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C. Runte and D. Dirksen
urement (Fig. 14.21). Symmetry is not a suf­cient condition for beauty as well as slight asymmetry is not for disgurement.
Less disguring features of the periorbital region like receding eyelids or an upward shift of the upper eye frame by fat atrophy can be cor-
Fig. 14.22 Benslimane et al. [78] described a “frame
ratio” dened by two distances: rst the distance (red line) from the shadow of tear trough and lid-cheek junction to the most superior peripheral shadow (dotted blue line) of the upper lid and second the inter-lid distance at the verti­cal mid-pupillary line. With aging, especially the upper margin is inuenced by fat atrophy leading to an upward shift. Furthermore, the upper margin is also usually more clearly visible because of a stronger curved surface result­ing in a higher contrast. Benslimane claims that a lower frame ratio is related to a higher females’ gaze attractive­ness. This example shows frame ratios of 2.26 (left) and
3.23 (right; artwork by Nina Runte)
rected with the help of proportion analysis (e.g., Benslimane etal. [78]; Fig.14.22).
At the lower edge of the Yarbus triangle, the white line of anterior teeth, exposed while smil­ing, has a highlighted position. Unesthetic malocclusions will draw more attention to the oral region and signicantly deteriorate facial attractiveness [79]. The beauty of a smile with reference to teeth can be summarized as the absence of discoloration, unbroken completeness (although the esthetic evaluation of a median dia­stema depends on the cultural background and may have changed in time [80, 81]), mirror sym­metry, and balanced alignment and proportions. Dental treatment can have a positive inuence on perceived disgurement. Especially the upper anterior teeth show a high contrast to the oral cavity behind if the person is smiling with slightly opened mouth. Discolored or missing teeth as well as an unusual gingival display or gingival height discrepancies and asymmetries can be per­ceived as disguring (Figs.14.2314.25). From the laypersons’ perspective [82], the highest level of agreement was found in variations concerning the overbite (in this context meaning the display of lower gingiva or intermaxillary inter-incisor space), the gingival display (i.e., the so-called
Fig. 14.23 Constructed variations of the esthetic smile: (a) original photograph, (b) discoloration indicating endodon-
tic disease, (c) asymmetrical loss of lateral incisor, and (d) symmetrical loss of lateral incisors
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Fig. 14.24 Constructed variations of the esthetic smile in
symmetry and proportion: (a) asymmetry by lateral shift, (b) asymmetry by inclination of the occlusal plane, (c)
Fig. 14.25 Constructed variations of the esthetic smile in
gingival height display: (a) asymmetric step in gingival display and (b) symmetrical step in gingival display as it
gummy smile), the width of the buccal corridor, and maxillary lateral incisor gingival height dis­crepancy (a step between the lateral incisor gin­giva and the central incisor gingiva). Interrater agreement reliability was only poor in the judg­ment of midline discrepancies. Though dentists pay attention to midline asymmetries, a close look at Michelangelo’s paintings in the Sistine Chapel, especially the Delphic Sibyl, will reveal that a symmetric mesiodens with no approximate contact in the midline does not necessarily affect the esthetic appearance of the face.
asymmetrical disproportion in lateral and central incisor width, and (d) symmetrical disproportion in lateral and central incisor width
was used similarly and identied as disguring by Ker etal. [82]
14.7 Conclusions
Although for centuries authors have tried to explain beauty, there are still many open ques­tions. Our esthetic judgment is on the one hand not free but determined by the mechanisms of perception and inuenced by experiences. On the other hand, people differ signicantly in their esthetic judgment. However, violation of funda­mental properties of the face like symmetry and average proportions leads to disgurement and
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may have serious psycho-social consequences. Treatment in these cases is essential for a suc­cessful rehabilitation.
