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Pediatric Oral Health

Managing the Cleft Lip and Palate Patient

Felix L. Kaufman, MD, FAAP*

Few problems challenge the depth and breadth of multidisciplinary pediatric knowledge more than the child with congenital anomalies. For the management of the child with cleft lip or palate, there are more than 200 national and 25 international multidisciplinary teams. A few of these teams have participating pediatricians, and a few are led by pediatricians. Yet pediatricians and family physicians must appreciate the complex problems of these patients if they are to fulfill their primary care role. After beginning with a general discussion of the basic science of clefting, this article examines, from a pediatric viewpoint, the roles of the various multidisciplinary team members for the purpose of gaining greater understanding of the multiple problems that these children face and the longitudinal issues in management. We conclude with a look specifically at the primary care responsibilities for all of these patients, whether or not there is the support of a cleft palate team.

BASIC SCIENCE Anatomy A basic understanding of clefting is critical to appreciating the issues faced by these patients, their families, and their physicians. As with most disorders, clefting encompasses a spectrum of severity. Anatomically, clefting varies from simple cleft lip to complete cleft of the lip and palate (Fig. 1). Table 1 describes the various anl)malies and their combinations along with their percentage prevalence, as seen in

* Pediatric

Consultant, Kernan's Cleft Palate Diagnostic Program, James Lawrence Kernan Hospital; and Clinical Assistant Professor, Department of Pediatrics, University of Maryland Hospital, Baltimore, Maryland

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8 Figure1.1.A,A,Unilateral Unilateralincomplete incompletecleft cleftlip. lip.B,B,Unilateral Unilateralleft leftcleft cleftliplipand andalveolus. alveolus. Figure

(Illustration continued on opposite page)

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Figure11(continued). (continued). C, C,Complete Completeleft leftcleft cleftlip lipand andpalate. palate. D, D, Bilateral Bilateral incomplete incomplete Figure cleftlip. lip. cleft

(Illustration continued on following page)

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F Figure Figure 11(continued). (continued).E, E,Bilateral Bilateralcomplete completecleft cleftlip lipand andpalate. palate.F,F,Cleft Cleftpalate palatealone. alone.

our unit and others. 27 • 29 Since the landmark work of Fogh-Anderson 18a in 1942, noting the etiologic distinction between cleft lip or palate or both (CL ± P) and cleft palate (CP) alone, most research has been in the context of these two most prevalent situations. Indeed, subsequent studies have consistently confirmed epidemiologic and genetic differences.

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Table 1. Distribution of Types of Defect KERNAN

Cleft lip alone Cleft of lip and palate 1. Incomplete (lip and anterior palate) 2. Complete a. Unilateral 1) Right 2) Left b. Bilateral Cleft palate alone 1. Soft and hard palate 2. Soft palate alone 3. Submucous cleft

OTHER TEAMS

6% 52%

16% 45% 45% 55% 73% 44% 56% 27%

42% 1+2

39% =86% 14%

1+2

=86% 14%

Incidence and Prevalence Clefting is the second most common of the major congenital defects, affecting approximately 1 in 700 individuals in the United States. When incidence is defined as the number of new patients entering the population, CL ± P = 1 in 600 live births, with CP = 1 in 1000. This incidence is sevenfold greater in CL ± P and threefold greater in CP when fetal data are included. 26 Translated into real numbers, there are approximately 15,000 newborns with clefts each year, with a total US population with clefts at anyone time (prevalence) of 330,000,17 It is reasonably certain, therefore, that practicing pediatricians and family physicians have to deal with more than one of these patients in a career. Geographic, racial, and sex differences are notable. The incidence of CL ± Pis 1.5 times greater in orientals than whites and 2.1 times greater in whites than blacks. 33 CL ± P occurs nearly twice as often in boys than girls, but occurs slightly less for boys than girls with CPo When unilateral, left-sided clefts are more common; when bilateral, the left side is usually the more severely affected side. There is no significant difference in the incidence of affected offspring as a function of the degree of severity of the cleft in an affected parent. Cause and Pathogenesis Although there remains no clear con census as to the cause and pathogenesis of clefting, investigations continue. Clearly there are both genetic and environmental components to the cause. The genetics of clefting, which probably involve multiple gene loci, are discussed in the section on genetics. Pathogenesis is probably caused by one or more of the following mechanisms: (1) anatomic obstruction; and (2) interference with cell differentiation or migration, either through hormonal defect, biochemical defect, or extrinsic biochemical interference. 17 Embryologically, the maxillary processes fuse to form the primary palate (upper lip and alveolus) by the seventh week after conception

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(Fig. 2). By the twelfth week, palatal processes have fused to form the secondary palate. In mammals, the maxillary process is formed by pharyngeal ectoderm combined with mesenchymal cells that have migrated from the neural crest. In addition to anterior fusion with nasal processes, palatal shelves form medially from the maxillary processes. 33 These shelves first migrate downward, then elevate to a horizontal position above the tongue. Neurotransmitters, growth factors, and cyclic adenosine-monophosphate control mesenchymal migration, palatal elevation, and eventual fusion. Although anatomic studies on human material are few, some animal and human studies support the tongue obstruction (of palatal shelf fusion) hypothesis, but only when associated with mandibular underdevelopment. 7 , 30, 33 Current thinking focuses at the cellular and biochemical level, i.e., cell migration and its control mechanisms. Numerous studies have substantiated the association between teratogens and clefting. Associations have been noted with ahticonvulsants, maternal smoking,28 and steroids. 33 Such teratogens may be individually operative in a subgroup of individuals that is genetically and biologically susceptible. Conversely, several different teratogens may act together on a single mechanism controlled by only a few genes. Glucocorticoid receptors have been demonstrated on palatal cells, suggesting a mechanism for the teratogenic effect of these agents. Phenytoin, retinoids, and lithium have been shown to interfere with growth factors and enzyme systems that are important in cell division and differentiationP Retrospective histochemical and electronmyographic studies of biopsied facial muscles that were obtained from cleft lip margins identified mitochondrial abnormalities. A defect in energy production that could influence cell proliferation, migration, or differentiation is suggested. 46

