Clinical and Radiographic Findings of Pyknodysostosis: Report of a Case

Pyknodysostosis is a rare sclerosing bone disorder with autosomal recessive inheritance. It is characterized by short stature, brachycephaly, short and stubby fingers, open cranial sutures and fontanelle and diffuse osteosclerosis. Here, we report a case of yknodysostosis with characteristic physical and radiographical findings. A large skull andgeneralized bonehyperdensification are typical features. This malformation shows a loss ofthe normal mandibular angle. The patient had a small face, with a big nose and a large head. Irregularity of the teeth and a wide arched furrowed palate are other intra-oral findings. Although the profile showed to be convex, the patient had a skeletal and dental class III relation.