Children with cleft lip and/or cleft palate commonly require multiple operations to improve cleft-specific aspects of appearance as a result of growth over time. Although much attention has been directed toward describing the technical aspects of cleft lip repair, little is known about patient and family preferences regarding cleft revision.1–3 Consequently, the precise indications and decision-making process surrounding revision procedures remain unclear and understudied.3–6 Importantly, however, these decisions can have enormous psychosocial and financial consequences.
A child with a cleft lip will undergo an average of eight surgical procedures throughout childhood; children with both a cleft lip and a cleft palate undergo revision at even higher rates than children with cleft lip alone.7–9 Previous studies demonstrate that children with cleft lip and/or cleft palate associate health-related quality of life to cleft-specific features of appearance.10,11 Children with visible craniofacial abnormalities have poorer perceptions of themselves and their appearances than children of a similar age without clefts. There is also evidence that this negative view of appearance is generalized to whole body appraisals.12–15 Children with visible cleft lip and/or cleft palate–related changes not only express lower self-esteem with appearance, but also report low self-perceived intellect compared with peers.13,14 For those children with poor health-related quality of life attributable to dissatisfaction with appearance, revision surgery may improve self-esteem and facial appearance.16 For example, adolescents undergoing revision surgery for nasal deformities experienced improved self-perception and quality of life after revision.17–19
Although many adolescents describe satisfaction after surgical revision, it is difficult to define which children will benefit most from revision, and how discussions regarding revision surgery should be conducted.17 Psychosocial adjustment and well-being is one important factor that can affect the desire for revision. Although many surgeons opt for reconstruction before school entrance, there are many individual and societal factors that affect the decision to perform or undergo cleft revision surgery.20–22 Preferences of surgeons and parents and broader cultural norms contribute to this process in a range of ways, depending on children’s circumstances. For example, although some surgeons do not perform revision procedures unless it is specifically requested by the child or parent, others routinely perform revision based on their personal preferences and training.1,17,23–25 It is also uncertain how patients’ preferences for revision compare to other groups, including parents and surgeons.
Understanding differences in perceptions between groups can inform the approach to decision-making before cleft revision surgery. In this context, we sought to compare satisfaction with orofacial appearance and the desire for cleft revision surgery among surgeons, patients, caregivers, and the general public. Our hypothesis was that parents, children, and surgeons would align in terms of the desire for revision and would demonstrate similar levels of satisfaction with cleft-specific aspects of appearance. Defining variations in perceptions among these groups can guide surgeon-patient interactions in the clinic and improve our ability to empower patients and families.
PATIENTS AND METHODS
We compared satisfaction with cleft-specific aspects of appearance and the desire for revision among parents, children, surgeons, and a community sample of adults. We surveyed children with cleft lip and/or cleft palate receiving treatment in a multidisciplinary cleft clinic (n = 100) and the children’s caregivers (n = 100). Children who met eligibility for inclusion were between 5 and 19 years of age and able to read and respond independently in English. A research assistant administered electronic versions of the instruments to patients and caregivers separately in the absence of the health care team. Plastic surgeons (n = 10) and a community sample of adults (n = 10) received photographs to rate according to a modified version of the scale administered to parents and children, as described below. Plastic surgeons did not evaluate their own patients and outcomes. The community sample of adults included participants who do not work professionally with children with cleft lip and/or cleft palate and do not have a family member with a cleft. This cohort was selected from within the hospital system (Fig. 1). Institutional review board approval was achieved before initiation of the study. All surgeons performed a rotation advancement type repair for the children with cleft lips included in this study.
Satisfaction with Appearance
To assess satisfaction with appearance, we used the Cleft Evaluation Profile. The Cleft Evaluation Profile is a 14-item instrument that is designed to delineate satisfaction with various aspects of cleft-specific appearance and function, including the upper lip, nose, and midface.17 Satisfaction was rated on a scale ranging from 1 to 7, with 1 being the most satisfied and 7 being the least satisfied. This instrument was administered to children and caregivers independently.
We also elicited ratings of appearance from plastic surgeons (n = 10) and the general cohort (n = 10) using photographs of these same children; each of these participants rated 10 children using five standardized photographs per patient. Using these photographs, surgeons and the community sample rated satisfaction with overall and cleft-specific appearance based on facial characteristics analogous to those defined by the Cleft Evaluation Profile. (See Appendix, Supplemental Digital Content 1, which shows a copy of the questionnaire used to rate photographs of children with cleft lip and/or cleft palate. This questionnaire was administered to plastic surgeons and the community sample of adults to measure satisfaction with appearance when reviewing standardized photographs of children with cleft lip and/or cleft palate, http://links.lww.com/PRS/D534.) This Cleft Evaluation Profile–based questionnaire required that surgeons and members of the general cohort evaluate their personal satisfaction with the patient’s upper lip, nose, maxilla, scar, and general facial appearance. The wording of the questions was modified to ensure that the surgeons/general cohort members would understand that they should document their own individual evaluation of the patient’s specified facial feature. To improve sensitivity, this instrument was based on a scale from 0 to 10, and patient/parent scores were converted to these values by a linear transformation to allow comparability. The surgeon responsible for an individual patient’s care was not allowed to evaluate that participant’s photograph.