NASOLATERAL PROCESS NASOMEDIAL PROCESS MAXILLARY MANDIBLE

PROCESS--~_-""'"",,-­

-------~-

Figure 2. Normal migration pathways for nasolateral, nasomedial, and maxillary processes. Failure of migration or fusion results in anterior clefts.

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THE CLEFT PALATE TEAM Plastic or Craniofacial Surgery The role of the plastic or craniofacial surgeon begins with early family consultation, either in the hospital or shortly after the affected infant's discharge. The surgeon is perceived by the family as the "one who will fix the problem." Families need both the reassurance that the cleft can be fixed and the initial information as to "what and when." Early involvement in this way allows the surgeon to play an important role in facilitating parent-infant bonding and provides the groundwork for a rapport that enhances the parents' understanding and acceptance of the difficulties that lie ahead. Primary Surgery. Primary surgical procedures consist of the initial repairs of the lip or palate. The achievement of normal speech, hearing, and maxillofacial growth is the goal. Because speech, hearing, and growth are developmentally dynamic issues, however, controversy continues as to optimal timing and optimal procedure. Conventional wisdom is to repair the lip at 2 to 3 months of age, either by simple lip adhesion or a combined lip and primary palate repair. Criticism of repair at an earlier age centers around increased risk of surgery and anesthesia before healthy growth is established, or optimal size is achieved; or incidence of restriction of anterior maxillary growth is increased. Palate repair, however, presents more of a dilemma. Speech and hearing are believed to be improved by palate repair before 2 years of age, but early (versus after 4 years of age) repair may adversely affect midfacial growth. Studies continue to try to further elucidate the effect of surgery on maxillofacial growth, but they have been confounded by variables of cleft type, differing surgical procedures,37 individual surgical technique,16 and perhaps the possibility of inherent growth deficiencies in some types of clefts. 43 Thus there is no general concensus as to the optimal timing of palate repair relative to maxillofacial growth. Speech, however, is clearly adversely affected if palate closure is delayed past 2 years of age, perhaps even after 1 year of age. Pharyngeal and glottal articulation errors are compensations common to cleft speech that are not only more likely to develop with later repair, but the longer they are established, the less amenable to therapy after repair. One study by Dorf and Curtin12 indicates a 10% incidence of such errors in children repaired before 1 year of age and 86% incidence in those repaired after that time. An 86% incidence of glottal and pharyngeal articulation errors was also noted by Bardoch et all in a group oflate repaired patients. Therefore, the concensus in 1990 is for definitive palate repair at approximately 1 year of age, with the choice of technique the decision of the individual surgeon. Secondary Surgery. After primary repair, the cleft palate patient generally faces a future of multiple subsequent surgeries. In a com-

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prehensive long-term study (birth to age 21 years) by Enemark et aP6 of 57 patients, 23% required pharyngeal flap, 97% required alveolar bone grafting, 40% required lip scar revision, 75% required correction of nasal deformity, and 12% required orthognathic surgery. Although no palatal fistulae were noted in this series, they are not uncommon. Pharyngeal flap procedures, i.e., attaching a pharyngeal flap to the soft palate, substantially alleviate hypernasal speech, which may result from a repaired palate that is insufficient in length or mobility to occlude the nasopharynx for normal speech production. As hypertrophied adenoids may facilitate velopharyngeal closure, hypernasality may not appear until adolescent adenoid involution. The need for a pharyngeal flap may be substantiated by nasoendoscopic or videofluoroscopic examination of the nasopharyngeal port during speech production. Lip scar revision is generally reserved for thattime when the lip becomes a cosmetic issue for the child. Correction of the nasal deformity should await the completion of the adolescent growth spurt, however. Orthognathic surgery for the correction of severe jaw discrepancies should also await the completion of mandibular growth. The need for and timing of fistula closure generally depend on the degree to which such fistulae affect speech, predispose to local infection, or cause distress to the patient with eating or drinking. Lastly, alveolar bone grafting is now accepted as a significant advance in management and is discussed in the section on orthodontics. Otology and Audiology The tensor veli palatini muscle is generally accepted as the eustachian tube muscle. The contribution of the levator veli palatini, which is primarily the muscle of velopharyngeal function, is controversial. 18 Nonetheless, this entire muscular sling of the soft palate is disrupted by posterior clefting. With nothing to pull against, failure of proper opening of the eustachian tube results in significant middle ear pathology in 50% to 100% of these patients. This incidence has been shown to be reduced with early cleft closure. Conductive hearing loss secondary to persistent middle ear effusion, particularly in the first year oflife, may result in significant delay in speech and language development. Thus, sequential, carefully done examinations by the otologist, with the support of the audiologist (pure tone audiograms, tympanometry, behavioral response), comprise an important component of the management of the cleft patient. Even with early palate closure, children with posterior clefts are likely to have recurring episodes of otitis media or effusion. Since the review by Bluestone 3 in 1978 confirming the high prevalence of these problems, many surgeons have advocated the early placement of tympanostomy tubes. With reports 23 , 38 based on myringotomy results of a 95% to 100% prevalence of effusion in children less than 20 months of age with posterior clefts, prophylactic or therapeutic placement of tubes at 3 months of age (if lip repair is to be done) has been recommended, thus "saving an anesthesia." Not all would agree, but would

