MULTIMODAL IMAGING OF ACUTE EXUDATIVE POLYMORPHOUS VITELLIFORM MACULOPATHY WITH OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY AND ADAPTIVE OPTICS SCANNING LASER OPHTHALMOSCOPY Purpose: To report a case of acute exudative polymorphous vitelliform maculopathy including the findings of optical coherence tomography angiography and adaptive optics scanning laser ophthalmoscopy. Methods: Findings on clinical examination, color fundus photography, spectral-domain optical coherence tomography, infrared reflectance, autofluorescence, optical coherence tomography angiography, and adaptive optics scanning laser ophthalmoscopy. Results: A 54-year-old white man with no significant medical history and history of smoking presented with bilateral multiple serous and vitelliform detachments consistent with acute exudative polymorphous vitelliform maculopathy. Extensive infectious, inflammatory, and malignancy workup was negative. Spectral-domain optical coherence tomography showed thickened, hyperreflective ellipsoid zone, subretinal fluid, and focal as well as diffuse subretinal hyperreflective material corresponding to the vitelliform lesions. Optical coherence tomography angiography showed normal retinal and choroidal vasculature, whereas adaptive optics scanning laser ophthalmoscopy showed circular focal “target” lesions at the level of the photoreceptors in the area of foveal detachment. Conclusion: Multimodal imaging is valuable in evaluating patients with acute exudative polymorphous vitelliform maculopathy.

SUCCESSFUL CLOSURE OF FULL-THICKNESS MACULAR HOLES SECONDARY TO MACULAR VITELLIFORM LESIONS Purpose: To describe the first reported cases of full-thickness macular holes secondary to vitelliform lesions that were successfully closed with vitrectomy surgery and gas tamponade. Methods: Two female patients developed visual loss secondary to bilateral vitelliform lesions and associated full-thickness macular holes. The patients underwent 25-gauge pars plana vitrectomy, internal limiting membrane peeling, and 26% sulfur hexafluoride gas, followed by 3 days of face-down positioning. Results: In both patients, the macular holes remain closed 3 and 25 months postoperatively. Conclusion: Vitrectomy surgery with gas tamponade may successfully close full-thickness macular holes secondary to macular vitelliform lesions.

CHOROIDAL MELANOMA, SECTOR MELANOCYTOSIS, AND RETINAL PIGMENT EPITHELIAL MICRODETACHMENTS IN BIRT–HOGG–DUBÉ SYNDROME Purpose: Birt–Hogg–Dubé Syndrome (BHDS) is a rare autosomal dominant condition that can predispose patients to numerous cutaneous fibrofolliculomas and other cutaneous lesions, pulmonary cysts with spontaneous pneumothorax, and multifocal renal tumors and cancer. The genetic mutations responsible for BHDS are related to tumor suppression and the mammalian target of rapamycin (mTOR) pathway. Previous reports of the ocular findings in BHDS include eyelid fibrofolliculomas, “flecked chorioretinopathy,” and one report of choroidal melanoma. We report a patient with BHDS who presented with choroidal melanoma, sector melanocytosis, and retinal pigment epithelial microdetachments. Methods: Observational case report. Results: A 38-year-old man with BHDS manifesting with facial fibrofolliculomas/tricodiscomas and pulmonary cysts with previous pneumothorax of both lungs was noted to have a large choroidal nevus, managed with observation elsewhere. On referral 1 year later, photopsia and visual field defect were noted, and the patient was discovered to have subtle patchy sector ocular melanocytosis of the iris and choroid with choroidal melanoma and dependent subretinal fluid. The melanoma measured 14 mm in basal dimension and 6.8 mm in thickness. In addition, pinpoint retinal pigment epithelial microdetachments were observed in both eyes at the equator and confirmed on optical coherence tomography. Custom-fit plaque radiotherapy was provided for tumor control. Conclusion: The BHDS can be associated with tumors of the skin and kidney. In this case, we noted ocular melanocytosis, malignant choroidal melanoma, and bilateral pinpoint retinal pigment epithelial detachments.

