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.