Intravitreal Fluocinolone Acetonide 0.19 mg Implant in a Patient with Resistant Blau Syndrome: A Case Report.

Introduction: Blau syndrome is a progressive disease with an unknown etiology and pathogenesis. It can cause severe damage, especially in the eye with severe involvement.

Case Presentation: A six-year-old female was referred to us complaining about blurry vision and floaters in both eyes for 1 year. She had been diagnosed with Blau syndrome and Blau syndrome-associated anterior uveitis. Her best-corrected visual acuity in the right and left eyes was 20/70 and 20/80, respectively. Slit-lamp exam revealed faint bilateral band keratopathy along with 1+ anterior chamber cells and posterior synechia 360° in both eyes. During dilated fundoscopy, 2+ haze in the media was observed, along with swollen and hyperemic disc OU. Based on changes in optical coherence tomography, fluorescein angiography, and indocyanine green angiography, she was diagnosed with panuveitis and retinal vasculitis. Given her complicated history, we decided to proceed with an intravitreal fluocinolone acetonide 0.19 mg implant implantation in both eyes. During the 1-month follow-up visit, vitreous haze, retinal vasculitis, and active choroiditis were resolved. At 6-month follow-up visit, no changes were observed compared to the 1-month follow-up visit.

Conclusion: In cases of Blau syndrome that display resistance to systemic immunomodulatory therapies, the inclusion of local treatments, such as the intravitreal fluocinolone acetonide 0.19 mg implant, should be considered as an adjunctive therapeutic option.