Igg4-related orbital tumour with eye enucleation-infra-orbital nerve enlargement

摘要

A 40-year-old Japanese man presented with loss of vision and exophthalmos in the right eye. The MRI showed the lesion was around the optic nerve in the right orbit, Infra-orbital nerve enlargement (IONE) was apparent in the left orbit, but it was not recognized at that time (Fig. 1). The lesions were diagnosed as meningioma, and the right eye and orbital tumour were removed. Pathological analysis revealed abundant infiltration of lgG4-bearing plasmacytes and storiform fibrosis without meningioma cells. The lesion had developed around the optic nerve. Then the patient was introduced to us. Physical examination disclosed sensory nerve dysfunction around the bilateral cheeks, and serological testing revealed elevated levels of serum lgG4. lgG4-related ophthalmic disease was diagnosed and the patient was prescribed prednisolone at a dose of 40 mg/day, with gradual improvement of the IONE.