Fungal Chorioretinitis: A Complication of Treatment of Leukemia Amanda N. Hale, OD, FAAO; William F. Deegan III, MD Abstract: Fungal chorioretinitis is a complication of systemic candidemia. A patient undergoing treatment for Acute Promyelocytic Leukemia presents with blurred vision and floaters following an episode of candidemia. Fundus examination and early detection are critical to successfully treat and decrease morbidity from this condition. Case History: 47 y.o. Asian female CC: Blurred vision and floaters OS for one month. Vision change initially attributed to Cytarabine (Ara-C) treatment. Ocular history: moderate myopia OU; no glaucoma, injury, or surgery Medical history: Acute Promyelocytic Leukemia (APL) diagnosed 7 months prior o Completed four rounds consolidation therapy for APL o Hospitalized x 6 weeks, received Cytarabine tx, contracted indwelling catheterassociated infection, positive blood culture for Candida Medications: Chemotherapy: Neupogen, Methotrexate, Mercaptopurine, Tretinoin; Zyrtec, Tylenol Pertinent Findings: Clinical: o BCVA OD 20/20, OS 20/20, Habitual Rx OS 20/40+ o Amsler Grid: OD no metamorphopsia, OS nasal metamorphopsia o Pupils round, equal, reactive; no APD o IOP: OD/OS 16 mmHg o Retinal exam: OD: isolated blot hemorrhage nasal to disc OS: Elevated creamy infiltrate with edema and fine infiltrate at base temporal mid-periphery B-Scan Ultrasonography: 1.26 mm elevation with edema Fluorescein angiography: Retino-choroidal anastamosis in the scar Radiology studies: o MRI: basal ganglia lesion seen, origin unclear, resolved with treatment Lumbar puncture: negative Differential Diagnosis: Primary/Leading: o Fungal chorioretinitis o Leukemic cellular infiltrate/retinal detachment o Choroidal melanoma Others: o Bacterial endophthalmitis o Toxoplasmosis o Acute Retina Necrosis o Cytomegalovirus retinitis o Resolving retinal hemorrhage Diagnosis and Discussion: Chorioretinitis is an exudative inflammatory process that involves the posterior uveal tract and affects the retina and vitreous body. It occurs as a congenital infection in newborns, and can result from the dissemination of bacterial, viral or protozoan infections in immunocompetent patients. In immunodeficient patients, it can be associated with Epstein-Barr virus, CMV, varicella-zoster, various fungi (Candida, Aspergillus, Fusarium, dimorphic fungi) and Toxoplasma. Given the patient’s history of candidiasis, the diagnosis of fungal chorioretinitis was made. Acute promyelocytic leukemia (APL) is a malignancy of bone marrow characterized by a deficiency of mature blood cells and an abundance of immature blood cells called promyelocytes. APL most commonly occurs in children between 2 and 3 years old, and adults over 40. It is caused by a translocation of chromosomes 15 and 17. Treatment is chemotheraphy with all-trans retinoic acid. Candida species fungi are a natural resident of the gastrointestinal system and skin, and candidiasis is a frequently reported complication of IV drug use or catheterization. 2-3% of patients with candidiasis develop endophthalmitis. Candida can be implicated as a cause of both exogenous endophthalmitis following ocular surgery or trauma, and endogenous endophthalmitis due to hematogenous seeding of the eye. Fungal endophthalmitis can be treated with oral fluconazole or intravenous amphotericin B. Corticosteroids can be used to treat vasculitis. Fluconazole is generally well-tolerated, with a lower risk of side effects than amphotericin B. Vitrectomy and retinal laser can also be required. Treatment/Management: The patient was treated with oral fluconazole, 400 mg for one day, then 200 mg qd. Chemotherapy was continued. Fluconazole was continued for two months, at which time the infiltrate had regressed significantly. Confirmed with B Scan Ultrasonography. Bibliography: o Wyckoff, C., et al. Exogenous fungal endophthalmitis: Microbiology and Clinical Outcomes. Ophthalmology 2008; 115:1501-7. o Akler, M.E., et al. Use of fluconazole in the treatment of candidal endophthalmitis. Clin. Infect. Dis.1995; 20:657-664. Clinical Pearls: Fungal chorioretinitis is a complication of leukemia, which was brought on in this case by infection of an indwelling catheter. There should be a low threshold for funduscopic examination in patients with cancer, as early detection of retinopathy is critical. In the case of heavy vitreous debris, fundus lesions are obscured, making diagnosis and treatment speculative. In some cases a diagnostic vitrectomy is needed, which carries a high risk of complication in these patients.