CLEFT LIP AND PALATE

Introduction: Cleft lip, cleft palate, and cleft lip and palate are separate anomalies that are closely related in etiology, pathophysiology, and nursing care. These distinct problems are all abnormal openings in the lip OP palate. The defects may occur unilaterally (on either side) or bilaterally and are the most common congenital craniofacial deformity.

Incidence The incidence ranges from 1 in 700 to 1000 births for cleft lip and palate and 1 in 2000 for cleft palate atone (Cleft Palate Foundation, 2005; March of Dimes, 2006). Cleft lip is seen predominantly in male infants and cleft palate in female infants. The prevalence of clefts is higher in Asians and Native Americans and has a lower frequency in African Americans. A genetic pattern or familial risk seems to exist (Tinaroff, 2004). Many infants who are affected with cleft lip and palate have other associated defects.

Definition: Cleft lip (cheiloschisis) and cleft palate (palatoschisis), which can also occur together as cleft lip and palate, are variations of a type of cleftingcongenital deformity caused by abnormal facial development during gestation. A cleft is a fissure or openinga gap. It is the non-fusion of the body's natural structures that form before birth. Approximately 1 in 700 children born have a cleft lip or a cleft palate or both. In decades past, the condition was sometimes referred to as harelip, based on the similarity to the cleft in the lip of a hare, but that term is now generally considered to be offensive.

Manifestations and Diagnostic Evaluation Cleft lip has the following manifestations: a notched vermilion border, variably sized clefts that involve the alveolar ridge, and dental anomalies (usually deformed, supernumerary, or absent teeth). Cleft palate includes nasal distortion, midline or bilateral cleft with variable extension from the uvula and soft and hard palates, and exposed nasal cavities. The diagnosis of cleft lip and cleft palate is based on observation at birth and. complete examination in the neonatal period. Diagnosis may also be made in taero with ultrasound. Cleft lip is readily diagnosed through inspection of the lip. The first sign of cleft palate may be formula coming from the nose. A gloved finger placed in the mouth to feel the defect or visual examination with a flashlight confirms the diagnosis.

Therapeutic Management Management is based on the severity of the defect. A number of professionals are involved in this process, including surgeons; nurses; geneticists; psychologists or psychiatrists; ear, nose, and throat specialists; audiologists; and occupational and speech therapists. Orthodontists and plastic surgeons become involved in the lengthy management. Pediatricians provide ongoing child health care. The first intervention involves modifying feeding techniques as needed to allow adequate growth. Use of special feeding techniques, obturators, and unique nipples and feeders can usually accomplish this goal and allow early discharge with parents . These modified techniques can decrease the energy required for the infant to take in adequate nutrition. Before surgical repair, removable orthopedic devices such as a Latham device may be used to expand and realign parts of the palate or decrease the size of a wide lip cleft. Cleft lip repair is usually performed by age 3 to 6 months. Early repair may

improve bonding and makes feeding much easier. The surgical technique involves the use of a staggered suture line to minimize scarring. Some cosmetic modifications may be needed again at age 4 to 5 years. Cleft palate repair is individualized and based on the degree of deformity and size of the child. Closure is completed between ages 6 and 24 months. Most teams recommend repair by 1 year. Earlier closure facilitates speech development. Concurrent treatment of altered dentition, recurring otitis media, speech dysfunction, emotional issues, and cosmetic concerns completes the ongoing therapy. Children with cleft palate are at high risk for developing chronic otitis media, which can cause long-term hearing loss.

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.

PATHOPHYSIOLOGY Cleft lip and cleft palate occur from embryonic developmental failures related to multiple genetic and environmental factors. These developmental failures result in an abnormal opening in the lip, palate and, sometimes, nasal cavity. Cleft lip results when the medial nasal and maxillary processes fail to join at 6 to 8 weeks of gestation. Cleft palate results from failure of the primary palatal shelves, or processes, to fuse at 7 to 12 weeks of gestation. Each of these abnormalities appears as a distinct mal- ! formation, but they may al'so appear together. Achieving suction during feedings may be impossible,

and fluids may enter the nose, putting the child at risk for aspiration, feeding difficulties, and respiratory distress.

