A systematic review of the use of psychological assessment tools in congenital upper limb anomaly management

Journal of Hand Therapy xxx (2019) 1e10

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A systematic review of the use of psychological assessment tools in congenital upper limb anomaly management Robert Miller BSc, MBChB, MRCS a, Harsh Samarendra BA, BMBCh b, *, Matthew Hotton BSc, MSc, DClinPsy (Oxon) c a

Plastic Surgery Department, The Royal Free Hospital, London St Mary’s Hospital, Imperial College NHS Trust, London c Specialist Surgery Psychology Team, Psychological Medicine Centre, John Radcliffe Hospital, Oxford b

a r t i c l e i n f o

a b s t r a c

Article history: Received 18 October 2017 Received in revised form 1 July 2018 Accepted 2 November 2018 Available online xxx

Study Design: This study is a systematic review. Introduction: Congenital upper limb anomalies (CULAs) are often associated with psychosocial difficulties including negative body image, low self-esteem, and withdrawal from social activities. Purpose of the Study: The purpose of the study was to identify, describe, and evaluate all published psychosocial assessment tools used in the assessment and management of CULAs, to direct the use of these tools in clinical practice, and to identify areas requiring development. Methods: A systematic search of Medline, EMBASE, Pubmed, and PsychInfo databases was performed. In total, 23 studies were included for analysis. Data extracted included study and population characteristics, psychosocial measures utilized, psychosocial outcomes reported, and the reliability and validity of measures. Results: Seventeen patient-reported measures were identified. The most commonly used tool was a Likert scale (n ¼7) with satisfaction with appearance and function, the most commonly evaluated outcome (n ¼ 18). Other evaluated domains included quality of life or psychosocial functioning (n ¼ 9), self-image (n ¼ 2), and psychological well-being (n ¼ 5). Discussion and Conclusions: There is no well-established, validated assessment tool in regular use to effectively address psychosocial outcomes for children with CULAs. Although the majority of children born with a CULA appear to adjust well, this is by no means the case for all children. There is a need for routine psychosocial evaluation preoperatively and postoperatively with long-term follow-up data to help direct patient-orientated management. A clear understanding of these, and how to measure them, is needed to help for a patient-centred, multidisciplinary, evidence-driven approach to CULA management. Crown Copyright Ó 2018 Published by Elsevier Inc. on behalf of Hanley & Belfus, an imprint of Elsevier Inc. All rights reserved.

Keywords: Congenital upper limb anomaly (CULA) Pediatric Patient-reported outcome Psychosocial Reconstructive surgery

Introduction Congenital upper limb anomalies (CULAs) result from abnormal formation of the upper limb during fetal development and present with a wide range of anomalies from minor polydactyly to complete absence of the upper limb. The exact prevalence is hard to establish due to a lack of population-based studies. However, figures from 5.25 to 27.2 per 10,000 live births have been reported.1,2 The upper limbs are vital for human interaction and social integration. Hand function is important for children to manipulate and communicate with their external environment, which in turn facilitates normal psychosocial development.3,4 In contrast to some * Corresponding author. St Mary’s Hospital, Imperial College NHS Trust, Praed St, London W2 1NY. Tel.: þ44 7508944330. E-mail address: [email protected] (H. Samarendra).

other congenital conditions, hand anomalies are routinely visible to both the individual and those they interact with. Visible congenital anomalies are often associated with a variety of psychosocial difficulties, such as negative body image, difficulties with social interactions, low self-esteem, and poor quality of life.5,6 Qualitative studies have demonstrated that individuals with CULAs experience stress related to their functional and esthetic differences, further augmented by difficulties with social interactions and the perceived negative reactions of their peers.7 Subsequently, research indicates that these individuals may be more likely to experience low self-esteem, anxiety, and depression,3,8 with older children, in particular, at higher risk of becoming withdrawn and disengaging themselves from social activities.9 There has been a recent drive to try and establish the full impact of CULAs on both the patient and family. Traditionally, research has focused on objective functional and radiological measurements of

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R. Miller et al. / Journal of Hand Therapy xxx (2019) 1e10

presurgical and postsurgical intervention outcomes. Fewer studies have focused on the psychological and social impacts of CULAs.5,7,10 Assessment and input from clinical psychology teams is becoming an increasingly recognized area of importance in the management of this patient group as an adjunct to traditional surgical management.11 Furthermore, there has been a trend in using PatientReported Outcome Measures (PROMs) to assess the impact of CULAs,12,13 and subsequent surgical correction, on activities of daily living, vocation, and recreation.14 The importance of using PROMS in this population has been highlighted in recent reviews.12,13 However, such reviews fail to identify the optimal characteristics of psychosocial assessment tools for use in routine clinical practice. Similar to reviews carried out in other population groups, such as neurodisability, there is generally a lack of evidence for key psychometric properties of included PROMs.15 A further concordance to the neurodisability literature is the value of identifying a PROM, which can be used across different CULAs and can assess a variety of psychosocial outcomes. This would serve to improve comparison across different CULA types and to enhance comparison with children without CULAs. We feel that to produce a versatile, multidimensional PROM, there must first be an assessment of the psychosocial tools currently used in CULA management. Purpose of study The purpose of this study is to identify, describe, and evaluate all published psychosocial assessment tools currently used in the assessment and management of CULAs. Through this, we hope to further improve our understanding of the experiences of these individuals and highlight challenges involved in capturing the psychosocial impact of CULAs. This will aid in future development of reliable and valid psychosocial PROMs for routine clinical use as part of CULA treatment, planning, and care provision.

