Surgical management for IgG4-related ophthalmic disease by a transcranial biopsy combined with extraorbital decompression: illustrative case

BACKGROUND: Immunoglobulin G4–related ophthalmic disease (IgG4-ROD) accounts for 22% of Japanese lymphoproliferative orbital disease and occurs in 4%–34% of patients with IgG4-related disease, according to the largest case series published to date. Because the optic nerve mass often appears as a tumor-like lesion, it is important, although difficult, to differentiate IgG4-ROD from other orbital tumors and diseases, and biopsy is essential for diagnosis. Here, the authors describe the surgical management of an IgG4-ROD case. OBSERVATIONS: A 63-year-old man presented to the authors’ hospital with proptosis and visual impairment. Ophthalmic examination revealed intraocular hypertension. IgG4-related disease with an ophthalmic lesion was suspected on the basis of a blood test and imaging studies. Transcranial biopsy with extraorbital decompression was performed. The patient’s symptoms, including visual impairment, improved 3 days after operation, and his IgG4-related disease resolved after corticosteroid treatment. LESSONS: The standard treatment for IgG4-related disease is systemic corticosteroid therapy. However, this treatment should not be administered to patients with IgG4-ROD who a high risk of blindness. In this case, the authors completed a diagnostic and symptom-relieving transcranial biopsy without affecting the patient’s aesthetic characteristics. This is the first study, to our knowledge, to report extraorbital decompression via a transcranial approach as a surgical option for IgG4-ROD.