Multi-Modality Imaging of Craniofacial Fibrous Dysplasia with Orbital Complications and Histopathology Correlation-A case report and short review of literature

Abstract

Craniofacial fibrous dysplasia [CF-FD] with orbital complications is a known but rare entity. This is a multi-modality imaging Case Report of extensive CF-FD in a 31-year-old male with right eye pain, swelling, and redness, along with histopathology correlation. In this patient, the CT scan demonstrates the classic ground glass bony appearance in great detail and helps with the diagnosis of FD, while excluding other bony pathology. The patient’s corroborative MRI brain with and without contrast and MRI brain perfusion images are presented to further characterize this pathology along with its orbital and ocular complications. Given the significant mass-effect on the ocular structures, the patient underwent orbital surgery with removal of as much of the lesion as possible. On macroscopic pathology evaluation, the affected bone was rubbery and gritty when sectioned. Microscopically, remnant fragments of woven bone of various size and shapes were seen with lack of an osteoblast rim. The bony fragments had a characteristic curvilinear, trabecular, and/or branching pattern. Post-surgical imaging demonstrated improvement in the mass-effect on orbital structures and proptosis, along with residual bony lesion.