John A. Nackashi
E. Rosellen Dedlow
Virginia Dixon-Wood
Children with cleft lip with or without palate (CL/P) have a wide array of needs. The most obvious consequence of CL/P is the facial difference. The impact of the cleft condition on the child and family can vary considerably. The child's long-term outcome is influenced by many factors, which include the underlying condition, strengths of the child and family, access to services, and completion of a comprehensive treatment plan. Children with CL/P have all of the needs and strengths of typical children, but many have special needs related to the cleft condition. Children with clefts can be referred to as children with special health care needs, i.e., “those who have or are at elevated risk for chronic physical, developmental, behavioral, or emotional conditions and who also require health and related services of a type or amount not usually required by children” (Maternal and Child Health Bureau, 1995). This definition helps to identify children who may need increased services. Through this identification process and to provide care, government agencies and health policymakers are given the foundation to build systems of care, health benefit packages, and services. For the clinician, recognizing that a child has special needs directs and emphasizes the importance of comprehensive and coordinated services.
The presence of CL/P is a marker indicating that there may be more associated concerns, which may be more significant and of greater consequence to the child than the cleft itself. Clefts may be associated with syndromes, early feeding problems, airway compromise, growth and developmental abnormalities, speech disorders, recurrent ear infections, and psychosocial concerns. An understanding of the underlying condition helps in predicting the complexity of the child's needs and possible outcomes. A child may have essentially no major concerns or may be unusually complicated. Early identification of a syndrome is extremely important and represents the first step in determining the child's needs and the complexity of care. Due to the increased risk for medical, developmental, psychological, surgical, and dental problems, a team of CL/P specialists can best serve the child and family. A team can also support the child's community providers in the provision of local healthcare services. Pediatric primary care providers, specialists, and cleft palate teams need to recognize that children with CL/P have the same needs as typical children in addition to their special needs. This conceptual framework provides the foundation for care.
Key times for care: a General Perspective
There are key times for the child and family that are associated with significant medical, development, psychosocial, surgical, and dental needs. The care for children with CL/P is very age-dependent. This is important to know because the final outcome is dependent on the correct timing of evaluations and procedures. As a child becomes older, the next step in care is built upon the prior completed step. During the life of the child, there are periods of time, which extend from weeks to years, in which a specific type of need (i.e., feeding, surgical, speech, or dental services) best describes the goals of treatment. There are also periods of time in which the only intervention is monitoring. Recognition of these key times is very useful in communicating with families to help them develop a frame of reference about their child's care and the treatment plan. These key times are linked to the specific goals and interventions.
There is a great commonality of needs among children and across cleft conditions and associated health concerns, but there also may be great variation of needs depending on the specific child and family. The following is a simplified way to view care for children with CL/P, providing a big-picture perspective that should be helpful to families. There is some variation in the timing of surgery by various centers and teams.
First Month
The first month is the time for evaluations to make the diagnosis, counsel families, and provide instruction to safely feed the infant. Weekly weight checks are recommended to monitor weight gain and ensure that the infant reaches birth weight by at least 3 weeks of age. A critical role for healthcare professionals is communication, information sharing, and referral to services.
Birth to 2 Years
For many children, the majority of surgery is completed by 18 months. In many centers, the lip is repaired by 3 months and the palate by 1 year. For all children, this is a time for screening growth and development with a special emphasis on language and speech, growth, middle ear disease, and hearing.
Age 2 to 7 Years
Screening of development with a special emphasis on speech and cognitive and psychosocial functioning is a pivotal need between the ages of 2 and 7. Initiation of dental care sets the stage for most of the treatment needs for the adolescent and teenage years. For many children, the occurrence of otitis media begins to wane. Some children continue to have middle ear disease and hearing loss. There are a significant number of children who require interventions for speech, development, and cognitive and psychosocial needs. Some children require surgical procedures to correct speech. Some families choose to have minor surgical lip and nose revisions.
Age 7 to Teenage Years
For children who have completed the needed and appropriately timed procedures, the primary needs are now dental. Dental care provides the final and necessary step to have the best long-term outcome. From the age of 7 to the teen years a period of extensive dental bracing is required. Bone grafting is routinely done to allow teeth to be moved into the cleft. Many families will choose this time period to have minor, “touch-up” surgery on the nose and lip. Due to a significant dental malocclusion, some children will have orthognathic surgery when they are finished growing, usually after 16 years of age. For most children, the treatment is now complete.
The Birth Experience and First Clinical Encounter
The birth of an infant with CL/P is usually unexpected. Prenatal diagnosis is becoming more common with transvaginal sonography. Accurate detection of a cleft lip is possible at 13 to 16 weeks' gestation. Cleft palate is difficult to detect in utero due to its posterior and midcephalic location (Habel et al., 1996). Prenatal diagnosis of CL/P represents only a very small number of children. As a result, families and healthcare providers are not prepared. In addition, healthcare providers commonly do not know the significance of the CL/P, what to tell the family, and how to provide care for the infant. Families are looking for a healthy baby and certainly not one that looks different and is difficult to feed. They are expecting healthcare providers who are confident and reassuring rather than confused and uncertain.
Many parents will describe their reaction to the birth of their baby with a cleft, especially when it involves a cleft lip, as one of “shock.” A consistent finding in studies involving the birth and diagnosis of CL/P is the families reporting shock, guilt, anxiety, and depression (Strauss, 1997). Families commonly state that there appears to be a great deal of confusion associated with the birth of their baby. Factors contributing to the difficulties encountered during the birth include cultural beliefs, the birthing experience, the level of experience of healthcare providers with CL/P infants, and the quality of communication with the family. Typically, the medical and nursing staff has little opportunity to develop competency in the early management of infants with CL/P, even though it is one of the most frequent congenital defects. The incidence of infants with CL/P is approximately 1/700 births in North America. Numerous community hospitals may have fewer than 1 or 2/1000 births in a year. Based on the incidence of cleft lip and palate, there may be only one or two such infants born in a year. In some community hospitals, a baby with CL/P may be delivered only every few years. Due to the limited number of births, physicians do not develop a high level of experience in providing care for the infant with CL/P. Typically, there is turnover of nursing and other hospital staff, which adds to the problem of developing competency in the care of newborns with clefts.
