DOCSLIB.ORG

Disc Oedema in Association with Hunter's Syndrome: Ocular Histopathological Findings

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Disc Oedema in Association with Hunter's Syndrome: Ocular Histopathological Findings Br J Ophthalmol: first published as 10.1136/bjo.68.8.590 on 1 August 1984. Downloaded from British Journal ofOphthalmology, 1984, 68, 590-594 Disc oedema in association with Hunter's syndrome: ocular histopathological findings MARILYN BECK' AND GILLIAN COLE2 From the 'Department of Ophthalmology, University Hospital of Wales, Heath, Cardiff CF4 4XW, and the 2Department of Pathology, Welsh National School of Medicine, Heath Park, Cardiff CF4 4XN SUMMARY The histopathology of the posterior half of one eye and the optic nerves of a man with Hunter's syndrome, who was known to have disc oedema without raised intracranial pressure for 11 years of his life, is reported. The possible pathogenesis of the disc oedema is discussed; an important contributory factor was the deposition of abnormal mucopolysaccharides within the sclera. This caused gross thickening of the sclera with compression of the optic nerve at the intrascleral level. Hunter's syndrome was first described in 1917' and is episodes of heart failure when he was aged 25 years. one of the mucopolysaccharidoses. McKusick2 sub- A cousin also had Hunter's syndrome and died of divided this group of inherited disorders ofconnective respiratory failure following dental surgery. tissue into seven types. Hunter's syndrome is classified He was first seen by an ophthalmologist in 1971. as type II MPS and may be further subdivided into His visual acuities with a hypermetropic correction type IIA (severe) and type IIB (mild) depending on were 6/6 right and 6/6 left eyes. Fundal examination the degree of intellectual impairment. This disease is was recorded as showing normal peripheral fundi but usually inherited as an X-linked recessive trait. 'bilateral disc blurring.' http://bjo.bmj.com/ Clinically this syndrome is characterised by short In 1972 he was found to have gross bilateral disc stature, a gargoyle facies, stiff joints, hepato- swelling, normal retinal vessels, and normal splenomegaly, and cardiac abnormalities. Variable peripheral fundi. Visual fields recorded on the degrees of mental subnormality occur, and pro- Goldmann perimeter were full peripherally, but the gressive deafness is a consistent feature. In the mild blind spots were enlarged. Fluorography confirmed form survival has been reported to the sixth decade, that this was true disc oedema. He was then seen at whereas in the severe form death usually occurs before six-monthly intervals until March 1982. The corrected on September 26, 2021 by guest. Protected copyright. 15 years of age. Ocular abnormalities described in acuities 6/6 right and left were retained despite the this condition include exophthalmos, hypertelorism, fact that his disc appearances remained unchanged. papilloedema, and a pigmentary retinopathy, but the The visual fields remained full peripherally, but there cornea is usually macroscopically clear. were increasing scotomata centrally due to the This patient with Hunter's syndrome has been pre- development of a pigmentary retinopathy which was viously described,- as he was known to have disc first noted in 1980. The patient died of respiratory oedema for 11 years but yet retained good visual complications following hip surgery in September acuity. 1982. Case report POST-MORTEM EXAMINATION The patient had the characteristic external appear- This man, born 9 December 1947, was diagnosed as ance of Hunter's syndrome. There was generalised having Hunter's syndrome when he was aged 4 years thickening of bone and collagenous structures, with because of his small stature, facial appearance, deaf- particular involvement of the hip joints, mitral valve, ness, hepatosplenomegaly, and joint restriction. He larynx, and trachea with marked tracheal stenosis. was of normal intelligence, but he suffered from Brain and spinal cord. The brain was large and recurrent chest infections as a child and had his first weighed 1700 g. The dura covering brain and spinal Correspondence to Mairilyn Beck, FRCS. cord was markedly thickened. Coronal sections did 590 Br J Ophthalmol: first published as 10.1136/bjo.68.8.590 on 1 August 1984. Downloaded from Disc oedema in association with Hunter's syndrome: ocular histopathologicalfindings 591 Fig. Sections ofposterior halfof eye. The sc/era is grossly thickened, and the intrascleralportion ofthe optic nerve is compressed. Bulging ofthe optic nerve is evident. (HE, x4- 7). not show any macroscopic abnormality. The ven- stained with haematoxylin and eosin (HE), Kluver tricles were of normal size. Microscopic examination Barrer (KLB) stain for myelin, phosphotungstic acid showed the only obvious pathological change to be a haematoxylin (PTAH) for glial fibres and collagen, mild deposition of mucopolysaccharides (MPS) with- periodic acid schiff reagent (PAS) and colloidal iron in vessel walls, with a consequent perivascular for neutral and acid mucopolysaccharides. rarefaction of tissue. No MPS accumulation within neurones or glial