This case-control study investigates the association between demographic and anatomic features of pediatric patients with cleft lip and the development of scars following surgical repair.
Key Points
Question
What pediatric patient demographic factors and anatomic scar features are associated with the overall cleft lip scar outcome after surgical repair?
Findings
In this case-control study of 58 pediatric patients who underwent surgical repair of cleft lip, black patients were observed to have worse overall scar outcomes than white patients, and a depressed scar height was significantly associated with overall scar appearance at both 6 and 12 months after surgical repair.
Meaning
This study’s findings suggest that black pediatric patients experience worse scar outcomes after surgical repair of cleft lip.
Abstract
Importance
Scar outcomes following cleft lip repair are an important component of pediatric patient and family satisfaction and indicate the need for future surgical interventions.
Objective
To assess the association of pediatric patient demographic factors and scar anatomic features with scar outcomes following cleft lip surgical repair.
Design, Setting, and Participants
A case-control study was conducted involving 58 pediatric patients who underwent surgical repair of a cleft lip from October 31, 2008, to August 4, 2016, at a tertiary care pediatric specialty hospital. Data on patient demographic factors, cleft type, and the surgical technique used were collected and analyzed from June 11, 2009, to November 21, 2017. Scar outcomes were subjectively rated by 3 physicians at 6-month and 12-month postoperative intervals.
Main Outcomes and Measures
Overall scar outcomes at 6-month and 12-month postoperative intervals were based on rating of scar appearance, color, width, height, and alignment by using a subjective, 5-point scar-assessment scale in which 1 indicated the poorest aesthetic appearance and 5, the ideal aesthetic appearance.
Results
A total of 58 pediatric patients who underwent cleft lip repair were evaluated; mean (SD) age at time of repair, 4.8 (3.0) months. Of these, 44 (76%) were male and 14 (24%) were female, 37 (64%) were white, 11 (19%) were black, 7 (12%) were Hispanic, 2 (3%) were Asian, and 1 (2%) was of another race/ethnicity. Scores on the Cohen κ interrater test indicated either a substantial or almost perfect strength of agreement among the physicians grading the scar outcomes. At 12 months, patients with black skin type had worse overall scar outcomes than patients with white skin type (odds ratio [OR], −0.31; 95% CI, −1.15 to −0.14; P = .03). A depressed scar height (OR, −0.54; 95% CI, −1.32 to −0.49; P < .001), and hypopigmented scar color (OR, −0.45; 95% CI, −1.34 to −0.32; P = .002) were associated with worse scar outcomes at 12 months following surgery. The overall median lip scar outcome significantly improved between the 6-month and 12-month follow-up assessments (scar-assessment scale score, 3.3; interquartile range [IQR], 2.7-4.0 vs 4.0; IQR, 3.3-4.3; P < .001). No association was observed between the anatomic type and severity of the cleft lip and scar outcomes (unilateral vs bilateral cleft, complete vs incomplete or microform cleft, and lip height ratio of the unilateral noncleft to cleft lip).
Conclusions and Relevance
This study’s findings suggest that, compared with white pediatric patients, black pediatric patients exhibited worse overall scar outcomes. A depressed scar and a hypopigmented scar also were associated with overall worse scar appearance after surgical repair. Cleft lip scar outcomes were not significantly associated with the type and severity of the cleft lip.
Introduction
Cleft lip with or without cleft palate is one of the most common congenital defects in newborns requiring surgical intervention, with an incidence of approximately 1 in 1000 births.1 The primary goals of cleft lip repair are to reestablish form and function of the lip. A natural aesthetic appearance of the lip can be achieved by creating an appropriate lip length, limiting transgression of anatomic boundaries, and establishing a symmetric Cupid’s bow width.2 These goals ideally are achieved at the time of the primary surgery; however, many pediatric patients with cleft lip require revision procedures. An unfavorable lip scar may account for the need to undergo a revision surgical intervention; therefore, it is critical to optimize scar outcomes during the primary surgery.
