Psammomatoid juvenile ossifying fibroma in a male patient with crouzon syndrome

Abstract

A 16-year-old male patient, with Crouzon syndrome and severe bone deformity was referred to a hospital after an orthodontics evaluation, which detected a hyperdense circumscribed image in the maxilla during a facial computed tomography examination. The patient had anosmia and clinically a slight painless bulging in the anterior maxilla. An excisional biopsy with peripheral osteotomy was performed and the material was sent for histopathological analysis with under hypothesis of osteoma or fibrous dysplasia. Microscopically, there were benign fibro-osseous lesions characterized by hypercellular fibrous connective tissue, containing multiple ossicles with variable sizes and shapes, from spherical, oval to irregular and eosinophilic, with few basophilic areas similar to calcification, many with a brush border. Vital bone trabeculae were observed bordering the lesion and erythrocyte extravasation was compatible with psammomatoid juvenile ossifying fibroma. There was no need for further treatment and the patient remains under oral and maxillofacial follow-up for future orthognathic surgery.