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Clinical & Refractive Optometry VOLUME 28, NUMBER 2, 2017

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A Comparison of In Vivo and In Vitro Osmometers for the Assessment of Dry Eye Disease Toxoplasma gondii: An Atypical Presentation of Optic Neuritis Spontaneous Malignant Glaucoma in a Phakic Patient Pars Planitis: A Review and Case Report A Case of Resolving Charles Bonnet Syndrome

Clinical&Refractive Optometry

Editorial Board • Volume 28, Number 2, 2017 Editor-in-Chief

Associate Editor

Associate Editor

Richard Maharaj, OD Toronto, Ontario

François Piuze, OD Quebec City, Quebec

Leonid Skorin, Jr., OD, DO, MS Albert Lea, Minnesota

Editors Emeriti Brad Almond, OD Calgary, Alberta

Barbara Caffery, OD Toronto, Ontario

John Jantzi, OD Vancouver, British Columbia

Yvon Rhéaume, OD Montreal, Quebec

Contributing Editors Scott D. Brisbin, OD Edmonton, Alberta

Gerald Komarnicky, OD Vancouver, British Columbia

Langis Michaud, OD Montreal, Quebec

Barbara Robinson, OD Waterloo, Ontario

Lorance Bumgarner, OD Pinehurst, North Carolina

Bart McRoberts, OD Vancouver, British Columbia

Rodger Pace, OD Waterloo, Ontario

Jacob Sivak, OD, PhD Waterloo, Ontario

Louis Catania, OD Philadelphia, Pennsylvania

Ron Melton, OD Charlotte, North Carolina

Maynard Pohl, OD Bellevue, Washington

Randall Thomas, OD Concord, North Carolina

Publication Staff Publisher Lawrence Goldstein

Managing Editor Mary Di Lemme

Senior Medical Editor Evra Taylor

Layout Editor Colin MacPherson

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Mission Statement Clinical & Refractive Optometry is a peer-reviewed professional journal dedicated to the publishing and disseminating of COPE approved CE credit scientific articles. The contents of each issue are composed of a mixture of original: state-of-the-art/technical, therapeutic/clinical, or practice management articles which are of particular interest to and use by practicing optometrists. Participants achieving 70% or more on the questionnaires that accompany each of the articles in the journal, will receive a course credit certificate.

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Clinical&Refractive Optometry

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Contents • Volume 28, Number 2, 2017

SCIENTIFIC STUDY 47 A Comparison of In Vivo and In Vitro Osmometers for the Assessment of Dry Eye Disease Henry Reis, MD; Stefanie Grenier, BSc; Daniela Albuquerque, MD CE CREDIT ARTICLES 50 Toxoplasma gondii: An Atypical Presentation of Optic Neuritis Nicky R. Holdeman, OD, MD; Steven Burnham, BSc; R. Alejandro Cruz, MD; Rosa A. Tang, MD ABSTRACT: The typical manifestation of toxoplasmosis is a retinochoroiditis, with a "headlight in the fog" appearance, due to dense inflammation of the vitreous; consequently, the diagnosis is often made clinically. This case describes a healthy 36-year-old Hispanic male who had an atypical presentation of ocular toxoplasmosis, with minimal vitritis and papillomacular involvement; thus serology was necessary for a definitive diagnosis. Treatment led to a rapid improvement in vision and ultimately a good prognosis.

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Spontaneous Malignant Glaucoma in a Phakic Patient Wess Jordan, OD; Lane Fujimoto, OD; Paul Vejabul, OD; Theresa Chong, OD; Michelle Matson, OD ABSTRACT: Malignant glaucoma is a rare form of secondary glaucoma characterized by an axially shallow anterior chamber angle and elevated intraocular pressure despite a patent iridectomy.

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Pars Planitis: A Review and Case Report Leonid Skorin, Jr. OD, DO, MS, FAAO, FAOCO Kristopher K. Sherrill, OD ABSTRACT: Pars planitis is an idiopathic intermediate uveitis characterized by a vitritis with snow banking and snowballs. The diagnosis of pars planitis is one of exclusion and the many known systemic associations of intermediate uveitis need to be ruled out. It is usually a chronic condition with exacerbations and remissions. Pars planitis has a bimodal peak occurrence in children and young adults.

