Successful conservative treatment of a case of mandibular osteomyelitis in a patient with pycnodysostosis

Pycnodysostosis, first described by Maroteaux and Lamy in 1962, is a rare autosomal recessive disease characterized by generalized diffuse bone sclerosing, short stature, and bone fragility, occurring frequently in association with skeletal dysplasia. Complications of pycnodysostosis such as intractable maxillary and mandibular osteomyelitis are common and appear in various forms. We present a case of mandibular osteomyelitis in a patient with pycnodysostosis who responded successfully to 7 years of conservative treatment. A 48-year-old woman who was previously given a diagnosis of pycnodysostosis was referred to our hospital because of continuous swelling and pain around the mandibular left first molar. Osteomyelitis gradually progressed after removal of the first molar. After a long follow-up period, during which the patient received local irrigation and low-dose antibiotic therapy, sequestration occurred. The lesion was cured completely by additional application of hyperbaric oxygen therapy (20 sessions). Even after 10 months of follow up, there has been no recurrence of inflammation.