Search The literature search contained the search terms found in Figure 1 (see Supplementary Appendix for an example search strategy). Study selection All potential studies were identified based on the aforementioned search terms. Titles and abstracts were reviewed by two independent reviewers. Results were cross-referenced, and any discrepancies were discussed with a third, independent reviewer. Full-text articles were subsequently reviewed against the aforementioned eligibility criteria (see Fig. 2 for a flowchart of study selection). Data-collection process Data were extracted onto an electronic data-extraction sheet to identify author’s name, sample size, age, study design, CULA type and psychological measures(s) used, and key psychological findings. This extraction sheet was piloted on a sample of randomly selected articles and subsequently refined. The authors have adhered to the Preferred Reporting Items for Systematic Reviews and MetaAnalyses (PRISMA) guidelines in designing and reporting of studies. The principles of the Newcastle-Ottawa Scale were used to help evaluate the quality of included studies, particularly those which involved a surgical intervention. The Consensus-based Standards for the selection of health Measurement Instruments (COSMIN) checklist helped to guide appraisal of the outcome measures used in the included studies.17,18 As the aim of this review is not strictly to critique the methodological strengths or weakness of included studies, Newcastle-Ottawa Scale scores are not reported. Results Study selection

Materials and methods Eligibility criteria Studies were required to meet the following inclusion criteria:  The study was published in English.  The study included patients who were children (aged under 18 years) with a CULA. A CULA was defined as a congenital malformation, deformation, and/or dysplasia of an upper limb.16  The study examined psychosocial outcomes for children with a CULA using a novel self-/parent-reported measure developed by the authors and/or to use one or more already established self-/ parent-reported measures to assess psychosocial outcomes for children with a CULA. Case reports, letters, reviews, and studies using structured or semistructured interviews were excluded but were used to provide background to this area of research and were mined for references. Further exclusion criteria included studies that solely investigated functional outcomes. Information sources EMBASE, PubMed, Medline, and PsycINFO databases were searched from inception to March 2018 to identify eligibly studies. References of included studies were mined for additional references for inclusion.

The search strategy returned 390 results (Fig. 2). After the removal of duplicates and article screening, 30 full-text articles were retrieved for further analysis studies. Of these studies, 18 met inclusion criteria and were included for analysis. Five additional articles were identified from references. Study characteristics Twenty-three studies were included for full analysis (Table 1). Most studies were retrospective cohort studies, with only three studies prospectively identifying the patient groups and setting a defined follow-up period.19,25,30 Three studies were comparative, using a control group or population norm.34,37,38 There was large heterogeneity with sample sizes, with sizes ranging from 523 to 116,37 with an average of 32. Four studies focused on focused parental perspective.16,19,22,23 Six studies asked for patients’ perspective only; however, it is not clear if parental help was permitted or given to facilitate completion.3,20,24,27,29,33 The remaining studies addressed both parent and patient perspective, either by direct questioning each group or asking parents of patients unable to complete answering the questions due to age or disability.21,25,26,28,30-32,34-39 The congenital anomaly in question varied between the studies, 14 studies focused on one condition16,21-24,26,27,29-31,33,35,36,39 and 4 focused on single operative interventionespecific conditions.19,20,25,32 The remaining 5 investigated a variety of conditions.3,28,34,37,38 A range of tools were used to assess the psychosocial impact of the procedure on the children (Table 2). The

R. Miller et al. / Journal of Hand Therapy xxx (2019) 1e10

Subheading Population

Outcome

Measures

3

Search Terms hand deform*, hand anomal*, congenital hand difference, congenital

psychological, social, well-bring, wellbeing, well-being, self-concept, selfesteem, self-confidence, body image, appearance, Body, physical appearance, aesthetic Instrument, measure, questionnaire, inventory, scale, score, survey, checklist, outcome, interview psychological, interview

Fig. 1. Search terms used in literature search.

most common methods of assessment were Likert (n ¼ 7)16,19,21,24,27,35,36 or visual analog scales (n ¼ 4)26,29,31,33 designed by the study authors. Such methods fail to meet standards from PROMs set out in the COSMIN checklist and are at risk of social desirability bias, acquiescence bias, and end-aversion bias.40,41 Six authors designed novel questionnaires for the purpose of their studies20,22,23,25,32,39; however, mostly these were not included alongside the publication. The Pediatric Quality of Life Inventory (PaedsQoL),30,34,37,38 Childhood Experience Questionnaire (CEQ),19,25 and a questionnaire developed by Bellew et al

(1999)20,25,32 were the only assessment tools to be used in multiple studies. Of the included studies, none of the psychosocial measures had been shown to be valid or reliable in CULA populations. No studies specifically commented on the ease of use of the assessment tool; however, Bellew and Kay20 commented that some of the children in their study were too young to complete aspects of their questionnaire which made reference to the preoperative period. In general, studies assessed psychosocial outcomes across four main domains: (1) quality of life and psychosocial functioning; (2) adjustment to or satisfaction with function, appearance, and

Fig. 2. Flowchart of study selection.

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R. Miller et al. / Journal of Hand Therapy xxx (2019) 1e10

Table 1 Summary table of all included studies Author

Sample Patient ageb,c sizea

Study design

CULAs involved or intervention used

Measures used

Psychosocial constructs measured

Main psychosocial findings

IPACD Likert scale (5 point) General Health Questionnaire-28 Child behavior checklist Childhood experience questionnaire Hand distress score

Adjustment to/satisfaction with function, appearance, and surgery Psychosocial functioning Psychological well-being

Significant improvement in parents’ satisfaction with appearance after surgery (P ¼ .03) Nonsignificant improvement on CBC and Childhood Experience Questionnaire (CEQ) scores after surgery. Poor parental adjustment was associated with worse CBC and CEQ scores before surgery. 91% children reported being “happier” or “much happier” after surgery, with 82% reporting an increase in self-confidence Parents:  14/22: 5/5 for function  5/22: 5/5 for appearance Patient:  7/22: 3/5 for appearance Parents satisfied with appearance (1.6/15) of thumb and report favorable social interactions (4.8/ 15) 3 ¼ best possible score 15 ¼ worst possible score Results consistent with findings 5 years earlierdStaines et al, 200522 e see above (raw data not available in follow-up study)

Bradbury et al (1994)19

19

5.08

Prospective cohort

Microvascular toe transfers

Bellew & Kay (1999)20

32

8.5

Cohort

Variousdall undergone Novel 33-item toe-to-hand transfer questionnaire

Kemnitz et al (2002)21

22

Cohort

Preaxial polydactyly

Likert scale (5 point)

Staines et al (2005)22

10

5.2

Cohort

Thumb aplasia undergoing pollicization

Novel 17-item parental questionnaire Questions ranked on scale of 1 (best) to 5 (worse)