IMMUNE RECONSTITUTION INFLAMMATORY SYNDROME CAUSING PROGRESSIVE OPTIC NERVE EDEMA IN CRYPTOCOCCAL MENINGITIS Purpose: To report an human immunodeficiency virus–positive patient undergoing therapy for cryptococcal meningitis who developed progressive optic disk edema that was steroid responsive. Methods: Observational case report. Results: One month after commencing antifungal treatment for cryptococcal meningitis, the patient developed bilateral, progressive, recurrent optic disk edema with subretinal fluid that coincided with initiation of highly active antiretroviral therapy and recovery of CD4+ cell counts. Lumbar puncture revealed normal opening pressure, and cerebrospinal fluid showed no recurrence of cryptococcal infection. There was no evidence of uveitis. The patient rapidly improved with a 5-day course of high-dose intravenous methylprednisolone. Conclusion: Recurrent optic disk edema with loss of vision after treatment of cryptococcal meningitis in the setting of normal intracranial pressure may represent a unique manifestation of immune reconstitution inflammatory syndrome localized to the optic nerve without uveitis. This is consistent with the temporal relationship between starting highly active antiretroviral therapy, CD4+ count recovery, and the development of progressive disk edema in the study patient. Isolated optic nerve inflammation as a manifestation of immune reconstitution inflammatory syndrome has not been widely reported.

SEROUS RETINOPATHY ASSOCIATED WITH CISPLATIN TREATMENT Background/Purpose: To describe a case of serous retinopathy and associated photoreceptor atrophy after intravenous cisplatin therapy. Methods: Evaluation was performed using electroretinogram, optical coherence tomography, fundus autofluorescence, and funduscopic examinations to assess the extent of retinal disease, toxicity, and eventual atrophy. Results: A 56 year-old man with metastatic small cell carcinoma with vision changes after initiation of cisplatin therapy. The patient developed loss of vision to 20/400. A serous retinopathy was found on spectral domain optical coherence tomography with associated outer retinal atrophy and subretinal fibrosis. He developed outer ellipsoid layer atrophy after discontinuation of cisplatin therapy. He had patchy hypoautofluorescent areas in his macula on fundus autofluorescence and decreased cone response and slowed b-wave on electroretinogram. The serous retinopathy resolved with discontinuation of cisplatin and the malignancy was further managed with etoposide without recurrence of subretinal serous fluid or further vision loss. Conclusion: Commonly used to treat various solid tumors, cisplatin is not without significant neurologic, ocular, and retinal toxicities. Multimodal imaging may further the authors' understanding of toxicity and this case highlights the benefits of optical coherence tomography, especially with color vision deviation or visual acuity change.

BILATERAL VISUAL FIELD DEFECTS IN A PATIENT TREATED WITH THE MEK AND BRAF INHIBITORS TRAMETINIB AND DABRAFENIB FOR MELANOMA OF UNKNOWN ORIGIN Introduction: Although the introduction of BRAF and MEK inhibitors has greatly enhanced treatment possibilities in advanced BRAFV600-mutated melanoma, class-related toxicities are rather frequent and often involve the eye. Ophthalmologic side effects most commonly include central/diffuse serous retinopathy and retinal vein occlusion. Affection of the optic nerve head however has not been described clinically. Case report: A 29-year-old man presented in our eye clinic with bilateral blurred vision. Seventeen days earlier, he had been started on trametinib and dabrafenib combination therapy for metastasized melanoma of unknown origin. Visual field testing revealed diffuse bilateral defects, which regressed spontaneously on pause of MEK and BRAF inhibitor treatment. Discussion: In addition to the widely known class-related retinal toxicity, MEK and BRAF inhibitor-associated adverse events may also involve the optic nerve head, causing visual field defects probably regressing spontaneously after discontinuation of targeted oncologic therapy. In such cases, repeat brain imaging and exclusion of melanoma-associated retinopathy is recommended. Reinitiation of treatment and subsequent dose escalation seem to be feasible, but should be monitored by an ophthalmologist.

