A submicroscopic unbalanced subtelomeric translocation t(2p;10q) identified by fluorescence in situ hybridization: fetus with increased nuchal translucency and normal standard karyotype with later growth and developmental delay, rhombencephalosynapsis (RES)

Ann Genet. 2004 Oct-Dec;47(4):405-17. doi: 10.1016/j.anngen.2004.07.005.

Abstract

Reaching an accurate diagnosis in children with mental retardation associated or not with dysmorphic signs is important to make precise diagnosis of a syndrome and for genetic counseling. A female case with severe growth and development delay, dysmorphic features and feeding disorder is presented. Antenataly, the fetus was observed to have increased nuchal translucency and a slight hypoplastic cerebellum. A standard karyotype was normal. RES and a submicroscopic unbalanced subtelomeric translocation t(2p; 10q) were demonstrated after birth. We show that within the framework of a collaborative approach, a concerted research of submicroscopic subtelomeric rearrangements should be performed in case of mental retardation associated with facial dysmorphic features, and when other etiologies or non-genetic factors (iatrogenic, toxic, infectious, metabolic...) have been ruled out.

Publication types

  • Case Reports

MeSH terms

  • Cerebellum / abnormalities
  • Child Development
  • Child, Preschool
  • Chromosome Banding
  • Chromosome Disorders / genetics*
  • Chromosomes, Human, Pair 10*
  • Chromosomes, Human, Pair 2*
  • Feeding and Eating Disorders / genetics
  • Female
  • Fetus / abnormalities
  • Humans
  • In Situ Hybridization, Fluorescence
  • Infant
  • Intellectual Disability / blood
  • Intellectual Disability / genetics*
  • Karyotyping
  • Magnetic Resonance Imaging
  • Nuchal Translucency Measurement
  • Rhombencephalon / abnormalities*
  • Telomere / genetics
  • Translocation, Genetic*