For many years, literature on children with disabilities has shown that families have expressed major concerns about how physicians communicate and deliver needed information. A study on parental recall about how they were told of their child's diagnosis of CL/P (Strauss, 1997) revealed that there were significant differences between what parents experienced and what they wanted in their communication with physicians. Parents related the need for more time to talk, more of an opportunity to show their feelings, and more of an effort on the part of the physician to make them feel better. Parents wanted more information, referral to another parent of a child with CL/P, and more opportunity to discuss the possibility of mental retardation (Strauss et al., 1995).
The first clinical encounter with healthcare providers has profound significance for the child and family. Since the birth of an infant with CL/P is typically unexpected, the role of the pediatrician or other healthcare providers is complicated by the special needs of the infant and the emotional and psychosocial impact on the family.
Pediatric providers have a critical role that involves evaluating the medical and feeding needs of the infant. The first responsibility is to evaluate the infant's medical stability and need for immediate treatment, referral to specialists, and/or transferral to a neonatal intensive care unit (NICU). Once it is determined that the infant is medically stable (which represents the majority of infants), the next step is to search for the presence of major and minor malformations or findings suggesting an associated syndrome. This involves a detailed review of the infant's medical history and the family's history of CL/P, syndromes, congenital defects, or conditions that have affected children. The prenatal history is important to elicit information about prenatal care, illnesses, chronic conditions, use of medications, use of alcohol, use of tobacco, or substance abuse during pregnancy. A newborn is examined with an emphasis on detecting subtle variations and obvious anomalies while focusing on the craniofacial, cardiac, skeletal, and genitourinary systems. Meticulous observation and documentation is the key to quickly determining the correct diagnosis. At the same time, the pediatrician should not feel pressured to make a definitive diagnosis. Geneticists and cleft palate team experts are best at making a final diagnosis. The detailed birth history and physical examination will enable the geneticist to determine if the cleft is a sporadic occurrence, associated with a syndrome, genetically transmitted through single-gene or multifactorial inheritance, chromosomal in nature, or caused by teratogens (Domet, 1986). References are available which will be of assistance in determining whether physical exam findings suggest a syndrome (Pashayan, 1983; Smith, 1997) and which link major and minor anomalies with syndromes (Pashayan, 1983; Smith, 1997). For the infant who has findings suggesting the presence of a syndrome, genetic consultation should be obtained as soon as possible.
If the history reveals no concerns and the physical exam is normal, the pediatric provider can tell the family that the baby most likely has an isolated CL/P. The family can be told that the infant can be referred to a cleft palate team and that comprehensive services directed by the team can maximize the outcome for the child. Even though a child appears to have only an isolated CL/P, many infants will benefit from a genetics evaluation, which can be scheduled at a convenient time for the family. This is an important time for families to gain information about CL/P in general and about their infant's specific needs. They may also have questions about future childbearing, which can be addressed by the genetics counselor. Pamphlets, web sites, and contact with families who have a child with CL/P are a great help to the family. These resources are an effective way to provide information and support to the family. A critical responsibility at the first clinical encounter is assessing the infant's feeding skills.
Provision of Pediatric Health Services: Community and Cleft Palate Team
All children with oral clefts should have primary care through a community medical home that provides continuity of care which is accessible, compassionate, and culturally competent. The American Academy of Pediatrics and the National Center of Medical Home Initiatives for Children with Special Health Care Needs promote this concept for children with special healthcare needs. The primary care well-child evaluation consists of a history, review of medications, allergies, immunizations, development and psychosocial status, family and social history, complete physical exam, anticipatory guidance, laboratory tests, immunizations, clinical impression, plan, and follow-up.
Coordinated, comprehensive services are best provided by a partnership between the primary care provider, the cleft palate team, and the family. This partnership is critical for the optimum outcome. Pediatric healthcare providers may be pediatricians, family practitioners, pediatric nurse practitioners, and physician assistants. The functions of the community and team pediatric healthcare providers overlap, but the roles are different. The community pediatrician uses this comprehensive approach to provide primary care. The cleft palate team pediatrician uses this comprehensive approach to gain a familiarity with the child and to support the team's evaluation and the development of an appropriate and safe treatment plan. The team benefits from the pediatrician's ability to give advise about special health concerns, the need for diagnostic evaluations and treatments for known or previously undetected problems, general developmental and psychosocial functioning, and the determination that surgery and procedures can be safely conducted. The team pediatrician has a unique role of overseeing the total needs of the child during the team evaluation and treatment process.
Roles and Responsibilities of the Primary Care Provider
Provide Early Identification and Diagnosis of Cleft Lip with or without Palate and Associated Conditions and Refer to Appropriate Specialists
The primary care provider can support the family following the prenatal diagnosis by collaborating with geneticists and specialists. Key responsibilities include aiding the communication and parental education process so that the family understands the significance of the suspected condition for the life of the child and family, required treatments, complexity of care, probable outcome with appropriate care, and the family's options. The primary care provider's role is to facilitate communication with experts in the field and to listen to the family's concerns and questions. The primary care provider may have a unique role as gatekeeper, to support the family's search for expert care. The most important initial responsibility at birth is determining that the infant is medically stable or has complex health conditions requiring special support or transfer to an NICU experienced with children with CL/P. The next step is to provide a medical diagnosis. Early communication with a cleft palate team can be important in determining the need for special testing (i.e., renal ultrasounds, cardiac consultation, immediate genetic evaluations, and cranial computed tomographic scans). Infants with isolated CL/P may need only a genetic evaluation, which can be scheduled at a time convenient for-the family. Other infants may need early or immediate extensive evaluation.
Evaluate Infant's Ability to Feed and Refer to a Professional with Expertise in Feeding of Infants with Cleft Lip with or without Palate
The vast majority of infants with CL/P will need assistance to be successfully fed orally. The primary care provider, through the initial medical examination, needs to determine that the infant can protect the airway and coordinate feeding and breathing. Consultation with a professional, knowledgeable about feeding infants with CL/P, is important to instruct the family, nurses, and hospital staff how to feed the infant (see Feeding Infants with Cleft Lip with or without Palate, below, for specific information).
