Abstract
Figure. A: A follow-up CT scan obtained 4 years after the patient's initial presentation demonstrates patchy areas within the right and left mandible, a finding that is consistent with Paget disease. B: Another view demonstrates lytic areas within the right and left mandible, a finding that is consistent with osteomyelitis.
A 67-year-old woman was referred to us by an infectious disease specialist for evaluation of chronic osteomyelitis of the mandible. She had been diagnosed with and treated for Paget disease of the mandible some 30 to 40 years earlier. Approximately 16 years prior to presentation, she had been diagnosed with osteomyelitis of the mandible. Since then, she had experienced multiple bouts of osteomyelitis that were treated with intravenous antibiotics. The diagnoses of both Paget disease and osteomyelitis had been confirmed by previous cultures and biopsies.
The patient was put on antibiotic therapy at that time. At a follow-up visit 4 years after her initial presentation to us, we found on examination that the right side of her mandible was swollen and sensitive to palpation, both externally and intraorally. The swelling was firm, and it extended from the parasymphyseal area to the midbody. Computed tomography (CT) demonstrated patchy, cotton-ball-like infiltrates of the maxilla and mandible bilaterally, a finding consistent with Paget disease (figure, A). Lytic areas within the bilateral mandible were also seen, a finding consistent with osteomyelitis (figure, B).
The patient subsequently underwent four bilateral debridements of her mandible, along with hyperbaric oxygen therapy, multiple courses of antibiotic treatment, and calcitonin therapy. Three years later, she was asymptomatic. Her most recent imaging study, obtained in 2007, showed no evidence of osteomyelitis, although evidence of Paget disease was still present.
Paget disease of bone (osteitis deformans) is the second most common bone disease (osteoporosis is the most common). It is characterized by a period of highly aggressive osteoclast-mediated bone resorption followed by abnormal osteoblast-mediated bone deposition.1,2 The average age of patients at the time of diagnosis is 50 years.1,2 The typical signs and symptoms include bone pain, bone deformity, fractures, and nerve root compression. However, most cases are discovered incidentally.1,2 Paget disease typically involves just one bone (monostotic), but there are cases in which several bones are involved (polyostotic). In the head and neck, the most commonly affected location is the skull. The maxilla and mandible are affected less frequently. 2 An untreated patient will have an elevated alkaline phosphatase level. The diagnosis can be made on plain radiography with a finding of a cotton-balllike appearance of the cortical bone. When the diagnosis is uncertain, a biopsy can be performed to confirm the pathology. 2
Histologic analysis during the active phase will demonstrate a disorganized bone architecture with clusters of osteoclasts. During the late phase, thick trabeculae with a mosaic pattern are seen. Medical treatment for this disease includes bisphosphonate and calcitonin therapy. 2
Osteomyelitis has been reported infrequently as a complication of Paget disease. Our review of the literature found only a few cases of chronic osteomyelitis of the mandible as a complication of Paget disease. 3 Treatment of osteomyelitis as a complication of Paget disease is no different than the treatment of osteomyelitis of other etiologies. Long-term intravenous antibiotics are the initial step. Biopsy and cultures allow for tailoring of the antibiotic regimen. Finally, debridement might be necessary to address any existing necrosis. 3