Complete Simple Syndactyly
Journal Title: INTERNATIONAL JOURNAL OF RECENT TRENDS IN SCIENCE AND TECHNOLOGY - Year 2014, Vol 9, Issue 3
Abstract
Syndactyly is the most common congenital malformation of the limbs, with a large aesthetic and functional significance. Syndactyly is a failure of differentiation in which the fingers fail to separate into individual appendages. This separation usually occurs during the sixth and eighth weeks of embryonic development. The syndactyly word is derived from Greek words syn, meaning together, and dactyly, meaning fingers or digits. The incidence of syndactyly 1 in 2000-3000 live births.There is currently nine types of phenotypically diverse non syndromic syndactyly, an increase since the original classification by temtamy and McKusick [12]. Non-syndromic syndactyly is inherited as an autosomal dominant trait, although the more severve presenting types and sub types appear to have autosomal recessive and in some cases X-linked hereditary [6]. Gene research has found that these phenotypes appear to not only be one gene specific, although having individual localized loci, but dependent on a wide range of genes and subsequent signaling pathways involved in limb formation. Research into the individual phenotypes appears to complicate classification as new gens are found both linked, and not linked, to each malformation. Consequently anatomical, phenotypical and genotypical classification can be used, but variable in significance. Currently, management is surgical, with a technique unchanged for several decades, although future development will hopefully bring alternatives in both earlier diagnosis and gene manipulation for therapy.
Authors and Affiliations
Selukar Mangesh S , Alka K. Bhandare , Kulkarni Promod R
Keywords
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