Provide Screening and Monitoring with Each Well-Child Visit for Early Detection of Problems for which Children with Cleft Lip with or without Palate Are at Increased Risk
Weight Gain in the First Month of Life
In general, feeding an infant with CL/P is initially difficult and stressful for the family, but with appropriate instruction and monitoring, the infant will do fine. Parents can be told to expect that their infant will not only take longer to feed but be a noisy feeder compared to the typical newborn. A weekly weight check for the first month of life ensures that the infant is gaining weight adequately. Birth weight should be regained by 2 to 3 weeks of life. Documented weekly weight gain is of importance to the infant's physiological health and the psychological well-being of the family, indicating that the infant is growing normally. Usually, after the first 4 to 5 weeks of age, the baby develops the ability to effectively orally feed. Infants with isolated CL/P should be able to orally feed and not require tube feedings. Infants who are unable to successfully feed without tubefeeding support should be evaluated for associated anomalies, perinatal illnesses, or injuries. Children who drop off their own growth curve need to be evaluated for failure to thrive.
Stature
Investigators have reported on the relationship between CL/P and children's stature. Short stature is defined as height less than two standard deviations below the mean or height below the third percentile for same sex and age peers. An expected outcome for children with CL/P is attainment of normal stature consistent with the family. A review of the literature shows great variability of findings about the stature and growth patterns of children with CL/P.
Increased incidence of short stature has been reported (Ranalli and Mazaheri, 1975; Roitman and Laron, 1978; Rudman et al., 1978; Duncan et al., 1983; Cunningham and Jerome, 1992; Felix-Schollaart et al., 1992). Investigators have found variations in the growth patterns, e.g., poor early growth with attainment of normal growth by 2 years of age (Roitman and Laron, 1978; Rudman et al., 1978; Duncan et al., 1983, Felix-Schollaart et al., 1992; Nystrom et al., 1992). Increased incidence of severe short stature due to associated growth hormone deficiency has been reported (Roitman and Laron, 1978; Rudman et al., 1978). Normal growth has also been reported (Nackashi et al., 1988). The variation in findings may be due to sample size, accuracy of measurements, ascertainment bias, cultural differences (country of origin), and definition of short stature.
Explanations for growth pattern variations and excessive short stature include feeding difficulties during the neonatal period and infancy (Drillen et al., 1966; Jensen et al., 1983) and decreased feeding after palatal surgery (Drillen et al., 1966; Ranalli and Mazahari, 1975; Hunter, 1981; Jensen et al., 1983, 1988; Seth and McWilliams, 1988). Felix-Schollaart et al. (1992) reported that feeding difficulties, airway infections, and surgery affect growth but did not account for differences among the CL/P groups and controls reported in the literature. Danish infants with some combination of CL/P were found to have birth weights and lengths close to those considered average for Danish newborns, but infants with severe palatal clefts demonstrated increased incidence of delayed growth.
Due to the variation in findings, many suggesting that children with CL/P have short stature, it is wise to view these children as at risk for being somewhat short but not meeting the criteria for short stature. The primary care provider, through continuity of care, is afforded the best opportunity to monitor growth by serial measurements and standardized growth charts.
After the newborn period, weight and length, in particular length, need to be closely monitored. Families need to be informed that children with syndromes commonly are small for age. Children with true short stature (height less than two standard deviations below the mean or below the third percentile) should be referred for an endocrinological evaluation. Children who are smaller than their peers or family members but are between the 5th and 95th percentiles for stature, have adequate nutrition, and have no other health risks need to be routinely monitored. The family and child should be counseled that the child is normal and healthy.
Development, Behavior, and Psychosocial Functioning
Children with CL/P experience many psychosocial stressors starting in early childhood. Multiple investigators have examined a diverse set of psychological factors, including emotional and behavioral adjustments (Padwa et al., 1991); peer relationships (Krueckeberg et al., 1993), learning disabilities, and neuropsychological development (Kapp-Simon et al., 1993; Richman and Eliason, 1993; Kapp-Simon, 1994); self-perception and body image (Broder and Strauss, 1989; Leonard et al., 1991; Kapp-Simon et al., 1992; Pope and Ward, 1997); and social interaction patterns (Kapp-Simon and McGuire, 1997). An extensive review of the literature on CL/P and other craniofacial anomalies involving parental acceptance, social competence, self-concept, emotional adjustment, and cognitive functioning indicates that 30% to 40% of children in most studies experience difficulties with internalizing and/or externalizing problems, learning disorders, and social competence (Endriga and Kapp-Simon, 1999).
Screening of development and behavior by the primary care provider involves standard developmental approaches. Appropriate approaches include parental report of skills and concerns, use of milestones and developmental screening tools (e.g., the Denver II), and clinical observation to detect developmental delay and affirm particular strengths of the infant and young child. Screening by a cleft palate team is also of great help to the primary care provider. The team has the advantage of seeing many children with CL/P, resulting in a rich experience and expertise. When concerns arise, referral to a developmental pediatrician, psychologist, speech-language pathologist, and other specialists is critical to reduce morbidity and improve outcome.
Developmental screening of school-aged children focuses on learning problems, school-related issues, and psychosocial functioning. The primary care provider can screen the child and family for psychosocial stressors and well-being by asking about family and peer relationships, social and educational concerns, and strengths. Recommendations to the family to intervene with peer-related concerns by visiting with schoolteachers and counselors can be helpful. Parents can talk to their child's classmates about CL/P with the hope that an understanding of the condition will extinguish negative behaviors and teasing. The primary care provider should counsel the family prospectively through anticipatory guidance. Persistent, disturbing, and pervasive psychological problems may require referral to a psychologist or licensed mental health professional.
Speech-Language Skills
Cleft palate puts the child at high risk for a speech disorder known as velopharyngeal insufficiency (VPI), the cause of classic “cleft palate speech.” Even after surgery, the velum (soft palate) and pharyngeal walls may be unable to create a velopharyngeal port that regulates the correct amount of airflow through the nose. Excess airflow through the nose results in hypernasality. If not treated, the hypernasality contributes to the development of compensatory speech patterns, which can be severe and lifelong. Most speech pathologists are not trained in the care of VPI, and this makes the involvement of an experienced cleft palate team even more important. The role of the primary care provider is to conduct routine speech-language screening and to refer to professionals for therapy when indicated. Indications for referral include speech-language delay, articulation problems, hypernasality, and unintelligible speech.