MANAGEMENT OF METASTATIC BREAST CARCINOMA OF IRIS WITH INTRAOCULAR BEVACIZUMAB INJECTIONS Purpose: To describe intraocular use of bevacizumab for a metastatic breast carcinoma of the iris resistant to advanced systemic chemotherapy protocols, for which conventional treatment would be local radiotherapy or brachytherapy. Methods: Case report. Results: A 65-year-old woman, who was previously diagnosed with breast carcinoma and treated with radical mastectomy coupled with radiotherapy and chemotherapy, presented with an iris mass in her left eye. Four successive intravitreal injections of bevacizumab resulted in progressive regression of the tumor to an almost indiscernible size at 8 months, along with blunting of the highly complex tumor vascular network on fluorescein angiography. At 12 months, the patient's visual acuity remained 20/20, and no ocular or systemic adverse effects were encountered. Conclusion: Intravitreal bevacizumab can offer a simpler and safer solution to treat metastatic iris tumors compared with other treatment options. This report of bevacizumab for treating iris metastasis from breast carcinoma may broaden the treatment options for similar neoplasms of the iris.

ACUTE IDIOPATHIC MACULOPATHY: 7-YEAR FOLLOW-UP WITH DEVELOPMENT OF OUTER RETINAL TUBULATIONS Purpose: To describe the development of outer retinal tubulations in a patient with acute idiopathic maculopathy. Methods: Chart review was performed on a single patient to collect all relevant clinical information and diagnostic imaging. Results: A case of outer retinal tubulations in a patient with idiopathic maculopathy is documented. Conclusion: Patients with acute idiopathic maculopathy may develop outer retinal tubulations similar to other entities that cause disturbance of retinal pigment epithelium or photoreceptors.

CHANGES IN THE POSTERIOR VITREOUS AFTER CATARACT SURGERY ASSESSED BY SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY Purpose: To assess changes in the posterior vitreous caused directly by phacoemulsification with implantation of an intraocular lens, using the enhanced vitreous swept-source optical coherence tomography system (Topcon, Tokyo, Japan). Methods: Consecutive patients with cataract without posterior vitreous detachment were enrolled. Swept-source optical coherence tomography examinations were performed 1 day before and several days after surgery, using enhanced vitreous visualization. We compared preoperative and postoperative posterior vitreous status and measured the distance between the internal limiting membrane and the posterior vitreous membrane at 26 locations at the posterior pole. Results: Images of 33 eyes (21 patients) could be analyzed. The perifoveal posterior vitreous detachment was not extended in any eyes, and the internal limiting membrane to posterior vitreous membrane distance before and after surgery did not differ at any location measured (P = 0.071–1.000). The posterior precortical vitreous pocket was dilated in three eyes. Age, gender, axial length, preoperative visual activity, nuclear sclerosis, duration of surgery, and duration between surgery and swept-source optical coherence tomography did not differ between the dilated vitreous pocket group (n = 3) and the unchanged group (n = 30). Conclusion: The height of the posterior vitreous membrane remained unchanged after surgery, although the posterior precortical vitreous pocket was dilated in three patients. Cataract surgery procedures seem to have little influence on the posterior vitreous membrane.

CIRCINATE PARTITION-LIKE FINDINGS ON CONE MOSAIC IMAGED BY ADAPTIVE OPTICS SCANNING LASER OPHTHALMOSCOPY IN EYES WITH INNER NUCLEAR LAYER MICROCYSTIC CHANGES Purpose: To report cases that showed partition-like, dark areas in the cone mosaic on adaptive optics scanning laser ophthalmoscopy (AO-SLO) images in eyes with inner nuclear layer (INL) microcystic changes. Methods: Eyes with INL microcystic changes were imaged by prototype AO-SLO. Results: An eye with Leber hereditary optic neuropathy, an eye with traumatic optic neuropathy, and an eye with retinitis pigmentosa that showed microcystic lesions in the INL were imaged by AO-SLO. The images revealed characteristic, dark, partition-like lesions in the cone mosaic of all the eyes in areas where microcystic changes in the INL were shown by spectral domain optical coherence tomography. The AO-SLO findings in eyes with optic neuropathy were quite similar in shape and size to those seen in eyes with retinitis pigmentosa. Conclusion: We report cases that manifest dark, partition-like areas in the cone mosaic on AO-SLO images. Microcystic lesions in the INL may affect the images of the cone mosaic.