Treatment of Apert Hand Syndrome: Strategies for Achieving a Five-Digit Hand

Background: Apert hand reconstruction requires complex surgical planning. The purpose of this study was to describe the authors’ 8-year surgical experience with Apert syndrome hand reconstruction, and provide specific surgical strategies for achieving a five-digit hand in Upton type I and II hands. Methods: A retrospective analysis of consecutive Apert syndrome patients who underwent web-space releases between 2007 and 2015 was performed. Demographic, surgical, and outcome data were verified through medical records, clinical photographs, radiographic images, and patient interviews. Results: A total of 41 Apert syndrome patients [23 boys (56.1 percent) and 18 girls (43.9 percent)] have been treated at our hospital since 2007. A five-digit hand was achieved in all patients (100 percent) with Upton type I and II hands, and in eight patients (72.7 percent) with Upton type III hands. A four-digit hand was obtained in three of 11 patients (27.3 percent) with Upton type III hands. Four of 20 patients (25 percent) with Upton type I hands, three of 10 patients (30 percent) with Upton type II hands, and six of 11 patients (54.5 percent) with Upton type III hands required subsequent revision for aesthetic reasons. Conclusions: Upton type III hands have demonstrated higher revision rates than type I and II hands, regardless of whether a four- or five-digit hand is obtained. Treatment strategies for Apert syndrome hands based on hand type are offered to guide four–web-space release in all patients with Upton type I and II hands. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.