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A Case of Resolving Charles Bonnet Syndrome Following Treatment of Intraocular Posterior Capsular Opacification Meggie N. Nguyen, OD, MPH ABSTRACT: Charles Bonnet syndrome (CBS) is a phenomena in which visual hallucinations are perceived in patients with ocular pathology and/or damage along the visual pathway, absent of other conditions. Patients with CBS are fully aware that the hallucinations are not real, and may find them to be pleasant. CBS is not well documented nor reported in the literature due to varying criteria for diagnosis, in addition to diagnoses made by different disciplines in medicine. From literature review, this is the first reported case of CBS secondary to a posterior capsular opacification after cataract surgery and some resolution after treatment.

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NEWS & NOTES

ISSN: 2371-7017; Date of Issue: April / May 2017

Courtesy of: Dr. Leonid Skorin, Jr. High magnification of classic snowball appearance, right eye.

Scientific Study A Comparison of In Vivo and In Vitro Osmometers for the Assessment of Dry Eye Disease Henry Reis, MD; Stefanie Grenier, BSc; Daniela Albuquerque, MD

ABSTRACT

INTRODUCTION

Purpose: To evaluate the efficacy of two commercially available osmometers in the assessment of dry eye patients. Methods: We included 100 eyes of 50 patients diagnosed with dry eye disease (DED). The study assessed 20 patients with mild DED and 30 with moderate DED at Focus Eyecare Centre, a full-scope optometric practice located in Burnaby, BC, Canada.

All subjects completed the Ocular Surface Disease Index (OSDI) questionnaire and underwent comprehensive examinations including tear osmolarity [TearLab® (TearLab Corp) and i-Pen® (I-MED Pharma Inc.)], Keratograph® 5M (Oculus Inc.) dry eye assessment, slit lamp examination and SM Tube.

Subjects were divided into 2 groups: Group 1 had the osmolarity assessed with i-Pen, followed by a TearLab osmolarity measurement 30 minutes later. Group 2 was initially assessed with TearLab and a second osmolarity measurement with i-Pen was acquired 30 minutes after the initial one. Results: Tear osmolarity values (mOsm/L) for Group 1 were 321.10 +/- 34.50 for i-Pen and 321.40 +/- 35.00 for TearLab. Group 2 yielded osmolarity values of 323.78 +/- 30.05 for i-Pen and 332.82 +/- 29.10 for TearLab. Patients diagnosed with mild DED presented with average tear osmolarity readings of 307.67 with i-Pen and 316.52 with TearLab. Those with moderate DED averaged 332.28 with the i-Pen and 334.16 with the TearLab. Conclusions: Both devices showed similar performance in a clinical setting for the diagnosis of dry eye disease. Tear osmolarity values were comparable in mild dry eye patients, but i-Pen acquired lower measurements in moderate dry eyes when compared to TearLab. The order in which these tests were performed also influenced the results in Group 2, due to reflex tearing induced by TearLab. Tear osmolarity should be considered as the key biomarker in the diagnosis of DED.

Dry eye disease (DED) is a complex, multifactorial condition that affects the anatomy and physiology of the eyelids and ocular surface. Intrinsically an inflammatory condition, it disrupts the lacrimal functional unit and leads to tear film instability and ocular surface damage. Patients commonly experience symptoms that range from reduced visual acuity to discomfort, pain and epiphora.

Approximately 5% to 30% of adults over 50 years of age present with DED according to the 2007 International Dry Eye Workshop.1 This chronic aggression to the cornea leads to an inflammatory cascade that results in goblet cell apoptosis and increased tear film osmolarity.

A variety of studies have been published which demonstrate that including tear osmolarity assessment in every comprehensive dry eye protocol is of paramount importance (Fig. 1).2-16 Furthermore, some authors postulate that, in patients with dry eye symptoms who present with normal tear osmolarity values (290 mOsm/L or lower with an inter-eye difference inferior to 5 mOsm/L), a cause other than dry eyes should be present 90% of the time.4 The Canadian Association of Optometrists has stated in its National Dry Eye Disease Guidelines for Canadian Optometrists that osmolarity is the most accurate and objective test for dry eye disease. The aforementioned rationale leads to a logical debate; while tear osmolarity is an integral component in current DED protocols, studies comparing commercially available tear film osmometers in a clinical setting are scarce. This study seeks to elucidate

H. Reis; S. Grenier; D. Albuquerque — Focus Eyecare Centre, Burnaby, BC Correspondence: Dr. Henry Reis, Focus Eyecare Centre, 104-7188 Kingsway, Burnaby, BC V5E 1G3; E-mail: focus@myoptometrist.ca This article has been peer reviewed and accepted for publication in May 2017.