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suggest that to wait until chronic effusion is established as a problem has not been shown to carry increased risk of long-term hearing or developmental problems. The real question, therefore, is not whether to place tympanostomy tubes, but when, i.e., prophylactically at the time of some other surgery or only as indicated by the presence of middle ear pathology. If the latter course is followed, the expertise of an experienced examiner is a vital component of management of these patients. One should not be misled into believing that with the placement of tympanostomy tubes the otologic component of management is concluded. These tubes are eventually extruded and must be replaced, many times in some patients. Tympanosclerosis or chronic perforation may result, with some children ultimately requiring tympanoplasty. Additionally, our experience has been that once speech and language have developed, careful ear follow-up seems to lose its priority for the family in the face of the multitude of other ongoing speech, dental, and cosmetic problems. Consequently, some patients may present later with glue ear, adhesive otitis, or cholesteatoma, which require late placement of ventilatory tubes or surgery. One spinoff of studies on these otologic issues comes from the work of Watson et al,55 who found a higher incidence of sensorineural hearing loss in patients with late palate closure. Bluestone 3 also noted studies indicating a 31 % incidence of sensorineural or mixed hearing loss in adults with cleft palates. The pathogenetic mechanism of sensorineuralloss in patients with clefts remains unclear, but this problem alone underscores the need for these patients to be followed through adolescence and into adulthood. Speech and Language Pathology U nrepaired clefts result in articulation errors characterized by omissions, substitutions, or distortions. Plosive or pressure consonants that require velopharyngeal closure to produce intraoral pressure may be absent. Glottal stops or pharyngeal fricatives may be substituted for some plosive or tongue tip sounds. Consonant distortion occurs when air is emitted improperly through the nose. These faulty speech patterns, once firmly learned, may be retained indefinitely.4 The data seem clear that early palate closure, before such abnormal patterns are established, improves outcome significantly.36 When surgery is not feasible, use of an obturator or speech bulb to achieve palate closure has been investigated, but may not be effective. 36 Prosthetic success depends on the child's tolerance of the device, dental status relative to device retention, and the availability of a skilled maxillofacial prosthodontist. Even with early palate repair, not all subsequent speech problems are articulation errors amenable to speech therapy. There must be continual periodic assessment of the adequacy of velopharyngeal closure, with techniques such as taped periodic speech samples and endoscopic or video fluoroscopic studies. lO Velopharyngeal incompetence (i.e., inappropriate nasal escape or talking through the nose)

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caused by a short palate, poor palate mobility, or adenoid involution32 occurs in 20% of patients. This compares to 80% occurrence in patients with late repaired clefts. Nasal escape during speech production may also be attributable to fistulae. The speech pathologist plays the pivotal role in determining the need for secondary management of the repaired soft palate (pharyngeal flap procedure) or fistula repair. The speech pathologist must also sort out those speech problems caused by dental deviations and advise the dental members of the team accordingly. There is a high probability that articulation problems will exist and will be difficult to correct when they are caused by a short or narrow maxillary arch. 45 An open bite or inappropriate shifting of teeth may affect tongue tip (alveolar) sounds. Children with clefts are at increased risk for language delays, receptive or expressive. Early intervention with parental counseling and a language stimulation program may initially be more important for some patients than remediation of articulation errors. Noting that some articulation and language deviations may persist into adulthood, the role of the speech and language pathologist is seen as an ongoing one. The speech and language pathologist is supported in this longterm effort by the knowledge that these patients have the capacity for further improvement beyond the age at which noncleft individuals achieve speech maturity. 52 Pediatric Dentistry In one sense, the pediatric dentist on the cleft palate team has the same responsibility to patients with clefts as with any other patient, i.e., to ensure the best of dental health. Besides the obvious importance for the patient, excellent dental health is a prerequisite to optimal orthodontic care. Unfortunately, the patient with a cleft is faced with a multitude of both anatomic and physiologic problems. Defects of tooth development can occur, such as supernumerary teeth, pegshaped teeth, hypoplastic teeth, or congenitally missing teeth (Fig. 3). When the alveolar ridge is clefted, the lateral incisor may be absent, replaced by small supernumerary teeth that may be already erupted at birth. Interestingly, individuals with cleft lip alone, without involvement of the alveolar ridge, may have a germinated, fused, or supernumerary tooth in the lateral incisor position on the cleft side. 25 Physiologically, there is a high incidence of enamel hypoplasia in the incisors adjacent to the cleft in both deciduous and permanent dentition. Enamel defects may also be present in some cuspids and molars. These defects may be congenital. Alternatively, they may be acquired secondary to surgical trauma from early lip or anterior palate repair, or they may be caused by the feeding difficulties, infections, febrile episodes, or drug treatment incurred in the first few months of life. In addition to enamel defects, children with cleft lip or palate are at higher risk for gingivitis, crossbite, and crowding. That children with clefts should be considered at increased risk for caries has been substantiated by Dahllof et a1 9 who documented nearly twice the rate of decayed and filled surfaces in a group of children with clefts as

