Photopsia and a temporal visual field defect

Accepted Manuscript Photopsia and a temporal visual field defect Marcela Marsiglia, M.D., Ph.D., Jeffery G. Odel, M.D., Danielle S. Rudich, M.D., Stephen H. Tsang, M.D., Ph.D., Gordon T. Plant, MD (Cantab) FRCP FRCOphth PII:

S0039-6257(15)30059-X

DOI:

10.1016/j.survophthal.2015.11.003

Reference:

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To appear in:

Survey of Ophthalmology

Received Date: 9 November 2015 Accepted Date: 11 November 2015

Please cite this article as: Marsiglia M, Odel JG, Rudich DS, Tsang SH, Plant GT, Photopsia and a temporal visual field defect, Survey of Ophthalmology (2015), doi: 10.1016/j.survophthal.2015.11.003. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Title: Photopsia and a temporal visual field defect

Authors:

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Marcela Marsiglia, M.D., Ph.D.1 Jeffery G. Odel, M.D. 1 Danielle S. Rudich, M.D. 2

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Stephen H. Tsang, M.D., Ph.D. 1

Gordon T Plant MD (Cantab) FRCP FRCOphth 3,4,5

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Affiliations:

Department of Ophthalmology, Columbia University. New York, NY, USA.

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The Eye Care Group. New Haven, CT, USA.

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The National Hospital for Neurology and Neurosurgery, Queen Square,

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London, WC1N 3BG, UK

Moorfields Eye Hospital, City Road, London, EC1V 2PD, UK

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St Thomas' Hospital, Lambeth Palace Road, London, SE1 7EH, UK

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(In keeping with the format of a clinical pathologic conference, the abstract and key words will appear at the end of the article.)

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Case Report A 30-year-old white female ophthalmologist presented with intermittent photopsia in her left eye. Three days later, while doing a confrontation visual field on herself, she

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noticed a temporal visual field defect just below the horizontal in the same eye and

concurrently experienced flu-like symptoms. Her past medical and family history was

medications or recreational drugs.

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unremarkable. She was myopic and astigmatic in both eyes and was not taking any

On initial examination her visual acuity was 20/20 in both eyes. Amsler grid and the color vision testing were normal, and there was no relative afferent pupillary defect.

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Humphrey 30-2 threshold perimetry and 120 point screening visual field demonstrated a defect in the left eye and a normal field of vision in the right eye (Figure 1). The slit lamp examination was unremarkable, without cells in the anterior chamber or in the vitreous,

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and the intraocular pressure was 12 mmHg in both eyes. Fundus examination by multiple

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retinal consults and fluorescein angiography were unremarkable, (Figure 2)

What is your differential diagnosis? What study or studies would you perform?

Comments by Gordon Plant, MD

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The history is of a young female ophthalmologist who is a myope presenting with phosphenes (photopsia) of recent onset and a self-reported temporal visual field defect.

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There is a suggestion of a possible viral infection.

I would always try to establish to my satisfaction whether the phosphenes are originating in retina/optic nerve or cortex as patients sometimes interpret positive symptoms

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localized to a hemifield as being localized to an eye. Generally retinal phosphenes are

more visible in the dark, while occipital phosphenes appear equally bright whether in the

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dark or looking at the noon sky. If caused by posterior vitreous detachment (PVD) phosphenes are often influenced by eye movements or jolting.

One wonders why an ophthalmologist would wait three days before presenting with

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photopsia as the first priority is to exclude a retinal detachment. PVD would be possible, but would not be associated with a visual field defect, and this finding would make a retinal detachment also possible whether associated with a PVD or not. PVD becomes

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less common at younger age, but more common with increasing degrees of myopia (We are not told what was the refractive error in this case). There is also recent interest in

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vitreous traction giving rise to optic disc related visual field defects mimicking (or some say, the entire cause of) anterior ischemic optic neuropathy.

