Maxillofacial osteonecrosis in a patient with multiple "idiopathic" facial pains

Previous investigations have identified focal areas of alveolar bone tenderness, increased mucosal temperature, abnormal anesthetic response, radiographic abnormality, increased radioisotope uptake on bone scans, and abnormal marrow within the quadrant of pain in patients with chronic, idiopathic facial pain. The present case reports a 53-year-old man with multiple debilitating, "idiopathic" chronic facial pains, including trigeminal neuralgia and atypical facial neuralgia. At necropsy he was found to have numerous separate and distinct areas of ischemic osteonecrosis on the side affected by the pains, one immediately beneath the major trigger point for the lancinating pain of the trigeminal neuralgia. This disease, called NICO (neuralgia-inducing cavitational osteonecrosis) when the jaws are involved, is a variation of the osteonecrosis that occurs in other bones, especially the femur. The underlying problem is vascular insufficiency, with intramedullary hypertension and multiple intraosseous infarctions occurring over time. The present case report illustrates the extreme difficulties involved in the diagnosis and treatment of this disease.