OSTEOMYELITIS IN A PATIENT WITH PYCNODYSOSTOSIS: A RARE CASE REPORT AND REVIEW OF THE LITERATURE

Pycnodysostosis (PYCD) is a rare autosomal recessive disease occurring due to a mutation in the gene that codes for the enzyme cathepsin K. Osteomyelitis of the jaws is a common complication of the PYCD. A 30-year-old female patient with PYCD presented with mandibular osteomyelitis which was treated by sequestrectomy and with a buccal fat pad as free graft (BFPFG) to fill the bone defect. The 24-month of follow-up demonstrates clinical and radiographic healing. A literature search was undertaken in Medline (through PubMed) in March of 2018 on PYCD case reports. Seventeen contributions were included in this study and discussed. To the best of our knowledge, this is the first case using BFPFG in the management of osteomyelitis of the jaws in a patient with PYCD. Despite the good results, further studies using BFPFG as adjuvant therapy for the management of jaw osteomyelitis are necessary to elucidate its clinical efficiency and safety. Support: FAPEMIG.