Variations in Apert Hand Treatment Outcomes by Upton Classification
Niki K Patel, MSc1; Anchith Kota, BA2; Zoe E. Belardo, BA2; Emily M. Graham, BSN3; Apurva S. Shah, MD MBA4; Shaun D. Mendenhall, MD2; Benjamin Chang, MD5
1University of Mississippi Medical Center, Jackson, MS; 2The Children's Hospital of Philadelphia, Philadelphia, PA; 3University of Utah, Salt Lake City, UT; 4Children's Hospital of Philadelphia, Philadelphia, PA; 5University of Pennsylvania, Philadelphia, PA
Introduction: Apert syndrome is characterized by craniosynostosis and varying presentations of syndactyly, necessitating distinct surgical interventions and resulting in various complications. Nonetheless, previous literature lacks thorough evaluation of such differences by hand type. Utilizing the Upton classification scheme, this study sought to better characterize the variations in intervention and outcomes of Apert syndromic hands.
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 at presentation with upper extremity syndactyly were included and analyzed by individual webspace. Variables of interest included hand type, age at presentation, commissure flap type, early complications (unplanned cast/dressing change, infection, dehiscence, etc.), and late complications (web creep, flexion contracture, angulation deformity, etc.). Frequencies, proportions, Chi-square tests, ANOVA, and multinomial logistic regressions were used to analyze findings.
Results: The cohort consisted of 106 webspace releases from 17 patients of mostly White and non-Hispanic backgrounds. Average ages at presentation and surgery were 10.2 months and 15 months, respectively, and mean follow-up length was 3 years and 2.5 months. Predictors of late presentation included Type II hands (p=0.018), non-White race (p=0.001), high Area Deprivation Index (p=0.008), and decreased complexity (p=0.001). The majority of the webspace releases were performed with volar and dorsal triangular flaps for the commissure, and straight-line incisions for the fingers with full thickness skin grafting. Type II hands were more likely to have early post-operative complications than Type III hands (26.8% and 3.7%, p=0.014), with no difference in late complications. Long-term complications included increased hypertrophic scarring in Type II hands (13.33%, p<0.05) only. Type III hands had higher rates of ROM deficits (73.3%) than all other hand types and flexion contracture (70%) than Type II hands (p=0.004 and p=0.006, respectively). Type I hands had no late complications. No other differences in complications were identified between hand types or flap shapes.
Conclusions: Significant distinctions in both clinical intervention and post-operative outcomes exist by hand types. Hand type and level of complexity served as prognostic indicators of age at initial presentation. Higher rates of complications were found early in Type II hands and late in Type III hands. Therefore, our findings suggest that Apert syndromic hand type can be predictive of early versus late post-operative findings.
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