Otitis Media and Hearing Loss
Children with CL/P are at increased risk for otitis media and hearing loss. Most children with CL/P experience some degree of middle ear inflammation, otitis media, or recurrent middle ear pathology during their early childhood years. Eustachian tube dysfunction is the physiological cause for this increased incidence of middle ear pathology and hearing loss. It is generally thought that eustachian tube dysfunction is caused by disruption of the entire muscular sling of the soft palate due to the formation of the posteriorly located cleft. This results in abnormal muscle attachments of the tensor veli palatini to the eustachian tubes. The tensor veli palatini muscles cannot contract effectively, resulting in inadequate opening of the eustachian tubes, which leads to middle ear disease. One of the goals of early palatal repair is to create a palate that is functional for speech and improves eustachian tube function. Investigators have reported that eustachian tube function improves after cleft palate repair (Doyle et al., 1986; Sweitzer et al., 1968). Children with a cleft palate can recover normal eustachian tube function after repair of the palate, but the recovery may take many years (Smith et al., 1994). For children who do not regain normal eustachian tube function, recurring otitis media or chronic otitis media with effusion continues to be a problem. Paradise et al. (1969) found that the most prevalent form of otitis media in children with cleft palate is chronic otitis media with effusion (COME). Persistent middle ear effusion increases the risk of conductive hearing loss, especially during the first year of life, increasing the chance of delayed speech and language development (Habel et al., 1996).
This is even more problematic since it is generally painless and therefore can go undetected except by an otological evaluation. In addition, the child with cleft palate may have COME, which increases the chances for serious middle ear pathology, including tympanic membrane atelectasis, perforation, and cholesteatoma (Bluestone and Kline, 1990).
Otological and audiological monitoring to detect middle ear pathology and hearing loss need to begin in infancy and continue through the teenage years. Children should receive biannual otological and audiological testing consisting of at least an ear exam, tympanometry, and audiogram during the first year of life and preferably up through 3 years of age. This same battery of screening tests should continue annually until care is completed. Many children have stabilization of their middle ear status by 7 years of age and primarily require monitoring. Depending on the complexity of the child's otological and audiological status, testing may need to be more frequent and extensive.
Contemporary approaches for the treatment of acute otitis media include minimizing the use of antibiotics in asymptomatic cases. Antibiotics are used for febrile episodes and when hearing loss is detected. If otitis media persists or continues to recur regardless of antibiotic treatment, the child needs more extensive follow-up. Recurrent otitis media, COME, and hearing loss are commonly treated by placement of tympanostomy tubes. Tympanostomy tubes can be effective at clearing middle ear effusion and correcting hearing impairment, but they can also cause sequelae, including scarring and tympanic membrane perforation. Adenoidectomy is contraindicated because it can exacerbate VPI. The outcome for middle ear function and hearing is maximized by long-term monitoring and testing as part of the comprehensive care provided by a cleft palate team.
Dental
The primary care provider can emphasize the importance of developing good dental hygiene habits toward the long-term outcome of cleft care. Weaning from the bottle early in the second year of life can enhance dental care. Other important strategies involve introduction of early cleaning and brushing of teeth. Since dental malocclusion is common, long-term use of a pacifier and thumb sucking should be discouraged. The family needs to know that the child's care from around 6 years of age to the completion of treatment in the mid-teens is primarily dental. Completion of properly timed dental care and procedures results in the best outcome. Families need to understand that dental care is a necessary component of the total treatment plan and that a combination of surgery and dental care will result in the best outcome for the child.
Special Conditions
There are over 300 syndromes associated with CL/P. All children with CL/P can benefit from a genetics evaluation and referral to a cleft palate team. Two of the more common associated conditions have implications for the primary care provider.
Velocardiofacial syndrome (VCFS) is the most frequent clefting condition (Shprintzen et al., 1981). There are numerous features, which are not always present, including cleft palate, cardiac anomalies, typical facial features, slender hands and digits, ophthalmological abnormalities, psychiatric illness, VPI, and medial displacement of the internal carotid arteries. The condition is caused by deletions of the human chromosome 22qll region. These deletions are also common in DiGeorgesyndrome, suggesting an etiological connection. DiGeorge syndrome is considered a sequence found in VCFS and involves thymic aplasia, immune deficiency, hypocalcemia, and congenital heart defects. Children who have these clinical features and 22qll deletions have VCFS. The implications of VCFS are broad, spanning physical, cognitive, psychosocial, speech, and developmental domains. These children are at high risk for poor outcomes, including school failure, behavioral concerns, and psychiatric illness. This is an example of a condition that can be missed at birth and has long-term consequences.
Robin sequence (RS) is a well-known condition that includes micrognathia, cleft palate, and glossoptosis, causing airway obstruction. Many infants with RS have some degree of airway compromise and are at risk for airway-related feeding problems. A large number of infants are managed with positioning, careful feeding, and close monitoring. When calm, infants are generally engaged in quiet breathing and appear well. Upper respiratory infections, vigorous crying, and feeding can cause obstruction or partial obstruction of the airway, which leads to airway compromise.
At birth, infants with severe airway compromise need NICU management, which may include tracheal intubation, supplemental oxygen, mechanical ventilation, orogastric tube feedings, and 24 h monitoring. Surgical management of the airway may involve tracheostomy, tongue-lip adhesion, or mandibular distraction. Some infants may require months of hospitalization before they are stable enough to be discharged home. The vast majority of infants will stabilize and have normal breathing and feeding abilities although, some will require a protracted period of time to outgrow their need for airway and nutrition support. Some children with RS may also have Stickler syndrome, which involves orofacial findings consistent with RS along with myopia and musculoskeletal abnormalities. After many years, arthritis may become a significant problem and progressive myopia may lead to retinal detachment and blindness. Some children are at risk for mitral valve prolapse. Children with RS, and especially those thought to have Stickler syndrome, should have a chest x-ray, electrocardiogram, genetic evaluation, eye examination, and a sleep study. Robin sequence is an example of a condition often apparent at birth, due to the airway compromise and feeding difficulties, but that can have an excellent outcome with appropriate supportive care. Stickler syndrome is an example of a condition that cannot be fully evaluated at birth because the features evolve over time. Children with this condition are at risk for worsening vision and need regular vision screening in the primary care office.
