Short rib-polydactyly syndrome

**Definition:** Short rib-polydactyly syndromes are a group of bone malformations characterised by a narrow thorax and polydactyly (usually preaxial). Prevalence as a group is unknown. The group is heterogeneous and includes Jeune syndrome and Ellis-Van Creveld syndrome, neither of which are lethal, together with lethal chondrodysplasias: Saldino-Noonan (type 1), Majewski (type 2), Verma-Naumoff (type 3) and Beemer-Langer (type 4).