Prenatal ultrasound findings observed in the Wolf-Hirschhorn syndrome: data from the registry of congenital malformations in Auvergne

Birth Defects Res A Clin Mol Teratol. 2013 Dec;97(12):806-11. doi: 10.1002/bdra.23194. Epub 2013 Nov 6.

Abstract

Background: Wolf-Hirschhorn syndrome (WHS) is associated with facial dysmorphism including high forehead, high nasal bridge, hypertelorism and severe mental retardation. WHS results from a 4p16.3 deletion. Only a small number of reports have been made on the prenatal ultrasound findings observed in WHS.

Cases: Here we report our experience on 10 cases of WHS ascertained prenatally between 1983 and 2009 through the CEMC-Auvergne registry of congenital malformations.

Conclusion: The assumption that a "Greek warrior helmet" facies is pathognomonic of WHS could lead to misdiagnosis. Other clinical findings such as severe and early onset intrauterine growth retardation, facial dysmorphism (high forehead, high nasal bridge, low-set ears, micrognathia, hypertelorism), atrial or ventricular septal defect, and renal dysplasia should help obstetricians to suspect the diagnosis of WHS prenatally.

Keywords: Wolf-Hirschhorn syndrome; fetus; prenatal ultrasound examination.

MeSH terms

  • Chromosome Deletion
  • Chromosomes, Human, Pair 4*
  • Female
  • Fetus / abnormalities*
  • France
  • Humans
  • Karyotyping
  • Phenotype
  • Registries
  • Ultrasonography, Prenatal
  • Wolf-Hirschhorn Syndrome / diagnosis*
  • Wolf-Hirschhorn Syndrome / genetics*
  • Wolf-Hirschhorn Syndrome / pathology