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Psychosocial Adjustment
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ofPatients withCongenital Craniofacial Malformations
ThomasMeyer
15
15.1 Psychological Factors Aecting Surgical Decision-Making
In children and adolescents with congenital orofacial malformations, the risk of self- and parent- perceived stigmatization and social dis­crimination is an important determinant in the decision to undertake reconstructive and orthog­nathic surgery. The indication for restorative intervention to correct for orofacial malformation and single- suture, nonsyndromic craniosynosto­sis in paediatric patients is based on the expected postoperative anatomical outcome as well as the improvement in quality of life and overall mental health [1, 2]. In a signicant number of patients with visible craniofacial differences, advances in plastic and orthognathic surgery have placed these individuals at a lower risk of experiencing social stigmatization, most likely by activating an arsenal of pre-existing effective coping strategies that can help foster a positive self-image and bet­ter health-related well-being [3]. Vulnerability to social stigmatization is particularly high in early adolescence and puberty when physical attrac­tiveness and outward appearance become impor-
T. Meyer (*) Psychosomatic Medicine and Psychotherapy, University of Göttingen, Göttingen, Germany e-mail: thomas.meyer@med.uni-goettingen.de
tant aspects of forming interpersonal interactions in peer relationships [4, 5].
Disguring conditions in patients with con­genital craniofacial anomalies may result in vari­ous age-dependent psychosocial problems such as elevated anxiety, appearance-related social avoidance, and poorer quality of life [6]. However, trajectories of psychosocial function­ing from childhood to early and later adulthood have not been well studied, and the long-term effects of restorative interventions need to be addressed [7, 8]. Particularly, the moderating effects of surgical corrections on the develop­ment of resilience and social functioning require further research efforts.
Given the complex relationships between the degree of orofacial disgurement, the experi­enced pre-operative distress, and the expected postoperative achievement in mental well­being, the surgeon has to meet the psychologi­cal needs of a patient before considering a surgical treatment approach [9, 10]. The pro­cess of decision- making on surgery requires a comprehensive understanding of the patient’s and his/her proxy’s ability to cope with the social meaning of the disgurement, the level of family support, and the age-dependent devel­opmental stage [11]. The surgeon needs to understand the feelings of the patient and par­ents, which often differ with respect to the impact of the facial disgurement on psycho­logical vulnerability.
© Springer Nature Switzerland AG 2021 U. Meyer (ed.), Fundamentals of Craniofacial Malformations,
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15.2 Psychological Adjustments toOrofacial Disgurement
Although the majority of infants and children with craniofacial malformations develop in a typ­ical manner without major psychological prob­lems, a signicant number of affected children experience internalizing and externalizing prob­lems and develop behavioural disorders, such as fearful shyness, depressive symptoms, and somaticizing disorders [1214]. The available lit­erature on psychological adjustments to congeni­tal craniofacial abnormalities shows inconsistent results due to the lack of consensus on psycho­logical constructs and diagnostic criteria as well to the heterogeneity of clinical phenotypes and disease entities [1519]. The age-dependent development of social interaction skills may be impaired in socially inhibited children with severe craniofacial birth defects. Low self­esteem, reduced quality of life, and altered men­tal and emotional adjustment including the risk of social inhibition have been described in patients with craniofacial conditions [20].
Although numerous studies have suggested that children and adolescents with non­intellectually impairing craniofacial malforma­tion may have an elevated risk of some types of psychosocial adjustment problems, it must be noted that most of these children develop nor­mally and do not experience signicant prob­lems at a clinical level [21, 22]. The individual diagnosis and severity of the craniofacial anom­aly may elicit specic patterns of psychosocial adjustment [23]. The majority of subjects with craniofacial conditions have learned to accept their visible facial difference and nd ways to develop internal strength as a signicant source of comfort and meaning in their lives [24]. Numerous publications studying psychological adjustments in children and adults with nonsyn­dromic orofacial malformations found compara­ble results to unaffected reference groups, while other papers reported signicant variations in psychometrically assessed key psychological domains, such as mental well-being, health­related quality of life, and social functioning among patients with congenital craniofacial deformity [20].