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Figure Figure3.3.A, A,Repaired Repairedright rightcleft cleftlip lipand andpalate. palate.Note Notethe themidline midlinefistula fistulaafter afterpalate palate expansion expansionand andthe themissing missingupper upperright rightlateral lateralincisor. incisor.B,B,Repaired Repairedbilateral bilateralcleft cleftlip lipand and palate. palate.Again, Again,note notemissing missingupper upperright rightlateral lateralincisor incisorand andcanine. canine.Also Alsonote notethe thebilateral bilateral maxillary maxillaryarch archcollapse, collapse,with withthe theneed needfor forpalatal palatalexpansion expansiontotohelp helpcorrect correctcrossbite. crossbite.

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compared to healthy controls. Dahllof et al conclude their paper by noting the importance of preventive (and treatment) programs to facilitate later secondary surgical corrections and orthodontic treatment.

Orthodontics Orthodontic management of the patient with a cleft must be well integrated with the surgical management, the two working closely together to determine optimal timing of intervention. Timing determinations are based on an understanding of the skeletal and dental components of the problem and an appreciation of the critical periods in growth and development. Thus, orthodontic involvement occurs at four stages: preprimary dentition, full primary dentition, mixed dentition, and full permanent dentition. In the immediate newborn period, use of obturators has been advocated by some. 31 Obturators are believed to improve feeding, and, therefore, parental bonding and growth; improve speech by allowing for early development of sounds for the child with a cleft; maintain maxillary arch width and dental alignment; and improve palatal growth by retaining the tongue in proper position. Trenite et al 51 believe that obturators actually stimulate palatal growth and avoid palatal and midfacial growth inhibition presumed to be a consequence of early palate surgery. Much of this has yet to be substantiated by controlled studies, and none have yet been done to determine the longterm effects of obturators on maxillofacial growth. 47 In the stage of full primary dentition, orthodontics may be needed to correct anterior crossbite (upper incisors behind lower) caused by maxillary collapse. Additionally, some would start palatal expansion at 5 to 6 years of age, depending on the amount of collapse (medially) of the lateral palatal segments. In these younger patients, retention of expansion is then achieved with the use of a removable appliance. 16 Many orthodontists prefer to do palatal expansion a few years later, just before bone grafting,37 one of the most significant advances in cleft management in recent years. Palatal expansion may result in the opening of an oral-nasal fistula in the alveolar process and anterior palate. 16 Grafting of cancellous bone from the iliac crest into the alveolar defect results in a restored maxillary arch functionally able to allow teeth to erupt normally or be moved into the former cleft site. Thus, the mixed dentition stage timing for bone grafting is important. Eight to 9 years of age is early enough to allow for eruption of the permanent maxillary canine through the transplant site, and late enough to avoid interference with anterior maxillary growth, which is nearly complete by this age. 47 Once the maxillary canines have erupted and full permanent dentition is achieved, conventional orthodontics may then proceed with fixed appliances and prosthetic tooth replacement and bridgework when indicated.

Social Work Perhaps the greatest obstacle to achieving optimal results in the management of children with cleft lip or palate is patient and family

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compliance problems. A whole host of socioeconomic issues may cause misunderstandings, missed appointments, and so forth. When faced with such a multitude of problems in the child and a multiplicity of (expensive) interventions, it is little wonder that many families are overwhelmed. The social worker is counted on by the team to help families understand the importance of compliance. At the same time, the team relies on the social worker to keep the team apprised of family circumstances that may be seen by the family, or in fact may be of higher immediate priority than the next planned intervention. Although most health insurance covers most of the surgical care of cleft patients, many families have little or no coverage. The social worker has the knowledge of other resources, such as crippled children's funds, that are accessible in many states. A more fundamental issue is simple transportation costs to the clinic site or specialists offices, a real problem for some families. Perhaps of greatest importance is for someone on the cleft palate team to be a good listener. Optimal care is achieved by knowing the patient and family well. Because each member of the team is a specialist, they are perceived by some families in just that way, communicating only to the social worker those issues that may, in fact, have significant impact on overall management. Genetics The geneticist's role on the cleft palate team is as one would predict: to advise the patient and family as to the likely cause of the malformation, its recurrence risks, and the possibility of associated anomalies. The heterogeneity of clefting patterns and the difficulties in elucidating the cause have been noted. It is no surprise then that clefting inheritance is believed to be multifactorial or multigenic. Although simple mendelian genetics rarely apply, certain patterns emerge that are helpful in a practical genetic approach to these children. Patients with cleft lip or palate may be divided, for genetic purposes, into three groups: syndromic, familial, or isolated. The simplest genetic approach to these patients is first by syndrome identification, then by family history. From a genetic counseling standpoint, the recognizable syndromes are the most straightforward to deal with because many of these are well recognized as following mendelian patterns that predict 25%,50%, or 100% recurrence risks. For an excellent, well-organized approach to over 150 syndromes, utilizing the cleft as the malformation starting point, the reader is referred to the classic work of Cohen. 8 Once a syndrome classification has been ruled out, family history defines the next group. Familial classification is defined as a situation in which the patient has a clefted first- or second-degree relative. Recurrence risk in this group is at least 16%.2,24,33 If no syndrome has been identified and the family history is negative, classification is said to be isolated, with a recurrence risk of 1% to 3%2 (acknowledging the possibility that the child may be the first in a future familial pattern). Statistically, isolated has been believed to be

