Main feature is a unilateral congenital hypoplasia of the
proximal row of carpal bones in the right hand. Dysplasia of the distal
carpal bones, radius, and ulna on the right side were also present. Both patients
had marked micrognathia, high-arched palate, and small mouth opening
(microstomia). However, several otherwise healthy family members (i.e., no limb
abnormalities) were retrognathic, thus, it is difficult to
unequivocally link this feature to the congenital carpal bone hypoplasia.