cells ofthe brain or cord was evident. MICROSCOPIC EXAMINATION Eye and optic nerves. The posterior half of the right Sclera. The sclera was grossly thickened. Measure- http://bjo.bmj.com/ eye and both optic nerves were available for exam- ment of the thickness of the sclera at the posterior ination. Sections of the eye and optic nerves were pole revealed it to be 2-9 mm, which was nearly three embedded in paraffin and cut at 5 .tm. Sections were times thicker than the average 1 mm thickness of a similar site in three control eyes (Fig. 1). The sclera was thickened by irregular bands of densely col- lagenous tissue. Scattered throughout the sclera were clumps of granular deposits, which were present in macrophages, and also apparently lying free in the on September 26, 2021 by guest. Protected copyright. tissue. The granular material proved to be MPS, staining positively with PAS and colloidal iron. Choroid. The posterior choroid was the only uveal tissue available for examination and was essentially normal. There were sparsely scattered deposits of MPS confined to vessel walls. Bruch's membrane was intact. Retina. Numerous levels of the posterior retina were examined. The retinal pigment layer had been completely destroyed, and there was marked loss of rods and cones. In those areas where rods and cones could still be detected the normal architecture was lost. Occasional eosinophilic amorphous structures Fig. 2 Retina showing loss of rods and cones, and were evident, and this was interpreted as a degenera- formation ofamorphous structures (arrow). The outer and tive change (Fig. 2). The appearance of the rod and inner nuclear layers and the ganglion cells are reduced in cone layer was not considered to be artefactual or due number. (HE, X250). to poor fixation, since the other layers of the retina Br J Ophthalmol: first published as 10.1136/bjo.68.8.590 on 1 August 1984. Downloaded from 5'92 Marilyn Beck and Gillian Cole were well preserved. The outer and inner nuclear thickened at the lamina cribrosa and throughout its layers were considerably reduced, being about half extent. Finely scattered MPS granules which were the normal cell thickness seen in controls. The PAS and colloidal iron positive were present in the ganglion cells were likewise reduced in number septa. The vessels within the optic nerve contained (Fig. 2). small amounts of positively staining material, indica- There was moderate gliosis within the ganglion cell tive of MPS within their walls, but the bulk of MPS layer. No deposits of acid mucopolysaccharide were appeared to be in the region of the collagenous septa evident within the retinal layers or ganglion cells. within the nerve. The presence of scanty MPS There was, however, a mild deposition of MPS within granules within macrophages in the region of the and around the vessel walls. Retinitis pigmentosa was septa was best demonstrated on thin toluidine blue present with accumulation of pigment within macro- sections. Sections of the optic nerve in its peripheral phages and lying free in the tissue. The pigment extent from the optic chiasm revealed essentially the tended to accumulate around blood vessels. same features of thickened septa and gliosis. The Optic nerves. At the intrascleral level the optic gliosis, however, was mostly confined to the external nerve was considerably compressed by the thickened fibres beneath the thickened meningeal coverings, sclera (Fig. 1). There was marked gliosis of the nerve and was less extensive than that seen in the optic at the papilla (Fig. 3) and at the intrascleral level. nerve head and intrascleral portion. Microscopic evidence of disc oedema was most con- vincingly demonstrated by changes in the optic nerve ELECTRON MICROSCOPY head, which bulged forwards and laterally with some Formalin fixed tissue was rehydrated from paraffin displacement of the retina away from the disc margins blocks, and refixed in phosphate buffered glutaralde- (Fig. 1). The collagenous septa of the nerve were hyde. After post-osmication and post-fixing in 1.5% A http://bjo.bmj.com/ on September 26, 2021 by guest. Protected copyright. Fig. 3 Optic nierve papilla with marked glialfibreformnatioti. (PTA H, x250). Br J Ophthalmol: first published as 10.1136/bjo.68.8.590 on 1 August 1984. Downloaded from Disc oedema in association with Hluter's synidrome: ocular histopathologicalfindings 593 '4!M. ' ;' <F te ; '$ , u I Ar, '4 v-. http://bjo.bmj.com/ Fig. 4 Scleralfibroblast withi initracloplasmini membratne bolund vacuoles conitaininlg lamellar atnd electron- detise iniclusion.s. N=nucleus. L =latnellar inclusions. (=collagen. (X 15000). aqueous uranyl acetate, pieces of choroid, sclera, and oedema was not a feature in these reports, and the optic nerve were dehydrated and embedded in Epon. optic nerves were found to be normal. We have Ultrathin sections were stained in lead citrate and described the histopathology and ultrastructure of on September 26, 2021 by guest. Protected copyright. viewed under a Philips 300 electron microscope. the posterior half of the eye of a patient with Hunter's In the choroid and sclera abnormal mucopolysac- syndrome who had unexplained disc oedema.
