Granulomatosis With Polyangiitis Presenting as Occlusive Retinal Vasculitis.

Purpose: We discuss an unusual case of granulomatosis with polyangiitis (GPA) presenting as anterior uveitis with occlusive retinal vasculitis. Methods: A case report is presented. Results: A 60-year-old woman with a history of autoimmune disease presented to the retina clinic with red eyes and blurry vision in both eyes. An examination showed anterior uveitis with retinal vasculitis, and topical steroids were started in both eyes. One month later, the patient's vision worsened and an optical coherence tomography scan showed new central cystoid macular edema in the left eye. An antivascular endothelial growth factor injection was given. The next day, her vision was "black" in the left eye and a fundus examination showed global ischemia. A comprehensive uveitis workup was positive for cytoplasmic-staining antineutrophilic cytoplasmic antibody. A diagnosis of GPA was confirmed with a renal biopsy. Conclusions: Physician awareness of ocular GPA presentation is vital, and GPA management is most successful with a multidisciplinary team.

ANTERIOR MIGRATION OF INTRAVITREAL FLUOCINOLONE ACETONIDE IMPLANTS: A CASE REPORT.

BACKGROUND/PURPOSE: To describe management of anterior migration of a fluocinolone acetonide(FAc) intravitreal implant. METHODS: A retrospective case report. A 61-year-old man with diabetic macular edema and prior vitrectomy had anterior migration of a FAc implant. Anterior segment photos and optical coherence tomography were performed. RESULTS: Approximately 3 months after FAc implant was administered, it was noted to have migrated into the anterior chamber. Vision, intraocular pressure, and optical coherence tomography imaging initially remained stable, and no evidence of detectable corneal edema developed in 30 months of follow-up. However, at 36 months of follow-up, after second FAc implant injection, mild corneal edema developed,suspected to be related to the migrating implants. CONCLUSION: Anterior migration of a FAc implant may lead to less rapid and severe corneal decompensation compared with other steroid implants. Despite this, delayed corneal edema may occur. Careful monitoring of the cornea and intraocular pressure is recommended in cases of anterior FAc migration.

Case Report: Retinal Infarction Associated with Migraine.

SIGNIFICANCE: Retinal migraine and migrainous infarction are distinct clinical entities delineated by the International Headache Society. Presented is a novel case report demonstrating unique optical coherence tomography evidence of retinal ischemia experienced during a migraine with effects across retinal vascular territories. This may represent evidence of migrainous infarction within the retina. PURPOSE: The purpose of this study is to present clinical and quasi-histologic optical coherence tomography features of retinal ischemia associated with migraine. CASE REPORT: Presented is a case of profound monocular vision loss coincident with a migraine episode. Optical coherence tomography with novel features of acute inner retinal thinning, increased delineation of the inner plexiform and outer plexiform layers, and increased signal intensity of the photoreceptor layer is reported. These discriminating characteristics contrast those of retinal artery occlusions and other primary ocular vasculopathies such as Susac syndrome and acute macular neuroretinopathies. CONCLUSIONS: A case of permanent vision loss with retinal thinning and ischemic hyperreflectivity of retinal layers on optical coherence tomography in different vascular territories is shown to be associated with migraine. These features may provide clinical evidence of migrainous pathophysiology within the retina.

Bilateral exudative retinal detachments due to thrombotic microangiopathy associated with intravenous abuse of Opana ER.

PURPOSE: To report the only known case, to our knowledge, of bilateral exudative retinal detachments in the setting of thrombotic microangiopathy associated with intravenous abuse of extended-release oxymorphone (Opana ER). OBSERVATIONS: A 35-year-old male presented with headaches and acute, painless vision loss in the context of daily IV abuse of crushed oral Opana ER. The patient was found to have microangiopathic hemolytic anemia (MAHA), acute kidney injury in conjunction with hypertensive crisis and bilateral exudative retinal detachments. CONCLUSIONS AND IMPORTANCE: Bilateral exudative retinal detachments are rare ophthalmic complications that have been reported with thrombotic thrombocytopenic purpura (TTP). Non-TTP thrombotic microangiopathy, initially described as a "TTP-like illness" consisting of MAHA and thrombocytopenia, has been associated with the IV abuse of Opana ER. We report a case of bilateral exudative retinal detachments due to thrombotic microangiopathy in the setting of IV abuse of Opana ER.

Bilateral Central Retinal Artery Occlusion Associated with Bilateral Lymphoproliferative Infiltrative Optic Neuropathy.