Adjustment to/satisfaction with function, appearance, and surgery

Aliu et al (2008)23

5

10.8

Cohort

Thumb aplasia undergoing pollicization

Adjustment to/satisfaction with function, appearance, and surgery

Goldfarb et al (2008)24

12

Not reported

Cohort

Central ray deficiency

Novel 32-item parental questionnaire (discrepancy in questionnaire question number with Staines et al22, unclear) Likert scale (5 point)

Andersson et al (2011)3

92

10.6

Case-control

Hand deformities and upper limb deficiencies

Piers-Harris Children’s Self-Concept scale

Psychosocial functioning Self-image

Bellew et al (2011)25

21

Not reported

Prospective cohort

Various but all treated with toe transfer

Novel 35-item questionnaire Hospital Anxiety and Depression Scale (HADS): Rosenberg Self-Esteem Inventory Birleson depression scale State-Trait Anxiety Inventory (STAI) Self-image profile (SIP) Childhood experience questionnaire (CEQ: 10, child; CEQ: 15, teenager)

Adjustment to/satisfaction with function, appearance, and surgery Psychosocial functioning Self-image Psychological well-being

Not reported

Adjustment to/satisfaction with function, appearance, and surgery Psychosocial functioning Adjustment to/satisfaction with function, appearance, and surgery

Adjustment to/satisfaction with function, appearance, and surgery

Most patients satisfied and consider appearance to have improved postsurgery 9 ¼ very satisfied 4 ¼ satisfied 3 ¼ somewhat satisfied (16 hands total in 12 patients) Total scores:  Comparison group ¼ 65.2  Mild deformity group ¼ 63.3  Severe deformity group ¼ 68.2 (no significant difference groups) Subgroups:  Children with ‘mild’ hand deformities had significantly worse scores in ‘popularity’ subgroup vs comparison group.  Children with severe deformities had significantly worse scores in ‘behavior’ and ‘intellectual and school status’ subgroup vs mild deformities 20/22 parents and 22/25 patients were “very satisfied” or “satisfied” with the results of surgery Depression and anxiety scores fell within normal ranges. Patients report higher self-esteem after surgery:  Mean Rosenberg self-esteem score: 15.46 (normative data mean ¼ 34.7) Patients did not have more negative social experiences  Mean CEQ scores: CEQ 10: 48.1 CEQ 15: 45.8 (normative data mean ¼ 50.1)

R. Miller et al. / Journal of Hand Therapy xxx (2019) 1e10

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Table 1 (continued ) Measures used

Psychosocial constructs measured

Main psychosocial findings

Cross-sectional Various case-control

Pediatric Quality of Life Inventory

Quality of life

11.8

Cross-sectional Various

Pediatric Quality of Life Inventory

Quality of life

20

Not reported

Cohort

Macrodactyly

Novel questionnaired‘Reach out’  Questions not stated  Parent or child completed depending on age

Adjustment to/satisfaction with function, appearance, and surgery

de Kraker et al, (2013)26

24

14

Cohort study

Thumb hypoplasia (type IIIB-V)

Adjustment to/satisfaction with function, appearance, and surgery

Aleem et al (2014)27 Kaplan and Jones (2014)28

18

Not reported

Cohort

Cleft hand

Visual analog scale (010) 0 ¼ worst 10 ¼ best Likert scale (5 point)

Age group 10-12 total score:  Control: 82.1  Case: 82.4, no significant difference between groups Age group 13-14 total score:  Control: 83.5  Case: 81 no significant difference between groups Only significant difference: Social functioning age 13-14  Control: 90  Case: 81.8 (P ¼ .01) PedsQL total score:  Parent: 82  Patient: 83 no significant difference between groups Large degree of variation observed. Mean limitation:  Environment and attitudes, 15  satisfaction, 35  future expectation, 11 (0 ¼ nonlimitation/best outcome; 100 ¼ total limitation/worst outcome) No difference between those managed conservatively or surgically Function rated highly by both patients and parents (7.5/10) Parents rate appearance higher than patients (8.1/10 vs 7.2/10) 21 of 23 patients ‘satisfied’ or ‘very satisfied’ with function

Cohort

Various

Pediatric Outcomes Data Collection Instrument (PODCI)

Singer et al (2014)29 Stutz et al (2014)30

32

Not reported

Cohort

Ulnar polydactyly

Visual analog scale (0100)

43

17

Prospective cohort

Radial polydactyly

Pediatric Quality of Life Inventory

Tonkin et al (2015)16

35

8.7

Cohort

Thumb hypoplasia

Likert scale (4 point)

Adjustment to/satisfaction with function, appearance, and surgery

Vuillermin et al (2015)31

16

11.6

Cohort

Radial longitudinal deficiency with softtissue release and bilobed flap treatment

Visual analog scale (010) PODCI

Adjustment to/satisfaction with function, appearance, and surgery Psychological well-being

Nikkhah et al, (2016)32

19

Not reported

Cohort

Various but all treated with toe transfer

Adjustment to/satisfaction 8-item questionnaire with function, appearance, adapted from Bellew et al, 201125 and Bellew and surgery 20 and Kay 1999 (parent and child reported).

Author

Sample Patient sizea ageb,c

Study design

Ardon et al (2012a)

116

11.8

Ardon et al, (2012b)

106

Hardwicke et al (2012)

12

4.4

CULAs involved or intervention used

Adjustment to/satisfaction with function, appearance, and surgery Adjustment to/satisfaction with function, appearance, and surgery

Adjustment to/satisfaction with function, appearance, and surgery Quality of life

Mean happiness as measured by PODCI:  Parent response (for child), 80*  Parent response (for adolescent), 84.5*  Adolescent response, 96.5** * No significant difference compared to “normal” population ** significantly higher compared to “normal” population Mean overall satisfaction was 89/ 100 (high satisfaction) Average Pediatric Quality of Life Inventory score:  Parents ¼ 87  Patients ¼ 87 No significant difference between polydactyly type and score. 94% believed appearance to be good or excellent 77% parents rated function as good or excellent VAS mean ratings:  Happiness ¼ 1.4  Satisfaction ¼ 1.2 Mean happiness as measured by PODCI ¼ 86.0 Parents  11/12 strongly agreed the procedure improved function.  5/12 strongly agreed the procedure improved appearance  11/12 very satisfied with surgery and would recommend to others Patient:  0/12 self-conscious about donor site  11/12 strongly agreed procedure improved function  5/12 strongly agreed procedure improved appearance (continued on next page)