Load more

Recommended publications
  • Optic Disc Edema, Globe Flattening, Choroidal Folds, and Hyperopic Shifts Observed in Astronauts After Long-Duration Space Flight
    University of Nebraska - Lincoln DigitalCommons@University of Nebraska - Lincoln NASA Publications National Aeronautics and Space Administration 10-2011 Optic Disc Edema, Globe Flattening, Choroidal Folds, and Hyperopic Shifts Observed in Astronauts after Long-duration Space Flight Thomas H. Mader Alaska Native Medical Center, [email protected] C. Robert Gibson Coastal Eye Associates Anastas F. Pass University of Houston Larry A. Kramer University of Texas Health Science Center Andrew G. Lee The Methodist Hospital See next page for additional authors Follow this and additional works at: https://digitalcommons.unl.edu/nasapub Part of the Physical Sciences and Mathematics Commons Mader, Thomas H.; Gibson, C. Robert; Pass, Anastas F.; Kramer, Larry A.; Lee, Andrew G.; Fogarty, Jennifer; Tarver, William J.; Dervay, Joseph P.; Hamilton, Douglas R.; Sargsyan, Ashot; Phillips, John L.; Tran, Duc; Lipsky, William; Choi, Jung; Stern, Claudia; Kuyumjian, Raffi; andolk, P James D., "Optic Disc Edema, Globe Flattening, Choroidal Folds, and Hyperopic Shifts Observed in Astronauts after Long-duration Space Flight" (2011). NASA Publications. 69. https://digitalcommons.unl.edu/nasapub/69 This Article is brought to you for free and open access by the National Aeronautics and Space Administration at DigitalCommons@University of Nebraska - Lincoln. It has been accepted for inclusion in NASA Publications by an authorized administrator of DigitalCommons@University of Nebraska - Lincoln. Authors Thomas H. Mader, C. Robert Gibson, Anastas F. Pass, Larry A.
    [Show full text]
  • 98796-Anatomy of the Orbit
    Anatomy of the orbit Prof. Pia C Sundgren MD, PhD Department of Diagnostic Radiology, Clinical Sciences, Lund University, Sweden Lund University / Faculty of Medicine / Inst. Clinical Sciences / Radiology / ECNR Dubrovnik / Oct 2018 Lund University / Faculty of Medicine / Inst. Clinical Sciences / Radiology / ECNR Dubrovnik / Oct 2018 Lay-out • brief overview of the basic anatomy of the orbit and its structures • the orbit is a complicated structure due to its embryological composition • high number of entities, and diseases due to its composition of ectoderm, surface ectoderm and mesoderm Recommend you to read for more details Lund University / Faculty of Medicine / Inst. Clinical Sciences / Radiology / ECNR Dubrovnik / Oct 2018 Lund University / Faculty of Medicine / Inst. Clinical Sciences / Radiology / ECNR Dubrovnik / Oct 2018 3 x 3 Imaging technique 3 layers: - neuroectoderm (retina, iris, optic nerve) - surface ectoderm (lens) • CT and / or MR - mesoderm (vascular structures, sclera, choroid) •IOM plane 3 spaces: - pre-septal •thin slices extraconal - post-septal • axial and coronal projections intraconal • CT: soft tissue and bone windows 3 motor nerves: - occulomotor (III) • MR: T1 pre and post, T2, STIR, fat suppression, DWI (?) - trochlear (IV) - abducens (VI) Lund University / Faculty of Medicine / Inst. Clinical Sciences / Radiology / ECNR Dubrovnik / Oct 2018 Lund University / Faculty of Medicine / Inst. Clinical Sciences / Radiology / ECNR Dubrovnik / Oct 2018 Superior orbital fissure • cranial nerves (CN) III, IV, and VI • lacrimal nerve • frontal nerve • nasociliary nerve • orbital branch of middle meningeal artery • recurrent branch of lacrimal artery • superior orbital vein • superior ophthalmic vein Lund University / Faculty of Medicine / Inst. Clinical Sciences / Radiology / ECNR Dubrovnik / Oct 2018 Lund University / Faculty of Medicine / Inst.
