Unilateral mandibular atrophy in neurofibromatosis-1: case report

Abstract

Neurofibromatosis is an autosomal dominant neurocutaneous syndrome characterized by skin lesions and central or peripheral nervous system tumors. Although neurofibromatosis is a neurocutaneous disease, it also involves multiple systems. For example, bone lesions have been reported in 40% of patients. As in this case, pathologies associated with the mandible and temporomandibular joint in neurofibromatosis are rarely reported in the literature. In our case, we aimed to emphasize that skeletal malformations may also be present in the rich clinical picture of neurofibromatosis. Maxillofacial computed tomography of a 24-year-old female patient who was followed up at Selçuk University Hospital with a diagnosis of neurofibromatosis revealed an appearance compatible with atrophy in the right half of the mandible. The mandibular ramus was 41.27 mm on the right and 53.44 mm on the left; the diameter of the condyloid process was 10.31 mm on the right and 15.71 mm on the left. The joint distance was increased on the right. Radiologic examinations in neurofibromatosis syndrome should be performed considering the possibility of bone lesions. These examinations are especially important for the prevention of pathologic fractures in bones.

Keywords

• bone lesion
• mandible
• neurofibromatosis

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