Another possible cause of a visual field defect with phosphenes localized to the defect is a local retinitis. These syndromes are named according to the location of the scotomas: acute zonal occult outer retinopathy, the idiopathic big blind spot syndrome and macular

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neuroretinitis. These may be post-viral phenomena, but as such the onset would be some weeks after a viral infection. Often there is no preceding illness. The phosphenes are clearly located to the scotoma, and the scotoma itself may be colored and visible in the

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dark as a colored shape. Thus patients are more aware of the precise dimensions of the scotoma than with optic nerve disorders or even occipital lesions where “filling in”

phenomena are common. Other types of retinitis and choroiditis such as the “white spot”

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syndromes cause phosphenes, but the visual loss is more diffuse, although there is some

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overlap with IBBSS and AZOOR.

The results of the automated perimetry illustrate well that perimetry that is “tailored” for a particular disorder (in this case glaucoma) can be quite insensitive at detecting other types of defect. The Humphrey 30 – 2 protocol has a handy extension nasally to pick up

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the nasal step, but there is insufficient information temporal to the optic disc. We are fortunate that the patient picked up the scotoma being more extensive than this, but now the 120 point screen has so few points in the temporal field that we cannot see what is

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going on.

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Another disorder which can cause acute optic disc related defects associated with phosphenes is optic disc drusen. Usually, however the phosphenes are provoked by eye movement, and there is an acute hemorrhage associated – either in the nerve fiber layer on the disc or a deeper peripapillary crescentic hemorrhage. The examination of the retina has ruled out a retinal detachment, but I am not sure if we are assured that there is no PVD. There are further comments, however, to be made

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regarding the optic discs. The right disc has no cup, and the left only a small cup. The right disc looks elevated to me, and there is an early branching patter in that the central retinal artery is not visible and is branching either deep in the disc, or perhaps there is a

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glial remnant of some kind on the disc. Any anomalous branching pattern should always lead to a hunt for buried optic disc drusen.

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Of greater relevance to the current clinical story, however, is the appearance of the left optic disc that is also anomalous, but in a different way. There is a branch artery at the

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upper pole of the disc that appears to be emerging probably as a very deep branch of the CRA and, even more significant, is the emergence of the main vessel sprout close to the nasal edge of the disc. This appearance is suggestive of prenatal sectoral disc hypoplasia and this would be associated with a visual field defect temporal to the blind spot, which is

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what the patient has herself discovered. The “topless” optic disc associated with inferior altitudinal visual field defects (superior segmental hypoplasia) is well recognized. Here we have the less evocative “sideless” optic disc. The patient is an ophthalmologist – we

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need to know if she has ever had a perimetric examination of any kind in the past. Please note though that an HFA 24-2 or 30-2 could well have been passed as normal.

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The patient needs kinetic perimetry. The visual field temporal to the optic disc is exceptional because the horizontal raphe of the retina does not extend beyond the optic disc. The retinal nerve fiber arrangement is radial so that defects from damage at the nasal border of the optic disc will result in fan-shaped defects quite unlike anything that would be seen in the other three quadrants. We need to know if this defect is disc related – in which case we will see a fan-shaped defect with its apex at the blind spot – or due to

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a local retinitis, in which case the defect will be geographic in the sense that its boundary will be created by a local retinal process that will not correspond to nerve fiber trajectory

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anatomy.

Case report (Continued)

The examination ruled out a retinal detachment, and the acute onset of photopsias

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and a temporal visual field defect related to the blind spot in the setting of a flu-like

syndrome suggested the diagnosis of Acute Idiopathic Blind Spot Enlargement Syndrome

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(AIBSES). 2,10 AIBSE may follow the Multiple Evanescent White Dot Syndrome. 3-5, 7, 9,11 Seeking evidence of an outer retinal disorder, her doctors ordered fundus autofluorescence, OCT of the macula, full-field electroretinogram (ERG) and an electrooculogram. All of these tests were normal on review.