15.3 Clinical Studies onPsychosocial Adjustments
In a cohort of 724 children and adolescents with congenital craniofacial anomalies aged 2–18 years from an urban hospital, who com­pleted Child Behavior Checklists, most subjects did not report experiencing psychosocial adjust­ment problems, but may still be at an elevated risk of internalizing problems [22]. In adoles­cents with a cleft aged between 11 and 16years from 145 families, Berger and Dalton found no signicant psychosocial adjustment difculties above that of the normal population [25]. In a later publication, these authors reported that psy­chosocial adjustment in adolescents was pre­dicted by both their former social experiences and maternal well-being [26]. Using a cross­sectional postal questionnaire design, they dem­onstrated that dissatisfaction with appearance, speech problems, and the use of avoidant coping strategies were also important negative predictors for psychosocial adjustment [26]. In boys with clefts of the lip and/or palate aged 7–12 years, there was evidence from magnetic resonance imaging which suggested that aberrant develop­ment of the ventral frontal cortex was correlated with social dysfunction, but not with psychomet­rically assessed measures of self-concept [27].
Using the Strengths and Difculties Questionnaire (SDQ), Brand and co-workers demonstrated that study participants with and without cleft lip and/or palate did not signi­cantly differ with respect to emotional problems, conduct problems, or hyperactivity [28]. However, difculties in interactional competence as measured by the PIELCQ questionnaire were more frequently observed in 32 children and ado­lescents from the group with clefts as compared to the 34 controls. Furthermore, the authors reported irregular sleep patterns to be associated with psychosocial strain rather than the presence of the cleft lip and palate deformity [28].
In a small sample of 25 adults with cleft lip and palate, Gassling and co-workers found no evidence of an abnormal habitual emotion regu­lation, as compared to an equal-sized control group of unaffected volunteers [29]. Scores from the Emotion Regulation Questionnaire (ERQ)
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and the Ambivalence over Emotional Expressiveness Questionnaire G 18 (AEQ-G18) questionnaire showed no differences between patients and subjects from the control group. Likewise, using the Facially Expressed Emotion Labeling (FEEL) test, the authors reported that facial emotion encoding was similar between the two groups. In a Chinese sample of 94 patients with cleft lip and palate aged between 10 and 40 years, patients suffered from signicantly lower general and social self-esteem as compared to 116 healthy controls with no dentofacial defor­mities [30]. In a recently published paper from the Whole of Life Survey in adults born with cleft lip and/or palate from the United Kingdom, the authors demonstrated that affected adults are at a risk of emotional distress from an early age that may persist to adulthood [31].
Interestingly, scientists from the Erasmus University Medical Center, Rotterdam, under­lined the signicance of patient satisfaction with facial appearance for social functioning rather than the objective severity of the deformity [32,
33]. Van der Elzen etal. showed that while social
anxiety and distress did not signicantly differ between adult patients with facial disgurement and a reference group without facial deformities, the patient’s own subjective appearance was a predictor of social functioning. The authors sug­gested that less frequent interpersonal behaviour was observed in those affected subjects who avoided stress caused by stigmatization. Van den Elzen and co-workers stated that disgured patients used more often what they classied as immature defence styles, suggesting that low self-esteem may result in less frequent utilization of mature defence styles [32, 33].
15.4 Quality ofLife inPatients
withCraniofacial Abnormalities
Patients with major craniofacial malformations resulting in severe forms of deformity may be at a particularly high risk of experiencing sociopsy­chological stress, enduring low quality of life and/or developing psychopathological comorbid-
ity [34]. Schliephake and colleagues from the University of Göttingen reported that, in their sample of 170 consecutive paediatric patients with orofacial clefts aged between 8 and 12years, the quality of life was superior as compared to that of an age- and sex-matched control group of unaffected schoolchildren [35]. The quality of family functions affecting quality of life was lower in parents with cleft lip and/or palate chil­dren as compared to a control group, particularly when their children reach adolescence [36]. While the level of satisfaction with facial appear­ance is often reduced in congenital and acquired facially disgured adults, any attempts to improve satisfaction with facial appearance either by sur­gery or by enhancement of self-esteem should probably improve long-term psychological func­tioning [37, 38].
