A case of pycnodysostosis complicated by a pathological fracture with mandibular osteomyelitis

Pycnodysostosis is an uncommon hereditary bone disorder characterized by general bone sclerosis, dwarfism, dysplasia of the skull, partial or total aplasia of the terminal phalanges, dysplastic clavicles, and an obtuse mandibular angle. Osteomyelitis of the jaws caused by odontogenic infection also seems to be a characteristic complication of pycnodysostosis. Since the bone is very brittle, oral infection may cause pathological or iatrogenic fractures that are very difficult to control. We report a case of pycnodysostosis in a 40-year-old man, whose mandible was fractured because of osteomyelitis. Segmental mandiblectomy and immediate reconstruction with a titanium plate was performed under general anesthesia. After a year, the clinical course was satisfactory. We discuss the treatment of mandibular osteomyelitis with pycnodysostosis.