Pyknodysostosis is a rare sclerosing bone disorder with autosomal recessive mode of inheritance. The condition is characterized by continuous bone deposition without physiological resorption, occurring mainly in the trabecular and not the cortical bone. The skeletal features include short stature, brachycephaly, open cranial sutures and fontanelles, diffuse osteosclerosis and obtuse mandibular angles. Among the oral features observed were grooved or furrowed palate and disturbances in eruption and exfoliation of teeth. Multiple fractures of long bones and osteomyelitis of the jaw are frequent complications. These are attributed to the increased brittleness and reduced vascularity of the bone as a result of continuous endosteal bone deposition. Treatment is usually successful with surgical management and sustained bactericidal antibiotic therapy, although hyperbaric oxygen has been recommended as adjunct treatment in the more refractory form.
The prevalence of developmental defects of enamel was assessed in 4805 16-year-old schoolchildren in fluoridated and non-fluoridated areas in Malaysia. In this sample, the mouth prevalence was 56.0%; tooth prevalence was 21.8%. There were significant differences between children in fluoridated and non-fluoridated areas, urban and rural areas as well as between the ethnic groups. However, there was no significant gender difference. Prevalences were lower than those reported in most other Asiancountries. However, direct comparisons could not be madedue to differences in indices and methodology used.