    [Show full text]
  • Eye60. Instrumental Eye Examination.Pdf
    INSTRUMENTAL EYE EXAMINATION Eye60 (1) Instrumental Eye Examination Last updated: May 9, 2019 “BEDSIDE” EXAMINATIONS ..................................................................................................................... 1 OPHTHALMOSCOPY (FUNDUSCOPY) ........................................................................................................ 1 DIRECT OPHTHALMOSCOPY .................................................................................................................... 1 INDIRECT OPHTHALMOSCOPY ................................................................................................................. 2 OPHTHALMOSCOPIC FINDINGS ............................................................................................................... 2 Hypertensive retinopathy ................................................................................................................. 6 Diabetic retinopathy ......................................................................................................................... 7 PEDIATRIC ASPECTS ............................................................................................................................... 9 APPLANATION TONOMETRY .................................................................................................................... 9 SLIT LAMP EXAMINATION (BIOMICROSCOPY) ........................................................................................ 9 ULTRASONOGRAPHY ..............................................................................................................................
    [Show full text]
  • Anatomy and Physiology of the Afferent Visual System
    Handbook of Clinical Neurology, Vol. 102 (3rd series) Neuro-ophthalmology C. Kennard and R.J. Leigh, Editors # 2011 Elsevier B.V. All rights reserved Chapter 1 Anatomy and physiology of the afferent visual system SASHANK PRASAD 1* AND STEVEN L. GALETTA 2 1Division of Neuro-ophthalmology, Department of Neurology, Brigham and Womens Hospital, Harvard Medical School, Boston, MA, USA 2Neuro-ophthalmology Division, Department of Neurology, Hospital of the University of Pennsylvania, Philadelphia, PA, USA INTRODUCTION light without distortion (Maurice, 1970). The tear–air interface and cornea contribute more to the focusing Visual processing poses an enormous computational of light than the lens does; unlike the lens, however, the challenge for the brain, which has evolved highly focusing power of the cornea is fixed. The ciliary mus- organized and efficient neural systems to meet these cles dynamically adjust the shape of the lens in order demands. In primates, approximately 55% of the cortex to focus light optimally from varying distances upon is specialized for visual processing (compared to 3% for the retina (accommodation). The total amount of light auditory processing and 11% for somatosensory pro- reaching the retina is controlled by regulation of the cessing) (Felleman and Van Essen, 1991). Over the past pupil aperture. Ultimately, the visual image becomes several decades there has been an explosion in scientific projected upside-down and backwards on to the retina understanding of these complex pathways and net- (Fishman, 1973). works. Detailed knowledge of the anatomy of the visual The majority of the blood supply to structures of the system, in combination with skilled examination, allows eye arrives via the ophthalmic artery, which is the first precise localization of neuropathological processes.