Specific scar features that are associated with an unfavorable outcome include color mismatch, increased thickness, firmness, widening, contraction, and contracture.3 When present, these features may lead to a diagnosis of scar hypertrophy, which is characterized by redness, elevation, widening, and stiffness without extension of the scar beyond the boundaries of the original incision site.4 Cleft lip scar hypertrophy typically develops within 3 to 6 months after the surgical intervention5; it is reported that between 8% and 47% of pediatric patients with cleft lip develop this condition.5,6,7,8 The scar outcome is secondary to the use of multiple surgical techniques, patients’ demographic and anatomic features, and environmental factors. The method of repair, intraoperative tissue handling, type of suture material used, postoperative lip tension, wound care, and genetic predisposition9 have the potential to be associated with the scar outcome. Unfavorable cleft lip scar features may have functional, aesthetic, and psychological consequences; therefore, their presence often necessitates revision surgical intervention.
The variables associated with the development of an unfavorable cleft lip scar are incompletely understood. Soltani et al5 reviewed the results from 180 cleft lip surgical interventions. The overall rate of hypertrophic lip scarring was 25% and ethnicity was found to be the only independent factor associated with a hypertrophic result. White pediatric patients had the lowest rate of hypertrophic scarring (11.8%), while Hispanic (32.2%) and Asian (36.3%) pediatric patients had the highest.
Thus, the objective of the present study was to further investigate the association of patient demographic factors and scar anatomic features with the development of unfavorable cleft lip scars after surgical repair.
Methods
Institutional medical records were used to identify pediatric patients who underwent surgical repair of cleft lip between October 2008 and August 2016, performed by one of 2 surgeons (C.M.D. or K.G.P.). Either a Millard10 or Fisher11 cleft lip surgical repair technique was used. Patients with at least 6 months of follow-up and with front-view photographs taken at 2 separate postoperative clinical encounters were included in the study. Postoperative appointments are typically completed at 6-month and 12-month intervals. Race/ethnicity was categorized per the US Census Bureau’s protocol based on the 1997 Office of Management and Budget standards on race and ethnicity.12 Accordingly, patient race/ethnicity is categorized as white, black, Asian, or Hispanic. Three reviewers (A.P.M., M.S.C., and A.W.M.) assessed the postoperative photographs to minimize potential measuring errors of a single observer and to obtain good reliability.3 A subjective scar-assessment scale was designed to produce a reliable and accurate measure of cleft lip scar outcomes.13 Scars were graded for overall aesthetic appearance on a 5-point scale, with 1 representing the poorest appearance and 5, the ideal aesthetic appearance. Independent scar features were assessed by each reviewer for the following characteristics: scar color (normal pigmentation vs hypopigmentation vs hyperpigmentation), height (flush with the surrounding skin vs depressed vs raised), width (thin vs wide), and alignment (aligned vs malaligned). Examples of cleft lip scar features are shown in Figure 1 and Figure 2. The scar analysis pertained only to the lip scar and did not incorporate the nasal anatomy into the scoring system. We did not validate the scar scoring system devised for this study. The institutional review board at the Medical University of South Carolina approved the study design and protocol, and written informed consent was obtained from the families of all patients.
Figure 1. Cleft Lip Scar Height and Color Features.
A, B, and C, Cleft lip scar height features. D, E, and F, Cleft lip scar color features.
Figure 2. Cleft Lip Scar Alignment and Width Features.
A and B, Cleft lip scar alignment features. C and D, Cleft lip scar width features.
