Outcomes of Syndactyly Release in the Management of Apert Syndrome
John A. Tipps, BA1; Anchith Kota, BA1; Niki K Patel, MSc2; Emily M. Graham, BSN3; Zoe E. Belardo, BA2; Benjamin Chang, MD4; Shaun D. Mendenhall, MD2; Apurva S. Shah, MD MBA1
1Children's Hospital of Philadelphia, Philadelphia, PA; 2The Children's Hospital of Philadelphia, Philadelphia, PA; 3University of Utah, Salt Lake City, UT; 4University of Pennsylvania, Philadelphia, PA
Introduction: Apert syndrome is characterized by a combination of craniosynostosis and varying degrees of syndactyly. Timing of clinical treatment and management of Apert syndactyly continues to be debated in the literature, and a thorough understanding of Apert-associated complications is yet to be described. Building on previous literature, this study aims to provide a detailed investigation of complications associated with Apert syndactyly treatment, as well as stratify complications by modality and timing of treatment.
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 included early complications (unplanned cast/dressing change, infection, dehiscence, etc.) and late complications (web creep, flexion contracture, angulation deformity, etc.). Frequencies, proportions, Chi-square tests, and multinomial logistic regressions were used to analyze findings.
Results: A total of 106 webspace releases from 17 patients were included with majority representation being White and non-Hispanic. 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. Among the early post-operative complications, 14.2% consisted of either unplanned cast/dressing changes, delayed wound healing, or graft failure and 0.9% with infection. Late complications were more prevalent, including 53.8% of patients with ROM deficits, 46.2% flexion contracture, 18.9% angulation deformity, and 13.3% with web creep. Additionally, graft donor site demonstrated significant association with web creep rates (4.76% groin, 14.7% lower abdomen, p=0.031), while rectangular flap compared to triangular flap usage in the dorsal commissure was correlated with an increase in late complications such as scarring and web creep (p<0.001). Furthermore, age at initial presentation and surgery did not influence early or late complications.
Conclusion: Early post-operative complications are typically limited and well-managed, with the majority occurring secondary to cast/dressing placement rather than incision technique. In contrast, late complications remain persistent and include ROM deficits, scar/flexion contracture, and angulation deformities. Variations in clinical intervention by flap shape (triangular vs. rectangular) and graft site (groin vs. abdominal) are associated with few complications aside from web creep and scar formation. Timing of surgical intervention played no role in surgical outcomes. Our findings, therefore, suggest late complications are more prevalent in the treatment of Apert hands; however, the burden of early complications should not be ignored.
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