Diagnosis 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.

Treatment Cleft lip and palate is very treatable; however, the kind of treatment depends on the type and severity of the cleft. Most children with a form of clefting are monitored by a cleft palate team or craniofacial teamthrough young adulthood. Care can be lifelong. Treatment procedures can vary between craniofacial teams. For example, some teams wait on jaw correction until the child is aged 10 to 12 (argument: growth is less influential as deciduous teeth are replaced by permanent teeth, thus saving the child from repeated corrective surgeries), while other teams correct the jaw earlier (argument: less speech therapy is needed than at a later age when speech therapy becomes harder). Within teams, treatment can differ between individual cases depending on the type and severity of the cleft. Within the first 23 months after birth, surgery is performed to close the cleft lip. While surgery to repair a cleft lip can be performed soon after birth, often the preferred age is at approximately 10 weeks of age, following the "rule of 10s" coined by surgeons Wilhelmmesen and Musgrave in 1969 (the child is at least 10 weeks of age; weighs at least 10 pounds, and has at least 10g hemoglobin). If the cleft is bilateral and extensive, two surgeries may be required to close the cleft, one side first, and the second side a few weeks later. The most common procedure to

repair a cleft lip is the Millard procedure pioneered by Ralph Millard. Millard performed the first procedure at a Mobile Army Surgical Hospital (MASH) unit in Korea. Often an incomplete cleft lip requires the same surgery as complete cleft. This is done for two reasons. Firstly the group of muscles required to purse the lips run through the upper lip. In order to restore the complete group a full incision must be made. Secondly, to create a less obvious scar the surgeon tries to line up the scar with the natural lines in the upper lip (such as the edges of thephiltrum) and tuck away stitches as far up the nose as possible. Incomplete cleft gives the surgeon more tissue to work with, creating a more supple and natural-looking upper lip.

Pre-surgical devices In some cases of a severe bi-lateral complete cleft, the premaxillary segment will be protruded far outside the mouth. Nasoalveolar molding prior to surgery can improve long-term nasal symmetry among patients with complete unilateral cleft lip-cleft palate patients compared to correction by surgery alone, according to a retrospective cohort study.[42] In this study, significant improvements in nasal symmetry were observed in multiple areas including measurements of the projected length of the nasal ala (lateral surface of the external nose), position of the superoinferior alar groove, position of the mediolateral nasal dome, and nasal bridge deviation. "The nasal ala projection length demonstrated an average ratio of 93.0 percent in the surgeryalone group and 96.5 percent in the nasoalveolar molding group" this study concluded.

Often a cleft palate is temporarily 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. Approximately 2025% only require one palatal surgery to achieve a competent velopharyngeal valve capable of producing normal, non-hypernasal speech. However, combinations of surgical methods and repeated surgeries are often necessary as the child grows. One of the new innovations of cleft lip and cleft palate repair is theLatham appliance.[43] 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. If the cleft extends into the maxillary alveolar ridge, the gap is usually corrected by filling the gap with bone tissue. The bone tissue can be acquired from the patients own chin, rib or hip.

NURSING CARE PLAN Focused Assessment Cleft lip and cleft palate are readily apparent at birth, and the degree of involvement should be documented during the newborn examination. After identifying this condition, assess the infant's ability to suck, swallow, and breathe without distress and handle normal secretions. Because the occurrence of cleft lip and palate is usually unexpected and its appearance can be frightening to parents and families, assess and record parents' reactions as well as their interactions with the neonate. Parents of an infant with a cleft lip or palate may need help to resolve feelings about their infant's appearance. The parents might need to deal with many question's

from family members, stares from strangers, and expressions of pity from other new parents. Providing information about the cause of the defect and showing them pictures of other children before and after surgical repair can give them some relief from these fears and concerns. In addition, modeling and encouraging bonding through touching, holding, and examining their newborn can be very reassuring. Pointing out the newborn's positive attributes can help decrease the focus on the defect. For example, emphasize how alert the baby is or the infant's responsiveness or beautiful eyes.