Feeding Infants with Cleft Lip with or without Palate
The goal of feeding the newborn is to provide adequate calories to support life, growth, and development. Mother-infant bonding is an important, but physiologically secondary, part of the feeding process. It has been suggested by Rybski et al. (1984) that feeding the newborn is contingent on the coordinated interaction of a complex set of behaviors, including the participation of a caregiver. It is a common clinical observation that parents of a newborn with CL/P often experience considerable anxiety due to the problems they encounter when attempting to feed their baby and that these feelings can interfere with the normal interaction between parent and child.
Biology of Sucking
Normal Sucking
Sucking, one of the earliest organized behaviors, and rhythmical bursts of jaw opening and closing associated with movements of the tongue have been identified even in fetal life (de Vries et al., 1988). Fetuses have been observed in activities of nonnutritive sucking of thumbs and toes as early as 28 weeks while in utero (Shaker, 1990; Medoff-Cooper and Ray, 1995).
For the neonate of 32 to 34 weeks, the mechanisms of creating a partial vacuum intraorally, controlling an effective stripping motion of the nipple by the tongue, and coordinating an efficient swallow have matured into a well-integrated system (Goldson, 1987; Shaker, 1990). The infant encompasses the nipple with the lips, creating an airtight seal around the nipple or areola. With an effective anterior seal, the mandible is pulled down along with the central dorsum part of the tongue, resulting in more space intraorally and subsequently a decreased or negative intraoral air pressure (suction). The infant then presses the nipple against the alveolus and hard palate and, with a rolling or stripping movement of the tongue tip across the nipple from front to back, extracts milk from the nipple. The milk bolus is forced by this rolling action of the tongue into the pharynx, where it enters into the esophagus, and through the process of peristalsis (and gravity), is carried to the stomach (Bu'Lock et al., 1990).
Suction or the Partial Vacuum Created Intraorally as Part of the Sucking Process
Various techniques have been utilized to define the sucking and swallowing process, including cineradiography (Ardran et al., 1958a,b), ultrasound (Smith et al., 1985; Weber et al., 1986; Bu'Lock et al., 1990; Nowak et al., 1995), videofiberoscopy (Eishima, 1991; Iwayama and Eishima, 1997), and assessment of pressure changes intraorally, with or without measurement of fluid flow, using pressure transducers (Goldson, 1987; Jain et al., 1987; Medoff-Cooper et al., 1989, 1993; Fadavi et al., 1997; Waterland et al., 1998). Of the three primary components in feeding [lip seal, tongue stripping, and intraoral negative pressure (suction)], it is suction with its several dimensions that can be relatively easily and objectively measured.
Research has been directed at quantifying various components of sucking, such as the intensity of each suck, the duration of each suck, and the duration of rest periods between sucks. These measures have been obtained using a pressure transducer to determine changes in air pressure within the bottle while the infant is feeding (Linder, 1991; McGowan et al., 1991; Medoff-Cooper, 1991; Palmer, 1993; Medoff-Cooper and Ray, 1995). As growth occurs, the infant can generate greater negative intraoral pressure and stripping pressure (Linder, 1991; Medoff-Cooper et al., 1993). By the end of the first year, there is generally a change in diet and a weaning off the bottle, with sucking activities and reflexes becoming repressed (Iwayama and Eishima, 1997).
Biology of Sucking during Breast-Feeding
Functionally, breast-feeding differs from bottle-feeding in the wide-open position of the jaw, the position of the nipple in the mouth, the muscles used, and the response or elasticity of the nipple in the mouth in response to the sucking (Turgeon-O'Brien et al., 1996). Most of the research on sucking has been done on infants fed from bottles equipped with transducers. Great care is taken to remove confounding variables by offering the same nipple, the same fluid, in the same position, etc., to all infants in a study. Research on breast-fed infants is more complex as it is harder to measure negative intraoral pressure and the infant-mother interaction and physiological responses of the mother to sucking cannot be quantified. Research and clinical observation indicate that the sucking pattern of infants changes over the course of the feed, as do the availability and composition of the breast milk (Chetwynd et al., 1998). The amount of milk extracted is not determined solely by the baby and the sucking pressure patterns but is affected by the let-down reflex and other maternal variables (Prieto et al., 1996). Infants who are breast-fed use negative intraoral pressure to position and stabilize the breast in the mouth. A combination of negative intraoral pressure, the stripping action of the tongue against the breast supported by the palate, and the pumping movements of the jaw and gums empties the milk ducts (Clarren et al., 1987; BuLock et al., 1990).
Biology of Sucking in a Neonate with a Cleft
There has been extensive research in the sucking patterns of preterm and term infants, and descriptions of the developmental stages of the sucking pattern to the age of 14 months (DeMonterice et al., 1992; Medoff-Cooper and Ray, 1995; Turgeon-O'Brien et al., 1996; Fadavi et al., 1997; Hafström et al., 1997; Iwayama and Eishima, 1997; Finan and Barlow, 1998; Waterland et al., 1998). There has been very little research on the sucking patterns of infants with cleft lip or cleft palate preoperatively or postoperatively (Choi et al., 1991). One report examined palatal motor movement pre-and postoperatively (Igawa et al., 1998). The sucking characteristics of these groups must be described prior to comparison to noncleft children. Dixon-Wood et al. (1999) measured the intraoral pressure of infants with CL/P during feeding. Special nipples were developed (Medoff-Cooper et al., 1993), which contained two tubes connected to a transducer. Intraoral pressure was measured during bottle-feeding. None of the children with cleft palate were able to generate any negative intraoral pressure. An interesting finding was that these children learned very early to position the nipple in the mouth so as to mechanically strip it with the tongue against the alveolus, cheek, or palate.
This supports what we know about anatomy, the development of negative intraoral pressure during sucking, and the effect of cleft palate on the ability to develop negative intraoral pressure. Therefore, we can assume that a child with a cleft palate preoperatively cannot create effective negative intraoral pressure due to the open communication between mouth and nose.
Additionally, infants with cleft palate lack the support against which the tongue can effectively strip the nipple or breast. Depending on the position of the premaxilla and its stability, the infant may be further hampered by an inability to use movements of the jaw and gums to assist with feeding. Clarren et al. (1987) separated subjects into groups by diagnosis and made feeding recommendations for each cleft type. This technique has been adapted Table 25.1.
