Assistant Professor of Department Orthodontics, School of Dentistry, Shiraz University of Medical Sciences
Abstract
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 pyknodysostosis with characteristic physical and radiographical findings. A large skull and generalized bonehyperdensification are typical features. This malformation shows a loss of the 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.Key words: Pyknodysostosis, Dwarfism, Hyperdensification