    [Show full text]
  • Using the Ophthalmoscope: Viewing the Optic Disc and Retina
    Using the Ophthalmoscope: Viewing the Optic Disc and Retina Judith Warner, MD University of Utah THE OPHTHALMOSCOPE DIRECT OPHTHALMOSCOPY • Jan Purkinje 1823 • Hermann von Helmholtz 1851 • Hand held ophthalmoscope • Direct up-right image Dials of the Ophthalmoscope RED-FREE FILTER (GREEN LIGHT) 450 nm monochromatic light nerve fiber layer optic nerve drusen OTHER DIALS • Used for measuring lesion size • Looking for the center of fixation OTHER DIALS: SLIT BEAM The wheel has lenses of power Panoptic-ophthalmoscope Direct type Wider field of view Distance from pt greater Similar apertures Not as easy to carry Slightly dimmer light source Not as magnified view of Disc Clean the rubber cup between patients Photographs: http://panoptic.welchallyn.com/faq.html WHEN EVER POSSIBLE: DILATE THE PATIENT Steps to Direct Ophthalmoscopy • Dimly lit room • Dilating drops • Patient fixates distant target • Align yourself • Red reflex • Dial in HOW TO USE THE DIRECT Ophthalmoscope.avi ophthalmoscope.wmv THE RED REFLEX The layers you will go through to see the optic disc THE OPTIC NERVE WHAT YOU SHOULD OBSERVE IN EVERYONE RIGHT EYE AND LEFT EYE THE NORMAL DISC • The disc is 1.62 mm or 1 million fibers • Central retinal artery and vein • Lamina Cribrosa • The optic cup The Normal Disc Appearance The lamina cribrosa is an important disc structure --Means Sieve --Anatomically present in all discs --Visible in about 1/3 --Shallow in myopia Look at the Cup-to-disc ratio: WHAT IS THE CUP-TO-DISC RATIO? .7 NO CUP 0.1 CUP 0.3 CUP 0.7 CUP 0.9 CUP What is the cup
    [Show full text]
  • The Horizontal Raphe of the Human Retina and Its Watershed Zones
    vision Review The Horizontal Raphe of the Human Retina and its Watershed Zones Christian Albrecht May * and Paul Rutkowski Department of Anatomy, Medical Faculty Carl Gustav Carus, TU Dresden, 74, 01307 Dresden, Germany; [email protected] * Correspondence: [email protected] Received: 24 September 2019; Accepted: 6 November 2019; Published: 8 November 2019 Abstract: The horizontal raphe (HR) as a demarcation line dividing the retina and choroid into separate vascular hemispheres is well established, but its development has never been discussed in the context of new findings of the last decades. Although factors for axon guidance are established (e.g., slit-robo pathway, ephrin-protein-receptor pathway) they do not explain HR formation. Early morphological organization, too, fails to establish a HR. The development of the HR is most likely induced by the long posterior ciliary arteries which form a horizontal line prior to retinal organization. The maintenance might then be supported by several biochemical factors. The circulation separate superior and inferior vascular hemispheres communicates across the HR only through their anastomosing capillary beds resulting in watershed zones on either side of the HR. Visual field changes along the HR could clearly be demonstrated in vascular occlusive diseases affecting the optic nerve head, the retina or the choroid. The watershed zone of the HR is ideally protective for central visual acuity in vascular occlusive diseases but can lead to distinct pathological features. Keywords: anatomy; choroid; development; human; retina; vasculature 1. Introduction The horizontal raphe (HR) was first described in the early 1800s as a horizontal demarcation line that extends from the macula to the temporal Ora dividing the temporal retinal nerve fiber layer into a superior and inferior half [1].
    [Show full text]
  • Molecular Regulation of Visual System Development: More Than Meets the Eye
    Downloaded from genesdev.cshlp.org on September 30, 2021 - Published by Cold Spring Harbor Laboratory Press REVIEW Molecular regulation of visual system development: more than meets the eye Takayuki Harada,1,2 Chikako Harada,1,2 and Luis F. Parada1,3 1Department of Developmental Biology and Kent Waldrep Foundation Center for Basic Neuroscience Research on Nerve Growth and Regeneration, University of Texas Southwestern Medical Center, Dallas, Texas 75235, USA; 2Department of Molecular Neurobiology, Tokyo Metropolitan Institute for Neuroscience, Fuchu, Tokyo 183-8526, Japan Vertebrate eye development has been an excellent model toderm, intercalating mesoderm, surface ectoderm, and system to investigate basic concepts of developmental neural crest (Fig. 1). The neuroectoderm differentiates biology ranging from mechanisms of tissue induction to into the retina, iris, and optic nerve; the surface ecto- the complex patterning and bidimensional orientation of derm gives rise to lens and corneal epithelium; the me- the highly specialized retina. Recent advances have shed soderm differentiates into the extraocular muscles and light on the interplay between numerous transcriptional the fibrous and vascular coats of the eye; and neural crest networks and growth factors that are involved in the cells become the corneal stroma sclera and corneal en- specific stages of retinogenesis, optic nerve formation, dothelium. The vertebrate eye originates from bilateral and topographic mapping. In this review, we summarize telencephalic optic grooves. In humans, optic vesicles this recent progress on the molecular mechanisms un- emerge at the end of the fourth week of development and derlying the development of the eye, visual system, and soon thereafter contact the surface ectoderm to induce embryonic tumors that arise in the optic system.
