Journal of Family Medicine and Primary Care

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

Split-hand/feet malformation: A rare syndrome


Bahubali D Gane, P Natarajan 
 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
India

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.


How to cite this article:
Gane BD, Natarajan P. Split-hand/feet malformation: A rare syndrome.J Family Med Prim Care 2016;5:168-169


How to cite this URL:
Gane BD, Natarajan P. Split-hand/feet malformation: A rare syndrome. J Family Med Prim Care [serial online] 2016 [cited 2021 Apr 11 ];5:168-169
Available from: https://www.jfmpc.com/article.asp?issn=2249-4863;year=2016;volume=5;issue=1;spage=168;epage=169;aulast=Gane;type=0