Abstract

Osteopetrosis (OP), also known as marble bone disease is an extremely rare inherited skeletal disorder, characterised by abnormal functioning of the osteoclasts that results in incremental bone deposition with lack of normal bone remodelling. This leads to the formation of hard and brittle bone can readily fracture. The compromised quality of marrow tissue with poor vascularity reduces bone healing and increases the risk of infections. The risk of jawbone osteomyelitis in patients with OP is high and invariably involves mandible. Involvement of maxilla is rare and has been sparingly reported in the literature. This paper highlights a case of extensive necrosis of maxilla and mid-face skeleton following tooth extraction in a patient with OP. Review of the English literature identifying 23 previously published reports of maxillary osteomyelitis in patients with OP is also presented. Demographic details, history of tooth extraction, extent of involvement, clinical presentation, imaging finding, treatment provided and the outcome have been discussed.