Osmolarity All Studies 1978-2005

Probability

Normal eyes Mean = 302.2 SD = 9.7

260

Dry eyes Mean = 326.9 SD = 22.1

280

300

320

340

360

380

400

Osmolarity (mOsm/L) Fig. 1 Distribution of osmolarity (mOsm/L) in studies from 1978 to 2005.8

A Comparison of In Vivo and In Vitro Osmometers for the Assessment of Dry Eye Disease — Reis et al

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370

i-Pen OD i-Pen OS TearLab OD TearLab OS

350

330

310

290

270 11 11 11 12 12 12 12 12 13 13 13 14 14 14 14 14 15 15 15 15 16 17 17 17 18 18 18 18 18 18 19 19 19 19 19 19 20 20 20 20 20 21 21 21 21 22 22 23 24 24

Fig. 2 Osmolarity values (mOsm/L) versus OSDI scores

if TearLabÂŽ (TearLab Corp, San Diego, CA) and i-PenÂŽ (I-MED Pharma Inc., Montreal, QC) yield comparable results in a clinical setting, in patients diagnosed with mild to moderate dry eye disease.

METHODS The authors have included 50 patients referred to the Focus Eyecare Dry Eye Centre (n=100). Subjects ranged from age 30 to 65. 21 were male (42%) and 29 female (58%).

The selection criteria included both objective and subjective examinations traditionally used for the diagnosis of DED17: 1. Ocular Surface Disease Index (OSDI) 2. Keratograph 5M Non-Invasive Break Up Time (K5M NIBUT)) 3. Slit lamp assessment (SLE) of corneal and conjunctival staining (Oxford).

4. SM Tube.

OSDI from 9 to 16 and from 17 to 24 were included in the study. Patients with normal OSDI or values of 25 and higher were excluded according to the Modified Thompson Tau method. K5M NIBUT was considered positive when values ranged from 1 to 9. Patients with unmeasurable BUT or ranging 10 and higher were excluded from the study. SLE Oxford Protocol was utilized to include patients ranging from Grade I to IV. SM Tube was also utilized to confirm K5M NIBUT findings and patients ranging from Grade 1 to 5 were considered for the study. It is important to point out that tear osmolarity was not included in the selection criteria to prevent selection bias.

The exclusion criteria eliminated from the study subjects with positive history for ocular surgery, active ocular infection, use of contact lens and/or artificial tears (preserved or not) in the previous 24 hours.

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Subjects had the severity of their condition assessed and were subsequently classified as having mild, moderate and severe DED. Severe DED patients were not included in the study mainly due to the criteria that excluded patients who had used artificial tears within the last 24 hours.

The study included 20 patients with mild DED and 30 with moderate DED (n=100). Subjects were divided into 2 groups. Group 1 underwent tear osmolarity assessments with i-Pen and TearLab, 30 minutes apart. Group 2 was tested in reverse order, also respecting a 30-minute interval between measurements.

A trained optometric technician with CCOA designation ensured proper technique, and the devices were utilized according to their manufacturer manuals.

RESULTS Tear osmolarity values (mOsm/L) for Group 1 were 321.10 +/- 34.50 for i-Pen and 321.40 +/- 35.00 for TearLab. Group 2 yielded osmolarity values of 323.78 +/- 30.05 for i-Pen and 332.82 +/- 29.10 for TearLab. Patients diagnosed with mild DED presented with average tear osmolarity readings of 307.67 with i-Pen and 316.52 with TearLab. Those with moderate DED averaged 332.28 with the i-Pen and 334.16 with the TearLab (Fig. 2). Inter-eye difference averaged 6.66 with i-Pen and 6.74 with TearLab. This value was lower among mild dry eye patients, with i-Pen yielding 6.3 and TearLab 5.25. Moderate DED patients showed a higher inter-eye osmolarity difference of 6.9 measured with i-Pen and 7.73 with TearLab (Fig. 3) No statistically significant differences between the devices were found for the group mean and group standard deviation (paired t-test, p=0.04).