Determining an Infant's Ability to be Orally Fed
After the birth of an infant with CL/P, the physician and newborn nursery staff need to make a rapid determination of the infant's ability to be safely and successfully fed by breast or bottle. The clinician can use Table 25.1 to guide the feeding evaluation by asking four physiological questions: (1) Can the infant protect the airway and coordinate swallowing with breathing? (2) Is the ability to swallow intact? (3) Can the infant generate negative intraoral pressure? (4) Is the sucking mechanism normal? Table 25.1 lists a series of cleft conditions and compares them across these four physiological elements.
A review of Table 25.1 reveals the following basic concepts:
· Most infants with CL/P can be safely and successfully orally fed using breast or bottle, usually bottle.
· The presence of a syndrome, multiple anomalies, perinatal illness, or neurological complications puts the infant at risk for not being able to be safely orally fed.
· Infants with RS may be unable to generate negative intraoral pressure or even suck/swallow normally due to anatomy (a posteriorly placed tongue due to micrognathia).
· Infants with isolated cleft lip are the easiest to feed.
· The presence of an isolated cleft palate suggests that an infant can safely orally feed but will need assistance due to an inability to generate negative intraoral pressure.
· Some infants with symptomatic submucous cleft palate may have difficulty generating negative intraoral pressure.
Feeding Practices in a Clinical Setting
What to Feed Infants with Cleft Lip with or without Palate
Breast Milk is Best
The American Academy of Pediatrics (1997) guidelines state “human milk is uniquely superior” nutritionally for both sick and well infants, and every mother should be encouraged to breast-feed. Research has shown breast milk to have multiple health benefits, including a decreased incidence of diarrhea and otitis media (Scariati et al., 1997), inhibition of colonization by respiratory bacterial pathogens (Hokama et al., 1999), and increased protection against viral illness (Englund et al., 1998), among many others. Breast milk provides protection against otitis media even in otitis-prone infants with CL/P (Paradise et al., 1994). Several researchers have even found breast-feeding to be responsible for improved cognitive development (Anderson et al., 1999; Uauy and Peirano, 1999). In addition to health benefits for the infant, health benefits for the mother and socioeconomic benefits for families, employers, and the nation are expounded (American Academy of Pediatrics, 1997; Ball and Wright, 1999). The primary care provider's role is to provide parents with complete and current information about the benefits of breast-feeding so that a fully informed decision can be made. Mothers of infants with CL/P should be encouraged to provide breast milk as the preferred source of nutrition if they are able to do so.
Encouraging breast-feeding has been a controversial subject in this population (Chibbaro, 1998). Societal parenting pressures and breast-feeding expectations compounded by the difficulties in feeding an infant with CL/P, anxieties about the infant's underlying condition, concerns about upcoming surgeries, and the already stressful postpartum period leave many parents feeling overwhelmed, anxious, and guilty. Professional concerns about already fragile parent-infant attachment in infants with a facial difference and about further increasing stress in this difficult postpartum period have led some to suggest that breast-feeding should not be recommended. The increasing findings of health benefits from breast milk cannot be ignored. Some parents have reported feeling good about expressing breast milk because they know this is something they can do for their child's health. Individual preference is paramount; however, the American Academy of Pediatrics (1997) states “before advising against breastfeeding or recommending premature weaning, the practitioner should weigh thoughtfully the benefits of breastfeeding against the risks of not receiving human milk.”
Formula Is Satisfactory
Although not providing all of the health benefits attributed to breast milk, formula feeding is an acceptable alternative. Historically, formula supplementation and assisted feeding techniques have been the mainstay of feeding an infant with CL/P. Extended periods of time to directly breast-feed, then express additional breast milk, give expressed milk by assisted feeding, followed by the additional timeconsuming chores of cleaning the breast pump, bottles, and the baby's bottom, all while keeping the mother well fed, rested, and hydrated are minimized. In households where other children also require care, the demands of breast-feeding an infant with a cleft may contribute to family stress or neglect of other family members. Benefits of formula include the ability to accurately measure the volume and caloric intake and to provide changes in concentration or additives to increase the caloric density. Under a pediatric nutritionist's guidance, formula has the unique ability to be reconstituted from concentrate or powder to create a more concentrated formulation. Pediatric nutritionists can also prescribe a variety of substances to add to formulas and expressed breast milk to increase caloric density. Simple sugars, Polycose®, corn oil, and medium chain triglycerides (MCT oil®) oil are just a few.
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TABLE 25.1. Feeding Characteristics and Suggested Feeding Techniques by Orofacial Diagnosis* |
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At first glance, a calorically dense formula is inviting since the infant needs less volume to meet caloric needs. A careful balance is necessary, however. If too dense, the fluid is hard to digest. This can increase transit time, causing constipation and other gastrointestinal symptoms. Maintaining enough free water for adequate hydration and protection of the kidneys is another important factor.
Whatever feeding method is chosen by the family, the primary care provider's responsibility is to support the decision and put the family in contact with the appropriate community support professionals. The American Cleft Palate-Craniofacial Association maintains a registry of cleft and craniofacial teams that provide care for people with clefts. The Cleft Palate Foundation (1992) has written educational materials for parents, healthcare providers, and hospitals.
Recommended Feeding Strategies
Initial feeding instruction is often provided to parents of a newborn with CL/P at a time when they are experiencing shock, anxiety, and grief. Newborn nursery staff, who may have little experience with feeding an infant with CL/P, often provide this initial instruction. All too often, parents are discharged to take their baby home without having received any training in feeding or time to practice these techniques. However, when feeding instruction is provided by cleft palate teams, it is generally based on trial-and-error strategies that may have worked for some families, rather than on proven physiological principles of sucking/feeding (Richard, 1994). Most instruction is limited to positioning the infant during feeding so as to minimize choking and gagging. Parents also are often advised to assist fluid flow from the bottle by enlarging or cross-cutting the hole in the nipple or by using a plastic bottle that can be squeezed to force a more rapid flow (Elster et al., 1994). Such instruction is offered, knowing that parents run the risk of “flooding” their baby in a flow of milk. This may result in undesired compensatory oral motor strategies in an attempt to cope with the excess flow and prevent choking and gagging and/or to gain momentary control to enable swallowing and breathing. Finan and Barlow (1998) and Fadavi et al. (1997) demonstrated the disruptive impact of such environmental stimuli on sucking motor patterns.
Rather than espousing only one feeding technique, it is important to communicate to parents that there is no “right” way to feed an infant with cleft. Each infant and parent diad will develop their own relationship and strategy during feeding, which works best for them. Infants (especially those with a cleft) are very adaptable and can tolerate being fed in a variety of ways by different care providers, parents, and other family members. However, some general guidelines or strategies are commonly used.
