Radiological findings in the hand in seckel syndrome (bird-headed dwarfism)

A. K. Poznanski*, G. Iannaccone, A. M. Pasquino, B. Boscherini

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

14 Scopus citations

Abstract

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 carplas 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.

Original languageEnglish (US)
Pages (from-to)19-24
Number of pages6
JournalPediatric radiology
Volume13
Issue number1
DOIs
StatePublished - Jan 1983

Keywords

  • Bird-headed dwarfism
  • Disharmonic skeletal maturation
  • Intrauterine growth retardation
  • Ivory epiphyses
  • Metacarpophalangeal pattern profile
  • Seckel syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Radiology Nuclear Medicine and imaging

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