Pycnodysostosis was first described as an independent entity by Aoike (1954) and named by Marouteaux and Lamy (1962). This lesion is a hereditary and sclerosing bone disease characterized by dwarfism, open cranial sutures, peculiar facial profile and blunt mandibular angle.
In the present report, two cases of pycnodysostosis in two brothers, aged 33 and 31, were studied. They had a history of multiple spontaneous fractures of lower extremities and their parents were first cousins. The elder (Case 1) was admitted to our hospital for treatment of pathological fracture of the mandible that may have been caused by severe osteomyelitis following the extraction of the left lower second molar. The patient was successfully treated by mandibulectomy and immediate reconstruction with A-O plate under antibiotic therapy.
In the patients with pycnodysostosis, a severe osteomyelitis in the jaws seems to easily happen after dental infection because of extreme bone density and abnormal dentition. This fact may indicate a necessity of careful dental treatment and surgery in the patients with sclerosing bone dysplasia such as pycnodysostosis.
