Overlap between VACTERL and hemifacial microsomia illustrating a spectrum of malformations seen in axial mesodermal dysplasia complex (AMDC)

Am J Med Genet A. 2003 Aug 30;121A(2):151-5. doi: 10.1002/ajmg.a.20167.

Abstract

We describe a male infant born to consanguineous healthy parents with multiple congenital anomalies of the skeleton and internal organs. His phenotype displays an overlap between VACTERL and hemifacial microsomia (oculo-auriculo-vertebral spectrum/OAV). In addition, striking asymmetry of the malformations further supports the classification as part of the "axial mesodermal dysplasia complex" (AMDC) which is supposed to arise from disturbed mesodermal cell migration during early blastogenesis. The present case was instructive to review the continuous spectrum of AMDC related anomalies.

Publication types

  • Review

MeSH terms

  • Abnormalities, Multiple / pathology*
  • Arm / abnormalities
  • Consanguinity
  • Ear / abnormalities
  • Facial Asymmetry / pathology*
  • Goldenhar Syndrome / pathology*
  • Humans
  • Infant, Newborn
  • Lung / abnormalities
  • Lung / diagnostic imaging
  • Male
  • Mesoderm*
  • Phenotype
  • Radiography
  • Spine / abnormalities
  • Spine / diagnostic imaging