The windblown hand - diagnosis, clinical picture and pathogenesis

Handchir Mikrochir Plast Chir. 2004 Apr-Jun;36(2-3):117-25. doi: 10.1055/s-2004-817873.

Abstract

The syndrome of the windblown hand deformity is a complex constellation of malformations affecting not only the head and the feet but also the hands in a quite distinct manner. In the hand, it involves congenital bilateral flexion contracture with ulnar deviation of the metacarpophalangeal joints. The thumb is characteristically adducted (reaching the palm; "thumb-in-palm deformity") with flexion of the MP joint and hyperextension of the IP joint. The etiology is basically unknown. We present two theories based on knowledge derived from the disciplines of evolution biology and embryology. We believe that the atavistic appearance of phylogenetically primitive muscle groups in conjunction with an impaired rotation of the extremities during embryological development account for this malformation syndrome.

Publication types

  • Review

MeSH terms

  • Abnormalities, Multiple / diagnosis*
  • Abnormalities, Multiple / embryology
  • Abnormalities, Multiple / genetics
  • Adult
  • Animals
  • Contracture / congenital*
  • Contracture / embryology
  • Craniofacial Abnormalities / diagnosis*
  • Craniofacial Abnormalities / embryology
  • Craniofacial Abnormalities / genetics
  • Disease Progression
  • Foot Deformities, Congenital / diagnosis*
  • Foot Deformities, Congenital / embryology
  • Foot Deformities, Congenital / genetics
  • Functional Laterality / physiology
  • Hand Deformities, Congenital / diagnosis*
  • Hand Deformities, Congenital / embryology
  • Hand Deformities, Congenital / genetics
  • Humans
  • Infant
  • Metacarpophalangeal Joint / abnormalities*
  • Metacarpophalangeal Joint / embryology
  • Pedigree
  • Phylogeny
  • Syndrome
  • Thumb / abnormalities*
  • Thumb / embryology