Jeune syndrome

1 It is a rare genetic disorder, with a poor survival rate beyond the neonatal period, 2 known to be genetically heterogeneous and characterised by short limbs, underdeveloped iliac wings and a narrow rigid thoracic cage, which often results in asphyxiation. 1 Survival has been reported to the fourth decade of life, 3 with no race or sex predilection. Some success has been seen by expanding the thorax through a longitudinal division of the sternum held open with bone graft, acrylic implants, metal plates or donor bone grafts.