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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. [Hand Microsurg 2015; 4(3.000): 53-57]

Interdigital cell death is a physiological regression process responsible for sculpturing the digits in the embryonic vertebrate limb. Changes in the intensity of this degenerative process account for the different patterns of interdigital webbing among vertebrate species. Here, we show that Reelin is present in the extracellular matrix of the interdigital mesoderm of chick and mouse embryos during the developmental stages of digit formation. Reelin is a large extracellular glycoprotein which has important functions in the developing nervous system, including neuronal survival; however, the significance of Reelin in other systems has received very little attention. We show that reelin expression becomes intensely downregulated in both the chick and mouse interdigits preceding the establishment of the areas of interdigital cell death. Furthermore, fibroblast growth factors, which are cell survival signals for the interdigital mesoderm, intensely upregulated reelin expression, while BMPs, which are proapototic signals, downregulate its expression in the interdigit. Gene silencing experiments of reelin gene or its intracellular effector Dab-1 confirmed the implication of Reelin signaling as a survival factor for the limb undifferentiated mesoderm. We found that Reelin activates canonical survival pathways in the limb mesoderm involving protein kinase B and focal adhesion kinase. Our findings support that Reelin plays a role in interdigital cell death, and suggests that anoikis (apoptosis secondary to loss of cell adhesion) may be involved in this process.