    [Show full text]
  • The Optic Disc in Glaucoma I: Classification
    Br J Ophthalmol: first published as 10.1136/bjo.60.11.778 on 1 November 1976. Downloaded from Brit. Y. Ophthal. (1976) 6o, 778 The optic disc in glaucoma i: Classification R. A. HITCHINGS Moorfields Eye Hospital, City Road, London AND G. L. SPAETH Wills Eye Hospital, Spring Garden Street, Philadelphia I9I30, USA The pale, cupped disc found in the final stages of deepening of the cup exposing the lamina cribrosa glaucomatous disease has been known from the (the 'laminar dots' sign (Reed and Spaeth, 1974)); time of Von Graefe (I854). Since then many and change in the course of the retinal vessel which observers have described changes that occur at the is seen as a sharp change in direction ('kinking' or optic disc in chronic, simple glaucoma. There has bayoneting (Reed and Spaeth, 1974)). In addition, been particular interest in changes which may the presence of localized regions of nerve fibre loss occur in the early stages of the disease. or 'gutters' has been demonstrated in some cases Studies of the normal population have shown of early glaucoma by Hoyt, Frisen, and Neuman that while there is a wide diversity in the appearance (1973). copyright. of the normal optic disc, in a single subject (Armaly, The aim of this paper was to assess systematically I969; Fishman, I970; Kolker and Hetherington, glaucomatous discs and look for those features that 1970; Portnoy, 1973) the two discs are remarkably are commonly associated. As a result, five different similar (Witsiuk, I966; Kronfeld, 1970; Woodruff, types of optic disc appearance are described. 1970; Portnoy, I973).
    [Show full text]
  • Peri-Papillary Atrophy
    Peri-Papillary Atrophy Maryke Neiberg, OD Author’s Bio Dr. Maryke Neiberg is an associate professor and full-time faculty at Western University College of Optometry in Pomona, California. Any opinions expressed in this article are her own and she has no financial interest to disclose. ________________________________________________________________________________________________ As optometrists, the optic nerve and rim tissue are of great interest to us. We look very carefully at the disc margins, color, and cup-to-disk ratio. We certainly look at the nerve fiber layer, and we may even pay special attention to the tissue directly surrounding the optic nerve. What can this area tell us? To answer this question, we would have to know a little about the unique anatomy of this area. This area is significant in terms of its vascular supply. It shares its vascular supply with that of the prelaminar, laminar and retrolaminar optic nerve. This section of the optic nerve and the peri-papillary area is supplied by the circle of Zinn-Haller, which represents anastomosis of the short posterior cilliary arteries.1 Usually, this area appears uniform in pigmentation with a clear demarcation between retina and optic nerve. In some cases, the optic nerve is surrounded by an area of hyper and hypopigmentation that may encircle the disc margin, commonly more visible in the temporal disc area.2 This pigment disturbance or mottling represents chorioretinal atrophy around the optic disc. This chorioretinal atrophy is known as peri-papillary or para-papillary atrophy (PPA), and is a relatively common finding. 3 Fluorescein or indocyanogreen angiopathy show characteristic filling patterns that can be used to further differentiate the peripapillary atrophy.
