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Year : 2016  |  Volume : 5  |  Issue : 1  |  Page : 168-169

Split-hand/feet malformation: A rare syndrome

Department of Paediatrics, Sri Venkateshwara Medical College, Puducherry, India

Correspondence Address:
Bahubali D Gane
Department of Paediatrics, Sri Venkateshwara Medical College, Ariyur, Puducherry - 605 102
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2249-4863.184656

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Split-hand/split-foot malformation (SHFM) is mainly inherited as an autosomal dominant trait with incomplete penetrance and characterized by malformation of the limb involving the central rays of the autopod. It presents with a deep median cleft of the hand and/or foot, aplasia/hypoplasia of the phalanges, metacarpals, and metatarsals. Pathogenic mechanism is a failure to maintain signaling from the median apical ectodermal ridge. Without this signaling, cells of the underlying progress zone stop proliferation and differentiation which in turn results in defects of the central rays. We describe a case of SHFM in 10-year-old boy.

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