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She was then referred for a multifocal ERG (mfERG) that showed no evidence of decreased amplitudes or delays in the area of the visual field defect. At the time of the mfERG, a swept source OCT of the macula and of the nerve fiber layer was performed.

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Fine line scans of the outer retina in the area of the visual field defect showed normal

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structure, with no evidence of outer retinal alterations.

At this point what is your diagnosis and how would you proceed? Comments (Continued) Comments by Dr. Plant I would not have carried out any of these tests without first doing a Goldmann visual field.

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Case Report (Continued) At this point we became involved. While interpreting the mfERG and the OCT,

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we reviewed the optic nerve photographs. Neither disc had a swollen nerve fiber layer,

but the right disc had a mildly anomalous vascular pattern. The retinal vessels on the left disc exited the nerve head on the nasal edge of the disc. The appearance of the left optic

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nerve head, in conjunction with the visual field defect, suggested nasal hypoplasia of the optic disc. The circle scan of the nerve fiber layer OS was markedly thinned nasally.

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Goldmann kinetic perimetry revealed a sector shaped, dense defect breaking out from the blind spot to the temporal periphery just below the horizontal in the left eye. (Figure 3: Goldmann VF) The swept source OCT fine line scan radially through the left nerve head extending into the nasal retina corresponding to area of the visual field defect showed an

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intact outer retina and a remarkably thinned nerve fiber layer compared to the right eye (Figure 3: ssOCT). Review of the OCT revealed peripapillary vitreous traction in the left eye (Figure 3: OCT of vitreous traction), accounting for the photopsia. After 2 years of

Discussion

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follow-up, the visual field defect remains unchanged.

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Our patient had nasal hypoplasia of the optic disc (NHOD)1 with coincidental

peripapillary vitreous traction causing photopsias, which contributed to the presentation mimicking AIBSES.1 Patients with optic nerve hypoplasia and other congenital optic neuropathies are typically unaware of their visual fields defects unless they are tested or in a visual circumstance that simulates confrontation visual fields.1 In this case, the patient performed a confrontation visual field test on herself after experiencing photopsia.

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Nasal hypoplasia of the optic disc (NHOD) is less frequently reported than generalized optic nerve hypoplasia and superior segmental optic nerve hypoplasia.6 Buchanan and Hoyt described NHOD in 1981 as consisting of 1) temporal wedge visual

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field defect(s) breaking out the blind spot, 2) nasal entrance of the central retinal artery, 3) thinning of the nasal nerve fiber layer, 4) decreased nasal retinal vascularity, 5) stable non-progressive visual field defect(s) and 6) unilateral or bilateral occurrence. No known

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systemic association or toxicity was noted.1 Ohguro et al reported 5 patients with NHOD, 3 patients with glaucoma and NHOD, and two patients with glaucoma with temporal

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wedge visual field defects resembling those seen in NHOD and noted an association with 5 diopters or more of myopia and a double pigment ring around the nasal disc.8 During the 1980s Goldmann kinetic visual field testing was being replaced by static threshold perimetry testing of the central 30 or 24 degrees. Central threshold visual

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field testing would miss the essential character of the temporal wedge defect and would reveal a slightly increased size of the blind spot that might be ignored, especially in myopic patients. Thus, unless Goldmann visual field testing was performed,

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technological change could prevent the recognition of this syndrome. The temporal wedge defect corresponds to the direct path taken by the nasal nerve

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fibers into the nasal optic nerve. OCT evidence of outer retinal alterations would be expected in AIBSES as well as in MEWDS, but were not present in this patient’s scans. 7,10

This is not a geographic visual field defect as seen in outer retinal disease as in the

AIBSES2 and has also been reported in glaucoma, though less frequently, with threshold static central testing. References

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1. Buchanan TA, Hoyt WF. Temporal visual field defects associated with nasal hypoplasia of the optic disc. British Journal of Ophthalmology. 1981;65(9):636-640. 2. Fletcher WA, Imes RK, Goodman D, Hoyt WF. Acute idiopathic blind spot

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enlargement. A big blind spot syndrome without optic disc edema. Arch Ophthalmol. 1988 Jan;106(1):44-9.

