Psychological adjustment to craniofacial conditions (excluding oral clefts): A review of the literature

“Parents of children and adolescents with syndromic craniosynostoses had significantly lower scores on QoL measures than norms, especially for those parents of children with Apert, Crouzon and Pfeiffer syndromes (Bannink et al., 2010; de Jong et al., 2012), as well as complex craniosynostoses (Bannink et al.”

“Another important aspect of living with a visible difference such as a CFA is repeated exposure to the social reactions of others, such as staring, comments, questions or teasing (Rumsey & Harcourt, 2004).”

“The assessment of cognitive function would also provide an important basis for school arrangements, in order to optimise learning strategies (Broder, 2001).”

“In addition, articles focussing on cognitive function and development alone were excluded since the literature on cognitive and neurobehavioural development in CFA is extensive and several reviews have been published on this domain during the last decade (Kapp-Simon et al., 2007; Knight et al., 2014; Speltz et al., 20…”

“In addition, articles focussing on cognitive function and development alone were excluded, since the literature on cognitive and neurobehavioural development in CFA is extensive and several reviews have been published on this domain during the last decade (Kapp-Simon et al., 2007; Knight et al., 2014; Speltz et al., 2…”

“literature (e.g. Klassen et al., 2012; Stock & Feragen, 2016; Stock, Hammond, Owen, Kiff, Shanly, & Rumsey, 2016).”

“2. deletion syndrome (di George or velo-cardio-facial syndrome) were excluded since the specificity of this condition and the high number of recent studies (Jonas, Montojo, & Bearden, 2014; Philip & Bassett, 2011; Swillen & McDonald-McGinn, 2015) suggests a need for a review on this condition alone.”

“Importantly, parent perceptions of treatment outcome did not often correlate with surgeons’ ratings, pointing to a well-known discrepancy between subjective and more ‘objective’ ratings of appearance within the visible difference literature (Moss, 2005).”

“literature (e.g. Klassen et al., 2012; Stock & Feragen, 2016; Stock, Hammond, Owen, Kiff, Shanly, & Rumsey, 2016).”

“2. deletion syndrome (di George or velo-cardio-facial syndrome) were excluded since the specificity of this condition and the high number of recent studies (Jonas, Montojo, & Bearden, 2014; Philip & Bassett, 2011; Swillen & McDonald-McGinn, 2015) suggests a need for a review on this condition alone.”

“…UK Limited, trading as Taylor & Francis Group Psychology & Health, 2017 Vol. 32, No. 3, 253–288, http://dx.doi.org/10.1080/08870446.2016.1247838 structures within the first and second branchial arches (Posnick & Ruiz, 2000) and affects approximately one in 50,000 live births (Orphanet, 2015).”

“Craniosynostosis results when one or more cranial structures fuse prematurely, and is relatively common, occurring in approximately one in every 2000 live births (McCarthy et al., 2012).”

“In addition, articles focussing on cognitive function and development alone were excluded since the literature on cognitive and neurobehavioural development in CFA is extensive and several reviews have been published on this domain during the last decade (Kapp-Simon et al., 2007; Knight et al., 2014; Speltz et al., 20…”

“Cognitive-behavioural techniques have shown promise in this area (Clarke et al., 2013).”

“No differences were found between subtypes of non-syndromic trigonocephaly in relation to the frequency of behavioural problems (Kelleher et al., 2006), while some differences were found across subtypes of craniosynostoses (Becker et al., 2005).”

“Two studies on young children with single-suture craniosynostoses reported differences on externalising (Kapp-Simon et al., 2012) and internalising behaviours (Kapp-Simon et al., 2012; Snyder & Pope, 2010).”

“Behavioural problems were also shown to be associated with underlying cognitive impairments in several studies (Maliepaard et al., 2014; van der Vlugt et al., 2009, 2012).”

“There were no associations between QoL and age (Geirdal et al., 2013; Warschausky et al., 2002) or gender (Roberts & Mathias, 2013; Warschausky et al., 2002).”

“Parents of children and adolescents with syndromic craniosynostoses had significantly lower scores on QoL measures than norms, especially for those parents of children with Apert, Crouzon and Pfeiffer syndromes (Bannink et al., 2010; de Jong et al., 2012), as well as complex craniosynostoses (Bannink et al.”

“Further, interesting differences in mothers’ and fathers’ reports of QoL indicate that both parents’ perspectives should be included, in a research field which has historically been dominated by mothers’ reports (Nelson & Kirk, 2013; Stock & Rumsey, 2015).”

“Qualitative studies are less common than quantitative methods in health research more generally, possibly because these approaches are not well understood and/or because lesser value is placed on them (Nelson, 2009).”

“Two studies on young children with single-suture craniosynostoses reported differences on externalising (Kapp-Simon et al., 2012) and internalising behaviours (Kapp-Simon et al., 2012; Snyder & Pope, 2010).”

