Hareem Qadeer, Sebastiano Stellino, Luigi Angelo Vaira, Jerome R Lechien, Antonino Maniaci, Fabio Maglitto, Giuseppe Consorti, Łukasz Woźniak, Bożena Antonowicz, Jan Borys, Giovanni Salzano, Giacomo De Riu
Osteonecrosis of the jaw is most commonly discussed in relation to antiresorptive or antiangiogenic medications and head and neck radiotherapy. However, a heterogeneous group of jaw osteonecrosis cases occurs in the absence of these recognized triggers and remains poorly systematized. This review summarizes the current evidence on jaw osteonecrosis occurring outside the classical settings of antiresorptive/antiangiogenic medication exposure and head and neck radiotherapy, with the aim of clarifying its etiological spectrum, clinical presentation, diagnostic challenges, and management principles. The available literature is largely composed of case reports, small case series, and retrospective studies, reflecting the rarity and heterogeneity of these conditions. Reported causes include local traumatic or idiopathic oral ulceration with bone sequestration, viral infections, immunosuppression, chemotherapy, hematological malignancies, hematopoietic stem cell transplantation, hemoglobinopathies, metabolic or genetic bone disorders, vascular and thrombotic abnormalities, and toxic or recreational drug exposure. Clinical manifestations range from small, self-limited sequestra over bony prominences to extensive necrosis with tooth exfoliation, fistulae, infection, pathological fracture, and midfacial involvement. Management should be tailored to the underlying cause and disease extent, ranging from conservative sequestrectomy to radical debridement or resection combined with systemic treatment. Recognition of these entities is essential to avoid misdiagnosis and to guide appropriate multidisciplinary care.