15.5 Anxiety andDepression inSubjects withCongenital Orofacial Malformation
A recently published meta-analysis identied 11 studies reporting on psychosocial symptoms in adolescents with a visible difference as compared to unaffected peers [39]. The authors found that adolescents with a visible difference had experi­enced more symptoms of anxiety, but not depres­sive mood. Fear of negative evaluation by others, the perceived social support, and self-esteem are important predictors for anxiety in adult patients with congenital craniofacial conditions [40]. Interestingly, periods of depressive mood were more common in a cohort of 28 Scandinavian patients with Apert syndrome (acrocephalosyn­dactyly type 1), but patients did not differ with respect to a generally positive attitude towards life from a matched control group [41]. In a Norwegian study of 196 adolescents with a visible cleft, Feragen and colleagues found that affected boys, when compared to 1832 controls, reported signi­cantly more positive perceptions of friendships and fewer depressive symptoms than the compari­son group [42]. Besides sleep irregularities, patients with orofacial clefts were considered to have elevated levels of anxiety and depression and
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to be at a higher risk of development of chronic pain states [43, 44]. The authors interpreted these results in the context of perceptions of social acceptance and emotional resilience.
15.6 Problems inSocial Interactions Related toCraniofacial Abnormalities
Psychometric assessment using well-validated questionnaires, such as the self- and patient­reported outcome measure CLEFT-Q or the Craniofacial Experiences Questionnaire (CFEQ), provides valuable clinical information regarding the need for future surgical interventions [4549,
19]. In addition, these instruments can be used to
study associations between speech problems, the degree of unhappiness with facial differences, and health-related quality of life [50].
Negative self-perception of physical appear­ance is often found in young patients with con­genital craniofacial anomalies, and concerns about their appearance are at a peak in adoles­cence [51]. Usually children with facial malfor­mation rst notice their difference at a mean age of 3years [52]. An overall negative view of the self and difculties with social interaction are particularly prevalent in preadolescents and adolescents [3, 53, 54]. The transition from childhood to early adulthood is the time when a subject feels increased strain to conform to social constructs of beauty standards and physi­cal attractiveness. There is a signicant societal pressure for these age groups to conform to cul­tural standards for both feminine and masculine beauty ideals. Damiano and colleagues analysed data from telephone interviews with mothers of children with nonsyndromic oral clefts and found that speech and aesthetic concerns became more important as their children got closer to adolescence, probably because the psychosocial burden related to the acceptance by peers generally becomes more critical in pre­adolescence [55].
There are well-established links between physical attractiveness and the likelihood of
social acceptability, and personal achievement may be disadvantageous for subjects with orofa­cial malfunctions. Individuals with orofacial dis­gurement have not only an abnormal facial appearance, but often additionally speech prob­lems with atypical consonant production, abnor­mal nasal resonance and nasal airow, termed hypernasality [56]. The imperfect physical appearance and the phonation disorder may result in subtle changes in the normal patterns of verbal and non-verbal communication. Patients with orofacial abnormalities often experience unfa­vourable social responses including teasing, bul­lying, and unwanted questioning, which they interpret as a form of not being fully accepted [5759]. While the number of operations was not related to the overall psychological functioning, adult patients with a higher degree of residual facial deformity displayed more dissatisfaction with their facial appearance and usually had more frequent experiences of discrimination [8].