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R. Miller et al. / Journal of Hand Therapy xxx (2019) 1e10

Table 1 (continued ) Author

Sample Patient sizea ageb,c

CULAs involved or intervention used

Measures used

Psychosocial constructs measured

Main psychosocial findings

Syndactyly

Visual analog scale (1100)

Adjustment to/satisfaction with function, appearance, and surgery

Cross-sectional Various (including lower limb) comparative study

Pediatric Quality of Life Inventory

Quality of life

Cohort

Duplicate thumb

Likert scale (3 point)

Adjustment to/satisfaction with function, appearance, and surgery

Cohort

Symbrachydactyly (with and without opposable digits)

Likert scale (10 point) Quality of life in neurological disorders (Neuro-QoL)

Adjustment to/satisfaction with function, appearance, and surgery Psychological well-being Quality of life

Median satisfaction:  Appearance ¼ 79  Function ¼ 91 Condition had minimal effect on profession/education or leisure activities and how these could be performed (all medians ¼ 98) Parent reported: - Unilateral CULA mean score ¼ 83.5*, multiple/lower limb deficiency mean score ¼ 70**, Norwegian school children mean score ¼ 86.5 Child reported: - Unilateral CULA mean score ¼ 84.5, multiple/lower limb deficiency mean score ¼ 69***, Norwegian school children mean score ¼ 83.5 * Significant lower score for school functioning subgroup ** Significant lower score for social and school functioning subgroup *** Significantly lower score for physical and social functioning 65% of patients/parents felt esthetic result was good. 70% patients/parents felt functional result was good. 98% of participant results within 1 standard deviation of population norm for all Neuro-QoL domains No significant difference among those with or without opposable digits.

Study design

Widerberg et al (2016)33

19

19 Cohort (median)

Johansen et al (2016)34

67

11

de Almeida (2017)35

20

Goodell et al (2017)36

53

12

CBC ¼ child behaviour checklist; CULA ¼ congenital upper limb anomalies; IPACD ¼ indicators of parental adjustment to congenital disfigurement; VAS ¼ Visual Analogue Scale. a N ¼ number of individuals with congenital hand anomalies only, excludes other patient groups (ie, trauma). b Ages expressed as ‘mean’ unless otherwise specified. c Age at follow-up and time of assessment, if explicitly stated.

surgery; (3) self-image; and (4) psychological distress. The qualitative comparisons from the included studies are summarized in Table 1 and discussed in more detail elsewhere in the article. Adjustment to/satisfaction with function, appearance, and surgery This was the most commonly assessed type of outcome included in 18 of the studies.16,19-29,31-33,35,36,39 All of these studies measured satisfaction with function and/or appearance through the use of visual analog (n ¼ 4) or Likert (n ¼ 7) scales, and all focused on satisfaction as a consequence of surgical intervention rather than satisfaction with appearance independent of surgery. Those studies focusing on satisfaction with function consistently found patients and/or their parents to be generally satisfied after surgery.16,20,21,23,25-27,29,33,35,36 Similarly, there were generally high levels of satisfaction with appearance after surgery.16,19-21,2326,29,31,33,36 De Kraker et al26 and Ardon et al38 additionally compared parent and child satisfaction with function and appearance, finding a high level of consistency between parents and children across both domains. The study by Bradbury et al19 was the only study to assess the statistical significance of parental satisfaction ratings before and after surgery and observed a significant improvement in ratings of appearance after surgery. Furthermore, Bradbury et al19 developed the Indicators of Parental Adjustment to Congenital Disfigurement for use in their study, with the authors reporting a significant

association between parental adjustment to their child’s condition and their family/social support. Unfortunately, the Indicators of Parental Adjustment to Congenital Disfigurement relied on interviewers to score parents on a three-point scale based on a semistructured interview rather than parents themselves providing a direct rating, introducing the potential for experimenter bias in this study. Quality of life and psychosocial functioning Quality of life was examined by 5 groups of authors.30,34,36-38 Both Ardon et al37 and Johansen et al34 examined the impact of various congenital hand differences on health-related quality of life using the Pediatric Quality of Life Inventory.42 Ardon et al37 found that children with congenital hand differences provided similar ratings of quality of life to their peers. This was with the exception of 13- to 14-year olds who rated their social functioning as significantly lower. Johansen et al34 found no significant difference in overall quality of life scores between a control population and unilateral CULA for both child- and parent-reported scores, apart from regarding parental reporting of school function, which was significantly lower (P ¼ .005). Furthermore, those with multiple lower limb deformities had significantly lower scores than controls with regard to physical and social function (child reported) and physical, school, and social functioning (parents reported), with only emotional function being comparable to controls. In all cases

R. Miller et al. / Journal of Hand Therapy xxx (2019) 1e10 Table 2 Summary of assessment tools used in included studies Tool used

Number of times used

References

Likert scales

7

Novel questionnaires

6

Pediatric Quality of Life Inventory (PaedsQoL)

4

Visual analog scales

4

Childhood experience questionnaire Pediatric Outcomes Data Collection Instrument (PODCI) Birleson Depression Scale Child Behavior Checklist General Health Questionnaire-28 Hand distress score Hospital Anxiety and Depression Acale (HADS): IPACD Piers-Harris Children’s SelfConcept Scale Quality of Life in Neurological Disorders (Neuro-QoL) Rosenberg Self-Esteem Inventory State-Trait Anxiety Inventory (STAI) Self-Image Profile (SIP)

2

1 1 1 1 1

Bradbury et al (1994)19 Kemnitz et al (2002)21 Goldfarb et al (2008)24 Aleem et al (2014)27 Tonkin et al (2015)16 de Almeida (2017)35 Goodell et al (2017)36 Bellew and Kay (1999)20 Staines et al (2005)22 Aliu et al (2008)23 Bellew et al (2011)25 Hardwicke et al (2012) Nikkhah et al, (2016)32 Ardon et al (2012a) Ardon et al, (2012b) Stutz et al (2014)30 Johansen et al (2016)34 de Kraker et al, (2013)26 Singer et al (2014)29 Vuillermin et al (2015)31 Widerberg et al (2016)33 Bradbury et al (1994)19 Bellew et al (2011)25 Kaplan and Jones (2014)28 Vuillermin et al (2015)31 Bellew et al (2011)25 Bradbury et al (1994)19 Bradbury et al (1994)19 Bradbury et al (1994)19 Bellew et al (2011)25

