The prevalence is unknown but
it seems to be less frequent than the other types of acromesomelic
dysplasia. Dwarfism is present at birth. The adult height is approximately
120 cm. The trunk is normally proportioned. Severe dwarfism is mostly
limited to the limbs (extremely short stature), with the legs more severely
affected than the arms. The middle segment (forearm with bowing of the
radius and posterior dislocation of its head; lower leg with marked
shortening of tibia and fibula) and distal segment (short hands and feet)
show the most severe changes. Dislocation of the large joints (elbow, hip,
knee, ankle) is frequently observed. The fingers usually are very short
secondary to cuboidal shape of the metacarpals and shortening of the middle
and proximal phalanges. Intelligence, facial appearance, and head
circumference are normal. Corneal opacities may occur.