15.7 Gender Eects Related toCoping Strategies inCongenital Disgurement
Although the view is widely held that females will have more trouble with orofacial disgure­ment, this assumption may not be true since boys and young men are especially vulnerable to bullying when aficted by facial disgure­ment, which makes them feel physically weaker and less attractive to girls [11]. In 170 consecu­tive patients with nonsyndromal orofacial clefts, Kramer and co-worker found that, although gender was not signicantly associated with family functioning, boys experienced a lower quality of life than girls, as measured using the Impact on Family Scale and the KINDL ques­tionnaires [35]. In a sample of 74 children with craniofacial abnormalities, Shapiro and col­leagues demonstrated that concerns about peer relationships were particularly prominent for boys, whereas girls reported the quality of their peer relationships as being comparable to non­affected peers [60, 61]. The authors demon­strated that self- and proxy ratings of child
15 Psychosocial Adjustment ofPatients withCongenital Craniofacial Malformations
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satisfaction were uncorrelated and that dissatis­faction with the appearance of their faces was signicantly associated with negative psychoso­cial outcomes in girls but not boys [60, 61]. Health-related quality of life in adolescents with oral cleft, as measured through the Short-Form Health Survey (SF-36) questionnaire, showed females in the three domains Bodily Pain, Vitality, and Mental Health statistically lower than males [62]. One study reported that parents of females with orofacial clefts expressed more concerns about their daughter’s appearance than parents of males, whereas, in contrast, parents of boys were more concerned about vocational problems in their offspring [63]. Nidey and col­leagues demonstrated that perceived social sup­port was reported to be higher among parents of male affected children compared with female affected children. In contrast, parental psycho­social functioning was unrelated to the cleft type [64].
Shapiro and colleagues demonstrated that more complex diagnoses of craniofacial differ­ence were associated with increased parenting stress and that there was a positive association between parental exibility with respect to gender views and child-reported parent-child relationship quality [65]. Caregivers with more exible gen­der attitudes were seen more supportive by their daughters but not their sons [65]. A longitudinal study in 47 children with craniofacial anomalies showed that parenting stress in early infancy pre­dicted psychosocial adjustment in later toddler­hood, suggesting that dysfunctional patterns in parent-child interactions persist in some families with a child with a craniofacial anomaly [66].
having a stable attachment at 12months, whereas at 24months, no signicant group differences in the attachment classication were observed [67]. In children aged between 5 and 6years, the self­reported KINDL scores were higher in all dimen­sions than the proxy- rated estimation by their parents, demonstrating that self-rated quality of life in the children is superior to that which their caregivers estimated. These ndings suggest that patients with craniofacial differences more fre­quently than their parents have developed the ability to implement a variety of effective coping strategies in order to counteract social stigma [3]. They consider themselves to be well adapted to their condition, having achieved positive self­esteem and developed stable interactional com­petence. There may also be a shift among various coping strategies in the mothers of affected chil­dren, as mothers of 13- to 18-year-old patients with nonsyndromic clefts reported greater use of a problem-solving coping strategy when com­pared with mothers of 8- to 12-year-old, younger patients [68].
One study suggested a reciprocal relationship between parenting stress and child adjustment [66]. Mothers of newborns and toddlers with cra­niofacial anomalies may be at an increased risk of experiencing clinically relevant depression and anxiety symptoms [69]. Perceived social support mediates the relationship between mater­nal psychological distress and their quality of life [69]. In 287 parents of children with oral clefts, fathers had a higher self-esteem and lower con­cern of being negatively judged by others than mothers [64]. However, fathers also reported a lower perception of communicating their prob­lems to others than the mothers did.
15.8 Parenting Stress inCaregivers ofChildren withCongenital Orofacial Conditions
Maris et al. tested for insecure mother-child attachments in infants with orofacial clefts versus non-affected controls using the Strange Situation procedure, and they found that children with a palate cleft were less likely to be classied as
15.9 Psychological Problems inSubjects withCraniosynostosis
In the existing literature, there are some reports on altered psychological development in chil­dren with single-suture craniosynostosis [16]. As compared to non-affected controls, patients with complex congenital malfunctions and impaired