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C. Runte and D. Dirksen
urement (Fig. 14.21). Symmetry is not a sufcient condition for beauty as well as slight
asymmetry is not for disgurement.
Less disguring 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” dened 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 inuenced 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 etal. [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 signicantly 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 inuence on
perceived disgurement. 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 disguring (Figs.14.23–14.25). 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 identied as disguring by Ker
etal. [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 inuenced by experiences. On the
other hand, people differ signicantly in their
esthetic judgment. However, violation of fundamental properties of the face like symmetry and
average proportions leads to disgurement and

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C. Runte and D. Dirksen
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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ofPatients withCongenital
Craniofacial Malformations
ThomasMeyer
15
15.1 Psychological Factors
Aecting 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 signicant 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].
Disguring 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 disgurement, 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 disgurement, 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 disgurement on psychological vulnerability.
© Springer Nature Switzerland AG 2021
U. Meyer (ed.), Fundamentals of Craniofacial Malformations,
https://doi.org/10.1007/978-3-030-46024-2_15
239

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T. Mey er
15.2 Psychological Adjustments
toOrofacial Disgurement
Although the majority of infants and children
with craniofacial malformations develop in a typical manner without major psychological problems, a signicant number of affected children
experience internalizing and externalizing problems and develop behavioural disorders, such as
fearful shyness, depressive symptoms, and
somaticizing disorders [12–14]. 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 [15–19]. 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 signicant problems at a clinical level [21, 22]. The individual
diagnosis and severity of the craniofacial anomaly may elicit specic 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 signicant 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 signicant 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
onPsychosocial 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 16years
from 145 families, Berger and Dalton found no
signicant psychosocial adjustment difculties
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 Difculties
Questionnaire (SDQ), Brand and co-workers
demonstrated that study participants with and
without cleft lip and/or palate did not signicantly differ with respect to emotional problems,
conduct problems, or hyperactivity [28].
However, difculties 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)

15 Psychosocial Adjustment ofPatients withCongenital Craniofacial Malformations
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241
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 signicantly
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 signicance of patient satisfaction with
facial appearance for social functioning rather
than the objective severity of the deformity [32,
33]. Van der Elzen etal. showed that while social
anxiety and distress did not signicantly differ
between adult patients with facial disgurement
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 disgured
patients used more often what they classied as
immature defence styles, suggesting that low
self-esteem may result in less frequent utilization
of mature defence styles [32, 33].
15.4 Quality ofLife inPatients
withCraniofacial
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 12years,
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 disgured 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 andDepression
inSubjects withCongenital
Orofacial Malformation
A recently published meta-analysis identied 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 signicantly 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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T. Mey er
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 inSocial
Interactions Related
toCraniofacial
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 [45–49,
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 3years [52]. An overall negative view of the
self and difculties 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 signicant 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 disgurement have not only an abnormal facial
appearance, but often additionally speech problems with atypical consonant production, abnormal nasal resonance and nasal airow, 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
[57–59]. 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 Eects Related
toCoping Strategies
inCongenital Disgurement
Although the view is widely held that females
will have more trouble with orofacial disgurement, this assumption may not be true since
boys and young men are especially vulnerable
to bullying when aficted by facial disgurement, 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 signicantly 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 ofPatients withCongenital Craniofacial Malformations
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satisfaction were uncorrelated and that dissatisfaction with the appearance of their faces was
signicantly 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 12months, whereas
at 24months, no signicant group differences in
the attachment classication were observed [67].
In children aged between 5 and 6years, 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
inCaregivers ofChildren
withCongenital 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 classied as
15.9 Psychological Problems
inSubjects
withCraniosynostosis
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
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