The cleft lip surgical repairs were completed between October 31, 2008, and August 4, 2016. The data were analyzed from June 11, 2009, to November 21, 2017, using statistical analysis software (SPSS, version 25; IBM Corporation and MedCalc, version 12.0.2.0; MedCalc Software). The Cohen κ correlation coefficient was calculated to measure interrater agreement between the 3 independent raters. The following scale was used for the categories of reference values for the κ coefficient: less than 0, no better than chance; 0.01 to 0.20, poor; 0.21 to 0.40, fair; 0.41 to 0.60, moderate; 0.61-0.8, substantial; and 0.81 to 1.00, almost perfect. A multivariate logistic regression analysis was performed to assess the association of the following variables with the overall cleft lip scar outcomes: sex, race/ethnicity, laterality of the cleft lip, completeness of the cleft, ratio of the unilateral noncleft lip to the cleft lip height, preoperative use of nasoalveolar molding, skin adhesive vs placement of skin sutures, and age at the time of repair. The scar features (color, height, width, and alignment) were not graded on a continuous scale (eg, width was categorized as either thin or wide). Therefore, the scar feature ratings ascribed by one of the investigators (A.P.M.) were used for the statistical analysis. A multivariate logistic regression analysis was used to evaluate the association of the individual scar features with the 6-month and 12-month scar-assessment scale scores. For all multivariate logistic regression analyses, the 3 reviewers’ overall scar-assessment scale scores were averaged. Finally, the median and 95% CIs of the patients’ overall scar-assessment scale scores, averaged for all 3 raters, were compared at 6-month and 12-month intervals using the Mann-Whitney rank sum test.
Results
Fifty-eight pediatric patients with cleft lip underwent surgical repair and met the inclusion criteria. Of these, 44 (76%) were male, and 14 (24%) were female, 37 (64%) were white, 11 (19%) were black, 7 (12%) were Hispanic, 2 (3%) were Asian, and 1 (2%) was of another race/ethnicity. Additional patient demographic data are given in Table 1. The Cohen κ coefficient test results showed a substantial to almost perfect strength of agreement for both the 6-month and 12-month independent cleft lip scar data (Table 2). At 6 months, the Cohen κ coefficients ranged from 0.64 (95% CI, 0.44-0.78) to 0.89 (95% CI, 0.82-0.93); at 12 months, coefficients ranged from 0.66 (95% CI, 0.46-0.79) to 0.87 (95% CI, 0.80-0.92). The multivariate regression analysis pertaining to the association of patients’ demographic and scar anatomic variables with the overall lip scar outcome showed that, at 12 months, being of black vs white race was significantly associated with a worse scar outcome (OR, −0.31; 95% CI, −1.15 to 0.14; P = .03) (Table 3). No significant association was found between Hispanic and white race/ethnicity with regard to the overall cleft scar-assessment scale scores. Sex, laterality of the cleft lip, completeness of the cleft, ratio of the unilateral noncleft lip to the cleft lip height, preoperative use of nasoalveolar molding, placement of skin adhesive vs skin sutures, and age at the time of repair were not significantly associated with the overall cleft lip scar outcome. The multivariate linear regression analysis of the association of the individual scar features (color, height, alignment, and width) with the overall scar outcome suggested a significant association for scar height, alignment, and width at the 6-month interval and for scar height and color at the 12-month interval. The strongest associations at the 6-month postoperative interval were for height (OR, −0.54; 95% CI, −1.21 to −0.49; P < .001) followed by alignment (OR, −0.47; 95% CI, −1.18 to −0.37; P < .001) and width (OR, −0.36; 95% CI, −0.90 to −0.16; P = .006). These results indicate a statistically worse scar outcome to be associated with a depressed, malaligned, and wide scar. At the 12-month interval, depressed scar height (OR, −0.54; 95% CI, −1.32 to −0.49; P < .001) again was associated with worse scar outcomes and had the strongest association of any of the 4 scar features with worse scar outcomes, followed by hypopigmented scar color (OR, −0.45; 95% CI, −1.34 to −0.32; P = .002). The overall median scar outcome significantly improved between the 6-month and 12-month follow-up intervals (scar-assessment scale score, 3.3; interquartile range [IQR], 2.7-4.0 vs 4.0; IQR, 3.3-4.3; P < .001).
