Good Syndrome: Cytomegalovirus retinitis case challenge.

Abstract

To describe cytomegalovirus retinitis in a patient with Good syndrome (hypogammaglobulinemia and thymoma), ocular progression despite treatment, and fatal outcome. A 71-year-old woman with unilateral panuveitis of unknown cause and a history of thymoma resection was referred to our clinic. Visual acuity (VA) was 20/100 in her right eye (OD) and light perception in her left eye (OS). In slit-lamp examination, OD had inferior fine pigmented keratic precipitates, 2+ anterior chamber (AC) cells, cataract, and 2+ vitreous cells, no fundus detail, and normal ocular ultrasound. OS presented a white cataract, chronic hypotony, and increased choroidal thickness with calcifications. Laboratory evaluations were normal or negative for common causes of infection and inflammation. Prednisolone acetate eye drops were started, with improvement of AC inflammation. Cataract surgery was performed, reaching VA of 20/30. Two years later, VA had decreased and 2+ vitritis and retinitis were found. On clinical suspicion of masquerade syndrome, a vitrectomy biopsy was performed; pathology reported no data on ocular lymphoma. Leukopenia and lymphopenia were found: immunoglobulin levels, CD4 count, and viral load revealed systemic immunosuppression. The aqueous tap was positive for cytomegalovirus. Oral valganciclovir and intravitreal ganciclovir were initiated. In a patient with previous resection of thymoma and hypogammaglobulinemia, final diagnosis was Good syndrome, with CMVR being secondary to immunosuppression. Despite treatment, CMVR progressed and systemic deterioration resulted in mortal outcome. Good syndrome is an extremely rare disease and association with CMVR is uncommon. To our knowledge, only 14 cases exist in the literature.