“…with craniosynostosis (Kapp-Simon, Speltz, Cunningham, Patel, & Tomita, 2007; Knight, Anderson, Spencer-Smith, & Da Costa, 2014; Speltz, Kapp-Simon, Cunningham, Marsh, & Dawson, 2004), or surgical outcomes in a range of congenital and acquired conditions (Habal, 2013; Sarwer & Crerand, 2002).”

“Other studies have also found children with non-syndromic craniosynostoses (Cloonan et al., 2013) and hemifacial microsomia (Dufton et al.”

“…on children with hemifacial macrosomia reported no differences between parent ratings and norms (Snyder & Pope, 2010), while teachers reported a higher frequency of internalising behaviours than parents compared to matched controls (Dufton et al., 2011; Werler, Starr, Cloonan, & Speltz, 2009).”

“Nonetheless, there remains a need for further rigorous research using appropriate outcome measures, in order to evaluate the effectiveness of such interventions (Jenkinson, Williamson, Byron-Daniel, & Moss, 2015; Norman & Moss, 2015).”

“Adults with syndromic craniofacial conditions reported mean scores of depression and anxiety within the normal range and comparable to norms in one study (Roberts & Mathias, 2012).”

“The same tendency was found in another study comparing self- and parent reports; however, differences were small and non-significant (Geels et al., 2008).”

“Further, interesting differences in mothers’ and fathers’ reports of QoL indicate that both parents’ perspectives should be included, in a research field which has historically been dominated by mothers’ reports (Nelson & Kirk, 2013; Stock & Rumsey, 2015).”

“Behavioural problems were also shown to be associated with underlying cognitive impairments in several studies (Maliepaard et al., 2014; van der Vlugt et al., 2009, 2012).”

“Such findings illustrate the complexity of the construct of ‘QoL’, and the multifaceted nature of adjustment (see Nelson, Glenny, Kirk, & Caress, 2012; Rumsey & Stock, 2013).”

“…corresponding measures have been advocated in the related fields of oral clefts and visible difference more generally (see Appearance Research Collaboration [ARC], 2009; Klassen et al., 2012; Stock et al., 2016), and may also offer guidance for the direction of research within the field of CFAs.”

“…and/or other medical problems (Bannink et al., 2010, 2011; Geirdal et al., 2013; Tovetjärn et al., 2012), cognitive function (de Jong et al., 2012; Raposo-Amaral et al., 2014), difficulties with vision or speech (de Jong et al., 2012), and parental emotional status (Warschausky et al., 2002).”

“…and adolescents with syndromic craniosynostoses had significantly lower scores on QoL measures than norms, especially for those parents of children with Apert, Crouzon and Pfeiffer syndromes (Bannink et al., 2010; de Jong et al., 2012), as well as complex craniosynostoses (Bannink et al., 2011).”

“Similarly, mothers of infants with single-suture craniosynostoses reported that the more visible they felt the condition was to others, the higher levels of stress they experienced (Rosenberg et al., 2011).”

“Social problems were also identified in young children with single-suture craniosynostoses (Kapp-Simon et al., 2012), although low effect sizes were reported.”

“In contrast, adults with a wide range of syndromic craniofacial conditions reported consistently positive perceptions of QoL comparable to norms (Prescott et al., 2013; Roberts & Mathias, 2012).”

“Parents of children with non-syndromic craniosynostoses (Cloonan et al., 2013) and hemifacial microsomia (Khetani et al., 2013) reported lower HRQoL compared to matched controls, and compared to young people with an oral cleft (Warschausky et al., 2002).”

“…satisfaction was confirmed among adults with a wide variation of syndromic craniofacial conditions (Roberts & Mathias, 2012), and among parents and children treated for trigonocephaly (Kelleher et al., 2007), and parents of children with sagittal craniosynostoses (Boltshauser et al., 2003).”

“Young people and adults with Apert syndrome also described positive feelings about their appearance (Raposo-Amaral et al., 2012).”

“Positive coping strategies that counterbalanced experiences of stigmatisation and teasing were also described in young people and adults with Apert syndrome (Raposo-Amaral et al., 2012).”

“Another study reported that less than 30% of young people with Apert syndrome had scores in the maladjustment range according to parent reports (Sarimski, 2001).”

“Similarly, a prospective study on young children with metopic suture synostoses (Kunz et al., 2014) indicated that 92% of the parents were highly satisfied with their child’s appearance after surgery.”

“In another study, adults with Treacher Collins syndrome reported less problems with anxiety, but more problems with depression than those with cherubism (Geirdal et al., 2015).”

“The assessment of cognitive function would also provide an important basis for school arrangements, in order to optimise learning strategies (Broder, 2001).”