Table 1. Demographic Data.
| Variable | Patients, No. (%) |
|---|---|
| Sex | |
| Male | 44 (76) |
| Female | 14 (24) |
| Race/ethnicity | |
| White | 37 (64) |
| Black | 11 (19) |
| Hispanic | 7 (12) |
| Asian | 2 (3) |
| Other | 1 (2) |
| Cleft type | |
| Unilateral | 45 (78) |
| Bilateral | 13 (22) |
| Cleft severity | |
| Complete | 35 (60) |
| Incomplete or microform | 23 (40) |
| Nasoalveolar molding | 5 (9) |
| Lip taping | 13 (22) |
| Age at time of repair, mean (SD), mo | 4.8 (2.9) |
Table 2. Cohen κ Coefficient Analysis.
| Scar Feature | κ Coefficient (95% CI)a | |
|---|---|---|
| 6-mo Interval | 12-mo Interval | |
| Overall | 0.89 (0.82-0.93) | 0.87 (0.80-0.92) |
| Width | 0.77 (0.63-0.85) | 0.77 (0.63-0.86) |
| Alignment | 0.76 (0.62-0.85) | 0.68 (0.49-0.80) |
| Color | 0.64 (0.44-0.78) | 0.73 (0.58-0.84) |
| Height | 0.74 (0.60-0.84) | 0.66 (0.46-0.79) |
See the Methods section for an explanation of the scale.
Table 3. Multivariate Regression Analysis for Association of Demographic and Anatomic Cleft Variables With Overall Scar Outcome at 12 Months.
| Characteristic | Odds Ratio (95% CI) | P Value |
|---|---|---|
| Male vs female sex | −0.20 (–0.82 to 0.14) | .16 |
| Race/ethnicity | ||
| White vs black | −0.31 (−1.15 to 0.14) | .03 |
| White vs Hispanic | 0.10 (–0.56 to 0.98) | .47 |
| Age at the time of repair, ≤4.8 vs >4.8 mo | −0.19 (–0.78 to 0.16) | .19 |
| Type of cleft | ||
| Unilateral vs bilateral | 0.13 (–0.28 to 0.77) | .35 |
| Incomplete or microform vs complete | −0.20 (–0.12 to 0.76) | .15 |
| Ratio of unilateral noncleft to cleft lip height, ≤1.4 vs >1.4 | 0.14 (–0.41 to 0.81) |
.51 |
| Preoperative use of NAM, no vs yes | 0.05 (–0.54 to 0.66) | .75 |
Abbreviation: NAM, nasoalveolar molding.
Discussion
The results of the present study suggest that, compared with white pediatric patients, black pediatric patients were at an increased risk of developing a worse overall aesthetic lip scar outcome at 12 months following surgery. No significant difference was observed in the overall scar outcome between Hispanic and white patients, and the study was underpowered to compare Asian patients with white patients. Soltani et al5 assessed cleft lip scar outcomes in 180 patients and found that Hispanic and Asian populations were at an elevated risk of developing scar hypertrophy. However, they were unable to statistically analyze the risk of scar hypertrophy in black patients owing to the small sample size. The varying conclusions regarding the associations of race/ethnicity with cleft lip scar outcome between the study by Soltani et al5 and the present study likely reflect the differences in patient demographics resulting in statistically underpowered analyses. However, there is a reported trend of an increased risk of scar hypertrophy in nonwhite patients based on studies in burn-related literature. Bombaro et al14 investigated hypertrophic scarring secondary to thermal injuries that either healed spontaneously or underwent skin grafting and found an incidence of 75% in nonwhite and 60% in white patients. Furthermore, Deitch et al15 investigated hypertrophic scarring following spontaneous healing of thermal injuries in pediatric patients younger than 14 years and found that black patients were at an increased risk of developing scar hypertrophy compared with white patients (31% vs 13%). The mechanism for increased hypertrophic scar incidence in nonwhite patients is incompletely understood. However, possible contributing genetic factors include inefficient downregulation of type 1 collagen synthesis16 and an increased sensitivity of melanocytes to the effects of pituitary-derived hormones.17
Substantial variability exists among the severity of cleft lip deformities and the available tissue for surgical transfer and rearrangement.18 This present study hypothesized that the type and severity of the cleft lip would be associated with the cleft lip scar outcome. However, the variables unilateral vs bilateral cleft, complete vs incomplete cleft, and ratio of the unilateral noncleft lip height to the cleft lip height were not associated with the overall aesthetic lip scar outcome. Consistent with the results of this study, Frans et al19 reported in 2012 that there was no difference in subjective scar outcomes between unilateral and bilateral cleft lip. The present analysis did reveal a statistical correlation between scar height and color and the overall scar outcome at the 12-month postoperative interval. Among the scar features evaluated, a depressed scar height was found to be consistently significantly associated with scar outcomes after surgical repair. These findings underscore the challenges of achieving optimal cleft lip scar outcomes based on consideration of cleft type and severity. However, the findings do suggest that the height of the lip scar (flush vs depressed vs raised) is the feature that contributes most to an unfavorable overall scar outcome compared with scar color, alignment, and width.
