Jeune syndrome (Asphyxiating thoracic dystrophy)
is a rare autosomal recessive skeletal dysplasia
characterized by small thorax, short ribs and
polydactyly. International Working Group on
Constitutional Diseases of Bone classified short rib
and polydactyly syndrome into 6 types: type 1
(Saldino- Noonan), type 11 (Magewski), type III (Verma-
Naumoff), type IV (Beemer langer), type V
(asphyxiating thoracic dystrophy ) and type VI
(chondroectodermal dysplasia) (Ellis van Creveld)
syndrome. Type 1-IV are lethal, however asphyxiating
thoracic dystrophy (ATD) and Ellis van Creveld
syndromes which have often but not always lethal
character1.