Pycnodysostosis- A Review

Abstract

Pycnodysostosis was coined by the French physicians Maroteaux and Lamy in 1962. The word "pyknos," in the Greek mean "dense" and word "dysostosis" mean abnormal bone formation. It characterized by osteoslerosis, short stature, wormian bones, last bone of the fingers usually short, open fontanel and several orofacial manifestations. It characterized by spontaneous fracture. This manuscript illustrates this condition with clinical features and management reported in the literature.