Background: Patients with Treacher Collins syndrome (TCS) and attendant airway dysmorphology may be predisposed to airway complications in the perioperative period. However, limited data correlates severity of mandibular hypoplasia and airway status. This study aims to improve risk stratification for perioperative airway insufficiency in TCS by using a previously proposed mandibular severity index. Methods: Patient demographics, perioperative airway status, difficulty of intubation, and Cormack Lehane grade were collected and compared using a TCS mandibular hypoplasia severity grading scale in patients with TCS treated between 2000 and 2022. Results: Twenty-six patients underwent 222 procedures with institutional mandibular severity gradings as follows: 23% Grade I, 31% Grade II, 39% Grade III, 8% Grade IV. Our severity index was associated with intubation difficulty ( P <0.001) and difficult airway status ( P <0.001), with 72% of difficult airways found in grade III and grade IV patients. Mandibular retrusion and ramal hypoplasia subscores were positively correlated with measures of airway severity ( P <0.001), whereas the gonial angle was negatively correlated ( P <0.001). Age was negatively correlated with difficult visualization for endotracheal intubation ( P =0.02) but had no association with difficult airway status ( P =0.2). Conclusions: This study found a positive correlation between severity of maxillomandibular dysmorphology and perioperative airway difficulty in TCS patients. Our findings suggest that severely affected patients require heightened vigilance throughout life, as difficult airways may not completely resolve with aging. Given the risk of morbidity and mortality associated with airway complications, proper identification and preparation for challenging airways is critical for TCS patients.
The “scarred villain” trope, where facial differences like scars signify moral corruption, is ubiquitous in film (e.g., Batman’s The Joker). Strides by advocacy groups to undermine the trope, however, suggest cinematic representations of facial differences could be improving with time. This preregistered study characterized facial differences in film across cultures (US vs. India) and time (US: 1980-2019, India: 2000-2019). Top-grossing films by country and decade were screened for characters with facial differences. We found that the scarred villain trope has actually worsened with time, although in tandem with progress in also representing non-villainous characters with facial anomalies. Country of origin did not predict the presence of facial differences in villains or heroes. “Action” and “fantasy” movies were the most likely genres to depict villains with facial differences. Finally, villains’ facial differences crossed more facial subunits and were more likely to involve lips, chin, and mandible than when present in heroes. Our findings underscore the need for critical reflection on the role of cultural practices—even when seemingly innocuous—in shaping and maintaining negative biases against already stigmatized groups.
Objective Characterize the interventions and assistance employed by a cleft nurse navigator (CNN) which have mediated improvement in care equity at our institution. Design Retrospective study. Setting Academic tertiary care center. Patients, Participants Patients presenting with cleft lip and/or cleft palate presenting between August 2020 and August 2021 with exclusions for syndromic diagnosis, Pierre-Robin sequence, late (> 6 months) presentation, and prior cleft surgery at outside institutions. Interventions Multidisciplinary cleft nurse navigator program Main Outcome Measure(s) Family interactions with the CNN by phone, text, and email across the first year of life including feeding support, nasoalveolar molding (NAM) assistance, appointment scheduling, financial assistance, addressing perioperative concerns, and facilitating physician consults. Patient weight and surgical timing were also recorded. Results Sixty-nine patients were included with a total of 639 interactions between the CNN and families. Scheduling support (30%), addressing perioperative concerns (22%), and feeding support (20%) were the most common interactions. Feeding support and NAM assistance were heavily distributed in the first 3 months of life compared to after 3 months ( P < .001). Median age at first contact was 1 week (range: 22 weeks gestation-14 weeks). There was no difference in the proportion of families receiving feeding support, NAM assistance, or scheduling assistance based on insurance status or race ( P > .05 for all). Conclusions Scheduling assistance, addressing perioperative concerns, and feeding support are the predominant methods by which the CNN interacts with and assists families of patients with cleft conditions. CNN service distribution is largely equitable between demographic groups.
Background: Facial proportionality and symmetry are positively associated with perceived levels of facial attractiveness.Objective: The aims of this study were to confirm and extend the association of proportionality with perceived levels of attractiveness and character traits and determine differences in attractiveness and character ratings between "anomalous" and "typical" faces using a large dataset.Methods: Ratings of 597 unique individuals from the Chicago Face Database were used. A formula was developed as a proxy of relative horizontal proportionality, where a proportionality score of "0" indicated perfect proportionality and more negative scores indicated less proportionality. Faces were categorized as "anomalous" or "typical" by 2 independent reviewers based on physical features.Results: Across the ratings for all faces, Spearman correlations revealed greater proportionality was associated with attractiveness ( ρ = 0.292, P < 0.001) and trustworthiness ( ρ = 0.193, P < 0.001), while lesser proportionality was associated with impressions of anger (ρ = 0.132, P = 0.001), dominance (ρ = 0.259, P < 0.001), and threateningness ( ρ = 0.234, P < 0.001). Mann-Whitney U tests revealed the typical cohort had significantly higher levels of proportionality (-13.98 versus -15.14, P = 0.030) and ratings of attractiveness (3.39 versus 2.99, P < 0.001) and trustworthiness (3.48 versus 3.35, P < 0.001).Conclusions: This study demonstrated that facial proportionality is not only significantly associated with higher ratings of attractiveness, but also associated with judgements of trustworthiness. Proportionality plays a role in evoking negative attributions of personality characteristics to people with facial anomalies.
