Apert Hand Syndactyly Presentation and Management: An Analysis of Demographic and Socioeconomic Factors in 106 Webspace Releases
Anchith Kota, BA1; Emily M. Graham, BSN2; Zoe E. Belardo, BA1; Niki K Patel, MSc1,3; Apurva S. Shah, MD MBA4; Benjamin Chang, MD1,5; Shaun D. Mendenhall, MD1
1The Children's Hospital of Philadelphia, Philadelphia, PA; 2University of Utah, Salt Lake City, UT; 3University of Mississippi Medical Center, Jackson, MS; 4Children's Hospital of Philadelphia, Philadelphia, PA; 5University of Pennsylvania Perelman School of Medicine, Philadelphia, PA
Introduction: Apert syndrome is a rare congenital disorder characterized by the presence of both craniosynostosis and syndactyly. While clinically well-described, its rarity has precluded the investigation of socioeconomic and demographic dynamics in cases of Apert syndrome, particularly with regards to disparities in Upton hand type presentation. We hypothesize Apert syndrome patients who identify as minorities and/or are parts of disadvantaged socioeconomic groups are associated with delayed care, increased initial presentation severity, and more frequent post-operative complications.
Methods: After IRB approval, a retrospective analysis identified patients diagnosed with Apert syndrome from 2007-2021 at a large children’s hospital. Patients 0-17 years of age with upper extremity syndactyly at time of presentation were included. Outcomes of interest including age at presentation, hand type distribution, early complications (infection, dehiscence, etc.), and late complications (web creep, flexion contracture, angulation deformity, etc.) stratified by race/ethnicity and Area Deprivation Index (ADI). Frequencies, proportions, Chi-square tests, Mann Whitney U tests, and multinomial logistic regressions were used to analyze findings.
Results: A total of 106 webspace releases from 17 patients were included. White and non-Hispanic patients represented the majority of the cohort. Average ages at presentation and surgery were 10.2 and 15 months, respectively, and overall follow-up length was 3 years and 2.5 months. With respect to race/ethnicity, White patients presented 2 years earlier and non-Hispanic patients 2.2 years earlier than non-White and Hispanic counterparts (p<0.001 for both). Type III hands, the most severe presentation, were proportionally greater in Black and non-Hispanic patients (p<0.001). On regression analysis, higher ADI, indicating greater socioeconomic disadvantage, was more predictive of Type III hands (p=0.002), but predicted no differences between Type I and II hands. Among complication rates, angulation deformity was more common in White populations and scar contracture in Hispanic populations (p=0.018 and p=0.01, respectively). However, socioeconomic status, race/ethnicity, and sex were not predictive of early complications.
Conclusion: Our study highlights the significance in racial/ethnic and socioeconomic differences among Apert patients from initial presentation to post-operative outcomes. Black and Hispanic patients presented at later ages with more severe initial presentations compared to their counterparts. Similarly, low socioeconomic status was prognostic for severe presentations. Few post-operative complications were found to be significantly different among racial/ethnic groups. Our findings, therefore, suggest that while treatment outcomes are largely independent of socioeconomic status or demographics, access to care and presentation severity are both significantly stratified.
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