CASE REPORT   

European Journal of Oral and Maxillofacial Surgery 2017 August;1(2):50-3

DOI: 10.23736/S2532-3466.17.00114-X

Copyright © 2017 EDIZIONI MINERVA MEDICA

lingua: Inglese

Management of mandibular fractures in pycnodysostosis

Maria ROMÁN RAMOS, Fernando MONSALVE IGLESIAS, Paolo CARIATI ✉, Miguel PEREZ de PERCEVAL TARA, Ildefonso MARTINEZ LARA

Department of Oral and Maxillofacial Surgery, Virgen de las Nieves University Hospital, Granada, Spain

Pycnodysostosis is an extremely rare genetic bone disorder characterized by open cranial sutures, short stature and dwarfism. It is a recessive genetic disorder due to cathepsin K deficiency, which provoke a reduced bone turnover. The patient head is usually large with open fontanelles, hypoplastic malar, maxilla and mandible bones. They have also low height and diffuse osteosclerosis. The major complications are represented by the multiple fractures of the long bones and osteomyelitis of the jaw. We present a rare case of pycnodysostosis with mandibular re-fracture and complicated postoperative course managed by surgery and antibiotic therapy (both intravenous and intralesional protocol).

KEY WORDS: Pycnodysostosis - Cathepsin K - Bone fractures - Osteomyelitis