Netan Choudhry

Images in clinical medicine. Valsalva retinopathy.

n engl j med 370;8 nejm.org february 20, 2014 e13 A 25-year-old woman at 11 weeks of gestation presented with sudden, painless, central “dark” vision after an episode of forceful vomiting (“morning sickness,” or emesis gravidarum). Visual acuity was 20/80 in the left eye. Funduscopy revealed sedimented preretinal hemorrhage that was enclosed by a domeshaped preretinal membrane, features consistent with Valsalva retinopathy (Panel A). Examination of the right eye was normal, as were the blood pressure, complete blood count, prothrombin time, activated partial-thromboplastin time, and fasting blood glucose level. Improvement was seen at 2 weeks (Panel B), 2 months (Panel C), and 5 months (Panel D) after presentation, and the hemorrhage finally resolved. Visual acuity in the left eye improved to 20/25. Typically self-limiting, Valsalva retinopathy is caused by retinal capillary rupture after abrupt rises in intraocular venous pressure, which may occur with violent coughing or vomiting. The dome-shaped area can represent the posterior hyaloid or internal limiting membrane, or both. These preretinal structures are closely apposed to the retinal surface in young adults, although hemorrhage can dissect tissue planes and fill the potential spaces. Pathological analysis of the preretinal membrane after surgery is necessary to unambiguously identify it as the posterior hyaloid or internal limiting membrane and to determine whether the hemorrhage is subhyaloid or beneath the internal limiting membrane. Subhyaloid and internal-limiting-membrane hemorrhages can also occur with hematologic dyscrasias and cancers, hypertension, the rupture of a retinal macroaneurysm, subarachnoid hemorrhage, and the shaken-baby syndrome. Treatment options are laser membranotomy, vitrectomy, or as in this patient, observation.

Enhanced Depth Imaging Spectral-Domain Optical Coherence Tomography Findings in Choroidal Neurofibromatosis

The authors report multimodal imaging findings, including enhanced depth imaging-optical coherence tomography, in an affected child with choroidal neurofibromatosis. Novel features such as choroidal vessel compression from choroidal nodules related to neurofibromatosis type 1 and an increased subfoveal choroidal thickness are identified. This is the first report to use EDI-OCT to analyze choroidal features in neurofibromatosis type 1.

Double optic pit maculopathy.

The author reports a case of a 16-year-old boy with decreased vision secondary to optic pit maculopathy. Clinical examination revealed two distinct optic pits in the optic nerve head with macular schisis and no subretinal fluid. Follow-up examination at 1 year demonstrated stable macular schisis and vision. This case illustrates visual impairment secondary to optic pit maculopathy arising from two optic pits in a single optic nerve head.

Regression of iris neovascularisation secondary to diabetic retinopathy with subconjunctival anti-VEGF therapy

A 48-year-old woman presented with asymmetric vision loss: the right eye being more aff ected than the left eye. She had a history of uncontrolled type 2 diabetes, 100% occlusion of the right carotid artery, and transient ischaemic attacks. She also had a history of proliferative diabetic retinopathy in both eyes, and had undergone multiple retinal surgeries for repair of retinopathy-related traction retinal detach ments in her right eye. Diabetic macular oedema was present in the left eye. Examination of the right eye showed traction of the pupillary edge by a fi brovascular membrane (fi gure A, arrow). Cataracts were present in both eyes: however, the cataract in the left eye was milder than the cataract in the right eye. Pathological iris neovascularisation extended onto the cataractous lens (fi gure A, B, arrowheads). Vascular leakage was confi rmed by iris angiography (blurred hyperfl uorescence; fi gure C, arrowhead). Ultrasonography did not show a tumour or retinal detachment. We diagnosed ocular ischaemic syndrome secondary to right carotid artery occlusion, related to systemic type 2 diabetes. Insuffi cient view was available for pan-retinal photocoagulation—the standard treatment for iris neovascularisation—because of the cataract. The patient received a subconjunctival injection of bevacizumab 2·5 mg (Genentech, South San Francisco, CA, USA), an antibody against vascular endothelial growth factor (VEGF; fi gure D, asterisk). 5 weeks later, regression of iris neo vascularisation was observed (fi gure E–G, arrowheads). The patient was referred to a primary-care physician to assist with control of diabetes and systemic anticoagulation. The anti-angiogenic eff ect was temporary, and the patient received three injections in the following 6 months to maintain regression and normal intraocular pressure. The patient continues to receive treatment. To date, no corneal epitheliopathy, conjunctival injection, or other toxic eff ects have been reported. Pathological iris neovascularisation occurs in 9–24% of patients with proliferative diabetic retinopathy, and can contribute to glaucoma, pain, and blindness, with surgical removal of the eye an outcome in some aff ected patients. Pathological iris neovascularisation occurs in two-thirds of cases of ocular ischaemic syndrome. This syndrome is often related to carotid disease secondary to diabetes mellitus, and is a serious disorder associated with a 5 year mortality of 40%, usually secondary to a cardiovascular cause.