“Further, commitment to social activities was found to protect adults with Crouzon syndrome in relation to feeling self-conscious about their appearance, which also increased well-being (Stavropoulos et al., 2011).”

“Concordantly, access to treatment and care was rated as satisfactory (Wong-Gibbons et al., 2009).”

“In two other studies, adults with Apert (Tovetjärn et al., 2012) and Crouzon syndrome (Fischer et al., 2014) reported fewer romantic and sexual experiences than matched controls.”

“In another study, adults with Treacher Collins had high levels of desire for future treatment, predominantly related to eyelid reconstruction (Plomp et al., 2013).”

“Perceived discrimination, for example, was found to be related to lower ratings of subjective appearance in adults with syndromic craniofacial conditions (Roberts, 2014).”

“Multi-centre studies (12 out of the 41 included articles) could potentially offer the optimal solution (Broder, 1997; Roberts & Shute, 2012), yet are not without their own difficulties (Sandy et al., 2011).”

“There were no associations between QoL and age (Geirdal et al., 2013; Warschausky et al., 2002) or gender (Roberts & Mathias, 2013; Warschausky et al., 2002).”

“Such strategies have useful implications for clinical practice; for example, staring could be interpreted as curiosity, rather than a threat (Partridge, 2010), thereby reducing the emotional impact of the event.”

“Behavioural problems were also shown to be associated with underlying cognitive impairments in several studies (Maliepaard et al., 2014; van der Vlugt et al., 2009, 2012).”

“The findings of this review therefore suggest that investigating positive, as well as negative impacts of CFAs could provide a better understanding of the complexity of adjustment, and useful insight for promoting resilience within clinical practice (Feragen, 2012).”

“Over time, laypersons’ appearance ratings decreased for both the patient and control groups (Collett et al., 2013).”

“Few condition-specific measures currently exist, and thus quantitative and qualitative work towards developing and evaluating such a measure may be beneficial (Holmbeck & Aspinall, 2015; Klassen et al., 2012; Patrick et al., 2007).”

“There were no associations between QoL and age (Geirdal et al., 2013; Warschausky et al., 2002) or gender (Roberts & Mathias, 2013; Warschausky et al., 2002).”

“…study on adults with Treacher Collins, participants were least satisfied with their ears, facial profile, and eyelids (Plomp, Versnel, van Lieshout, Poublon, & Mathijssen, 2013), while being relatively satisfied with nasal appearance (Plomp et al., 2013, 2015), but less so than the control group.”

“In another study, parents of young children also with craniosynostoses, reported an association between satisfaction with treatment and speech problems (Knudsen et al., 2012).”

“…with craniosynostosis (Kapp-Simon, Speltz, Cunningham, Patel, & Tomita, 2007; Knight, Anderson, Spencer-Smith, & Da Costa, 2014; Speltz, Kapp-Simon, Cunningham, Marsh, & Dawson, 2004), or surgical outcomes in a range of congenital and acquired conditions (Habal, 2013; Sarwer & Crerand, 2002).”

“One study identified an association between positive adjustment and active coping strategies, social support seeking and use of avoidance strategies (Roberts & Shute, 2012), associations that became non-significant, however, when controlling for baseline levels of adjustment (Roberts & Shute, 2012).”

“28 Many patients with craniofacial conditions require orthodontic treatment.”

“4 Speech dif fi culties and disorders can have a psychological impact 5,6 and signi fi cantly affect the quality of life.”

“Although previous research has found parents to be satisfied with the outcomes of their children’s appearance following surgery (Feragen & Stock, 2017), parents must nonetheless face 1Oslo University Hospital, Oslo, Norway 2University of the West of England, Bristol, Bristol, United…”

“…have found that experiences of teasing among adolescents with CL/P may be associated with psychosocial difficulties (Feragen & Borge, 2010; Feragen & Stock, 2016a) and that social experiences can predict the extent of psychosocial adjustment among adolescents with CL/P (Berger & Dalton,…”

“Although perceived teasing is a key influential factor in the development of appearance concerns and depressive symptoms in young people, challenging social interactions can promote resilience and personal growth if well managed (Feragen and Stock, 2016).”

“22,23 This was re fl ected in a large multisite study of children with CFM in which teachers reported higher internalizing concerns and lower social competence and adolescents self-reported more social concerns compared to their peers.”

“Whilst these children still demonstrated some difficulties, specifically in attention, executive function and arithmetic, the ability to draw generalizable conclusions from the research to date is limited by small samples, and inconsistent use of objective measures across domains.(6,12,13) Therefore, findings amongst…”

“Systematic reviews have highlighted the importance of psychosocial aspects of development and consideration of cognitive factors in the context of psychosocial factors for meaningful information to be obtained.(6) Furthermore, the importance of collaboration across health care institutions to undertake high-quality re…”

“Few studies have reported on self-concept (a term relating to one’s general self-perception, closely related to self-esteem) as a psychosocial outcome in the CS literature.(13,25) Where self-concept has been reported in patients with CS, studies have been qualitative,(26) or focused on more heterogeneous populations s…”

“Craniosynostosis patients have been reported to have a less pleasing aesthetical appearance [18] and encounter social difficulties related to visible differences in appearance, such as teasing in childhood and difficulties in romantic relationships in adulthood [19, 20].”

