A recurrence of a hydrop lethal skeletal dysplasia showing similarity to Desbuquois dysplasia and a proposed new sign: the Upsilon sign

Am J Med Genet A. 2010 Apr;152A(4):966-9. doi: 10.1002/ajmg.a.33264.

Abstract

We report on a recurrence of a lethal skeletal dysplasia with features similar to Desbuquois dysplasia (DD) to expand the phenotypic spectrum of DD-like conditions, to increase awareness of DD-like phenotypes in the differential diagnosis of prenatal onset skeletal dysplasias, and to suggest a new sign, the Upsilon sign, to aid in the differential diagnosis of skeletal dysplasias with an extra ossification centre distal to second metacarpal.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Bone Diseases, Developmental / complications*
  • Bone Diseases, Developmental / diagnostic imaging*
  • Edema / complications*
  • Edema / diagnostic imaging*
  • Epiphyses / pathology
  • Female
  • Fetus / abnormalities
  • Fetus / pathology
  • Humans
  • Male
  • Postmortem Changes
  • Pregnancy
  • Recurrence
  • Ultrasonography, Prenatal