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Neuropsychological Impact of Facial Deformities in Children Neurodevelopmental Role of the Face in Communication and Bonding

Bruce D. Perry, M.D., Ph.D.
Danita I. Czyzewski, Ph.D.
Molly Lopez
Laura C. Spiller
Diane Treadwell-Deering, M.D.


The ChildTrauma Academy
www.ChildTrauma.org


*This is an Academy version of an article originally appearing in Clinics in Plastic Surgery.

Official citationPerry, BD, Czyzewski, D, Lopez, M, Spiller, L, Treadwell-Deering, D:  Neuropsychologic Impact of Facial Deformities in Children-Neurodevelopmental Role of the Face in Communication and Bonding. Clinics in Plastic Surgery, 25:587-597, 1998.


Background: Communication in Human Living Groups

Communication between one human and another is the hallmark of our species. Communication was the critical capacity required for survival during the thousands of generations of our evolution. Naked, slow, weak and without biological armor or weapons, humans survived by living and hunting in groups. Interdependent individuals created a strong, flexible and adaptive whole? the band, the clan, the tribe.

Although physically separate and self-aware, individual humans are linked by the invisible, yet biologic, bonds of sensation, perception and communication into larger biological units ? collections of individuals -- groups. One individual may belong to many groups ? a couple, a family, a working group ? each with unique and dynamic properties. Each group has a set of tasks and a set of rewards for the individual and, as a whole, the integrity and function of the group is formed, maintained and changed by social interaction.

Central to the invisible biological processes which allow social interaction is communication -- the capacity to perceive and understand others and to express meaning and intention to others. As might be expected, after thousands of generations, the human brain developed remarkable biologic apparatus dedicated specifically to social perception and communication, verbal and non-verbal. These underlying biologic properties are continually at play in all human interactions including sensing, processing, perceiving, storing and acting on signals from other humans. All human interactions are governed by core principles of communication which, in turn, are the product of neurobiologic processes shaped by thousands of years of evolutionary pressures.


Nonverbal Communication

A primary key to survival and reproductive success is the amazing capacity of the human brain for nonverbal communication. Indeed, humankind has communicated with complex verbal language for only a short period in our history and only a fraction of our total brain is dedicated to verbal communication. Most of our communication with others is nonverbal, and a huge percentage of what our brains perceive in communication from others is focused (even without our being aware) on the nonverbal signals, including eye movements, facial gestures, tone of voice, latency to respond to question, the move of a hand or tip of the head. Even as one area of the brains is processing and attending to the words in an interaction, more areas are continually focusing on, and responding to, the nonverbal actions which accompany the words.

The Human Face-The Primary Tool of Human Communication

During the thousands of generations of the early history of our species, we lived in small bands of 20 to 50 members. Individual survival depended upon cooperation and communication. The remarkable expressive communication capacity of the face was further refined. Facial expressions became the most important of all social communication instruments. Facial expressions have the capacity to reflect the internal emotional state of the individual, and elicit a specific emotional and social response from an individual, including a smile, frown, glare, snarl, ignore, stare, "come hither," or "get lost." The face expresses pain, ecstasy, anger, fear, doubt, confidence and threat.

During the development of an individual, a small catalogue of familiar faces, the faces of the family and the band, are the stored templates for "familiar/safe." And in these familiar faces, the infant and child learns the nonverbal language of the group, just as surely as they learn the verbal language. An unfamiliar face will elicit a low-level alarm response in any individual (see box).   All new faces are judged to be threatening until proven otherwise because (1) in general, the brain?s information matching process is very conservative, and all novel situations and new information are judged to be threatening until proven otherwise; (2) the human brain evolved in a world where for thousands of generations, the major threats to any individual were other humans from other clans. A new person, a new face in the typical interaction from 100,000 years ago to 5,000 years ago meant that there were other humans around, competing for the same water, fruits, game, cave. This new person was as likely to attack you, drive you away, steal your camping site, take the young and rape the women of your band, as he or she was to decide to affiliate or cooperate. Across generations, wariness to new individuals, new groups and new ideas was selected and built into the circuits of the human brain?s alarm response.