1 1

Bradbury et al (1994)19 Andersson et al (2011)3

1

Goodell et al (2017)36

1 1

Bellew et al (2011)25 Bellew et al (2011)25

1

Bellew et al (2011)25

2

IPACD ¼ indicators of parental adjustment to congenital disfigurement.

where there was a difference, parental scores were lower. Ardon et al38 compared child and parent ratings on the Pediatric Quality of Life Inventory, and overall, there appeared to be good agreement between child and parent raters. Similarly, Stutz et al30 obtained the same mean score from both patients and parents. However, Ardon et al38 reported a large degree of variation on an individual basis and expressed a need for caution when relying on parental ratings on the Pediatric Quality of Life Inventory. Most recently, Goodell et al36 used the Quality of Life in Neurological Disorders measurement system to examine stigma and interaction with peers, finding almost all patients to be within 1 standard deviation of population mean. However, a recent systematic review of PROMs for use in neurodisability populations found the Pediatric Quality of Life Inventory measure to fall short of the COSMIN standard for proxy reliability, and no studies were identified evaluating this for the Neuro-QoL.15 Social and behavioral functioning was assessed in 5 of the included studies.3,19,20,25,36 The earliest of these was carried out by Bradbury et al who used the Child Behaviour Checklist43 and CEQ44 to assess the quantity and quality of friendships, as well as any social or behavioral issues with children who had undergone microvascular-free toe transfer. They observed (nonsignificant) improvements after surgery on both measures. Furthermore, presurgery scores were lower for those children whose parents demonstrated poorer adjustment to their child’s condition, and

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these children continued to have difficulties with social relationships after surgery.19 Most recently, Goodell et al36 used the NeuroQoL tool to demonstrate results for stigma, and depression, anger, and peer relationships were comparable to a population norm. Unfortunately, the CEQ has not been validated, making it fall short of COSMIN standards, and although Bradbury attempted to develop norms for this measure by administering it to 390 local school children, they failed to report these norms.19 Conversely, the Child Behaviour Checklist has been shown to have good internal consistency, as well as structural and criterion validity in populations other than those with CULA (eg, those with autism).45 Regarding the Neuro-QoL, Janssens et al15 demonstrated little assessment of its psychometric properties. Although it shows some content validity within neurological disorders, this is not known for CULAs. Bellew and Kay20 did not observe any general improvements in parental ratings of child behavior or friendships after surgery. At follow-up, these children had fewer negative social experiences than the norm, as measured by the CEQ.25 Andersson et al3 used the Piers-Harris Children’s Self Concept Scale (PHCSCS46) to demonstrate that children with CULA scored similarly to a comparison group on measures of behavior, popularity, and intellectual/school status. However, of note was the finding that for boys, but not girls, with mild hand deformity, their scores on these measures were worse than scores for those with severe hand deformity. Although the psychometric properties of the PHCSCS in the CULA population was not directly reported by Andersson et al, Piers and Harris47 have previously demonstrated good internal consistency, testretest reliability, and construct validity in the general population.3 Self-image Self-image, self-concept, and self-esteem were explored by Bellew et al25 and Andersson et al.3 Using the Rosenberg’s SelfEsteem Inventory,48 Bellew et al found that patients who had undergone toe transfer reported higher self-esteem than the general population norms. Furthermore, the same patients’ scores on the Self-Image Profile49 were in the normal range. These patients also reported greater self-confidence after surgery on a questionnaire designed by the authors.25 Similarly, Andersson et al3 used the PHCSCS to demonstrate that most children with CULA had “good” self-concept, which was equivalent to the comparison group. Both the Rosenberg Self-Esteem Inventory48 and Self-Image Profile24 have previously been shown to have strong psychometric properties; however, in keeping with the other studies included in this review, Bellew et al25 did not report on the psychometric properties for the CULA population. Psychological well-being Bellew et al25 and more recently Goodell et al36 were the only authors to assess anxiety and depression in young people with a CULA. Bellew et al, using a battery of well-established assessments, and Goodell et al, using the Neuro-QoL, demonstrated that patients who underwent toe transfer and symbrachydactyly surgery, respectively, reported anxiety and depression levels that fell in the “normal range” and 1 standard deviation of normal. Interestingly, Goodell et al36 also found no difference between those with and without opposable thumbs. Only Bradbury et al examined parental anxiety and depression, as measured by the General Health Questionnaire.50 They found that parents’ scores on the General Health Questionnaire-28 did not significantly improve after their child underwent microvascular toe transfer.19 The impact of soft-tissue release and bilobed flap treatment for radial longitudinal deficiency on happiness was investigated by

