The windblown hand - diagnosis, clinical picture and pathogenesis.
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.
Grünert
J
J
Department of Hand and Plastic Surgery, Kantonsspital, St. Gallen, Switzerland. joerg.gruenert@kssg.ch
Jakubietz
M
M
Polykandriotis
E
E
Langer
M
M
D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000196:embryology / Q000235:genetics; D000328:Adult; D000818:Animals; D003286:Contracture / Q000151:congenital* / Q000196:embryology; D019465:Craniofacial Abnormalities / Q000175:diagnosis* / Q000196:embryology / Q000235:genetics; D018450:Disease Progression; D005532:Foot Deformities, Congenital / Q000175:diagnosis* / Q000196:embryology / Q000235:genetics; D007839:Functional Laterality / Q000502:physiology; D006228:Hand Deformities, Congenital / Q000175:diagnosis* / Q000196:embryology / Q000235:genetics; D006801:Humans; D007223:Infant; D008662:Metacarpophalangeal Joint / Q000002:abnormalities* / Q000196:embryology; D010375:Pedigree; D010802:Phylogeny; D013577:Syndrome; D013933:Thumb / Q000002:abnormalities* / Q000196:embryology