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the most common group by some authors, comprising 70% to 80% of the cleft population, but as little as 40% by others who believe that multiple malformation syndromes are more common than those identified thus farP Some of this disturbing discrepancy is caused by problems in definition, the latter investigators failing to differentiate between malformations simply associated with cleft and those that fit into recognizable patterns. Another important role of the geneticist is an appreciation of other nonsyndromic malformations statistically associated with clefts. In 1981, Geis et al 19 screened a population of 282 patients with clefts for congenital heart disease, finding a tenfold increase in prevalence over the general population. In 1984, Strunz et al 49 identified varying degrees of pathologic alterations in the urinary tract in 26 of 68 children with clefts. Before concluding this section, special attention needs to be given to Stickler syndrome, an autosomal dominant disorder that may be extremely difficult to recognize in infants. Retinal detachment, severe myopia, or glaucoma may be devastating in these children, who present with isolated cleft palate associated with epicanthal folds and flat facies. 27 Pediatrician The pediatrician has a triple role on the cleft palate team. As the only true primary care individual on the team, the pediatrician has the charge of monitoring "the whole patient." With the pediatric foundation of growth and development in mind, the pediatric team member must deal with all those issues common to patients with clefts, e.g., feeding problems, developmental delays, associated anomalies, behavior disorders, or recurrent infections. Second, the pediatric member of the cleft palate team must be prepared to serve the family as a second opinion to the child's primary care provider, with respect to any and all of the child's problems. (We draw the line at doing school physicals.) Third, the pediatrician on the cleft palate team serves as an advisor to other team members. The team depends on the pediatrician to apprise them of other cleft or noncleft-related medical or behavioral problems, thus influencing the timing of procedures or even the selection of specific technique. For example, poor feeding and failure to thrive in the infant may necessitate delay in lip closure until better feeding technique and weight gain can be achieved or appropriate examination completed. Conversely, judgment may suggest proceeding with lip closure to improve mechanics or parental-infant bonding. Family or patient history of asthma, bleeding disorders, or medication allergies are preoperative risks that must be brought to the attention of the team when considering procedures requiring anesthesia. The pediatrician may be called on by the surgeon or orthodontist for advice on the retarded or hyperactive child's ability to tolerate certain surgical procedures (postoperatively) or dental and orthodontic appliances. All of the above notwithstanding, the most important aspect of the

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role of the pediatrician is to serve as a patient advocate. This may mean advising as to the child's ability to tolerate procedures or, more commonly, helping to prioritize procedures in a sequence that takes into account the wants and needs of the individual child and family.

PRIMARY CARE OF THE CLEFT PATIENT Regardless of whether support of a cleft palate team is available, the charge to the primary care physician remains that of fostering normal growth and development. Growth Three issues emerge as unique in regard to growth of the child with cleft lip or palate: infant feeding problems, normal (for the child with a cleft) growth patterns, and growth hormone. Feeding. It is difficult enough to achieve parental bonding with an infant who has a craniofacial defect. Without proper guidance and support, feeding problems in these infants can be an added exercise in frustration to a parent. Assuming no concomitant neurologic problems, feeding difficulties are anticipated as a function of the type of cleft. 14 Generally, children with cleft lip alone and children with isolated cleft of the soft palate have little trouble with breast or bottle feeding. In children with cleft lip, the breast may well help to fill the defect, allowing the infant to develop the negative intraoral pressure necessary to feed efficiently. When bottle fed, a large-based nipple may do the same. When only a posterior cleft is present, only the nipple shape may make a difference in bottle feeding. Conversely, children with a cleft oflip and palate are likely to have significant problems generating negative intraoral pressure, creating difficulty with both breast and bottle feeding. These children generally require some mechanical assistance in facilitating milk flow. Various nipples have been tried in the past, usually with an enlarged or cross-cut opening to allow for more gravitational milk flow, with varying degrees of success. Today the most su~cessful device is a simple soft plastic squeezable bottle, such as the Mead-Johnson cleft palate feeder, that affords control of milk volumes and flow rates that the infant can handle. Because some negative pressure may be achieved after surgical lip closure, many infants subsequently improve in feeding. Those mothers who prefer breast feeding and have been feeding pumped breast milk may, after lip closure, find success at breast feeding. 6 Keeping in mind that all babies are different and the diversity of cleft patterns, any given infant may be more successful with breast or bottle feeding than predicted. Mothers should be encouraged to try their own method of preference, and through experimentation they may well find a technique that works well for their child. It is up to the primary care physician to monitor for evidence of poor weight gain in

