= group of autosomal recessive disorders characterized by short limb dysplasia, constricted thorax, postaxial polydactyly (on ulnar / fibular side)

Type I = SALDINO-NOONAN SYNDROME

Type II = MAJEWSKI TYPE

Type III = NAUMOFF TYPE

Type IV = BEEMER

• severe micromelia
• pointed femurs at both ends (type I); widened metaphyses (type III)
• narrow thorax
• extremely short horizontally oriented ribs
• distorted underossified vertebral bodies + incomplete coronal clefts
• polydactyly
• cleft lip / palate

Prognosis: uniformly lethal