 

Clinical Issue: RECOGNITION

Over hundreds of thousands of years, protohominids and human beings lived in groups of no more than 30. Thus, the biologic capacity for the number of familiar or "safe" faces was quite small. And these "template" faces and facial features, like all other neurodevelopmentally-determined templates for emotional, behavioral and social functioning, were set during childhood. This tendency to have an alarm response when exposed to an unfamiliar face or mismatched facial features is the root of many human behaviors. For example, despite very minor differences in where facial features are placed, almost all people can immediately recognize the difference in a child with Down?s syndrome. This matching against previous template faces is at the root of racism (and a strong argument that children of different races should be together in school and play, allowing them to build in diverse set of internal templates).

This capacity to match diverse information with previous templates of multisensorial input is also at the root of recognition of deceit. When words do not match with familiar body movement, facial expression, or tone of voice, the brain senses a multisensory mismatch. This is, of course, why children raised with domestic violence or in multiple foster homes internalize patterns of communication and interaction that are very distorted and often destructive (e.g., they talk about association of intimacy, power, violence, threat, ). Often, children raised in these deceitful settings, can lie easily without detection. They have not internalized the same nonverbal templates associated with deceit. For these children, the development of sociopathic characteristics is merely an adaptation to the deceitful, inconsistent, and unrewarding world their caregivers have created for them.

 

Through these thousands of generations of evolutionary selection, the brain developed the capacity to read nonverbal cues, many of which are communicated via facial expression. The brain has special face and expression recognition capabilities, and, through a process of matching expressions and faces with previously known expressions and faces, makes decisions about the familiarity and intentionality of a specific interaction.

Because we have a limited capacity for categorizing and matching specific faces and facial expressions, the brain uses other body movements, postures, and other symbolic trappings of recognition (e.g., clothes, uniforms, style of hair cut), to make secondary decisions about recognition. For example, you may not recognize a face, but the haircut, clothes, or manner of interaction can readily identify him or her as familiar and good or as familiar and bad. This categorizing tendency, of course, is the basis for a host of well-described and common phenomenon in human interaction, including first impressions or using celebrities to sell products or ideas. A classic example of this in the mental health field is transference. This is phenomenon of attaching multiple attributes of a past relationship to one in the present, when only one of those attributes may truly be present (e.g., reacting to a male therapist with the intensity that was present in a paternal relationship).

The human brain is constantly sensing, perceiving and storing information from the outside world. In an almost simultaneous fashion, these new sensations and perceptions are being compared with previous patterns of sensation and perception.

The Human Face in Attachment and Bonding

In the infant brain, for which a given experience is truly new and the sensations and perceptions are completely novel, the patterns of neuronal activity which result from an experience (the combination of integrated sensations) create a template against which all future similar experiences will be compared. The first blurry images of mother?s face, the smell, warmth, caress, taste of mother?s breast, sound of the mother?s soothing hum ? the "somatosensory bath" ? create patterned neuronal activity throughout the brain, which, over time, becomes the child?s template against which a host of related templates will be created. In the newborn, the vast majority of waking experience is provided by the mother or primary caregiver. Thus this relationship and all of the somatosensory experiences associated with it in the very first weeks of a child?s life begin to create the templates for feeding, soothing, rocking, singing, touching and all future human social interactions.

Furthermore, because of the sequential development of the brain, these initial core templates begin to be the patterned core of neurobiologic organization that will mediate all brain-related functions, including thinking, feeling and behaving. The crucial role of experiences in the first 3 years of life should not be underestimated. Organizing experiences that provide the set of neurobiological templates against which all future experiences will be compared and categorized take place during infancy and early childhood.

If the first organizing experiences are rich in sensory stimulation, predictable, nurturing, and consistent, the child builds the flexible and strong templates against which future development can procede in a healthy way. If however, the first set of experiences are chaotic, inconsistent, and devoid of stimulation or emotional attention, the child will have templates that match the disorganized, chaotic and emotionally empty experiences.

Attachment is described as a "affectional tie that one person forms to another person" (Lewis & Volkmar, 1990). Infants are born with behaviors that help to draw their caregivers to them, such as orientation to faces, rooting reflexes, and preference for their caregiver?s voice. It is these preadapted behaviors that help to set the pace for early caregiver-child interactions. It is the caregiver?s job to help facilitate synchronous, mutual interactions and be responsive to the infant?s cues. Attachment develops and strengthens as the child begins to recognize their caregiver?s responsiveness to their needs and begins to trust that the caregiver will continue to provide for them.