The osmometers provided results that were statistically aligned with OSDI scores and SLE assessment, but did not show correlation with K5M NIBUT and SM Tube measurements.

18 i-Pen 16 TearLab Linear (TearLab) 14 Linear (i-Pen)

12

10

8

6

4

2

0 11 11 11 12 12 12 12 12 13 13 13 14 14 14 14 14 15 15 15 15 16 17 17 17 18 18 18 18 18 18 19 19 19 19 19 19 20 20 20 20 20 21 21 21 21 22 22 23 24 24

Fig. 3 Absolute inter-eye difference in osmolarity (mOsm/L) versus OSDI

The coefficient of variation CV%=(SD/Xbar)100 was 3.2% and 4.1% for i-Pen and TearLab, respectively, indicating good method performance.

It is important to note that Group 1 did not present a significant change in value when having the second test performed, whereas Group 2 showed a statistically significant lower mOsm/L even after a 30-minute interval. The authors attribute this finding to potential reflex tearing induced by TearLab. Subjects assessed with i-Pen, in contrast, presented with minimal reflex tearing. Inter-eye mOsm/L difference corresponded to OSDI scores, with similar significance between the two devices. Mean values differed 0.52, however TearLab showed greater inter-eye variance when compared to i-Pen, with 2.14 and 0.908, respectively.

2.

CONCLUSION

10.

TearLab and i-Pen, two commercially available osmometers, showed consistent correlation with OSDI and slit lamp examination, proving to be reliable and objective devices in DED management. No statistically significant difference in performance between the osmometers could be demonstrated in a clinical setting under a strict study protocol. Adequate technique and rigorous patient selection should be observed in order to achieve reliable results. The authors advise optometrists and ophthalmologists to include tear osmolarity in the assessment of every dry eye patient. ❏

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6.

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REFERENCES 1.

The definition and classification of dry eye disease: report of the Definition and Classification Subcommittee of the International Dry Eye WorkShop (2007). Ocul Surf 2007; 5(2): 75-92.

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Maharaj RL. In vivo ocular surface osmolarity in a dry eye population. Clinical and Refractive Optometry 2017; 28(1): 3-6.

Rocha G, Gulliver R, Borovik A, Chan CC. Randomized, masked, in vitro comparison of three commercially available tear film osmometers. Clinical Ophthalmology 2017; 11: 243-248.

Karpecki PM. Diagnosis and treatment of ocular surface conditions: focus on dry eyes. Clinical and Refractive Optometry 2016; 27(6): 236-241.

Versura P, Profazio V, Campos EC. Performance of tear osmolarity compared to previous diagnostic tests for dry eye diseases. Current Eye Research 2010; 35(7): 553-564.

Barrus BJ. The science of dry eye hyperosmolarity. Clinical and Refractive Optometry 2016; 27(5): 202-206.

Lemp MA, Bron AJ, Baudouin C, Benítez Del Castillo JM, Geffen D, Tauber J, et al. Tear osmolarity in the diagnosis and management of dry eye disease. Am J Ophthalmol. 2011; 151(5): 792-798 e1.

Baudouin C, et al. Role of hyperosmolarity in the pathogenesis and management of dry eye disease. Ocul Surf 2013; 11: 246-258.

Avinashi P. Principles of dry eye disease: diagnosis, treatment and management. Clinical and Refractive Optometry 2016; 27(4): 127-132.

Moore JE, Vasey GT, Dartt DA, McGilligan VE, Atkinson SD, Grills C, et al. Effect of tear hyperosmolarity and signs of clinical ocular surface pathology upon conjunctival goblet cell function in the human ocular surface. Invest Ophthalmol Vis Sci 2011; 52(9): 6174-6180.

Maharaj R. Advancing the diagnostic use of tear osmolarity in primary eye care: part 1. Clinical and Refractive Optometry 2016; 27(1): 22-26.

Maharaj R. Advancing the diagnostic use of tear osmolarity in primary eye care: part 2. Clinical and Refractive Optometry 2016; 27(1): 29-33.

