete cleft) or it continues into the nose (complete cleft) c. Cleft lip can be unilateral or bilateral. d. It is due to the failure of fusion of the maxillary and medial nasal processes (formation of the primary palate). 2. Cleft palate (palatoschisis) is a congenital anomaly that occurs in approximately 1 of every 2000 births, and it is more common in boys than girls.

a. It is a condition in which the two plates of the skull that form the hard palate (roof of the mouth) are not completely joined. b. It ranges in severity from soft palate involvement alone to a defect including the hard palate and portions of the maxilla. c. Cleft palate may or may not be associated with cleft lip. 3. Children with these structural disorders may have associated dental malformations, speech problems, and frequent otitis media, the latter resulting from improper functioning of the Eustachian tubes. B. Etiology 1. Many factors are associated with the development of cleft lip and cleft palate, and cleft lip with or without cleft palate is developmentally and genetically different from isolated cleft palate. 2. Most cases appear to be consistent with the concept of multifactorial inheritance as evidenced by an increase incidence in relatives and monozygotic twins. C. Pathophysiology 1. During embryonic development the lateral and medial tissues forming the upper lip palates fuse between weeks 7 and 8 of gestation; the palatal tissues forming the hard and soft palates fuse between weeks 7 and 12 gestation. 2. Cleft lip and cleft palate result when these tissues fail to fuse. D. Assessment findings 1. Clinical manifestations a. Cleft lip and cleft palate are readily apparent at birth. Careful physical assessment should be performed to rule out other midline birth defects. b. Cleft lip and cleft palate appear as incomplete or complete defects, and may be unilateral or bilateral. 2. Laboratory and diagnostic study findings. Obstetric ultrasound will reveal cleft lipwhile the infant is in utero. E. Nursing management 1. Assess for problems with feeding, breathing parental bonding, and speech. 2. Ensure adequate nutrition and prevent aspiration. a. Provide special nipples or feeding devices (eg, soft pliable bottle with soft nipple with enlarged opening) for a child unable to suck adequately on standard nipples. b. Hold the child in a semiupright position; direct the formula away from the cleft and toward the side and back of the mouth to prevent aspiration.

c. Feed the infant slowly and burp frequently to prevent excessive swallowing of air and regurgitation. d. Stimulate sucking by gently rubbing the nipple against the lower lip. 3. Support the infants and parents emotional and social adjustment. a. Help facilitate the familys acceptance of the infant by encouraging the parents to express their feelings and concerns and by conveying an attitude of acceptance toward the infant. b. Emphasize the infants positive aspects and express optimism regarding surgical correction. 4. Provide preoperative care. a. Depending in the defect and the childs general condition, surgical correction of the cleft lip usually occurs at 1 to 3 months of age; repair of the cleft palate is usually performed between 6 and 18 months of age. Repair of the cleft palate may require several stages of surgery as the child grows. b. Early correction of cleft lip enables more normal sucking patterns and facilitates bonding. Early correction of cleft palate enables development of more normal speech patterns. c. Delayed closure or large defects may require the use of orthodontic appliances. d. The responsibilities of the nurse are to: 1. Reinforce the physicians explanation of surgical procedures. 2. Provide mouth care to prevent infection. 5. Provide postoperative care.

a. Assess airway patency and vital signs; observe for edema and respiratory distress. b. Use a mist tent, if prescribed, to minimize edema, liquefy secretions, and minimize distress. c. Position the child with cleft lip on her back, in an infant seat, or propped on a side to avoid injury to the operative site; position the child with a cleft palate on the abdomen to facilities drainage. d. Clean the suture line and apply an antibacterial ointment as prescribed to prevent infection and scarring. Monitor the site for signs of infection. e. Use elbow restraints to maintain suture line integrity. Remove them every 2 hours for skin care and range-of-motion exercises. f. Feed the infant with a rubber-tipped medicine dropper, bulb syringe, Breck feeder, or soft bottle-nipples, as prescribed, to help preserve suture integrity. For older children, diet progresses from clear fluids; they should not use straws or sharp objects. g. Attempt to keep the child from putting tongue up to palate sutures. h. Manage pain by administering analgesic as prescribed. 6. Provide child and family teaching. Demonstrate surgical wound care. Show proper feeding techniques and positions. Explain that temperature of feeding formulas should be monitored closely because new palate has no nerve endings; therefore; the child can suffer a burn to the palate easily and without knowing it. Explain handling of prosthesis if indicated. Stress the importance of long-term follow up, including speech therapy, and preventing or correcting dental abnormalities. Discuss the need for, at least, annual hearing evaluations because of the increased susceptibility to recurrent otitis. The child may require myringotomy and surgical placement of drainage tubes. Teach infection control measures

Cause

The development of the face is coordinated by complex morphogenetic events and rapid proliferative expansion, and is thus highly susceptible to environmental and genetic factors, rationalising the high incidence of facial malformations. During the first six to eight weeks of pregnancy, the shape of the embryo's head is formed. Five primitive tissue lobes grow: a) one from the top of the head down towards the future upper lip; (Frontonasal Prominence) b-c) two from the cheeks, which meet the first lobe to form the upper lip; (Maxillar Prominence) d-e) and just below, two additional lobes grow from each side, which form the chin and lower lip; (Mandibular Prominence) If these tissues fail to meet, a gap appears where the tissues should have joined (fused). This may happen in any single joining site, or simultaneously in several or all of them. The resulting birth defect reflects the locations and severity of individual fusion failures (e.g., from a small lip or palate fissure up to a completely malformed face). The upper lip is formed earlier than the palate, from the first three lobes named a to c above. Formation of the palate is the last step in joining the five embryonic facial lobes, and involves the back portions of the lobes b and c. These back portions are called [19] palatal shelves, which grow towards each other until they fuse in the middle. This process is very vulnerable to multiple toxic substances, environmental pollutants, and nutritional imbalance. The biologic mechanisms of mutual recognition of the two cabinets, and the way they are glued together, are quite complex and obscure despite intensive scientific research