Young children can be described as having varying levels or types of attachment based on their behavior in interactions with their caregivers. Infants and young children with "secure" or healthy attachments can use their trust in their caregiver to begin to explore their environment and interact with others. Their caregiver provides a "safe base" from which they can explore and investigate. On the other hand, infants with insecure attachments are less likely to separate from their mothers and explore, seeming to need physical contact in order to be "attached".

Attachment can continue to have an important role in relationships in childhood and adulthood by providing the cognitive schemata for understanding how others will interact with an individual. For example, children with insecure attachments have been found to have poorer social skills, are less popular among peers, display more irritability and noncompliance, and are more aggressive towards peers and teachers (Arend, Gove & Sroufe, 1979; Sroufe, 1983; Waters, Wippman, & Sroufe, 1979). They have been found to be at greater risk for developing psychopathology (Lewis, Feiring, McGuffog, & Jaskir, 1984 ).

If the major tool for this crucial maternal-infant or caregiver-infant bonding process is altered by craniofacial deformities, there is potential for impaired bonding and attachment problems. The abnormal facial features of the infant are likely to elicit a different maternal pattern of gaze, eye-contact and interaction that has the potential to impair healthy neurodevelopment and result in significant neuropsychological problems.

Within this framework, the current research on the neuropsychological impact of craniofacial deformities is reviewed.


Research Findings

General Attractiveness

The role of physical attractiveness in society has been well documented. Studies have shown that attractive individuals have an advantage in employment settings and are more likely to be acquitted for a crime (Efran, 1974. This bias for beauty is also evident for children. Research suggests that unattractive children are generally treated more harshly by adults who do not know them (Berkowitz & Frodi, 1979) and are viewed as less developmentally competent (Ritter, Casey, & Langlois, 1991). Unattractive children are commonly not preferred as playmates by other children (Langlois & Downs, 1979) and are assumed by their peers and teachers to be more antisocial (Schneiderman, Harding, 1984; Lerner & Lerner, 1977). In addition, teachers expect attractive children to be more intelligent and well-behaved than their unattractive peers.

Yet the influence of attractiveness is widely believed to not influence parents of children. It is assumed all children are viewed as beautiful by a parent?s eye. And the phrase "a face only a mother could love" suggests this is true even for very unattractive children; however, recent studies have demonstrated that even parents are influenced by the attractiveness of their children.

In a study by Langlois and colleagues, 173 low-income mothers of newborns were observed in the hospital before discharge and again 3 months following the child?s birth. During the hospital observation, mothers of newborns rated as more attractive by independent raters engaged in more affectionate play than mothers of less attractive infants. Mothers of less attractive infants engaged in more routine caregiving and attending to others than mothers of attractive infants. When the infant was 3 months of age, mothers of attractive boys were shown to engage in more affectionate play than mothers of unattractive boys or attractive girls. The means for mothers of unattractive girls did not differ from the other means. Additionally, mothers of attractive girls participated in more affectionate grooming than mothers of attractive boys, whereas means for mothers of unattractive infants did not differ from the others. Mothers of unattractive infants saw more value in stimulating their infants than did mothers of attractive infants. These differing behaviors towards attractive versus unattractive infants could not be explained by differences in the infant?s behavior or by differences in the mother?s own attractiveness.

This influence of attractiveness on maternal behaviors and subsequently on mother-infant interactions can be viewed within the context of mother-child attachment.

Craniofacial Abnormalities: Research Findings

If mothers of average infants behave differently based on their infant?s attractiveness, having a child born with a craniofacial abnormality would be expected to negatively impact the mother-infant relationship; however, mothers of children with facial deformities have been found to rate themselves as more satisfied with their parenting role and had more positive life experiences following pregnancy than mothers of normal infants (Barden, Ford, Jensen, Rogers-Salyer, Salyer, 1989). In addition, mothers of craniofacially deformed infants rate their relationship with their child as average or above average (Barden, ). This contradiction between mothers? self-report and expected relational problems has been addressed in the literature.