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the infant or difficulties with bonding because of slow, frustrating feedings. Growth Patterns. There is no evidence for increased prevalence of intrauterine growth retardation in the cleft population when multiple malformation syndromes are excluded from the data. 13 Poor weight gain in the first 2 to 3 months of extrauterine life is, unfortunately, not an uncommon phenomenon. This is usually caused by the feeding problems noted. Subsequently, as the result of lip repair, neurologic maturation, discovery of successful technique, or some combination thereof, infants with a cleft generally begin to do well and show catchup weight gain. As more longitudinal growth studies have been completed, it has become increasingly clear that some children with clefts show short stature growth patterns, the explanation for which remains unclear. Consistent in the data among several investigators is that isolated cleft lip and bilateral cleft lip and palate children do grow normally. Conversely, some children with isolated cleft palate show a progressive decrease in height percentiles to 8 years of age. Beyond 8 years of age 26% are below the fifth percentile. 13 Some children with unilateral cleft lip and palate also show a decrease in height percentiles, to 4 years of age in this group; 24% of children with unilateral cleft lip and palate were found by Bowers et a1 4, 5 to be below the tenth percentile. They additionally found that boys in this group were thinner than their normal peers. Growth Hormone. Postulating a defect in Rathke's pouch or adenohypohysis as it would relate embryologically to clefting is inconsistent with the previously noted normal stature in children with bilateral cleft lip and palate. Although some investigators have found growth hormone (as well as adrenocorticotropic hormone and thyroidstimulating hormone) deficiencies at a rate 40 times that of children without clefts,44 these have been studies of very small numbers of children. Additionally, growth hormone deficiency did not correlate with type of cleft in a manner consistent with the distribution of short stature among cleft types, as noted earlier. The question of growth hormone deficiency and clefting is ripe for further investigation, particularly in view of the advances made in the past 10 years in the understanding and diagnosis of growth hormone deficiency. Development Developmental issues in patients with clefts are primarily neurologic or psychosocial. Again excluding multiple malformation syndromes, a higher incidence of psychomotor retardation and attention deficits have been noted anecdotally by many cleft palate teams. Whether this is in fact a true association with clefting or caused by other independent socioeconomic variables remains to be elucidated. Richman et al 42 in 1988 reported a prevalence of reading disability of 35% in a group of 172 children with clefts. Moreover, this disability was more prevalent at younger ages, suggesting an association with speech and language disorders, although the authors are careful to point out a number of other variables that might be factors. Addi-

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tionally, Richman et al also found a dichotomy between cleft lip and palate children and those with cleft palate alone. In this study, the former group's reading disability tended to improve with time, while the latter group's did not. Lastly, although the entire effort of the cleft lip and palate team is directed toward the achievement of normalcy, no result is perfect and all teams note varying degrees of surgical success. Social adjustment for the child with a cleft is directly influenced by positive parent-child interaction and by peer acceptance. Problems with self esteem and social inhibition increase as age increases, beginning to appear in these children as early as 7 years of age. Although improvement in psychosocial adjustment occurs after surgery, many children still demonstrate impaired peer relationships, overdependence on adults, low self esteem, and feelings of anger or depression. 39 The primary care physician, cognizant of these issues, must (1) work very hard to foster positive relationships between parents and their child with a cleft; (2) provide support and understanding, especially as the child approaches the added burden of adolescence; and (3) constantly look for the clues indicating depression or avoidance disorder, indicating a need for psychiatric intervention. One of the best resources available to both the pediatrician and the patient, to be used early and continuously, is a parent support group. There are 76 such groups in 30 US states as well as internationally.54 When available, there is no better resource for reassurance and understanding. Additionally, a variety of publications, both free and at a nominal charge, as well as a bibliography of other publications may be obtained from Cleft Palate Foundation publications: Cleft Palate Foundation, 1218 Grandview Ave, Pittsburgh, PA 15211, telephone (412) 481-1376.

SUMMARY We have attempted to elucidate, first through an examination of the basic science of clefting and then through a description of the roles of the various members of the cleft palate team, an understanding of the multiplicity of problems faced by this heterogeneous group of children with cleft lip or palate. With an incidence of cleft of 1 in 700 births, all practicing pediatricians will at some time be faced with a patient with a cleft. Pediatric management begins in the hospital nursery by ruling out possible associated anomalies (e.g., congenital heart disease or urinary tract) or syndromes. At the same time, cleft palate nursers may be needed to overcome feeding problems. Simultaneously, counseling for the family begins with a positive attitude toward outcome and an initial explanation of the schedule of corrective procedures, i.e., lip repair at 3 months of age and palate repair at about 1 year. The counseling should incorporate an understanding of the cause of clefting, both its genetics and teratogenetics, and then proceed to noting the actual recurrence risks of 1% for a new cleft and 16% if there is a positive family history in a first-degree relative. The

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recurrence risks in recognized syndromes may follow mendelian patterns. Later, the pediatrician can help the family deal with multiple ear infections and the likely need for tympanostomy tubes, anticipating the need for tubes in a way that might facilitate placement at the time of anesthesia for the lip or the palate repairs. An understanding of the speech and language difficulties that may be encountered in later infancy may relieve parental anxiety. Later, after palate repair, knowledge of velopharyngeal incompetence may avert premature hypernasal speech problems caused by adenoidectomy, which should be avoided if at all possible. The pediatrician needs to be aware of the tooth malformations that accompany clefting of the alveolus as well as the increased susceptibility to caries, so that the family may be directed to early intervention by the pediatric dentist. With support by pediatrics of the efforts of pediatric dentistry, the child is then in optimal condition for orthodontics. Continually keeping the child's emotional adjustment in mind, the pediatrician can encourage the child and family, knowing that with palatal expansion techniques plus possible bone graft surgery to the cleft alveolus at about 9 years of age, the child may be orthodontically corrected to very near normal. Cosmetically, the pediatrician may remind the family of the availability of lip revision surgery, while at the same time counseling the patient on the importance of awaiting the completion of the adolescent growth spurt before nasal reconstruction. Cognizant of the risk of emotional difficulties in children with facial deformities, the pediatrician must not lose sight of the added burdens of short stature, with or without growth hormone deficiency, for which these children are at risk. In addition to the growth issues, the pediatrician is also in a position to anticipate learning or attentional difficulties. If children with cleft lips or palates are to maximally approach the goal of normal growth and development, they and their parents need all the help we can provide. At a minimum, pediatricians can help families understand and cope with the complex and ongoing problems associated with a cleft deformity. With the aid of parent support groups, the pediatrician and the other cleft palate team management professionals have the gratifying opportunity to do a great deal for children with cleft lip or palate. ACKNOWLEDGMENT