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Vuillermin et al31 using the Pediatric Outcomes Data Collection Instrument (PODCI);51 however, the authors failed to provide an interpretation of their results. Bellew et al assessed happiness ratings after toe transfer, finding that patients reported greater happiness after surgery.25 Similarly, Kaplan and Jones28 reported significantly higher happiness scores using PODCI in adolescent responses, but no difference with parental responses for children or adolescents. Discussion The primary role of most surgical interventions for CULAs is to improve function while addressing esthetic concerns. Ultimately, the aim is to improve the patient’s quality of life, both in childhood and also their adult lives, by facilitating functional and psychosocial development. Understanding the impact of CULA treatment, be it surgical or nonsurgical, is vital to help direct person-centered care and help facilitate evidence-based decision-making. This review is the first to specifically investigate the psychosocial impact of CULAs on children. Its findings largely mirror those of more general reviews evaluating PROMs in this population.12 Although many studies reported that most children born with a CULA are satisfied with their appearance/function and do not experience poorer psychosocial functioning than children born without a CULA, many children still experience psychosocial difficulties.3,19,37 Our review identified only 23 studies addressing the psychosocial outcomes of CULA management. The majority were retrospective without a control group, reducing the internal validity of these studies. However, 8 of these only used a Visual Analogue Scale or Likert scale. Although these are easy to complete and provide some insights into satisfaction with surgery, they are crude. For example, when a three-point scale looking at only two domains are used,35 an accurate assessment of psychosocial adjustment is not possible, and there is a high risk of acquiescence bias. Furthermore, with so few domains addressed, the risk of measurement bias is high, particularly when little information is given regarding how the surveys are completed. In a comparison of Likert and visual analog scales, van Laerhoven et al41 found that although children show a preference for Likert scales, both types of measure can be prone to the effects of acquiesce and end-aversion bias in child populations, limiting the reliability and validity of such measures. Although we included these studies in our review, the primary aim of publications using only these measures was not to assess psychosocial outcomes of CULA management. A significant challenge for measuring psychosocial outcomes is the age of patients at assessment. Surgery is performed in children as young as 8.6 months29 when clearly it is not possible to take baseline self-report measurement for future follow-up and comparison. Without the ability to compare patients before and after reconstruction, either due to age or ability of the child to complete the assessment, studies need to provide control groups to allow comparison to other children with no CULA or, ideally, CULAs that have been managed nonoperatively. The only study to include nonoperative management of CULA was the one by Goodell et al36 addressing macrodactyly. This issue has been partially overcome in several studies by including parental-report measures. Importantly, children coconstruct the psychological meaning of visible and functional differences with their parents. Bradbury et al demonstrated the crucial role of parental adjustment on the psychosocial development of their child. The responses and behavior of parents during a child’s preschool years is a critical element in alleviating or exacerbating future stress and self-consciousness. Parental behaviors, such as covering an infant’s hand with mittens, are likely to lead the child

to develop negative beliefs about the appearance of their hand.19 Furthermore, if parents react with panic or distress in response to their child’s questions about their hand condition, then they are more likely to perceive their differences with negativity and shame.8 Early intervention and exploration of parental concerns and anxieties should also be an integral aspect of care for families to facilitate familial adjustment and subsequently the emotional and behavioral development of the child. It is therefore important to routinely assess parental attitudes and concerns with regard to their child’s condition. Although studies within this review have demonstrated a high degree of consistency of agreement between child and parent ratings of appearance and functioning,26,38 the proxy reliability of parental measures can vary and is likely to introduce a degree of bias.18 Furthermore, Ardon found that although there was no significant difference between parent and patient PedsQoL scores, there was a large variation with patients and their parents tending to disagree, but without a consistent pattern.38 Other groups have questioned the reliability of parental assessment of children’s health-related quality of life with greater agreement for physical functioning than emotional or social functioning.52 In our analysis, assessments were grouped based on the psychological construct measured: (1) adjustment to/satisfaction with function; (2) appearance and surgery; (3) quality of life and psychosocial functioning; and (4) self-image and psychological wellbeing. These are key areas that need to be addressed using assessment tools. The COSMIN checklist18 was used to appraise the psychometric quality of PROMs. Although many of the assessments used have been shown to be reliable and valid in a variety of patient groups, none of the assessment tools identified by our review have been validated in a CULA population. Unfortunately, no studies included control groups to allow assessment of appearance independent from surgery. The results of this study add further weight to the recent call for the development of a reliable, valid, and standardized multidimensional PROM that encapsulates biopsychosocial domains as such a measure is clearly lacking from the literature.12 The recent development and success of Face-Q53 highlight how it is possible to develop a set of reliable and valid PROMs for use across multiple biopsychosocial domains in patients undergoing facial surgery. As such, it is desirable to develop similar measures in the child CULA population. The only well-established measure to be used across multiple studies was the Pediatric Quality of Life Inventory,30,34,37,38 which measures health-related quality of life. The advantage of this tool is that there are both child-reported and parentalreported versions, which have previously been shown to be reliable.42 Ardon et al demonstrated that children with CULA aged 13 to 14 years reported worse social functioning than their peers. This was not found in the younger patient groups. Unfortunately, this study did not investigate whether such issues persist into adulthood,37 with 13 to 14 years being the eldest patient subgroup. In general, studies failed to assess the impact of CULA through to adulthood. This is an important area for future study to better understand the long-term impact of being born with CULA and different management strategies. Furthermore, there is a lack of agreement regarding the optimum time to carry out surgery for CULA. It is argued that earlier surgical intervention may minimize both physical and psychological burden54; however, again we lack assessment tools to assess this. None of the tools discussed have been validated in the CULA population and therefore may not be appropriate in this patient group. Goodell et al.36 commented on their use of the Neuro-QoL tool that it was designed for more debilitating conditions which may limit its sensitivity.