Using observational techniques rather than self report, researchers have found interactions between mothers and their facially deformed infants to be at some increased risk for relational problems. By examining the behaviors that help create and maintain mother-infant attachment, a preliminary understanding of the impact of facial disfigurement on this bond is possible. Field and Vega-Lahr (1984) studied behaviors of 3-month-old infants with (n=12) and without (n=12) cleft lip and palates and their mothers. When placed face-to-face and asked to play with their infants, mothers of infants with anomalies smiled, vocalized, and imitated their infants less frequently than mothers of normal infants. They also spent less time playing games and showed less contingent responsivity. Although both groups of mothers gazed at their infants an equal amount of time, infants with craniofacial anomalies returned their mother?s gaze less than comparison infants. They also spent less time smiling and vocalizing than normal infants. Barden and colleagues observed mother-4-month-old infant dyads, five infants with unspecified craniofacial anomalies and five infants with no problems. In comparison to mothers of infants without craniofacial anomalies, the mothers of infants with craniofacial anomalies scored consistently lower on all nurturant behaviors. These included behaviors such as touching the baby affectionately, time spent in a face-to-face position, and responding to infant cues. Infants with craniofacial anamolies consistently demonstrated less positive and more negative behaviors than comparison infants. For example, they touched their mothers less frequently, averted their heads and bodies from their mother more frequently, and were less likely to smile or laugh than comparison infants. Because of the small sample size and unclear homogeneity of the craniofacial group, these results must be interpreted carefully; however, their significant consistency is remarkable. The authors point out that this pattern of decreased reinforcement for social behaviors on the part of both the mother and infant may lead to a cycle of decreased responsiveness and interactiveness that weakens the attachment.

Research with infants with craniofacial anomalies generally includes infants with cleft lip and palate and frequently this is the only diagnosis included. A possible confound in investigating the impact of facial deformity on attachment behaviors is the difficulty associated with feeding these infants. Infants with cleft lip and palate can experience excessive air intake, nasopharyngeal reflux, and difficulty sucking which can result in less satisfying feeding interactions for both the mother and child (Clarren, Anderson, and Wolf, 1987; Speltz, Goodell, Endriga, & Clarren, 1994). In an attempt to provide some information about the impact of these deformities on mother and infant behavior during feeding interactions, Speltz and colleagues scored feeding episodes of mothers with their infants using the Nursing Child Assessment of Feeding Scale. Infants, 3 months of age, either had uncorrected cleft lip and palate (n=15), cleft palate (n=19), or no health problems (n=17). Mothers of infants with cleft lip and palate were observed to be less sensitive to their infants' cues than mothers of infants in the other two groups. Mothers were not found to differ on their response to distress, social-emotional growth fostering, or cognitive growth fostering. Infants with cleft palate or cleft lip and palate  scored lower on measures of their clarity of cues than control infants. This finding primarily occurred because of the decreased frequency of smiling of both cleft palate and cleft lip and palate infants. Despite these observed differences in the feeding interactions, maternal feeding behavior was not related to maternal reports of the infants "acceptability" and reinforcement value. This is consistent with other research suggesting that mothers may present an idealized image of their relationship to their imperfect child.

(In a study investigating somewhat older infants, Wasserman and Allen (1985) conducted videotaped observations of semistructured interactions between mothers and infants. Subjects were 14 infants with craniofacial or limb anomalies (2 with limb anomalies), 14 premature infants (mean gestation 31 weeks), and 14 healthy infants. Each mother-infant pair was videotaped when the child was 9, 12, 18, and 24 months old, with premature infants corrected for gestational age. Statistical analyses were limited to specific comparisons. At-risk infants (those with congenital anamolies and prematuitye combined) engaged in play that was less focused and elaborate than nonrisk infants? play, with this trend weakening as the infant grew older. At-risk infants were less likely to exhibit positive affect than nonrisk infants, with this trend becoming stronger as the children aged. Compared with premature infants, infants with congential anomalies were more distractible and less compliant during play and were more likely to be unwilling to separate from their mother. Mothers of at-risk infants were more initiating of activities than control mothers, especially when the infant was young. In addition, they were less responsive and less likely to use distraction than comparison mothers. Mothers of infants with congential anomalies were more likely to use attention management behaviors (e.g., "Look at this toy.") and encouragement than mothers of premature infants. The authors suggest that mothers may be responding to their infants? inhibition and lack of competence by increasing their attention management and verbal praise behaviors and taking more control of the interaction from the infant. Although this may be beneficial to the infant?s cognitive development, this lack of responsivity may negatively impact the mother-child attachment.