The results of this study suggest that the median overall appearance of the repaired lip scar statistically improved between the 6-month and 12-month postoperative assessments (scar-assessment scale score, 3.3 vs 4.0; P < .001). Although it is important to intervene in a timely manner when unfavorable or hypertrophic lip scars develop, the results of this study suggest that patience should be used when deciding to surgically revise the scar. A multitude of additional noninvasive treatments are also reported, including cutaneous laser therapy20 and botulinum toxin injections.21 In select patients, these conservative measures may be sufficient to achieve an acceptable lip result; however, some patients will eventually require a revision surgical intervention. In our experience, a revision cleft lip surgery is delayed until at least 4 years following the primary cleft lip repair. Previous reports have provided evidence indicating the significant association of poor scar outcomes with negative social interactions and poor self-esteem.22 Therefore, surgical scar revision should be discussed with patients and families if indicated and performed prior to patients’ matriculating into secondary school.
To minimize risk of poor scarring, the skin and soft tissue are carefully handled, skin edges are meticulously everted, and tension is minimized during surgery. The present study collected data on the use of topical skin adhesive vs suture for skin closure. However, nearly all patients had topical skin adhesive placed, and therefore, the group sizes were insufficient to be compared statistically. Evidence in the literature suggests that topical skin adhesive and suture closure of the skin lead to comparable scar outcomes and the risk of hypertrophic scarring.18 Postoperative scar minimization care such as application of a topical silicone-based product23 and massage are recommended. Patients are also closely monitored by the surgical team to allow for timely scar repair intervention, if necessary. Use of fluorouracil/steroid injection is considered a safe and effective technique for treatment of hypertrophic cleft lip scarring.24,25,26,27 In the clinical practice of the senior investigator (K.G.P.), these injections are used on the hypertrophic lip scars as early as 4 weeks after surgery when indicated. Of the 58 pediatric patients in the present study, 1 was injected with fluorouracil and another, with a combination of fluorouracil and triamcinolone acetonide for treatment of keloid formation at the cleft lip scar site.
Limitations
The small patient cohort, age at the time points chosen for follow-up evaluation, and use of a nonvalidated scar-assessment scale are limiting factors of this study. The limitations of a subjective scar-assessment scale used by the surgical team include the omission of patient and family perception and heterogeneity among the physicians grading the scars. The grading system used in this study is unvalidated and, therefore, less scientifically precise. A validated scar-assessment scale could not be used because no existing scale had all the scar features that were being evaluated in this study. However, the Cohen κ coefficient test results showed a substantial to almost perfect strength of agreement between the physician reviewers. Only front-view photographs were available to evaluate. A more thorough lip scar analysis would include multiple photographic vantage points; however, obtaining multiple images of different views is challenging in the infant population. Finally, the demographics of the geographic catchment area of our hospital system limited a complete analysis on the association of skin color with lip scar outcomes. Black and white patients were well represented, but only small numbers of Asian and Hispanic patients were included.
Conclusions
The results of this retrospective cleft lip scar analysis suggest that black compared with white pediatric patients had a worse overall aesthetic lip scar outcome at the 12-month interval following surgery. Of the individual scar features assessed, height and color had a significant association with the overall scar outcome at the 12-month interval, with a depressed scar height being the most predictive factor. No association was observed between age, sex, or the anatomic cleft variables (unilateral vs bilateral cleft, complete vs incomplete cleft, and lip height ratio of the unilateral noncleft to cleft lip height) and the overall lip scar outcome. Further investigation with a larger patient cohort and longer follow-up is necessary to more completely understand the results of the present study.
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