Tomlinson A, McCann LC, Pearce EI. Comparison of human tear film osmolarity measured by electrical impedance and freezing point depression techniques. Cornea 2010; 29(9): 1036-1041.

Tauber S. Treating and managing dry eye. Clinical and Refractive Optometry 2016; 27(1): 14-17.

Narayanan N. Osmolarity: a diagnostic test for dry eyes. Review of Optometry 2011.

Suzuki M, Massingale ML,Ye F, Godbold J, Elfassy T,Vallabhajosyula M, et al. Tear osmolarity as a biomarker for dry eye disease severity.

Invest Ophthalmol Vis Sci 2010; 51(9): 4557-4561.

Korb DR. Survey of preferred tests for the diagnosis of the tear film and dry eye. Cornea 2000; 19(4): 483-486.

A Comparison of In Vivo and In Vitro Osmometers for the Assessment of Dry Eye Disease — Reis et al

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CLICK HERE TO PRINT THIS CE CREDIT ARTICLE AND TEST

Clinical & Refractive Optometry is pleased to present this continuing education (CE) article

by Dr. Nicky R. Holdeman et al entitled Toxoplasma gondii: An Atypical Presentation of Optic Neuritis. In order to obtain a 1-hour Council of Optometric Practitioner Education (COPE) approved CE credit, please refer to page 56 for complete instructions.

Toxoplasma gondii: An Atypical Presentation of Optic Neuritis Nicky R. Holdeman, OD, MD; Steven Burnham, OD; R. Alejandro Cruz, MD; Rosa A. Tang, MD, MPH, MBA

ABSTRACT Toxoplasma gondii is a parasite whose natural host is the cat. Ocular toxoplasmosis can be categorized into two forms of infections: congenital, where an infant is infected in utero; and acquired, where an individual is typically infected by ingesting food contaminated with T. gondii oocytes. Although acquired infections are uncommon in the United States, toxoplasmosis should remain in the differential diagnosis of an infectious optic neuritis.

The typical manifestation of toxoplasmosis is a retinochoroiditis, with a "headlight in the fog" appearance, due to dense inflammation of the vitreous; consequently, the diagnosis is often made clinically.

This case describes a healthy 36-year-old Hispanic male who had an atypical presentation of ocular toxoplasmosis, with minimal vitritis and papillomacular involvement; thus serology was necessary for a definitive diagnosis. Treatment led to a rapid improvement in vision and ultimately a good prognosis.

N.R. Holdeman, S. Burnham — Medical Eye Service, University Eye Institute, University of Houston, Houston, TX; R.A. Cruz, R.A. Tang — Multiple Sclerosis Eye Center for Analysis, Research and Education, University Eye Institute, University of Houston, Houston, TX. Correspondence to: Dr. R.A. Cruz, 4901 Calhoun Blvd., Houston, TX 77204-2020; E-mail acruz@neuroeye.com Disclosures: Dr. R. Alejandro Cruz reports no disclosures. S. Burnham reports no disclosures. Dr. Rosa Tang serves as principal investigator for NORDIC Pseudotumor Cerebri Clinical Trial and receives research support

by NFI, NIH, Roosevelt Hospital NY, NORDIC, Biogen, Teva, Bayer, Eisai.

Dr. Tang receives honoraria from serving on the advisory committee membership of Amprya, Teva and Merck. Dr. Tang receives honoraria as a member of the speaker’s bureau for Biogen, Serona, Bayer, and Teva.

Dr. Nicky R Holdeman is a medical consultant for Bausch & Lomb Pharmaceuticals and the Cooper Institute in Dallas, Texas. Dr. Holdeman receives research support from Allergan Pharmaceutical and SARCode Bioscience, Inc. This article has been peer-reviewed.