Direct Breast-Feeding
Infants with cleft lip or cleft lip with incomplete cleft of the alveolus may have some difficulty with the orolabial seal around the breast or bottle. These infants may respond best to breastfeeding since the breast tissue is more malleable and can fill the opening, allowing for a tight seal. If there is no associated cleft palate, the infant should be able to strip the nipple against the roof of the mouth normally and seal the soft palate against the posterior pharyngeal wall to create negative intraoral pressure (suction). These infants should be able to feed and grow normally, and their intake is not affected by their cleft (Clarren et al., 1987; Coulter-Danner, 1992; Elster et al., 1994).
Infants who have clefts of the palate can provide some stripping action, depending on the size and placement of the cleft, but cannot create negative intraoral pressure. The breast must be held in the infant's mouth and the infant held up to the breast. Supporting the baby on pillows will decrease the strain on the mother. Experimenting with the infant body and head position, to get the best compression of the breast within the mouth, will assist with the mechanical emptying of the milk ducts. These infants can still be directly breast-fed by mothers with a strong let-down reflex who have a stream of milk from the breast. Massaging the breast to assist the letdown reflex or manually expressing milk from the breast into the infant's mouth are additional strategies (Cleft Palate Foundation, 1992). This may be easier in mothers who have previously breast-fed other infants. Using these strategies may allow a parent and infant to have some direct breast-feeding time but rarely provides adequate calories. Accurate measurement of actual intake (not just minutes of feeding on each breast) is essential since feeding efficiency is reduced. One way to estimate this is by obtaining prefeeding and postfeeding weights. The higher-calorie “hind milk” is not expressed by these methods, and the infant may miss out on the nutrient-dense portion of the feeding while expending higher calories over a longer period of time than with direct breast-feeding alone. This puts the infant at increased risk of dehydration, hyperbilirubinemia, failure to thrive, and weight loss (Clarren et al., 1987; Coulter-Danner, 1992; McCartney, 1996; Wide Smiles, 1997).
Subsets of the population at greatest risk for breastfeeding failure include infants with symptomatic but undiagnosed submucous cleft palate. Most children with submucous cleft palate are asymptomatic, and this is often not diagnosed until later. In this age group, symptomatic submucous cleft palate puts these infants at risk because the palatal musculature is not able to effectively and consistently seal against the posterior pharyngeal wall, impairing the ability to produce negative intraoral pressure and increasing feeding inefficiency. When the baby is put to breast or to bottle, appropriate rooting and sucking movements occur. If a gloved finger is placed in the mouth, appropriate placement of the tongue with normal peristaltic muscular movements and compression against the roof of the mouth are felt. This may mislead a healthcare provider or parent into believing that effective sucking is occurring. If a symptomatic submucous cleft palate is not diagnosed by physical exam at birth, it may not be diagnosed until feeding difficulty progresses to failure to thrive or poor weight gain.
Expressed Breast-Feeding
A combination of direct breast-feeding and expressed breast milk by assisted feeding or expressed breast milk by assisted feeding alone is the preferable way to feed most infants with CL/P. A mother's desire to provide breast milk to an infant is a commitment of time, energy, and fortitude. She will need the full support of the hospital staff, family, primary care provider, and cleft palate team to accomplish it. She needs better than average nutrition, adequate sleep, forced hydration, and a great breast pump. She will, in essence, be taking the time for two feedings for each infant feeding. She will need assistance from a spouse or family member to feed the infant with assisted feeding techniques while she expresses breast milk to be fed later. She will benefit from a lactation consultant familiar with feeding infants with cleft and from a friend who will support her decision to breast-feed or to begin formula supplementation if breast-feeding becomes too difficult for her.
For each feeding, a careful diary must be maintained with the number of minutes at breast, the volume of milk given by assisted feeding, and the length of time needed. In this way, if an infant shows poor weight gain at a follow-up visit, the energy expenditure at the breast and the total time of feeding with assistance can be evaluated and recommendations made to decrease energy expenditure or increase intake. It is important to stress to mothers that every infant is different and, in some ways, every feeding is different. Infants will vary in their ability to feed efficiently depending on many variables, including the extent of the cleft, their level of arousal at the time of feeding, development of respiratory infections, or just a change in schedule. By doing their best at each feeding and monitoring weight gain every week or so, mothers will be doing all they can to ensure that their baby has the best nutrition for growth and development (Wide Smiles, 1996).
Assisted Feeding
Assisted feeding can reduce calorie expenditure. Assisted feeding techniques use pulsesqueezed bottles, droppers, cups, and other devices to get the milk into the infant's mouth without sucking and with decreased energy expenditure. It should take approximately 15 to 30 min for infants to complete a feeding (Sidoti and Shprintzen, 1995). If an infant takes longer than 30 min, valuable calories needed for growth are being used and the infant may tire, fail to gain weight, or even lose weight (Balluff, 1986). Because infants with CL/P are inefficient feeders due to the structural problems of the cleft, assisted feeding is recommended to compensate for the decreased or absent negative intraoral pressure. The choice of feeding equipment must be based on what works well for the infant, is readily available in the community, and is affordable for the parent. Some examples are described below.
The Mead Johnson (Evansville, IN) Cleft Lip/Palate Nurser is a compressible bottle with a cross-cut straight nipple. It can be fitted with an orthodontic nipple. The orthodontic nipple is wider and has a larger surface to be compressed, but care must be taken to turn the nipple upside down so the stream of fluid is directed toward the tongue or the posterior pharnyx, not up into the cleft. This bottle works best with nipples that have the porthole on the tip. If a vertical slice is made on the tip, the formula will flow through the enlarged hole when the infant compresses the nipple between the gums, even without negative intraoral pressure. Alternatives are a horizontal slice for a lower flow rate or a cross-cut for a faster flow rate. The bottle can also be pulse-squeezed by the caregiver along with the infant's mouthing pattern to simulate sucking. The Mead Johnson CL/P Nurser is a disposable bottle and cannot be sterilized. It can be reused in the home by washing it in warm water with soap and sterilizing the nipples or washing it in the top rack of the dishwasher. Standard baby bottle nipples by most manufacturers fit.