One conflicting finding originates from research by Koomen and Hoeksma (1993) on the developmental effects of hospitalization to correct cleft palate. The researchers found that 9-month-old infants with cleft lip and palate did not differ from children with no anomalies on a maternal report of attachment behavior. This might be predicted by previously discussed research demonstrating that mothers of infants with craniofacial infants describe themselves as closer to their infants than mothers of healthy infants; however, 9-month-old infants with cleft lip and palate did not differ from control infants on rater-coded behavioral reactions to induced stress (i.e., separation from mother followed by a noxious sound). Maternal attachment behaviors were not coded or assessed during this observation. The authors found no differences in attachment classifications (i.e. secure, insecure-avoidant, insecure-resistant) between each of the two cleft lip and palate groups (n=13, n=14) and the control group (n=14) at 12 or 18 months of age, based on the Ainsworth Strange Situation Test. This finding is discrepant with evidence that mothers and infants interact differently when the child has a craniofacial anomaly; however, this finding needs to be replicated with a larger, more diverse sample.

The research suggests that infants with craniofacial anomalies tend to present fewer social cues during interactions with their mothers and maybe more difficult and less competent behaviorally than infants with no anomalies. Mothers of these infants seem to be somewhat more directive and less responsive during interactions with their infants. In addition, most studies suggest that mothers of facially deformed infants show less positive, nurturant behavior during interactions. Because behavioral differences can be noted shortly after birth, research can not adequately differentiate with which member of the dyad this pattern originates; however, it is likely the lack of reciprocity and "entunement" that develops will continue to negatively impact the behavior of both individuals. Attachment theory would predict that these mother-infant pairs would be at risk for developing insecure bonds. The one study reviewed that assesses infant attachment style using the standard observational method did not show facially deformed infants to be at a greater risk for insecure attachment than control infants; however, this study was limited by a small sample size and relatively "mild" facial deformity. It will be vital for future research to begin to examine in a standardized manner the "attachment outcome" of these children at various developmental stages.

Research: Older Children

Some indirect support for the hypothesis that children with craniofacial anomalies are at greater risk for attachment problems comes from the research conducted with somewhat older children with these diagnoses. Again, research is primarily conducted with children with cleft lip, palate, or both, and few studies include subjects with more severe facial anomalies. Most of the research of school-age children has looked at psychological adjustment in terms of self-concept, behavioral problems, or social competence. Studies have had very discrepant findings on all of these aspects of adjustment.

Brantley and Clifford (1979) found the self-concept of adolescents with cleft lip and palate to be higher than both normal and obese adolescents. In addition, Krueckbergand colleagues found preschool girls with craniofacial anomalies had significantly higher total self-concept scores than both girls with craniofacial anomalies and control females. Other investigators have found little to no impact of facial deformity on self-concept. Clifford (1969) found no difference in self-concept between children with cleft lip, palate, or both and children with asthma. Pillemer and Cook (1989) reported no difference on global self-concept scores between children age 6 years to 16 years with significant craniofacial disorders (n=25) and the normative sample. Several other studies have also reported the global self-concept of children with craniofacial anomalies to be well within the normal range, suggesting few broad difficulties (Campis, DeMaso, & Twente, 1995; King, Shultz, Steel, Gilpin, Cathers, 1993; Pertschuk & Whitaker, 1985). In an intriguing follow-up, Speltz and colleagues studied 23 5-to 7-year-old children with cleft lip, palate, or both or sagittal synostosis who had been observed with their mothers as infants and toddlers. These school-aged children with craniofacial anomalies did not differ from children in the normative group on global self-concept; however, mothers' teaching behaviors as observed in the mother-infant interaction several years earlier were negatively correlated with global self-concept, predicting nearly 50% of the variance in self-concept.

Other research has suggested, however, that children with craniofacial anomalies are at increased risk for poor self-concept. Jones (1984) found children and adolescents with cleft lip and palate, excluding subects with cleft lip or cleft palate only, demonstrated lower global self-concept, lower ratings of their behavior, lower perceived school status, lower ratings of happiness and satisfaction, and lower ratings of physical attributes and appearance than a matched noncleft group. Boys with cleft lip and palate reported less popularity than unaffected peers, and girls with cleft lip and palate reported higher levels of anxiety than unaffected peers. Kapp-Simon (1986) found young children with cleft lip, palate, or both to be more likely to score below the 50th percentile of the normative group on total self-concept than were control children with no facial anomaly. This study was replicated by Broder and Strauss (1989) who also found that children with cleft lip and palate scored significantly lower on global self-concept than children with cleft lip or cleft palate only. Although finding no problems in global self-concept, King and colleagues did find 8 to 14-year-old children with various physical disabilities, including cleft lip and palate, to report significantly poorer athletic competence and romantic appeal than children in the normative group. Boys with physical disabilities also reported lower scholastic competence than normative males, whereas girls with physical disabilities reported less social acceptance than normative females. Kapp (1979) also found adolescents with and without cleft lip, palate, or both did not differ significantly on global self-concept; however, girls with clefts were more likely to report anxiety, unhappiness, and dissatisfaction with their appearance and consider themselves less successful in school than girls without clefts. In addition, both boys and girls rated themselves lower in physical appearance.