BACKGROUND: Leukemic infiltration of the optic nerve is relatively rare. While previously described in acute leukemia, the infiltration in our case represents central nervous system (CNS) metastasis of Burkitt-type lymphoma that developed as a complication of solid-organ transplantation, resulting in a bilateral infiltrative optic neuropathy with sequential, bilateral central retinal artery occlusion (CRAO) and devastating vision loss. METHODS: The medical record, serial ophthalmic examination findings, clinical course, and imaging including magnetic resonance imaging (MRI), fundus photographs, and fluorescein angiography of a single patient were retrospectively reviewed. RESULTS: MRI demonstrated multifocal cortical and leptomeningeal CNS involvement, including the left optic nerve. Serial fundus examination/photography and fluorescein angiography showed that despite urgent whole-brain irradiation and systemic chemotherapy, CNS disease progressed to bilateral optic nerve infiltration and CRAO with no light perception vision in both eyes. CONCLUSION: CRAO can occur as a devastating and irreversible complication of lymphoproliferative optic nerve infiltration.

Sector Retinitis Pigmentosa Associated With Novel Compound Heterozygous Mutations of CDH23.

Usher syndrome is an autosomal recessive condition characterized by retinitis pigmentosa (RP) and congenital hearing loss, with or without vestibular dysfunction. Allelic variants of CDH23 cause both Usher syndrome type 1D (USH1D) and a form of nonsyndromic hearing loss (DFNB12). The authors describe here a 34-year-old patient with congenital hearing loss and a new diagnosis of sector RP who was found to have two novel compound heterozygous mutations in CDH23, including one missense (c.8530C > A; p.Pro2844Thr) and one splice-site (c.5820 + 5G > A) mutation. This is the first report of sector RP associated with these types of mutations in CDH23.

Retinopathy of Prematurity Versus Familial Exudative Vitreoretinopathy: Report on Clinical and Angiographic Findings.

BACKGROUND AND OBJECTIVE: Retinopathy of prematurity (ROP) and familial exudative vitreoretinopathy (FEVR) are classified as distinct diseases; however, emerging genetic research and fluoresce-in angiographic evidence suggest a margin of overlap between the two. PATIENTS AND METHODS This was an institutional review board-approved, retrospective case series. A literature review was performed, and medical records of all pediatric patients who underwent examination under anesthesia and laser for FEVR at the Bascom Palmer Eye Institute Pediatric Retina Service by one of the authors (AMB) from Jan. 1, 2006, to June 30, 2013, were analyzed retrospectively. RESULTS: Nine infants born prematurely had fluorescein angiograms and a clinical course most consistent with FEVR. Angiographic findings included irregular sprouts of vascularization at the vascular/ avascular junction, distinct pruning of vessels, pinpoint areas of hyperfluorescence, and segmental areas of vascular leakage. CONCLUSION: The authors propose a classification of ROPER (ROP vs. FEVR) to more accurately stratify these patients. Identification of this subset of patients will allow for sustained surveillance of infants with ROPER who demonstrate a continuing risk of disease activity.

Clinical Course of Vitreomacular Traction Managed Initially by Observation.

BACKGROUND AND OBJECTIVE: To investigate the clinical course and outcomes of patients with vitreomacular traction (VMT) managed initially by observation. PATIENTS AND METHODS: This noncomparative case series included patients with a diagnosis of VMT based on clinical symptoms and findings on spectral-domain optical coherence tomography (SD-OCT) between 2005 and 2014. VMT was documented using a standardized grading system based on the degree of distortion of the foveal contour. Data were collected at five retina clinics using standardized collection forms. Visual acuity, changes in SD-OCT findings, and timing of the release of VMT as seen on SD-OCT were recorded. RESULTS: The study included 230 eyes of 185 patients. Mean age was 72.5 years, and mean follow-up was 32 months. At baseline, VMT grading was grade 1 in 92 eyes (40%), grade 2 in 118 eyes (51.3%), and grade 3 in 20 eyes (8.7%). By last follow-up, spontaneous release of VMT occurred in 73 eyes (31.7%). Spontaneous release of VMT occurred at a mean of 18 months (median: 10.9 months) after initial visit. Mean logMAR best corrected visual acuity (BCVA) was 0.28 (20/55) (range: 20/20 to 20/400) at baseline and 0.25 (20/51) (range: 20/20 to 20/400) at last follow-up. Pars plana vitrectomy was performed in 10 eyes (4.1%) for macular hole (six eyes) and increased VMT (four eyes); BCVA was at least 20/40 in eight of the 10 eyes at last follow-up. CONCLUSION: Patients with VMT generally had a favorable clinical course when managed initially by observation. Spontaneous release of VMT occurred in approximately one-third of patients. At last follow-up, pars plana vitrectomy was performed in fewer than 5% of patients.

