Subretinal Phialophora Abscess in Chronic Granulomatous Disease

Purpose: To report a rare case of a subretinal Phialophora richardsiae abscess in a patient with chronic granulomatous disease (CGD). Methods: A 21-year-old male with CGD and a history of invasive pulmonary aspergillosis presented with progressive loss of vision and pain in his left eye. He was found to have a subretinal abscess with a macula involving serous retinal detachment. A diagnostic and therapeutic pars plana vitrectomy, subretinal biopsy with debridment, inferior retinectomy and silicone oil tamponade was performed. Intraoperative cultures grew Pleurostoma (Phialophora) richardsiae. He was treated with systemic liposomal amphotericin B and high-dose Posaconazole. However, eye eventually required enucleation. Results: Preoperative visual acuity (VA) was light perception on the left eye and improved to count fingers at 4 weeks post-operative. However, VA rapidly declined to light perception, he developed an opaque white cataract, iris neovascularization, posterior synechiae, and corectopia. The retina remained attached under silicone oil. Histopathology revealed granuloma formation and active fungal elements. Discussion/Conclusion: The case supports the importance of vitreoretinal surgery to determine a definitive systemic diagnosis. Treatment of Phialophora infection is surgically challenging to manage and has a poor visual prognosis in patients with CGD.