Many thanks to Hans Wilhelmsen MD, F ACS, plastic surgeon and team leader; to Pat Landis, speech pathologist and team spirit; and to the other fine volunteer members of the Kernan's Cleft Palate Diagnostic Team, for providing the author the opportunity to make a contribution to their excellent care of these children.

REFERENCES 1. Bardoch J, Morris H, Olin W: Late results of primary veloplasty: The Marburg project. Plast Reconstr Surg 73:207-215, 1984 2. Bixler D: Genetics and clefting. Cleft Palate J 18:10-18, 1981

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3. Bluestone CD: Prevalence and pathogenesis of ear disease and hearing loss. In Graham MD (ed): Cleft Palate, Middle Ear Disease, and Hearing Loss. Springfield, Charles C Thomas, 1978 4. Bowers E, Mayro R, Whitaker L, et al: General body growth in children with cleft palate and related disorders: Age differences. Am J Phys Anthropol 75:503-515, 1988 5. Bowers E, Mayro R, Whitaker L, et al: General body growth in children with clefts of the lip, palate, and craniofacial structure. Scand J Plast Reconstr Surg 21:7-14, 1987 6. Clarren S, Anderson B, WolfL: Feeding infants with cleft lip, cleft palate, or cleft lip and palate. Cleft Palate J 24:244-249, 1987 7. Clarke L, Hepworth W, Carey J, et al: Chondodystrophic mice with coincidental agnathia: Evidence for the tongue obstruction hypothesis in cleft palate. Teratology 38:565-570, 1988 8. Cohen M: Syndromes with cleft lip and cleft palate. Cleft Palate J 15:306-327, 1978 9. DahllofG, Ussisoo-Joandi R, Idelberg M: Caries, gingivitis, and dental abnormalities in preschool children with cleft lip and/or palate. Cleft Palate J 26:233-237, 1989 10. D'Antonio L, Marsh J, Province M, et al: Reliability of flexible fiberoptic nasopharyngoscopy for evaluation of velopharyngeal function in a clinical population. Cleft Palate J 26:223-224, 1989 11. Domet M: The general pediatrician and the craniofacial defects team. Ear Nose Throat J 65:296-304, 1986 12. Dorf D, Curtin J: Early cleft palate repair and speech outcome. Plast Reconstr Surg 70:75,1982 13. Duncan P, Shapiro L, Soley R, et al: Linear growth patterns in patients with cleft lip or palate or both. Am J Dis Child 137:159-163, 1983 14. Dunning Y: Feeding babies with cleft lip and palate. Nursing Times 82:46-47, 1986 15. Egyedi P: Timing of palatal closure. Journal of Maxillo-Facial Surgery 13:177-182, 1985 16. Enemark H, Bolund S, Jorgensen I: Evaluation of unilateral cleft lip and palate treatment: long term results. Cleft Palate J 27:354-361, 1990 17. Ferguson M: Palate development: Mechanisms and malformations. Ir J Med Sci 156:309-315, 1987 18. Finkelstein Y, Talmi Y, Nachmani A: Levator veli palatini muscle and eustachian tube function. Plast Reconstr Surg 85:684-692, 1990 18a. Fogh-Anderson P: Inheritance of Hare Lip and Cleft Palate. Copenhagen, Nordisk Forlag Arnold Busch, 1942 19. Geis N, Seto B, Bartoshesky L, et al: The prevalance of congenital heart disease among the population of a metropolitan cleft lip and palate clinic. Cleft Palate J 18:19-23,1981 20. Godel V, Nemet P, Lazar M: The Wagner-Strickler syndrome complex. Doc OphthalmoI52:179-188, 1981 21. Goldberg R, Shprintzen R: Hirschsprung megacolon and cleft palate in two sibs. J Craniofac Genet Dev BioI 1:185-189, 1981 22. Goldberg W, Ferguson F, Miles R: Successful use of a feeding obturator for an infant with a cleft palate. Special Care Dentistry 8:86-89, 1988 23. Grant HR, Quiney RE, Mercer DM, et al: Cleft palate and glue ear. Arch Dis Child 63:176-179,1988 24. Habib Z: Genetic counselling and genetics of cleft lip and cleft palate. Obstet Gynecol Surv 33:441-447, 1978 25. Hall R: Care of adolescents with cleft lip and palate: The role of the general dental practitioner. Int Dent J 36:120-130,1986 26. Hook E: "Incidence" and "prevalence" as measures of the frequency of congenital malformations and genetic outcomes: Application to oral clefts. Cleft Palate J 25:97-101, 1988 27. Jones M: Etiology of facial clefts: Prospective evaluation of 428 patients. Cleft Palate J 25:16-20,1988 28. Khoury M, Gomez-Farias M, Mulinare J:. Does maternal cigarette smoking cause cleft lip and palate in offspring. Am J Dis Child 143:333-337, 1989

