clinical features

Last reviewed 01/2018

This syndrome is characterised by:

  • distinct facies
  • abnormalities of hands and feet:
    • broad thumbs and toes
    • clinodactyly
    • dislocation of the radial head
    • hip dislocation
  • proportionate short stature
  • occasionally mental retardation
  • undescended testes

The distinct facies includes:

  • prominent supraorbital ridges
  • a broad nasal root
  • flattening of the midface
  • micrognathia
  • anti-mongoloid slant of eyes

There is an association between cleft palate and conductive deafness.