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. d. Palate cleft can occur as complete (soft and hard palate, possibly including a gap in the jaw) or incomplete (a 'hole' in the roof of the mouth, usually as a cleft soft palate). 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. The incidence is highest in Asians, followed by Caucasians. Clefts of the lip with or without cleft palate ate more common in males while clefts of the palate alone are more common in females Possible etiologies include genetic and environmental factors. If there is a family history of a cleft, the risk of other children also having a cleft is higher. Environmental factors includes parental age, maternal intake of excessive alcohol, maternal drug exposure and dietary factors such as folic acid and vitamin deficiencies. 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. 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. Laboratory and diagnostic study findings. Traditionally, the diagnosis is made at the time of birth by physical examination. Recent advances in prenatal diagnosis have allowed obstetricians to diagnose facial clefts in utero by ultrasound If the disorder is not discovered until birth, a cleft lip is repaired surgically shortly thereafter, often at the time of the initial hospital stay or between 2 and 10 weeks of age. Some infants may have a nasal mold apparatus applied before surgery to shape a better nostril, early repair also helps infants experience the pleasure of sucking as soon as possible A two-stage palate repair with soft palate repair at 3-6 months of age and hard palate repair at 15- 18 months of age may be recommended Do not use the older term for this condition, harelip, when talking with parents about the problem. The word harelip tends to be associated with these negative outcomes like large lip scars, gross speech impediments and a poor appearance after surgery 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 infant’s and parents’ emotional and social adjustment. a. Help facilitate the family’s 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 infant’s positive aspects and express optimism regarding surgical correction. 4. Provide preoperative care. a. Depending in the defect and the child’s 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 physician’s 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 olderchildren, 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. Cleft lip and palate is very treatable; however, the kind of treatment depends on the type and severity 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. Most children with a form of clefting are monitored by a cleft palate team or craniofacial team through young adulthood. Treatment procedures can vary between craniofacial teams. Within the first 2–3 months after birth, surgery is performed to close the cleft lip. While surgery to repair a cleft lip can be performed soon after birth, the often preferred age is at approximately 10 weeks of age. The most common procedure to repair a cleft lip is the Millard procedure pioneered by Ralph Millard. Often a cleft palate is temporarily closed, the cleft isn't closed, but it is covered by a palatal obturator (a prosthetic device made to fit the roof of the mouth covering the gap). Cleft palate can also be corrected by surgery, usually performed between 6 and 12 months. One of the new innovations of cleft lip and cleft palate repair is the Latham appliance. The Latham is surgically inserted by use of pins during the child's 4th or 5th month. After it is in place, the doctor, or parents, turn a screw daily to bring the cleft together to assist with future lip and/or palate repair.