Several studies have suggested that any differences in self-concept that do exist among children with craniofacial anomalies and their peers are likely caused by factors other than unattractiveness (Starr, 1980) or visability of defect (Kapp-Simon, 1986; Krueckberg, et. al., 1993). Quality of the mother-child relationship has been suggested as an important predictor of overall self-concept (Speltz, et. al., 1993). In addition, research by Arndt and colleagues suggests that children's adjustment to surgical procedures may also be important. The authors interviewed 20 patients with Treacher Collins (age 4 to 34 years) 6 months prior to surgery and again 1, 2, and 4 years postoperatively. Subjects were found to score lower on a measure of self-concept than the normative group before surgery. At 1 year postsurgery, self-concept scores were significantly higher than the normative group but returned to the normal range 2 and 4 years after surgery.

Research on the effects of attractiveness suggests that both adults and children expect unattractive children to demonstrate more behavior problems (Schneiderman & Harding, 1984; Tobiasen, 1987). It has been suggested that this assumption may be a self-fulfilling prophecy, resulting in children with facial anomalies to be at increased risk for psychopathology (Tobiasen, 1987). Early studies suggest very low rates of maladjustment or no differences between children with facial anomalies and nonimpaired control groups on measures of personality adjustment (Billig, 1952; Watson, 1964; Wirls & Plotkin, 1971). Using a sample of preadolescents with a variety of craniofacial anomalies, Pope and Ward (1997) found no significant elevations on the Child Behavior Checklist (CBCL) in any of the narrow band scales, the Internalizing Scale, or the Social Problems Scale compared to published normative data. Subjects, on average, were found to score 0.5 to 1.0 standard deviation below the mean on the Externalizing Scale, suggesting that children with craniofacial anomalies were reported by their parent to exhibit less "acting out" than children in the normative group. In contrast, Speltz and colleagues found that girls (age 5 through 17) with craniofacial anomalies had higher parent-completed CBCL total and externalizing scores than either girls in the control group or boys with craniofacial anomalies. This finding was not replicated in teacher-completed CBCLs, for which no significant differences were found.

Looking at behavioral problems of children with cleft lip and palate longitudinally from the age of 4 to 12, Richman and Millard (1997) found that boys demonstrated somewhat stable levels of internalizing behavior, about 1.0 to 1.5 standard deviations above the mean of the normative group after age 7. Girls exhibited significantly greater levels of internalizing behavior between the ages of 8 to 12 than at ages 4 to 6 years, with scores being 1.5 to 2.0 standard deviations above the mean of the norm group after age 7. Boys showed significantly greater levels of externalizing behavior at ages 6 and 7 years than at other ages and showed significantly lower levels of externalizing behavior at ages 11 and 12, a pattern not found in the normative group. Although the findings of this study are limited by a small sample size and lack of concurrent control group, it does underscore the important role of child development in the relationship between facial disfigurement and psychopathology.

Researchers have also been interested in the social competence or social behaviors of children with craniofacial anomalies. To some degree, this research is hampered by the finding that individuals' expectations and interpretations of a child's behavior are likely to be impacted by the child's attractiveness (Schneiderman & Harding, 1984; Tobiasen, 1987). This artifiact makes interpreting information provided by parents, teachers, and peers with regard to a child's social competence more complicated. In the study by Richman (1978), teachers rated children with cleft lip, palate, or both as significantly more inhibited in the classroom than was reported by parents. Krueckberg and colleagues found that young children (n=22) with various craniofacial anomalies provided "less friendly" responses to hypothetical social vignettes than children without facial impairment, but both groups provided equally assertive responses. Groups did not differ on parent- or teacher-completed measures of social skill or objective measures of facial encoding and decoding skills. Futher investigation in Krueckberg suggested that for both the group of children with craniofacial anomalies and those without, the child's level of social skill was best predicted by scores on the Parenting Stress Inventory, which includes both child temperament items and items related to the mother-child relationship. This measure accounted for 34% and 28%, respectively, of the variance in social skill. Other parent measures, such as child rearing practices and social support, were not as useful in this prediction. Pope and Ward (1997) also found children with cleft lip, palate, or both were as socially competent as the normative sample. They found that children with more social problems also had significant elevations in withdrawn behavior, suggesting this possible link to poor social adjustment.