Objective Determine interactions between geospatial and socioeconomic factors influencing cleft lip and/or cleft palate (CL/P) management and outcomes. Design Retrospective review and outcomes analysis (n = 740). Setting Urban academic tertiary care center. Patients 740 patients undergoing primary (CL/P) surgery from 2009 to 2019. Main Outcomes Measures Prenatal evaluation by plastic surgery, nasoalveolar molding, cleft lip adhesion, and age at CL/P surgery. Results Prenatal evaluation by plastic surgery was predicted by the interaction between higher patient median block group income and shorter patient distance from the care center (OR = 1.07, p = 0.022). Nasoalveolar molding was also predicted by the interaction between higher patient median block group income and shorter distance from the care center (OR = 1.28, p = 0.016), whereas cleft lip adhesion was predicted by higher patient median block group income alone (OR = 0.41, p < 0.001). Lower patient median block group income predicted later age at cleft lip (β = −67.25, p = 0.011) and cleft palate (β = −46.35, p = 0.050) repair surgery. Conclusions Distance from the care center and lower median income by block group interacted to significantly predict prenatal evaluation by plastic surgery and nasoalveolar molding for patients with CL/P at a large, urban, tertiary care center. Patients living farthest from the care center who received prenatal evaluation by plastic surgery or who underwent nasoalveolar molding had higher median block group income. Future work will determine mechanisms perpetuating these barriers to care.
Advancements in artificial intelligence and the development of shape models that quantify normal head shape and facial morphology provide frameworks by which the outcomes of craniofacial surgery can be compared. In this work, we will demonstrate the use of the Swap Disentangled Variational Autoencoder (SD-VAE) to objectively assess changes following midfacial surgery.
Abstract Introduction In an effort to maximize benefit and minimize morbidity when performing fronto-orbital distraction osteogenesis (FODO) for unilateral coronal synostosis (UCS), we have transitioned to an endoscopic-assisted approach (“endo-FODO”). This study compares photogrammetric outcomes of patients who underwent FODO via an endoscopic-assisted versus open approach. Methods We retrospectively reviewed patients treated for UCS from 2013 to 2023. Photogrammetric outcomes at one to three years postoperatively were compared between patients who underwent endo-FODO and age- and sex-matched controls who underwent open FODO. Differences between pre- and postoperative periorbital symmetry ratios, canthal tilt symmetry, and orbital dystopia angle (ODA) were calculated. Results Twenty patients (ten per group) underwent surgery at a mean age of 6.1 ± 1.8 and 5.4 ± 1.1 months ( p = 0.426) and were photographed at 1.6 ± 0.9 and 1.8 ± 0.9 years ( p = 0.597) postoperatively in the endo-FODO and open FODO groups, respectively. Patients who underwent endo-FODO demonstrated significant improvements in margin-reflex distance 1 (MRD1) symmetry ratio ( p = 0.004), palpebral height symmetry ratio ( p = 0.004), canthal tilt symmetry ( p = 0.020), and ODA ( p = 0.009). Patients who underwent open FODO likewise demonstrated significant improvements in MRD1 symmetry ratio ( p = 0.004), palpebral height symmetry ratio ( p = 0.033), and ODA ( p = 0.004). All postoperative measurements as well as degrees of improvement were similar between groups ( p > 0.05). Conclusions Endo- and open FODO were associated with significant and comparable improvements in soft tissue periorbital symmetry and orbital dystopia at nearly two years postoperatively. While continued follow-up until cranial maturity is needed to assess the durability of aesthetic results, these data support a minimally invasive, endoscopic alternative to fronto-orbital distraction.
Background: The CLEFT-Q is a validated instrument designed to elicit patient-reported outcomes among people affected by cleft lip and/or palate. However, it has not been reported how use of CLEFT-Q data alters routine cleft care. This study analyzed the impact of CLEFT-Q data integration on patient care and clinical decision-making. Methods: Patients were sequentially, prospectively evaluated during scheduled cleft team visits. The CLEFT-Q was completed before the clinic encounter, but results were initially masked from the surgeon and family. In the encounter, a study observer characterized patients’ verbalized attitudes across 7 specific domains of appearance and function, and the provisional assessment and plan was noted. CLEFT-Q data were then introduced into the clinical encounter and discussed. Discordance between patients’ initially verbalized attitudes and their self-reported scores on the CLEFT-Q was documented along with any resultant modifications to their care plan. Results: Seventy patient visits were observed; the mean patient age was 12.7 years (range, 8 to 19 years). Forty-one patients (59%) had cleft lip and palate/alveolus and 29 (41%) had isolated cleft palate. Discordance was observed in 36% of visits and in 9.2% of specific domains assessed. Highest discordance rates were observed in domains of psychosocial function (12.5%), speech function/distress (11.6%), and lips/lip scar appearance (11.6%). No age group or sex was associated with increased discordance. Integration of CLEFT-Q results altered the assessment and plan in 11 visits (16%). Conclusion: The CLEFT-Q provides clinically relevant insight into patient perspectives that are not captured by routine interview and examination alone, and regularly leads to a change in the management plan.
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