Surgical treatment results of hand deformities in patients with Apert syndrome

Abstract

Objectives: Hand deformities in Apert sydrome are complex pathologies and usually consist of complex syndactyly with distal bony fusion in the 2., 3. and 4th fingers, simple syndactyly in the 5th finger, and short thumb, radial clinodactily and synphalangism. Other accompanying deformities and the presence of craniosynosthosis make the treatment plan even more complex. Because it is such a rare disorder, no standard treatment protocol has yet been developed. Methods: In this study, our aim was to evaluate the early results of our standard treatment protocol for Apert’s hands. We evaluated 7 patients with Apert’s hands who were treated bilaterally between the years of 2008 and 2013. We performed a two-stage surgical protocol, the first of which was the bilateral opening of the border fingers and the second stage was the opening of the middle fingers and web space deepening, osteotomy and collateral release of the thumb. Patients were evaluated according to grasping and pinching ability, graft-flap necrosis and cosmetic satisfaction. Results: The mean age at the first operation was 2.7 years and the mean number of operations was 3 per patient. No patient developed graft-flap necrosis and no patients required amputations. All patients were able to perform grasping and pinching functions and families were satisfied with the cosmetic results. Conclusion: Using a two-stage surgical protocol, achieving satisfactory results with a minimal number of operations is possible in patients with Apert Syndrome.