Clinical Implications:

Case Sample

P is a 9 year old child with a craniofacial anomaly with no cognitive impairment who initially presented with behavior problems at school. She is an only child who lives with her biological parents. Following diagnosis at birth, P underwent several surgeries; however, more surgery is planned when P?s bone growth is completed. P has a hearing deficit and wears a hearing aid. She has noticeable malformation of the facial features including her eyes and ears. P?s sinus structures were also malformed, and P is often congested and breathes through her mouth. When she first came to the clinic, P?s hygiene was poor and often her nose and face were dirty with drainage. Previous testing by her school revealed that P is highly intelligent.

P?s parents sought psychological treatment primarily because of P?s behavior at school. Her teachers? complained of P's oppositional and defiant behavior, included not following rules, arguing with the teacher, not turning in assignments and pouting or stomping when angry. She was sent to the principal on multiple occasions for her in-class behavior. P?s peer relations were also poor. She had one friend who was a few years younger whom she played with at daycare. According to her teachers? descriptions, P had no friends at school and rejected the attempts of the other students to be nice to her. P expressed hostility towards the other students, particularly those who were popular. She said that she doesn?t like them, because they don?t like her. P drew pictures of bad things happening to children she doesn?t like (e.g. bird defecating on head of child). P frequently perceived that others were making fun of her, and she often became angry and reacted defensively and often aggressively.

As part of the evaluation, P?s parents were asked how they believed P?s craniofacial anomaly had affected P?s development and current behavior, and what strategies they had used to manage the impact of the problem. They reported that they did not consider her craniofacial anomaly to be a significant concern . The parents reported that they tell P that she may look a little different but she can do the same things other children do; however, they said that they have not talked with her about how she is different for two years. They said that P does not notice people staring at her because she is self-absorbed and rarely pays attention to other people. Her parents did remember P saying that kids at school were mean to her because she looks different. They reported that they have tried to protect her from negative reactions to her appearance by distracting her attention, and not talking about people?s reactions. For the most part her parents seemed to avoid thinking about or discussing P?s appearance and physical problems.

In general P?s parents are quite reserved and seemed uncomfortable discussing personal issues. They did not like to discuss negative feelings or thoughts and conveyed this to P. They seemed to handle negative feelings by withdrawing and isolating themselves from one another (and likely from P as well). By the time they sought therapy, there were few positive interactions, either between the parents or between parents and child. This avoidance of confrontation is consistent with the parents? permissive parenting. P?s parents would spend a lot of time talking to P about why she behaved as she did and trying to get her to explain to them what "is the matter" and understand what she did wrong. Similarly, the school was responding to her misbehavior by sending P to the principal or the counselor with whom P could talk about her "problems". This not only has reinforced P?s negative behaviors with attention, but seems to have contributed to her beliefs that her poor behavior is excusable, particularly if she is upset about something.

Thus while her parents report that they do not believe that P?s craniofacial anomaly has affected her in any way, it seems that, at least in the school, her acting out behavior is interpreted and handled differently from the same behavior in a child without an obvious "difference". Specifically her inappropriate behavior is regularly excused and treated as an uncontrollable part of her medical condition instead of P's receiving negative consequences. At age 9, P is much more egocentric than the average child; she seems to expect that she will be treated specially and that rules do not apply to her. It is likely that the perception by other children that P has more lenient rules than they do only adds to their negative feelings toward her.

P exhibited several deficits in emotional and social development consistent with this egocentricity and social immaturity. She had poor manners and was insensitive to others. She does not take the perspective of others or have empathic ability. She blames others for her problems and mistakes and does not take responsibility for her own behavior. When she did not get her way she becomes angry and yells, cries, whines, stomps around, or pouts. Her teachers complained that she has "tantrums" if she does not get to read aloud or perceives that another child was getting preferential treatment. They described behaviors such as crawling on the floor or hiding under the desk in response to reprimands.

