Prenatal features of mandibulofacial dysostosis Guion-Almeida Type

J Med Life. 2021 Sep-Oct;14(5):722-725. doi: 10.25122/jml-2020-0082.

Abstract

Facial dysostoses are clinically and genetically heterogeneous conditions characterized by congenital craniofacial anomalies which result from abnormal development of the first two pharyngeal arches and their derivatives during embryogenesis. Mandibulofacial dysostosis Guion-Almeida type (MFDGA) is a rare and relatively new syndrome described in the literature, first identified by Guion-Almeida et al. in 2000 and 2006. Another 108 cases have been documented after that. Prenatal diagnosis of this syndrome has not been described yet. Here we present the prenatal ultrasound findings in a case where MFDGA was confirmed after delivery. We suggest that MFDGA should be included in the prenatal differential diagnosis of syndromes with micrognathia and craniofacial anomalies.

Keywords: Guion-Almeida; mandibulofacial dysostosis; prenatal ultrasound; whole exome sequencing.

Publication types

  • Case Reports

MeSH terms

  • Female
  • Humans
  • Intellectual Disability*
  • Mandibulofacial Dysostosis* / diagnostic imaging
  • Mandibulofacial Dysostosis* / genetics
  • Peptide Elongation Factors
  • Pregnancy
  • Ribonucleoprotein, U5 Small Nuclear
  • Syndrome

Substances

  • Peptide Elongation Factors
  • Ribonucleoprotein, U5 Small Nuclear