    [Show full text]
  • Determining the Location of the Fovea Centralis Via En-Face SLO and Cross-Sectional OCT Imaging in Patients Without Retinal Pathology
    Article Determining the Location of the Fovea Centralis Via En-Face SLO and Cross-Sectional OCT Imaging in Patients Without Retinal Pathology Archana A. Nair1, Rebecca Liebenthal1, Shefali Sood1, Grant L. Hom2, Marc E. Ohlhausen3,ThaisF.Conti2,CarolinaC.S.Valentim2, Hiroshi Ishikawa1, Gadi Wollstein1,JoelS.Schuman1, Rishi P. Singh2, and Yasha S. Modi1 1 Department of Ophthalmology, NYU Langone Health, New York University, New York, NY, USA 2 Center for Ophthalmic Bioinformatics, Cole Eye Institute, Cleveland Clinic, Cleveland, OH, USA 3 Case Western Reserve University, Cleveland OH, USA Correspondence: Yasha S. Modi, 222 Purpose: The purpose was to establish the position of the fovea centralis to the optic E. 41st Street, 3rd Floor,NewYork,NY nerve via en-face, near-infrared spectral domain optical coherence tomography (NIR- 10017, USA. e-mail: OCT) in healthy patients. This may shed light on physiological variability and be used [email protected] for studying subtle cases of foveal ectopia in macular pathology and after retinal detach- Received: July 15, 2020 ment. Accepted: January 3, 2021 Methods: SD-OCT data of 890 healthy eyes were retrospectively analyzed. Exclusion Published: February 17, 2021 criteria included axial myopia causing tilting of the optic disc, peripapillary atrophy >1/3 Keywords: fovea centralis; OCT; the width of the disc, macular images excluding greater than half of the optic disc, retina and patients unable to maintain vertical head positioning. Two independent reviewers measured the horizontal and vertical distance from the fovea to the optic disc center and Citation: Nair AA, Liebenthal R, Sood optic disc diameter via cross-sectional and en-face scanning laser ophthalmoloscopy S, Hom GL, Ohlhausen ME, Conti TF, OCT imaging.
    [Show full text]
  • Visual Field Defects from Optic Disc Melanocytoma
    RETINAL ONCOLOGY CASE REPORTS IN OCULAR ONCOLOGY SECTION EDITORS: CAROL L. SHIELDS, MD; AND SARA LALLY, MD Visual Field Defects From Optic Disc Melanocytoma BY EMIL ANTHONY T. SAY, MD; NEELEMA SINHA, MD; AND CAROL L. SHIELDS, MD ptic disc melanocytoma is a darkly pigment- CASE DESCRIPTION ed mass of the optic disc characterized A 33 year-old white man perceived an inferior visual histopathologically by Zimmerman and field defect in his right eye for 1 month. Visual acuity O Garron1 in 1962 as a uniform accumulation was 20/20 in both eyes, and intraocular pressures were of heavily pigmented cells with abundant cytoplasm, 19 mm Hg in both eyes. External and anterior segment small nuclei, and inconspicuous nucleoli, all of which are examination was unremarkable for both eyes. The benign cellular characteristics resembling ocular patient had brown irides without ocular melanocytosis. melanosis. In 2004, Shields and colleagues2 described the Fundus examination of the left eye was unremarkable, clinical features in a large cohort of 115 patients and but the right eye showed a pigmented lesion occupying related, that melanocytoma, although benign, has the the superior half of the optic disc from 10 to 2:30 o’clock, capacity to cause significant visual morbidity. They with extension into the juxtapapillary retina superotem- reported that at 10 and 20 years, respectively 18% and porally and lacking a choroidal component (Figure 1). 33% will lose two or more lines of vision by Kaplan-Meier Optic disc edema was present but retinal vessels were estimates.2 We report a case of optic disc melanocytoma not dilated or tortuous.
    [Show full text]
  • Your Glaucoma Eye Examination: Part 2 Your Optic Disc
    Your Glaucoma Eye Examination: Part 2 Your Optic Disc In the last issue of Eyelights we discussed What happens in glaucoma? the significance of eye pressure measurement Glaucoma causes damage to the nerve fibres in glaucoma care. This time we look at the and although the exact mechanism is still importance of examining the optic disc. unknown, there are two main theories that may explain glaucoma damage. What is the optic disc? The optic disc or optic nerve head is the The mechanical theory proposes it is the portion of the optic nerve which can be seen effect of eye pressure that damages the on examination of your eye. nerve fibres. It is thought that the nerve fibres are compressed at their exit point and The optic nerve begins in the eye and is continued, long-term damage causes the composed of 1,200,000 tiny nerve fibres that nerve fibres to atrophy or slowly die. send signals from the eye to the brain. It is these nerve fibres that can be seen, almost The vascular or “blood flow” theory proposes end-on, within the eye. that the pressure of the eye has an effect on blood flow to the optic nerve. If the nerve Each nerve fibre receives visual signals from fibres are starved of blood, nutrients and a certain area of retina and thus represents oxygen, then they undergo atrophy. an area of your field of vision. The loss of nerve fibres means that there will The typical optic disc is a circular structure be a corresponding loss or defect in the visual where the nerve fibres exit the eye.
    [Show full text]