The authors conceptualized P?s social and behavioral problems and deficits as resulting from an interaction of P?s craniofacial anomaly, societal reactions to it, and her parents response to this situation, as well as their general management of emotional and behavioral issues. P?s parents and P admit that P receives at least occasional negative reactions to her appearance. The parents? response is to distract P from the responses of others; however, in helping P ignore the reactions of others around her appearance they may have encouraged her to ignore the reactions of others to her behavior as well. The reactions of others are an important influence of development and to ignore it deprives a person of important feedback. Furthermore, while not confronting directly the emotional consequences of the social difficulty that P experiences because of her appearance, the parents may have made it difficult for P to admit to negative feelings and deal with them. Instead, they have given her the idea that someone else is responsible and must resolve her negative feelings. Furthermore, not admitting and dealing with their own sadness about P?s anomaly may have let the parents overcompensate by lowering their standards for her behavior. These lowered standards have not helped her to progressively take more control over her impulses and desires as is required of any child in order to mature and adapt to the expectations around them. P was not required to tolerate negative emotions or to attend to the needs of others. Adults have allowed P to focus on her own emotional reactions and use these as an excuse for misbehavior. Getting upset over the behavior of others distracts attention away from her behavior and results in reinforcement as her parents and teachers try to make her "feel better". It is possible that the response to soothe P?s feelings was facilitated by the awareness that P is more vulnerable to negative reactions or rejection from others and a desire to protect her. In some ways, this protection has been successful. Measures of self-esteem show that P has above average levels of self-esteem; however, she does not have the skills of emotional regulation to get along with others.

Besides these problems, one has to wonder whether there were early problems with attachment.  Given P?s appearance at birth, it is likely that her parents experienced anxiety about her health and future functioning, as well as disappointment at having a less than perfect infant. If her parents responded then as they do now, they would have tried to avoid feeling these negative emotions, possibly to the extent of limiting their emotional and perhaps behavioral involvement with their infant. The withdrawal of P?s father from the family and particularly from his role as a parent has been a source of P?s parents? marital discord. The family displays an ambivalence that is characteristic of maladaptive attachment. P?s current interactions with her parents are generally instrumental, with few expressions of emotion either positive or negative. When asked about her parents, P often describes feeling angry or disappointed with them, complaining that they have not done something she wants them to do. Although P is responsive to positive attention from her parents, she does little to facilitate positive interactions with them. More often, she is argumentative and will try to debate even nonpersonal issues. Given her parents? reservation, P may be more successful at engaging her parents in negative rather than positive interactions. Furthermore, P?s parents have had difficulty completing assignments aimed at increasing positive family interactions, seemingly preferring to spend time even leisure time engaged in solitary activities.

Summary and Future Directions

Research todate suggests that children with craniofacial anomalies are not necessarily at significant risk for poor self-concept, psychopathology, or poor social competence merely on the basis of their facial disfigurement. It does suggest, however, that the impact of having a congenital facial anomaly, likely varies depending on factors specific to the child and his or her family, as well as the child's developmental level. Certainly, traditional risk and protective factors, such as socioeconomic status, intelligence, and family social support, continue to play an important role in the psychosocial development of children with craniofacial anomalies; however, it is important to determine other factors that may interact with a child's medical status to create a different or more significant risk factor for this group of children. For example, the increased teasing from peers experienced by children with facial anomalies (Jones, 1984) may lead to increased levels of social anxiety or inhibition. Rubin and Wilkins (1995) proposed a model suggesting that children with craniofacial anomalies who have poor social adjustment and problematic peer relationships have a history of insecure parental attachment and more frequent uncomfortable peer interactions, which leads to increased social anxiety, withdrawn behavior, and self-consciousness about their appearance.

The current research base is limited by its reliance on children with cleft lip, palate, or both. Although samples of children with other diagnoses of sufficient size to conduct statistical analysis are difficult to obtain, the generalizability of much of the current findings is questionable. Collaborative, multisite research programs are likey to be necessary to answer important questions about the similarity of the experiences of children with various craniofavial diagnoses and their subsequent psychological and social adjustment. In addition, the importance of development is evident in the current research, although longitudinal studies are rare. Research should begin to investigate theoretical models by following variables over the course of the child's development, allowing investigators to explore the importance of variables, such as attachment and peer relationships, at various points in the development of the child.


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