These bottles are the least expensive and require replacement after 15 uses. To increase the life of the Mead Johnson CL/P Nurser, do not heat milk or formula on the stove or in the microwave. Use a regular bottle to heat the milk or formula in a cup of warm or hot water, then transfer it to the Mead Johnson CL/P Nurser (Dixon-Wood, 1997; Mead Johnson Nutritionals, personal communication, 2000).
The Haberman Feeder (Medela, McHenry, IL) is a rigid bottle with an elongated, compressible nipple. It has an adjustable flow rate depending on the direction the nipple is inserted into the mouth. Making this adjustment does not require any alteration of the nipple hole. The feeder has a one-way valve between the nipple and the bottle to prevent formula from being pushed back into the bottle by compression and to decrease the amount of air ingested through the nipple. Some infants are able to adequately compress the enlarged nipple and do not need pulse-assisted feeds; however, the nipple can be pulse-squeezed for infants who need additional assistance. Due to the one-way valve, this nipple does not do well for infants who require thickened feedings for gastroesophageal reflux. The Haberman Feeder and nipple can be sterilized. As with the Mead Johnson CL/P Nurser, do not heat milk or formula in a Haberman Feeder in the microwave. The Haberman nipple is recommended for room-temperature liquids only. Complaints about this feeder center around the price (currently about $16 each if purchased directly from the manufacturer), the unusual appearance, and the lack of a travel cap (Medela, Inc., 1994; Wide Smiles, 1996).
The Cleft Palate Nipple System (Children's Medical Ventures, Norwell, MA) is a compressible bottle with a larger-than-usual tri-cut nipple that is stiffer on one side and very pliable on the other. It is positioned in the infant's mouth with the stiff side toward the cleft. The tongue then strips along the very pliable side, compressing against the stiffer side and maximizing each attempt at sucking. The bottle can also be pulsesqueezed by the caregiver along with the infant's mouthing pattern to simulate sucking. It also has a one-way valve between the nipple and the bottle to prevent milk from flowing back into the bottle by compression. Flow rate can be adjusted by tightening or loosening the cap, which opens or closes dual air holes. If the infant does not require pulse-squeezed assistance, this nipple will fit on any baby bottle. The Pigeon Feeder can be boiled or gas-sterilized. As with the Haberman Feeder, the one-way valve precludes the use of thickened feedings for infants with gastroesophogeal reflux (Children's Medical Ventures, 1999).
A review of the literature reveals a variety of other feeding innovations and anecdotal stories of infants fed successfully with eyedroppers, Dixie cups, shot glasses, and syringes with all manner of bulbs, catheters, and other attachments. An interesting adaptation of the Playtex Nurser with its wide-based nipple and collapsible formula bag was made by cutting away part of the hard plastic sleeve so the liner could be compressed by the parent. There is not room to review all of the possibilities here. Suffice it to say that if it gets a sufficient quantity of breast milk or formula in the baby with little calorie expenditure or risk of choking, it has probably been tried. If the infant fails to grow with conventional feeding strategies and after medical, psychosocial, and nutritional evaluations the growth failure is still felt to be a mechanical problem due to the cleft, then unconventional feeding techniques can be tried.
Additional Helpful Feeding Strategies
Feed in More Upright Position
Positioning the infant with CL/P in a more upright position (>65 degrees) is recommended for several reasons. One is that the more upright position allows gravity to assist with moving the formula bolus to the back of the mouth and down the esophagus. This gravity assistance helps to reduce infant energy expenditure and fatigue during feeding. It also reduces the amount of nasal regurgitation. Nasal regurgitation is common since the infant's tongue reflexively pushes up into the cleft when attempting to strip the nipple. Although nasal regurgitation is a sign of swallowing dyscoordination in children without clefts, it is not an indication of swallowing problems in an infant with a cleft palate (Clarren et al., 1987; Cleft Palate Foundation, 1992).
Frequent Burping
The soft palate cannot form an airtight seal during swallowing, and air freely flows through the nose during feeding and swallowing. In addition, coordination between the parent pulsing the formula (with an assisted feeding device) and the infant swallowing and breathing may not be fully developed for several weeks. For these reasons, infants with CL/P swallow more air during feeding. They require more frequent burping than infants without a cleft and may have more “wet burps,” nasal regurgitation, vomiting, or colic-like symptoms. Thankfully, as the infant grows and muscle tone, swallow-breath coordination, and parental skill at feeding and reading subtle cues from the infant improve, these symptoms usually resolve. Colic-like symptoms from air swallowing are common in this population and do not necessarily mean that true colic is developing. Antiflatulence drops (e.g., simethicone) are one possible medical intervention to consider.
At the same time, if feeding and burping are going well and the typical symptoms of colic continue, then counseling the parents on the development of colic in babies with or without CL/P may be indicated. A trial of some of the other common interventions for treatment of colic may work. In addition, the possibility of other etiologies (other than the cleft) should be explored. It cannot be too strongly stressed that infants with CL/P have the same incidence rates of associated problems like colic, reflux, and pyloric stenosis as the general population.
Plain Water for Mouth and Nose Rinse
Mucus may line the nasal area and cleft prior to a feeding. Formula (and later foods) may get trapped in the cleft after a feeding. A few sips or spoonfuls of water before and after feedings will rinse trapped mucus and food/formula particles away (Muir and Burton, 2000).
Obturators (“Feeding Appliances”)
Presurgical palatal appliances were at one time advocated because it was hoped that they would help the infant with CL/P feed more efficiently (Balluff and Udin, 1986; Jones, 1988). In as much as these appliances provide a solid surface against which the infant can compress the nipple, they do improve the ability to feed. Currently, these presurgical appliances are used to direct palatal segments into position for surgical repair or to prevent dental arch collapse from the pressure of the cheeks. These appliances do not allow the infant to create negative intraoral pressure, however, and the infant still needs assisted feeding (Choi et al., 1991). A palatal appliance may improve, but will not solve, feeding problems in an infant with CL/P. The benefit of a solid surface on which to compress the nipple must be weighed against the large percentage of intraoral space the appliance occupies. Infants with retrognathia, whose tongues are posteriorly positioned, are poor candidates for palatal appliances because of the risk for airway occlusion. In addition, palatal appliances must be monitored closely and refitted with growth, thus becoming expensive and time-consuming (Cleft Palate Foundation, 1992; Berkowitz, 1996).
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