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INTRODUCTION Toxoplasma gondii is an obligate intracellular protozoan parasite. Nearly one third of humanity has been exposed to this organism; however, only 1% to 3% will develop ocular manifestations.1-3 Despite the relatively low rate of ocular involvement, T. gondii is one of the leading causes of posterior uveitis and is the leading cause of infectious retinochoroiditis worldwide.1,2 The natural host of T. gondii is the feline family. Via fecal matter, cats release oocysts into the environment, which can remain viable for extended periods. Humans typically acquire infection by ingesting the oocysts found in contaminated soil, fruits, vegetables and undercooked meats or through congenital transmission in utero.4 The oocysts develop into tachyzoites, the primary infectious form of T. gondii, which cause acute tissue destruction and inflammation. Tachyzoites then transform into bradyzoites, which form protective cysts and may remain dormant until a precipitating event causes reactivation.3

CASE STUDY A 36-year-old, healthy Hispanic male presented to a local optometrist with complaints of an acute onset of constant, blurred vision and “black specks” in the left eye that started earlier that morning. Prior to this event, the patient had never experienced any vision abnormalities. His family ocular and medical history was unremarkable. He was not using any medications and had no known drug or environmental allergies. The patient was a police officer who had never smoked and consumed alcohol only occasionally.

He denied any sexually transmitted diseases.

Unaided acuities were 6/4.5- (20/15-) in the right eye and 6/6- (20/20-) in the left; refraction yielded a low simple hyperopia in both eyes. Gross inspection and slit lamp examination were unremarkable, with no cells detected in the anterior chamber or in the vitreous in either eye. Ophthalmoscopy revealed no abnormalities in the right eye but disclosed optic nerve edema in the left eye. Humphrey visual field, (HVF) 24-2 SITA Fast of the right eye was normal, but did show a generalized depression, with an enlarged blind spot in the left eye (Fig. 1).

Following the examination, further discussion revealed a history of cold sores, with a recent labial infection. The patient also reported that he had experienced flu-like symptoms, with a low-grade fever, which had

Fig. 1 Initial Humphrey visual field 24-2 SITA-Fast, performed by the referring optometrist, showed an overall depression and enlarged blind spot in the left eye. The right visual field was unremarkable and was not included.

persisted for approximately two months. In addition, he noted that his daughter and his stepdaughter both had cats, but he denied changing the litter or having any close contact with either pet. Lastly, he acknowledged that he enjoyed hunting and had recently handled deer meat. The patient was referred to his primary care physician (PCP), with a recommendation to obtain an MRI of the brain and orbits.

An MRI, with and without contrast, was performed as well as a CBC, CRP, ESR, thyroid panel, lipid profile and hepatic function testing. The MRI of the brain and orbits was unremarkable and clinical pathology revealed no abnormalities. Over the course of the following week, the patient's symptoms gradually worsened but there were no significant changes noted upon examination.

A referral was then made to a neuro-ophthalmologist, who noted a 0.6 log unit afferent pupillary defect (APD), 1+ vitreal cells, and 4+ optic nerve edema in the left eye; examination of the right eye was unremarkable (Fig. 2).

A visually evoked potential showed normal implicit times and amplitudes in both eyes. A multifocal electroretinogram (mfERG) was normal in the right eye but there was a reduced response in the inferior temporal field in the left eye (Fig. 3). Optical coherence tomography (OCT) of the right eye was normal but the left eye had significant subretinal fluid in the superior papillomacular bundle (Fig. 4). Intravenous fluorescein angiography (IVFA) showed no abnormalities in the right eye, but disclosed diffuse leakage of the left optic disc, with no vascular or macular irregularities (Fig. 5). With evidence of an inflammatory process, a chest X-ray, PPD, ACE levels, RPR, FTA-ABS, HIV testing, Bartonella antibodies, and Toxoplasma antibodies were requested. The patient was prescribed oral azithromycin while awaiting the results of the ancillary tests.

Fig. 2 At the initial neuro-ophthalmology visit, there was massive edema of the left optic nerve, with peri-papillary and pre-retinal hemorrhages, as well as venous engorgement and a large area of serous fluid superotemporally suggestive of neuroretinitis. The right eye was unremarkable.

Twelve days after the initial presentation, the patient requested to be seen emergently due to a sudden change in vision. His BCVAs remained 6/6 (20/20) in the right eye, but had declined to 6/60 (20/200), with eccentric fixation, in the left. The APD in the left eye had progressed to 0.9 log units and color perception by HRR plates was decreased in the left eye. Amsler grid showed a defect in the temporal half of the left eye and a HVF 24-2 SITA Fast disclosed a generalized, relative, deep scotoma centered on the blind spot in the left eye (Fig. 6). The visual field of the right eye had low test reliability, but showed no significant defects. A repeat OCT demonstrated persistent subretinal fluid in the papillomacular bundle of the left eye, while the right eye remained unremarkable (Fig. 7).

