Pierre Robin syndrome

Pierre Robin syndrome is a complex of congenital abnormalities of unknown cause. It is characterized by

  • a very small jaw (micrognathia)
  • high arched hard palate, which may be cleft
  • cleft soft palate
  • large tongue (in relation to the jaw) that tends to flop back and obstruct throat
  • cleft soft palate
  • natal teeth
Treatment involves maintaining the baby in a face-down position, which prevents the tongue from obstructing the baby's airway. Over a period of months to years, the jaw grows in relative size, relieving the tendency of the tongue to fall back and cause airway obstruction. More severe cases may require placement of a nasal tube to maintain a clear air passage for breathing, and even tracheostomy (surgical creation of an opening in the windpipe for breathing).

The condition can be associated with other genetic conditions, so children with Pierre Robin syndrome are usually referred to a genetic specialist to investigate the possibility of other problems.

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