2011 Volume 57 Issue 7 Pages 414-418
Pycnodysostosis is a rare autosomal recessive skeletal dysplasia characterized by short-limbed dwarfism, acroosteolysis, and a specific facial appearance associated with dysplasia of the jaw. We report the treatment of osteomyelitis of the mandible in a patient with pycnodysostosis that had not been diagnosed until 60 years of age. The patient visited our department because of buccal phlegmon arising from mandibular osteomyelitis. Pycnodysostosis was diagnosed on the basis of osteomyelitis of the jaw and other physical findings. He was treated under hospitalization. First, anti-inflammatory treatment of the right buccal phlegmon was performed. Subsequently, sequestrectomy was performed, and an external dental fistula was simultaneously constructed using the rhomboid-to-W technique. There has been no recurrence of acute inflammation, and the postoperative course has been favorable up to the present （2 years 10 months after operation）. However, the patient is being very closely followed up because the formation of a new sequestrum.