The patient was admitted to the hospital for observation. Soon thereafter, testing revealed T. gondii IgG and IgM antibodies, indicating a recently acquired toxoplasmosis infection. The patient was given a five-day course of pyrimethamine, sulfadiazine, leucovorin calcium, methylprednisolone succinate, valacyclovir, and pantoprazole. The patient was referred to the Center for Disease Control for evaluation of long-term antibiotic treatment.

Toxoplasma gondii: An Atypical Presentation of Optic Neuritis — Holdeman et al

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Fig. 3 Multifocal electroretinogram of the right eye was normal but demonstrated reduced responses centrally and in the inferior-temporal quadrant of the left eye.

Fig. 4 Optical coherence tomography of the right eye was normal but the left eye had significant elevation of the optic nerve, with subretinal fluid in the superior papillomacular bundle.

The patient was last seen for a scheduled follow-up evaluation four months after the initial onset. Best corrected visual acuities were now 6/6 (20/20) in both eyes. There was significant reduction in the optic nerve edema of the left eye, resolution of the pre-retinal and retinal hemorrhages, with mild residual retinal scarring (Figs. 8, 9). Humphrey visual fields demonstrated improvement in the left eye compared to prior testing

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(Fig. 10). Initial findings of what may have progressed to a necrotic and non-resolving neuro-retinitis secondary to a T. gondii infection were almost entirely resolved.

DISCUSSION Although acquired T. gondii is rare in the United States, it is still a potential cause of infectious optic neuritis, as demonstrated by this case. Clinicians often diagnose

Fig. 5 Intravenous fluorescein angiography showed no abnormalities in the right eye, but disclosed diffuse leakage of the left optic disc, with no vascular or macular irregularities.

Fig. 6 Humphrey visual field 24-2 SITA Fast of the left eye showing a deep, arcuate defect surrounding the left blind spot and encroaching central vision

ocular toxoplasmosis based on the signs of active retinochoroidal lesions, vitreous inflammation, and a “headlight in the fog� appearance, all of which are commonly found in immunocompetent patients.1,5,6 However, this healthy male had minimal vitritis, with optic nerve involvement, which is an unusual presentation for toxoplasmosis.5 Despite lack of significant vitreous inflammation, there is not necessarily reason to suspect that every patient is immunocompromised. It has been reported that deep retinal lesions (i.e., punctate outer retinal toxoplasmosis) are often associated with less vitreous inflammation, as in this case.5 This atypical manifestation required the exclusion of other infectious entities that can produce an optic neuritis, such as toxocariasis, cat scratch disease, tuberculosis, sarcoidosis, Lyme disease, syphilis, herpes, or cytomegalovirus.6,7 Serology is supportive and the presence of IgM antibodies, detected by IFA and

ELISA testing, indicates the presence of a recent infection.

However, these tests are not diagnostic in every situation, as 20% to 70% of the general population can show positive titers.8 It may be necessary to confirm difficult or atypical cases with paracentesis of the anterior chamber, using polymerase chain reaction, to detect T. gondii DNA in the aqueous.1,8 Indications for treatment vary considerably between practitioners,9 despite the fact that more than 94% of patients with ocular toxoplasmosis will have permanent visual defects, per automated perimetry.10 Many clinicians opt to monitor a peripheral lesion in an immunocompetent individual with good acuities, as the infections are typically self limiting.1,9 However, reduced acuities, associated with lesions threatening major vessels, the optic nerve, and/or perifoveal areas, are indicators for treatment, as was seen in this case.1,8 Treatment of vision threatening toxoplasmosis typically includes some combination of antimicrobial and anti-parasitic agents, although their efficacy has not been proven in clinical trials.11 Choices often involve a multidrug approach including pyrimethamine, with some combination of azithromycin, sulfadiazine, clindamycin, or sulfamethoxazole/trimethoprim. Patients treated with pyrimethamine should also receive folinic acid (leucovorin calcium) to minimize bone marrow suppression.1,7,11,12 The use of oral prednisone, with concurrent antimicrobial treatment, may be beneficial in immunocompetent patients, especially when there is optic nerve and/or macular involvement.1,7 However, one must consider that systemic therapy is expensive and can have significant side effects. A viable and potentially safer option for patients who cannot tolerate systemic therapy, is a combination of clindamycin and dexamethasone given as an intravitreal injection.1,13 Longer-term treatment of ocular toxoplasmosis is aimed at reducing the severity and frequency of recurrences. Use of intermittent trimethoprim/sulfamethoxazole for several