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It is important to note that this review has limitations. The inclusion of only studies written in the English language is likely to have led to the exclusion of broader international perspectives. Although the COSMIN checklist was used to guide appraisal of PROMs, the vast majority of studies failed to include sufficient information about the psychometric properties of their included PROMS. As a result of this and given the absence of PROMs that are standardized and validated for the CULA population, it is not possible to draw firm conclusions about the psychosocial outcomes of this population. Furthermore, owing to the large heterogeneity of study design across included studies, it was not possible to provide a quantitative synthesis of the outcome data or address publication bias. Conclusion In conclusion, there is no well-established, validated assessment tool or battery of tools in regular use that effectively addresses the psychosocial outcomes for children born with CULAs. Although the majority of children born with a CULA appear to adjust well to their condition, this is by no means the case for all children. Although surgeons and parents may be good judges of functional outcomes, quality of life can only really be accurately measured if self-reported.39 A consensus is therefore needed regarding the most appropriate tool to be used, with clear guidelines for different ages and an assessment of parental adjustment to their child’s condition. Such measures need to be validated in a CULA population and shown to be reliable and efficient for use within routine clinical practice. These measures should be used to facilitate the capture of longitudinal data into adult life so as to help guide evidence-based CULA management. This will also help determine which children and families will benefit from clinical psychological input, allowing a targeted approach for intervention to help address parental concerns and facilitate the psychosocial well-being of children born with CULAs. References 1. Koskimies E, Lindfors N, Gissler M, Peltonen J, Nietosvaara Y. Congenital upper limb deficiencies and associated malformations in Finland: a population-based study. J Hand Surg Am. 2011;36(6):1058e1065. 2. Goldfarb CA, Shaw N, Steffen JA, Wall LB. The prevalence of congenital hand and upper extremity anomalies based upon the New York congenital malformations registry. J Pediatr Orthop. 2017;37(2):144e148. 3. Andersson GB, Gillberg C, Fernell E, Johansson M, Nachemson A. Children with surgically corrected hand deformities and upper limb deficiencies: self-concept and psychological well-being. J Hand Surg Eur Vol. 2011;36(9):795e801. 4. Johnson SP, Sebastin SJ, Rehim SA, Chung KC. The importance of hand appearance as a patient-reported outcome in hand surgery. Plast Reconstr Surg Glob Open. 2015;3(11):e552. 5. Rumsey N, Harcourt D. Visible difference amongst children and adolescents: issues and interventions. Dev Neurorehabil. 2007;10(2):113e123. 6. Versnel SL, Duivenvoorden HJ, Passchier J, Mathijssen IMJ. Satisfaction with facial appearance and its determinants in adults with severe congenital facial disfigurement: a case-referent study. J Plast Reconstr Aesthet Surg. 2010;63(10): 1642e1649. 7. Franzblau L, Chung K, Carlozzi N, Chin A, Nellans K, Waljee JF. Coping with congenital hand differences. Plast Reconstr Surg. 2015;135(4):1067e1075. 8. Bradbury E. The psychological and social impact of disfigurement to the hand in children and adolescents. Dev Neurorehabil. 2007;10(2):143e148. 9. Hermansson L, Eliasson AC, Engström I. Psychosocial adjustment in Swedish children with upper-limb reduction deficiency and a myoelectric prosthetic hand. Acta Paediatr. 2005;94(4):479e488. 10. Bradbury E, Kay S, Tighe C, Hewison J. Decision-making by parents and children in paediatric hand surgery. Br J Plast Surg. 1994;47(5):324e330. 11. Tan J, Tu Y. Comparative study of outcomes between pollicization and microsurgical second toe-metatarsal bone transfer for congenital radial deficiency with hypoplastic thumb. J Reconstr Microsurg. 2013;29(9):587e592. 12. Adkinson JM, Bickham RS, Chung KC, Waljee JF. Do patient- and parentreported outcomes measures for children with congenital hand differences capture WHO-ICF domains? Clin Orthop Relat Res. 2015;473(11):3549e3563. 13. Bickham R, Waljee J, Chung K, Adkinson J. Postoperative patient- and parentreported outcomes for children with congenital hand differences: a systematic review. Plast Reconstr Surg. 2017;139(6):1422e1429.

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14. Waljee JF, Carlozzi N, Franzblau LE, Zhong L, Chung KC. Applying the patientreported outcomes measurement information system to assess upper extremity function among children with congenital hand differences. Plast Reconstr Surg. 2015;136(2):200ee207e. 15. Janssens A, Rogers M, Gumm R, et al. Measurement properties of multidimensional patient-reported outcome measures in neurodisability: a systematic review of evalutation studies. Dev Med Child Neurol. 2016;58:437e451. 16. Tonkin MA, Boyce DE, Fleming PP, Filan SL, Vigna N. The results of pollicization for congenital thumb hypoplasia. J Hand Surg Eur Vol. 2015;40(6): 620e624. 17. Wells G, Shea B, O’Connell D, Peterson J, Welch V, Losos M. The Newcastle-Ottawa Scale (NOS) for Assessing the Quality if Nonrandomized Studies in Meta-Analyses; 2009. Available at: http://www.ohri.ca/programs/clinical_epidemiology/ oxford.htm. Accessed March 28, 2018. 18. Mokkink L, de Vet H, Prinsen C, et al. COSMIN risk of bias checklist for systematic reviews of patient-reported outcome measures. Qual Life Res. 2018;27: 1171e1179. 19. Bradbury E, Kay S, Hewison J. The psychological impact of microvascular free toe transfer for children and their parents. J Hand Surg Br. 1994;6:689e695. 20. Bellew M, Kay S. Psychological aspects of toe to hand transfer in children. Comparison of views of children and their parents. J Hand Surg Br. 1999;6:712e 718. 21. Kemnitz S, De Smet L. Pre-axial polydactyly: outcome of the surgical treatment. J Pediatr Orthop. 2002;11(1):79e84. 22. Staines KG, Majzoub R, Thornby J, Netscher DT. Functional outcome for children with thumb aplasia undergoing pollicization. Plast Reconstr Surg. 2005;116(5):1314e1323. 23. Aliu O, Netscher DT, Staines KG, Thornby J, Armenta A. A 5-year interval evaluation of function after pollicization for congenital thumb aplasia using multiple outcome measures. Plast Reconstr Surg. 2008;122(1):198e205. 24. Goldfarb C, Chia B, Manske P. Central ray deficiency: subjective and objective outcome of cleft reconstruction. J Hand Surg Am. 2008;33(9):1579e1588. 25. Bellew M, Haworth J, Kay S. Toe to hand transfer in children: ten year follow up of psychological aspects. J Plast Reconstr Aesthet Surg. 2011;64(6):766e775. 26. de Kraker M, Selles R, van Vooren J, Stam H, Hovius S. Outcome after pollicization: comparison of patients with mild and severe longitudinal radial deficiency. Plast Reconstr Surg. 2013;131(4):544ee551e. 27. Aleem A, Wall L, Manske M, Calhoun V, Goldfarb C. The transverse bone in cleft hand: a case cohort analysis of outcome after surgical reconstruction. J Hand Surg Am. 2014;39(2):226e236. 28. Kaplan J, Jones N. Outcome measures of microsurgical toe transfers for reconstruction of congenital and traumatic hand anomalies. J Pediatr Orthop. 2014;34(3):362e368. 29. Singer G, Thein S, Kraus T, Petnehazy T, Eberl R, Schmidt B. Ulnar polydactyly an analysis of appearance and postoperative outcome. J Pediatr Surg. 2014;49(3):474e476. 30. Stutz C, Mills J, Wheeler L, Ezaki M, Oishi S. Long-term out- comes following radial polydactyly reconstruction. J Hand Surg Am. 2014;39:1549e1552. 31. Vuillermin C, Wall L, Mills J, et al. Soft tissue release and bilobed flap for severe radial longitudinal deficiency. J Hand Surg Am. 2015;40(5):894e899. 32. Nikkhah D, Martin N, Pickford M. Paediatric toe-to-hand transfer: an assessment of outcomes from a single unit. J Hand Surg Eur Vol. 2016;41(3):281e294. 33. Widerberg A, Sommerstein K, Dahlin LB, Rosberg HE. Long-term results of syndactyly correction by the trilobed flap technique focusing on hand function and quality of life. J Hand Surg Eur Vol. 2016;41(3):315e321. 34. Johansen DH, Andersen BØ, Andresen IL. Children with congenital limb deficiency in Norway: issues related to school life and health-related quality of life. A cross-sectional study. Disabil Rehabil. 2016;38(18):1803e1810. 35. de Almeida C. Analysis of surgical results and of residual postoperative deformities in preaxial polydactyly of the hand. J Plast Reconstr Aesthet Surg. 2017;70(10):1420e1432. 36. Goodell A, Bauer A, Oishi S, et al. Functional assessment of children and adolescents with symbrachydactyly: a unilateral hand malformation. J Bone Joint Surg Am. 2017;99(13):1119e1128. 37. Ardon M, Janssen W, Stam H, Selles R, Hovius S. Low impact of congenital hand differences on health-related quality of life. Arch Phys Med Rehabil. 2012;93(2): 351e357. 38. Ardon M, Selles R, Roebroeck M, Stam H, Janssen W, Hovius S. Poor agreement on health-related quality of life between children with congenital hand differences and their parents. Arch Phys Med Rehabil. 2012;93(4):641e646. 39. Hardwicke J, Khan M, Richards H, Warner R, Lester R. Macrodactyly - options and outcomes. J Hand Surg Eur Vol. 2013;38(3):297e303. 40. Garland R. The mid-point on a rating scale: is it desirable? Mark Bull. 1991;2: 66e70. 41. van Laerhoven H, van der Zaag-Loonen HJ, Derkx BH. A comparison of likert scale and visual analogue scales as response options in children’s questionnaires. Acta Paediatr. 2004;93:830e835. 42. Varni J, Seid M, Kurtin P. PedsQLTM 4.0: reliability and validity of the pediatric quality of life InventoryTM version 4.0 generic core scales in healthy and patient populations. Med Care. 2001;39(8):800e812. 43. Achenbach TM, Edelbrock C, Howell CT. Empirically based assessment of the behavioral/emotional problems of 2- and 3-year-old children. J Abnorm Child Psychol. 1987;15(4):629e650. 44. Pertschuk M, Whitaker L. Psychosocial outcome of craniofacial surgery in children. Clin Plast Surg. 1987;14(1):163e168.