“…social difficulties (teasing, isolation) in individuals with skeletal dysplasias (Thompson, Shakespeare, & Wright, 2008), craniofacial anomalies (Feragen & Stock, 2017), and short stature (Lee et al., 2009; Quitmann, Bullinger, Sommer, Rohenkohl, & Bernardino Da Silva, 2016), and these…”

“The potential impact of demographic factors has not been discussed, since these have been extensively described in previous reviews (see Feragen & Stock, 2017; Hunt et al., 2005; Stock & Feragen, 2016).”

“These concerns are in line with the psychological co-morbidity of school-aged children with other visible medical conditions [28 – 30].”

“15,39 Financial burden related to their child ’ s microtia care was reported by over 90% of parents in this sample.”

“…adjustment problems attribute these to difficulties in social situations (Beaune et al., 2004; Bessell et al., 2012; Bradbury, 2012; Feragen et al., 2009; Feragen & Stock, 2017; KappSimon, McGuire, Long, & Simon, 2005; Patrick et al., 2007; Topolski et al., 2005; Warschausky et al., 2002).”

“2,13 Although there can be negative effects associated with CFCs, growing evidence indicates that patients with CFCs may experience similar 9,14 – 16 or fewer 9,16,17 psychosocial challenges compared to their peers without CFCs.”

“Craniosynostosis patients have been reported to have a less pleasing aesthetical appearance [17] and encounter social difficulties related to visible differences in appearance [18, 19].”

“(J Craniofac Surg 2022;33: 1670–1673) A craniofacial condition and its long-term treatment is expected to bring about a range of psychosocial challenges for those affected and their families.(1) For parents, the diagnosis can evoke a number of conflicting and fluctuating emotions, as they come to terms with the implic…”

“Additional medical difficulties could also play a role in emotional and behavioral problems of children with CL + P or IH (Hoornweg et al., 2009; Feragen & Stock, 2016).”

“Furthermore, regarding psychological adjustment to craniofacial anomalies, children and adolescents with syndromic anomalies have lower scores than nonsyndromic anomalies, revealing a deeper problem associated with syndromic conditions.[21]”

“Empirical data relevant to these hypotheses are limited (Feragen and Stock, 2017), primarily because CFM is a rare condition and most studies of social–behavioral functioning have involved diagnostically heterogeneous samples of children with more prevalent craniofacial diagnoses (eg, oral clefts).”

“Previous findings suggest that others' perceptions of the infant's appearance could spark negative emotions in individuals (27).”

“11 Indeed, a key risk factor for poor medical and psychosocial outcomes in children with a chronic illness is parental psychological distress.”

“15,16 Meyer-Marcotty et al noted that emotional displeasure elicited from reflecting on participants’ own CL-relevant facial features may result in aversion to their images, yielding higher fixation times in non-pathological areas such as the eyes.”

“Conversely, under-repair of cranial defects can leave patients susceptible to brain injury and neurological damage, as well as cosmetic issues and associated social and psychological distress.(18) Without evidence to support which defects require repair, poor, under-resourced, marginalized communities may be especiall…”

“)e multiple factors that determine outcome among those with such defects are poorly understood, and existing research has significant methodological challenges [1–12].”

“19,20 While the CC-UK aims to address this across the 4 HSCCs, with the implementation of a clinical protocol in which the same measures are used consistently, harmony across the wider literature is needed to allow for greater comparisons and homogeneity.”

“a visible difference).(4,5) A visible difference can stem from scarring after injuries, burns or surgery, cancer treatment, skin conditions or congenital medical conditions that affect stature, limbs, face or skull.”

“3,4 Commonly reported social experiences (eg, teasing and bullying) can also impact psychological well-being and self-esteem.”

“5 Yet, understanding of the psychosocial impact of rare conditions, such as craniosynostosis, and how to improve psychological health in those affected and their families remains relatively poor.”

“…appearance-conscious world, it is well established that conditions or injuries which affect appearance (often referred to as visible differences) can have a significant impact on psychological well-being and quality of life (Fournier et al. 2023; Feragen and Stock 2017; Rumsey and Harcourt 2004 ).”

“…self, can be traced within the article: the authors have in fact found high levels of anxiety and depression, relationship difficulties and negative experiences in subjects with craniofacial conditions, with a high perception of social stigmatization and frequent bullying (Feragen & Stock, 2017).”