Clinical Applications and Advantages of Swept Source OCT

The Topcon DRI Triton is an optical coherence tomographer (OCT) with a built-in color fundus camera. It utilizes swept source technology and has a central wavelength of 1050 nm. The scanning speed is 100 kHz and the depth resolution is 8 μ. It provides high-speed, high-resolution B-scans of the anterior and posterior segments of the human eye. It also provides 3D volumetric cubes that can be viewed in cross-sectional or in an en face format. Thickness maps are automatically generated for various retinal layers including: (1) full retinal thickness; (2) retinal nerve fiber layer (RNFL); (3) ganglion cell layer (GCL) plus the inner-plexiform layer (IPL); (4) RNFL plus GCL plus IPL; and (5) choroid layer. Due to the rapid speed of scanning, large areas of the retina can be imaged in a single scan including 3D scans covering areas as large as 12 × 9 mm, which includes both the macula and optic disc regions.

Vitreous Invasion: Neovascular Frond in Proliferative Diabetic Retinopathy

Originally received: June 1, 2016. Final revision: August 18, 2016. Accepted: August 22, 2016. Available online: September 27, 2016. Manuscript no. 2016-1164. 1 Uveitis Unit, Department of Ophthalmology, University Hospital of León, León, Spain. 2 Instituto de Biomedicina (IBIOMED), University of León, León, Spain. 3 Immunology Service and Uveitis Unit, University Hospital of León, León, Spain. 4 Rheumatology Service, University Hospital of León, León, Spain. Financial Disclosure(s): The author(s) have made the following disclosure(s): M.C.-C.: Lecturer e Abbvie; Merck; Sharp & Dohme; Allergan; Advisory board e Abbvie; Allergan J.G.R.M.: Lecturer and Advisory board e Abbvie Supported in part by a grant from the Junta de Castilla y León (SACYL; Valladolid, Castilla y León, Spain; grant no.: GRS 964/A/14 [M.C.-C., J.G.R.M.]). Author Contributions: Conception and design: Cordero-Coma, Calleja-Antolin, Ruiz de Morales Analysis and interpretation: Cordero-Coma, Calleja-Antolin, Garzo-García, Nuñez-Garnés, Ruiz de Morales Data collection: Cordero-Coma, Calleja-Antolin, Garzo-García, NuñezGarnés, Alvarez-Castro, Franco-Benito, Ruiz de Morales Obtained funding: none Overall responsibility: Cordero-Coma, Ruiz de Morales

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Multimodal Ultrawide-Field Imaging Features in Waardenburg Syndrome

A 45-year-old woman was referred for bilateral irregular fundus pigmentation. Dilated fundus examination revealed irregular hypopigmentation posterior to the equator in both eyes, confirmed by fundus autofluorescence. A thickened choroid was seen on enhanced-depth imaging spectral-domain optical coherence tomography (EDI SD-OCT). Systemic evaluation revealed sensorineural deafness, telecanthus, and a white forelock. Further investigation revealed a first-degree relative with Waardenburg syndrome. Waardenburg syndrome is characterized by a group of features including telecanthus, a broad nasal root, synophrys of the eyebrows, piedbaldism, heterochromia irides, and deafness. Choroidal hypopigmentation is a unique feature that can be visualized with ultrawide-field fundus autofluorescence. The choroid may also be thickened and its thickness measured with EDI SD-OCT.

A Ghostly OCT.

1. Sproule DM, Kaufmann P. Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes: basic concepts, clinical phenotype, and therapeutic management of MELAS syndrome. Ann N Y Acad Sci. 2008;1142:133-58. 2. Daruich A, Matet A, Borruat FX. Macular dystrophy associated with the mitochondrial DNA A3243G mutation: pericentral pigment deposits or atrophy? Report of two cases and review of the literature. BMC Ophthalmol. 2014;14:77.