Desire for Cleft Revision
All groups also received a survey focused on understanding the desire to perform/undergo revision. The site of desired revision, timing, and reason for revision were evaluated. The desire for revision was characterized as a categorical variable (yes/no). Specifically, each participant was asked about recommending/desiring revision now or later, and at which anatomical region. (See Appendix, Supplemental Digital Content 2, which shows a copy of the questionnaire used to gauge desire for revision. This questionnaire was used to gauge the desire of children with cleft lip and/or cleft palate to undergo revision; a version of this questionnaire was administered to each group, http://links.lww.com/PRS/D535.)
Clinical and Demographic Data
Demographic and clinical data including sex, age, race, household income, and primary cleft diagnoses were collected from a survey and the medical record. Patient age was defined as a continuous variable. Household income was self-reported as less than $40,000, $40,000 to $100,000, and greater than $100,000. Race and sex were defined as categorical variables.
Univariate statistics were used to describe attributes of the study sample. Differences in desire for revision (1 = yes and 0 = no) were examined by means of chi-square tests and Fisher’s exact tests. Caregiver ratings of the child’s nose, lip, and maxilla (based on scale ranging from 1 to 7 and recoded to 1 = least satisfied and 7 = most satisfied) were compared to the child’s ratings using repeated measures/paired samples t tests. Child and parent scores were then rescaled to a scale ranging from 0 to 10 and were separately compared to surgeon and community sample scores using a repeated measures analysis of variance with rater treated as the within-subjects factor, followed by Bonferroni adjusted pairwise comparisons by rater. Lastly, age, sex, and race of the patients were added as between-subject factors followed by Bonferroni adjusted pairwise comparisons by rater to ascertain whether or not statistical significance persisted while adjusting for age, sex, and race. If the assumption of sphericity was not met, Huynh-Feldt correction was used to test within-subject effects. Normality was assessed by plotting and examining the saved residuals of each score by each rater on individual normal/quantile-quantile plots. If all residuals were on or fairly near the diagonal of the plot, the data were considered approximately normal. Repeated measures analysis of variance has been shown to be robust under minor normality violations. All analyses were conducted in Stata 13 (StataCorp, College Station, Texas) and IBM SPSS Version 22 (IBM Corp., Armonk, N.Y.). Significance was set at p < 0.05. Clinical significance was determined based on differences in score by at least one point between groups given the gradations used by the Cleft Evaluation Profile.
Of the 100 children with cleft lip and/or cleft palate, the average age was 10.5 ± 3.6 years (range, 6 to 18 years). Among these children, 53 percent were male; 69 percent were Caucasian, 26 percent were Asian, 3 percent were Indian/Alaskan, and 2 percent were Native Hawaiian. Unilateral cleft lip (54 percent) was the most common presentation, followed by bilateral cleft lip (23 percent), unilateral cleft lip and palate (17 percent), and bilateral cleft lip and palate (6 percent) (Table 1).
Repeated measures analysis of variance with Huynh-Feldt correction determined that satisfaction with the appearance of the nose differed significantly between raters (F2.83,249.1 = 5.70l, p < 0.001). Similarly, satisfaction with the appearance of lip and extent of maxillary hypoplasia also differed between raters (F3,264 = 5.03, p < 0.002; F3,261 = 2.83, p < 0.04, respectively). Tests for the interaction of age, sex and race with rater with respect to each outcome were not significant.
Children and Their Caregivers Were Similarly Satisfied with Most Cleft-Specific Aspects of Appearance
Children and caregivers expressed similar degrees of satisfaction with the appearance of the lip (5.48 ± 1.69 versus 5.6 ± 1.49; p > 0.5) and extent of maxillary hypoplasia (6.08 ± 1.1 versus 5.8 ± 1.2; p = 0.07). However, children were significantly more satisfied than caregivers with the appearance of their noses (5.5 ± 1.69 versus 5.1 ± 1.52; p = 0.03). Results between cohorts persisted after controlling for age, sex, and race in the multivariate analysis (Table 2).
Children Were Significantly More Satisfied with Their Appearance Compared with Surgeons and Adults from the Community
Post hoc tests using a Bonferroni correction indicated that children rated personal satisfaction with cleft-specific aspects of appearance including the nose, lip, and maxilla as significantly higher than surgeons (nose, 7.77 versus 5.51, p < 0.001; lip, 7.94 versus 5.90, p < 0.001; maxilla, 8.16 versus 6.56, p < 0.001) and control observers (nose, 7.78 versus 6.00, p < 0.001; lip, 7.80 versus 6.12, p < 0.001; maxilla, 8.16 versus 7.40, p < 0.001) (Table 2). Interestingly, there was no significant difference between ratings by plastic surgeons compared to the general cohort (p > 0.05). Differences between all cohorts persisted after controlling for age, sex, and race in the multivariate analysis.
