Pagetʼs Disease of Bone with Osteonecrosis of the Jaw and General Deterioration in a Very Old Patient：A Case Report

Abstract

　Pagetʼs disease of bone is characterized by abnormal bone remodeling, which results in deformity of the micro- and macro-structures of the bone, eventually diminishing the bone strength. This condition is extremely rare in Japan, affecting approximately 2 or 3 people per million. Frequently affected sites include the pelvis and spinal column, whereas the jaw is rarely affected. Here, we report the case of an very old patient with Pagetʼs disease of bone, diagnosed due to extensive osteonecrosis of the jaw. The patient was a 96-year-old male who was found at home and had difficulty in moving. The patient was diagnosed with septic shock due to urinary tract infection and was hospitalized. Since his mouth was filled with clotted blood, he was referred to the dentistry department for investigation of the oral infection. Significant bone exposure was observed in the right incisor and left molar regions of the maxilla. The various imaging findings were as follows：extensive sequestra and periodontal space degradation on panoramic radiography, a mixture of sclerotic and resorptive regions on posteroanterior radiography, a cotton wool-like appearance on computed tomography, and abnormal accumulation in the cranial and facial bones on bone scintigraphy. High alkaline phosphatase activity was detected in peripheral blood tests. The patient was diagnosed with Pagetʼs disease of bone concomitant with osteonecrosis of the maxilla. After sequestra excision and dental treatment, an oroantral fistula was found, which was covered using a denture in order to restore the velopharyngeal and swallowing functions. After instructing family members on the handling of the denture, the patient was discharged. During the 2-year follow-up period as an outpatient, no complications were reported. The patientʼs oral condition improved, which allowed him to consume regular meals. Since older patients with osteonecrosis may have undiagnosed Pagetʼs disease of bone, a careful systematic evaluation should be performed for timely management.