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29. Khoury M, Weinstein A, Panny S, et al: Maternal cigarette smoking and oral clefts: A population-based study. Am J Public Health 77:623-625, 1987 30. Latham R: The pathogenesis of cleft palate associated with the Pierre-Robin syndrome, an analysis of a seventeen-week human fetus. Br J Plast Surg 19:205-214, 1966 31. Markowitz J, Gerry R, Fleischner R: Immediate obturation of neonatal cleft palates. Mt Sinai J Med 46:123-129,1979 32. Mason R, Warren D: Adenoid involution and developing hypernasality in cleft palate. J Speech Hear Disord 45:469-480, 1980 33. Melnick J: Cleft lip with or without cleft palate: Etiology and pathogenesis. CDA J 14:92-98,1986 34. Molsted K, Palmberg A, Dahl E, et al: Malocclusion in complete unilateral and bilateral cleft lip and palate. Scand J Plast Reconstr Surg 21:81-85, 1987 35. Moss A, Jones K, Pigott R: Submucous cleft palate in the differential diagnosis of feeding difficulties. Arch Dis Child 65:182-184,1990 36. Noordhoff M, Kuo J, Huang H, et al: Development of articulation before delayed hard-palate closure in children with cleft palate: A cross sectional study. Plast Reconstr Surg 80:518-524, 1987 37. Nystrom M, Ranta R: Effect of timing and method of cleft palate repair on dental arches at the age of three years. Cleft Palate J 27:349-353,1990 38. Paradise JL: Otitis media in infants and children. Pediatrics 65:917, 1980 39. Pillemer F, Cook K: The psychosocial adjustment of pediatric craniofacial patients after surgery. Cleft Palate J 26:201-207, 1989 40. Review Article: Cleft palate and glue ear. Lancet 1:1262,1988 41. Richman L: Commentary. Cleft Palate J 26:117-118,1989 42. Richman L, Eliason M, Lindgren S: Reading disability in children with clefts. Cleft Palate J 25:21-25, 1988 43. Rohrich R, Byrd H: Optimal timing of cleft palate closure. Clin Plast Surg 17 :27-35, 1990 44. Rudman D, Davis T, Priest J, et al: Prevalence of growth hormone deficiency in children with cleft lip or palate. J Pediatr 93:378-382, 1978 45. Schaefer L, Sullivan M: The speech-language pathologist's role on the craniofacial team. Ear Nose Throat J 65:346-352, 1986 46. Schendel S, Pearl R, De'Armond S: Pathophysiology of cleft lip muscle. Plast Reconstr Surg 83:777-784,1989 47. Semb G: Effect of alveolar bone grafting on maxillary growth in unilateral cleft lip and palate patients. Cleft Palate J 25:288-295, 1988 48. Shaw W, Semb G: Current approaches to the orthodontic management of cleft lip and palate. Journal of the Royal Society of Medicine 83:30-33, 1989 49. Struntz V, Westesmann V, Biewald W: Incidence of malformations of the urinary tract in association with clefts of lip, alveolus, and palate. J Max-Fac Surg 12:14, 1984 50. Styer G, Freeh K: Feeding infants with cleft lip and/or palate. J Obstet Gynecol Neonatal Nurs 10:329-332, 1981 51. Trenite G, Weil J, Roos P: Observation: A comment on "A discussion of presurgical orthodontics in patients with clefts." Cleft Palate J 27:419-424, 1990 52. Van Denmark DR, Morris HL, Vandehar C: Patterns of articulation abilities in speakers with cleft palate. Cleft Palate J 16:230-239, 1979 53. Vig K: Orthodontic considerations applied to craniofacial dysmorphology. Cleft Palate J 27:141-145,1990 54. 1989 Membership Team Directory, American Cleft Palate-Craniofacial Association, Pittsburgh 55. Watson DJ, Rohrich RJ, Poole MD, et al: The effect on the ear oflate closure of the cleft hard palate. Br J Plast Surg 39:190-193,1986

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ADDITIONAL READING FROM THE CLEFT PALATE FOUNDATION 1. For parents of newborn babies with cleft lip/palate, 1980. Pp 4. $.20 each. 2. Cleft lip and cleft palate: The child from birth to three years, 1980. Pp 16. $.50 each. 3. Cleft lip and cleft palate: The child from three to twelve years, 1980. Pp 16. $.50 each. 4. Information for the teenager born with a cleft lip and/or cleft palate, 1980. Pp 12. $.50 each. 5. Feeding an infant with a cleft, 1986. Pp II. $.75 each. 6. The genetics of cleft lip and palate: Information for families, 1987. Pp 8. $.75 each. 7. Organizing support groups for patients and parents of children with cleft palate, 1987. Pp 20. $1.50 each. 8. CPF Newsletter for parents and patients, $5.00 per year. 9. Catalogue of audiovisual aids/cleft lip and palate, 1986. Pp 20. $1.00 each. 10. Free information about cleft lip and palate; choosing a cleft palate or carniofacial team; dental care; financial assistance; Crouzon disease; Pierre Robin malformation sequence; Treacher Collins syndrome; The National Cleft Palate Association.

Address reprint requests to Felix L. Kaufman, MD 120 Sister Pierre Dr. Suite 403 Towson, MD 21204

Managing the cleft lip and palate patient.

We have attempted to elucidate, first through an examination of the basic science of clefting and then through a description of the roles of the vario...
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