Surgeons and Caregivers Recommended Surgical Revision More Commonly Than Children Themselves Desired Revision
There was no significant relationship between children and surgeons in terms of the desire for revision surgery (p = 0.44) (Table 3). For children who desired revision, surgeons agreed 93 percent of the time and disagreed 4 percent of the time. More importantly, when the child did not desire revision, there was agreement with surgeons only 12 percent of the time. This is consistent with the fact that children were more satisfied with their appearance than surgeons (Fig. 1).
In this study, our group demonstrated that children have more favorable perceptions of personal appearance compared with surgeons and members of the larger community, and more similar ratings to their caregivers. In our cohort, surgeons recommended revision more frequently than children requested it. Although moderate agreement was noted between groups when children desired revision, there was much less agreement in situations in which children did not desire revision. As children in our cohort tended to be more satisfied with appearance than parents, surgeons, and lay people, this may have contributed to the reluctance to undergo revision.
Our findings build on those of previous studies that have examined variation between family members and providers involved in cleft care.26–28 Given the variation in degree of satisfaction with cleft-specific aspects of appearance among surgeons, parents, children, and the general populace, the process of incorporating the child’s perspective must be more clearly defined to align differing perceptions into unified treatment plans. Given the longitudinal nature of cleft care, surgeons/physicians must begin acknowledging the role of younger children in the decision-making process, as this may both empower and encourage compliance in this patient population.29 Allowing children to take part in decisions about reconstruction supports healthy psychosocial development for the child.30 Because specific plastic surgery interventions among children with craniofacial anomalies can improve psychosocial outcomes, we must educate patients accordingly.31,32 A large aspect of self-esteem and satisfaction arises from external perceptions formed from social construct, culture, and media definitions of normal and attractive. Changes in physical appearance following reconstructive procedures can alter the social perceptions of others and, in turn, may impact the psychosocial functioning of the patient.31,32 These concepts are important to our study, as our patient population including children and adolescents are markedly influenced by peers, their surroundings, and social and cultural norms. At our institution, we begin screening children for the desire to undergo revision surgery at 5 to 6 years of age, which coincides with the time point at which they begin school. The decision to perform cleft revision is based solely on the desire of the parent or child, regardless of whether or not the surgeon believes the patient will benefit from revision. This process is maintained throughout the patient’s care with the multidisciplinary cleft team. Counseling patients and families in a patient-centered manner for revision surgery is an area of ongoing research, to which this study adds areas of focus that may be most important to the patient.
According to the American Academy of Pediatrics, children as young as age 7 years are able to provide informed assent based on the process of normal growth and development.29 In our cohort, the average age was 10.5 years, with a significant proportion of patients between 6 and 10 years of age. Although it may be the case that children do not routinely understand the long-term consequences of their actions and need guidance from adults on many issues, the decision to undergo an elective operation for aesthetic concerns is perhaps one where the child’s perception is of critical importance. Although it may be more expedient for parents and surgeons to decide on timing and occurrence of reconstruction, it is important to ascertain the opinions of children and gain assent for the proposed procedure beginning at this age, particularly given that the children in our study expressed greater satisfaction with appearance than all other groups surveyed. Best practices for eliciting a child’s preferences in this regard have yet to be elucidated and are the aim of ongoing research. A future research study could assess how children view other peers in terms of need for surgical revision and compare children’s assessment of their peers to surgeon and caregiver assessment. Furthermore, assessing shared decision-making models and methods is an area of future study.
Although there may be differences in perceptions between patients, providers, caregivers, and the general populace, there are important limitations of this study to consider. As this is a single-center study at a large tertiary referral center, our results may not be generalizable to institutions with different approaches to cleft care. In addition, because of the cross-sectional nature of this study, it cannot be concluded that dissatisfaction with appearance and the extent of variation present between groups are what caused differences in the desire for cleft revision surgery. Finally, children may have varying levels of understanding regarding the potential impact that revision may have on their psychosocial development and satisfaction with appearance. This study does demonstrate, however, that differences in satisfaction with appearance are associated with measurable differences in clinical decision-making with regard to the desire for cleft revision surgery. Performing revision on children who are already satisfied imposes additional risks of anesthesia, increases scar tissue that may preclude future revision, and disempowers children during the course of cleft care. In this context, incorporating the patient’s and the family’s perspectives and motivations may empower children and afford caregivers the opportunity to play an active role in the process of determining when surgery may or may not be beneficial, increasing the likelihood of success for this otherwise elective operation.
Children, parents, surgeons, and control observers demonstrated varying degrees of satisfaction with cleft-specific aspects of appearance and function. Understanding the degree of this variation is critical during the discussion about cleft-revision surgery.
Parents or guardians provided written consent for use of patients’ images.
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