A case of pycnodysostosis complicated by intractable mandibular osteomyelitis and pathologic fracture

Pycnodysostosis is a rare, normal chromosomal recessive disease named in 1962 by Maroteaux and Lamy. We reconstructed the mandible in a 41-year-old man with pycnodysostosis who had complications of osteomyelitis and a pathologic fracture of the mandible. Treatment involved hyperbaric oxygen therapy, sequestrum removal, resection of the mandibular region, and the placement of titanium plates. The findings in this patient, who subsequently had repeated recurrence of osteomyelitis, are described.