Radiological findings in the hand in Seckel syndrome (bird-headed dwarfism).
Two patients with severe intrauterine growth retardation and bird-headed appearance are described. These two children had most of the clinical features of the so-called Seckel dwarfism. The radiological findings included: (1) ivory epiphyses affecting all phalanges in one patient and many phalanges in another; (2) cone-shaped epiphyses in the proximal phalanges; (3) marked disharmonic bone maturation between carpals and phalanges, between individual carpals, and from side to side; (4) alteration in the length of the hand bones, with considerable similarity of the metacarpophalangeal pattern between the two children; (5) relatively small carpals, which have an angular configuration; and (6) relatively normal or increased cortical thickness of the metacarpals.
D000365:Age Determination by Skeleton; D001846:Bone Development; D001842:Bone and Bones / Q000473:pathology*; D002348:Carpal Bones / Q000000981:diagnostic imaging / Q000473:pathology; D002648:Child; D003937:Diagnosis, Differential; D004392:Dwarfism / Q000000981:diagnostic imaging*; D004838:Epiphyses / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D005317:Fetal Growth Retardation / Q000000981:diagnostic imaging*; D006225:Hand / Q000000981:diagnostic imaging*; D006257:Head / Q000002:abnormalities*; D006801:Humans; D008297:Male; D008663:Metacarpus / Q000000981:diagnostic imaging / Q000473:pathology; D011247:Pregnancy; D011371:Progeria / Q000175:diagnosis; D013577:Syndrome