Peripheral retinal vasculopathy in childhood glaucoma.

PURPOSE: To report wide-field fluorescein angiography findings in the peripheral retina of eyes with childhood glaucoma. METHODS: Fluorescein angiograms were examined retrospectively in 12 patients with childhood glaucoma who underwent wide-field fluorescein angiography after surgical or medical treatment of glaucoma. This series included 12 eyes of 6 patients with primary congenital glaucoma, 5 eyes of 3 patients with glaucoma associated with congenital cataract surgery, 4 eyes of 2 patients with glaucoma associated with a systemic condition, and 1 eye of 1 patient with phakomatosis pigmentovascularis Type II. RetCam fundus photography images and digital fluorescein angiography were performed under general anesthesia. RESULTS: The most common finding observed in our series was peripheral retinal nonperfusion, which affected 20 eyes (91%). Other notable features seen were circumferential branching of the retinal vessels parallel to the ora serrata (77%), venous shunts (50%), and abnormal capillary branching patterns, including capillary dilatation (41%) and tortuous capillary tangles (18%). Leakage at the junction of vascular and avascular retina was not commonly seen (13.6%). None of the eyes had evidence of retinal neovascularization or fibrovascular proliferation. CONCLUSION: Retinal vascular abnormalities and peripheral nonperfusion are novel findings in childhood glaucoma. Further studies are needed to understand the significance and possible long-term consequences of these findings.

Internal drainage for chronic macula-involving serous retinal detachment in idiopathic central serous chorioretinopathy.

Conventional treatment of idiopathic central serous chorioretinopathy (ICSC) consists of argon laser, photodynamic therapy, or observation. However, in cases of atypical bullous ICSC with exudative detachment preventing any laser therapy, a surgical approach with external drainage of fluid has been performed. We present a case of ICSC with persistent macula involving exudative retinal detachment without evidence of uveitis that responded favorably to internal drainage by vitrectomy along with a scleral buckle placement. Our case, treated with internal drainage, also demonstrated successful long-term reattachment of the serous retinal detachment without any additional complications from the surgery.

Clinical course of vitreomacular adhesion managed by initial observation.

PURPOSE: The purpose of the study was to investigate the clinical course of patients with idiopathic vitreomacular adhesion (VMA). METHODS: A noncomparative case series of patients who had clinical symptoms and spectral-domain optical coherence tomography findings consistent with VMA. The VMA was graded based on the optical coherence tomography findings at initial and follow-up examinations. Grade 1 was incomplete cortical vitreous separation with attachment at the fovea, Grade 2 was the Grade 1 findings and any intraretinal cysts or clefts, and Grade 3 was the Grade 2 findings and the presence of subretinal fluid. RESULTS: One hundred and six eyes of 81 patients were identified as having VMA by spectral-domain optical coherence tomography at 3 retina clinics. The mean age was 73 years and the mean time of follow-up was 23 months. Forty-three eyes (41%) had Grade 1 VMA, 56 eyes (52%) had Grade 2 VMA, and 7 eyes (7%) had Grade 3 VMA. By the last follow-up, spontaneous release of VMA occurred in 34 eyes (32%), and pars plana vitrectomy was performed in 5 eyes (4.7%). Mean best-corrected visual acuity was 0.269 logarithm of the minimum angle of resolution or 20/37 at baseline (range, 20/20-20/200) and logarithm of the minimum angle of resolution 0.251 or 20/35 at the last examination (range, 20/20-20/400). CONCLUSION: In this selected patient cohort with mild symptoms, the clinical course of patients with VMA managed by initial observation was generally favorable.

Accidental Nd:YAG laser-induced macular hole in a pediatric patient.

After being exposed to an industrial Nd:YAG laser, an 11-year-old boy developed a full-thickness macular hole in the right eye with resultant visual acuity of 20/100. Five months after the initial injury, the vision was stable and the hole was still present. The patient underwent pars plana vitrectomy, internal limiting membrane peeling, and gas tamponade with prone positioning. Three months postoperatively, visual acuity improved to 20/25 with closure of the macular hole; however, there was a focal photoreceptor deficit. A review of the English-language literature identified 15 eyes with similar macular hole injuries after Nd:YAG exposure. Three eyes were surgically managed. While all holes were successfully closed, only two experienced visual improvement.