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Case report

A Brazilian man in his mid-30s presented to the emergency department with a 2-week history of floaters and worsening vision in his left eye, as well as redness and swelling of his right ear ( Fig. 1 ). His medical history was notable for chronic right-sided maxillofacial sinusitis and perichondritis secondary to biopsy-proven paracoccidioidomycosis diagnosed 15 years prior with intermittent flares on maintenance therapy of itraconazole 100 mg twice daily (BID) for the last 3 years. Two months prior to presentation, he ran out of itraconazole and was unable to obtain refills in the setting of the COVID-19 pandemic.

External photograph of right ear at presentation.

Snellen visual acuity at distance was 20/20 right eye (OD) and 20/200 left eye (OS). Intraocular pressures were 18 mmHg OD and 15 mmHg OS. Examination of the right eye was unremarkable. Slit lamp examination of the left eye was notable for 1+ conjunctival injection, corneal endothelial dusting with a Krukenberg spindle, and 1+ anterior chamber cell and pigment. There were no iris nodules, synechiae, or transillumination defects. Posterior segment examination revealed 1+ vitreous cell and 0.5+ inferior haze with a 4 disc-diameter elevated circular whitish-yellow subretinal lesion along the distal inferotemporal arcade with subretinal fluid and exudates extending to the fovea ( Fig. 2 A). There were numerous mid-peripheral small-to-medium white lesions with indistinct borders. Optical coherence tomography (OCT) confirmed a serous retinal detachment surrounding the lesion and involving the fovea. Fluorescein angiography (FA) showed early hypofluorescence over the lesion with late hyperfluorescence without evidence of neovascularization. Review of systems was notable for a perichondritis flare with increased pain, edema, and erythema of his right ear.

Wide-field fundus photographs and optical coherence tomography of the macula at various time points during the clinical course. A) Initial presentation with vitreous inflammation and chorioretinal lesions. B) Following three months of systemic antifungal and corticosteroid therapy, and prior to intravitreal therapy, with a plateau in lesion size reduction. C) Following three months of serial intravitreal voriconazole injections (14 total) with resolution of vitreous inflammation and lesion consolidation. The persistent subretinal fluid was attributed to vitreoretinal traction. D) Three months after pars plana vitrectomy for tractional retinal detachment with improvement in subretinal fluid. Four months after the initial surgery, he underwent repeat intervention to release the epiretinal fibrosis. E) One week post-operatively he had a significant reduction and near resolution of subretinal fluid.

He was started on oral intraconazole 200 mg BID and trimethoprim/sulfamethoxazole 160/800 mg BID, given his history of excellent response to systemic therapy in the past. Additionally, topical prednisolone acetate was added four times a day (QID). One month later, oral prednisone was started at 20 mg/day; this dose was chosen during the early stages of the novel coronavirus pandemic with concern for the risk of immunosuppression, but was escalated to 40 mg/day three weeks later due to persistent subretinal fluid. Over the next three months, the ear redness/swelling improved and the chorioretinal lesion gradually reduced in size on systemic therapy ( Fig. 2 B). However, the patient self-discontinued oral prednisone due to concerns over a rash. Due to persistent vitreous inflammation and macular traction with associated subretinal fluid, the patient was started on intravitreal voriconazole 100 μg injections. He underwent 14 injections, as frequently as twice a week, over the subsequent three months, which resulted in near-complete resolution of the vitreous inflammation and further consolidation of the border and reduction in the size of the chorioretinal lesion with some decrease in the surrounding subretinal fluid ( Fig. 2 C). The persistence of subretinal fluid was attributed to vitreoretinal traction from the chorioretinal lesion, so the decision was made to proceed with a pars plana vitrectomy. Intraoperatively the regressed lesion was strongly attached to the underlying choroid with surrounding retinal contraction and macular dragging. A biopsy was attempted, but the lesion was too friable to grasp any tissue. The retina could not be flattened without releasing the retinal traction from the lesion, so a retinotomy was performed 360° around the lesion allowing the retina to flatten intra-operatively, endolaser was applied to seal the retina around the excision site, and the eye was filled with silicone oil ( Supplementary Video ). Undiluted samples of vitreous fluid were negative for fungal stains by cytopathology, and fungal cultures remain negative to date. One month post-operatively, vision was 20/100 under silicone oil, and over the next few months, epiretinal fibrosis developed with retinal folding from the optic nerve to the retinotomy lesion, with some persistent subfoveal fluid ( Fig. 2 D). The retinotomy was confirmed to be well sealed by OCT, further indicating the source of the subretinal fluid was traction from the recurrent macular fibrosis. Four months after his initial vitrectomy, he underwent a repeat vitrectomy to remove the recurrent epiretinal fibrosis. The dense fibrosis was difficult to peel from the underlying retina leading to traction and limited detachment of the retina intra-operatively, perfluoron liquid was used to flatten the retina, and silicone oil was replaced. One week post-operatively he had significant improvement in subretinal fluid ( Fig. 2 E) and a vision of 20/400.

The following is the supplementary data related to this article:

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