3. Furino C, Boscia F, Cardascia N, Alessio G, Sborgia C. Fundus autofluorescence

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and multiple evanescent white dot syndrome. Retina. 2009 Jan;29(1):60-3.

4. Hamed LM, Glaser JS, Gass JD, Schatz NJ. Protracted enlargement of the blind

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spot in multiple evanescent white dot syndrome. Arch Ophthalmol. 1989 Feb;107(2):194-8.

5. Jampol LM, Sieving PA, Pugh D, Fishman GA, Gilbert H. Multiple evanescent white dot syndrome: clinical findings. Arch Ophthalmol. 1984;102671- 674

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6. Kim R, Hoyt W, Lessell S, Narahara M. Superior segmental optic hypoplasia. Arch Ophthal. 1989:107:1312-1315.

7. Nguyen MH, Witkin AJ, Reichel E, Ko TH, Fujimoto JG, Schuman JS, Duker JS.

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Microstructural abnormalities in MEWDS demonstrated by ultrahigh resolution optical coherence tomography. Retina. 2007 Apr-May;27(4):414-8.

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8. Ohguro H, Ohguro I, Tsuruta M, Katai M, Tanaka S. Clinical distinction between nasal optic disc hypoplasia (NOH) and glaucoma with NOH-like temporal visual field defects. Clinical Ophthalmology. 2010;4:547-555. 9. Singh K, de Frank MP, Shults WT, Watzke RC. Acute idiopathic blind spot enlargement. A spectrum of disease. Ophthalmology. 1991 Apr;98(4):497-502. 10. Sugahara M, Shinoda K, Matsumoto SC, Satofuka S, Hanazono G, Imamura Y,

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Mizota A. Outer retinal microstructure in a case of acute idiopathic blind spot enlargement syndrome. Case Rep Ophthalmol. 2011 Apr 1;2(1):116-22. 11. Yenerel NM, Kucumen B, Gorgun E, Dinc UA. Atypical presentation of multiple

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evanescent white dot syndrome (MEWDS). Ocul Immunol Inflamm. 2008 May-

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Jun;16(3):113-5.

Key Words: photopsia, nerve fiber layer, swept-source OCT, visual field, visual field defect, temporal visual field defect, flu-like symptoms, hypoplasia of the

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optic nerve, nasal hypoplasia of the optic nerve.

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Abstract: A 30-year-old woman presented with intermittent photopsia, a temporal visual field defect below the horizontal in her left eye, and ‘flu-like symptoms. Slit lamp and fundus examination were unremarkable. Humphrey 302 threshold perimetry and 120 point screening visual field demonstrated blind spot enlargement of the left eye and a normal field in the right eye. Fundus autofluorescence, optical coherence tomography (OCT) of the macula, fullfield electroretinogram (ERG), electrooculogram and multifocal ERG were normal. Swept source OCT scan of the left optic nerve showed an intact outer retina, a remarkably thinned nerve fiber layer nasally, and peripapillary vitreous traction. Goldmann kinetic perimetry revealed a sector- shaped dense defect breaking out from the blind spot to the temporal periphery just below the horizontal in the left eye. The patient had nasal hypoplasia of the optic nerve and peripapillary vitreous traction.

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Figure Legends Figure 1: Humphrey visual field (top row), 120 points screening visual field (top row).

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Figure 2: Color fundus photos centered in the macula (left) and in the optic nerve (right).

Figure 3. Goldman visual field (top row), SS-OCT (middle row), OCT showing

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peripapillary vitreous traction (bottom row, left) and fundus autofluorescence

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(bottom row, right).

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