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45. Pandolfi V, Magyar C, Dill C. An initial psychometric evaluation of the CBCL 6e 18 in a sample of youth with autism spectrum disorders. Res Autism Spectr Disord. 2012;6(1):96e108. 46. Piers E, Harris D. Age and other correlates of self-concept in children. J Educ Psychol. 1964;55(2):91e95. 47. Piers E, Harris D. PierseHarris Children’s Self-concept Scale Revised Manual. Los Angeles: West Psychol Serv; 1984. 48. Sinclair S, Blais M, Gansler D, Sandberg E, Bistis K, LoCicero A. Psychometric properties of the Rosenberg self-esteem scale: overall and across demographic groups living within the United States. Eval Health Prof. 2010;33:56e80. 49. Butler R, Gasson S. Development of the self-image profile for adults [SIP-AD]. Eur J Psychol Assess. 2006;22(1):52e58.

50. Goldberg DP, Hillier VF. A scaled version of the genreal health questionnaire. Psychol Med. 1979;9(1):139e145. 51. Daltroy L, Liang M, Fossel A, Goldberg M. The POSNA pediatric musculoskeletal functional health questionnaire: report on reliability, validity, and sensitivity to change. J Pediatr Orthop. 1998;18(5):561e571. 52. Eiser C, Morse R. Can parents rate their child’s health-related quality of life? Results of a systematic review. Qual Life Res. 2001;10(4):347e357. 53. Klassen AF, Cano SJ, Scott A, Snell L, Pusic A. Measuring patient-reported outcomes in facial aesthetic patients: development of the FACE-Q. Facial Plast Surg. 2010;26(4):303e309. 54. Watson S. The principles of management of congenital anomalies of the upper limb. Arch Dis Child. 2000;83:10e17.

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Appendix: S1. Example search strategy in full 1 Hand Deformities, Congenital/ 2 “congenital upper limb anomal*”.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 3 CULA.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 4 CHDs.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 5 “hand deform*”.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 6 “hand anomal*”.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 7 “congenital hand difference*”.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 8 (“congenital anomal*” adj3 (“upper limb” or hand*)).mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 9 1 or 2 or 3 or 4 or 5 or 6 or 7 or 8. 10 exp child/or exp infant/ 11 exp Parents/ 12 (child or children).mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 13 Infant*.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 14 parent*.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 15 (father or mother).mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 16 10 or 11 or 12 or 13 or 14 or 15. 17 exp Adaptation, Psychological/ 18 Social Adjustment/ 19 psychosocial.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 20 psychological.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier]

10.e1

21 social.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 22 (well-being or “well being”).mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 23 wellbeing.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 24 Self Concept/ 25 self esteem.mp. 26 self confidence.mp. 27 body image.mp. or Body Image/ 28 appearance.mp. or Body, Physical Appearance/ 29 aesthetic*.mp. 30 17 or 18 or 19 or 20 or 21 or 22 or 23 or 24 or 25 or 26 or 27 or 28 or 29. 31 checklist/or exp “surveys and questionnaires”/ 32 instrument*.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 33 measure*.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 34 questionnaire*.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 35 inventory.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 36 scale*.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 37 score*.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 38 survey*.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 39 checklist*.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 40 outcome*.mp. [mp ¼ title, abstract, original title, name of substance word, subject heading word, keyword heading word, protocol supplementary concept word, rare disease supplementary concept word, unique identifier] 41 Interview, Psychological/or Interview/or interview.mp. 42 31 or 32 or 33 or 34 or 35 or 36 or 37 or 38 or 39 or 40 or 41. 43 9 and 16 and 30 and 42.