Toxoplasma gondii: An Atypical Presentation of Optic Neuritis — Holdeman et al

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Fig. 7 Repeat optical coherence tomography documented persistent sub-retinal fluid in the papillomacular bundle, with a serous retinal detachment of the left eye, while the right eye remained within normal limits.

Fig. 8 Several weeks following treatment, OCT showed significant reduction of optic nerve edema, mild retinal scarring, and gradual dissipation of serous fluid in the left eye.

months has been shown to reduce the rate of recurrence.1,14Management of choroidal neovascularization includes photodynamic therapy or anti-VEGF agents (intravitreal bevacizumab or ranibizumab).1,15-17

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The ocular complications associated with toxoplasmosis include risk of recurrence, visual field defects, retinal detachment, sub-retinal neovascular membranes, cataracts, cystoid macular edema, papillitis, glaucoma,

Fig. 9 Fundus photography of the left eye, four months after onset of symptoms, showed resolution of optic nerve edema, fading of pre-retinal and retinal hemorrhages and mild residual retinal scarring.

Fig. 10 Humphrey visual field 24-2 SITA Fast showed a persistent inferior arcuate defect in the left eye, but was somewhat improved compared to prior tests.

and chronic posterior uveitis.1,7 Since this patient had a newly acquired lesion, with optic nerve and macular involvement, frequent progress exams were indicated to properly manage potential complications. Fortunately, early diagnosis and intervention led to a good visual prognosis.

9.

10.

11.

CONCLUSION Although acquired ocular toxoplasmosis is uncommon in the United States, infection via contaminated food or water does occur.18,19 Consequently, clinicians should consider toxoplasmosis in cases of atypical optic neuritis and use appropriate tests to assist in the diagnosis and management. â??

12.

13.

REFERENCES 1.

2.

3.

4.

5.

6.

7.

8.

Holdeman NR. Ocular Toxoplasmosis. In: Onofrey BE, Skorin L, Holdeman NR, eds. Ocular Therapeutics Handbook.

Philadelphia, PA: Wolters Kluwer Health Lippincott Williams & Wilkins, 2011 p. 477-481.

Petersen E, Kijlstra A, Stanford M. Epidemiology of ocular toxoplasmosis. Ocul Immunol Inflamm 2012; 20: 68-75.

Holland GN. Ocular toxoplasmosis: a global reassessment. Part 1: epidemiology and course of disease. Am J Ophthalmol 2003; 136: 973-986.

Jones JL, Dubey JP. Foodborne toxoplasmosis. Food Safety 2012; 55(6): 845-851.

Wakefield D, Cunningham Jr. ET, Pavesio C, Garweg JG,Zierhut M. Controversies in ocular toxoplasmosis. Ocul Immunol Inflamm 2011; 19(1): 2-9.

Bodaghi B, Touitou V, Fardeau C, Paris L, LeHoang P. Toxoplasmosis: new challenges for an old disease. Eye 2012; 26(2): 241-244.

Holland GN. Ocular toxoplasmosis: A global reassessment. Part 2: disease manifestations and management. Am J Ophthalmol 2004; 137(1): 1-17.

Ongkosuwito JV, Bosch-Driessen EH, Kijlstra A, Rothova A. Serologic evaluation of patients with primary and recurrent ocular toxoplasmosis for evidence of recent infection. Am J Ophthalmol 1999; 128(4): 407-412.

14.

15.

16.

17.

18.

19.

Holland GN, Lewis KG. Perspective. An update on current practices in the management of ocular toxoplasmosis. Am J Ophthalmol 2002; 134(1): 102-114.

Scherrer J, Iliev ME, Halberstadt M, Kodjikian L, Garweg JG. Visual funtion in human ocular toxoplasmosis. Br J Ophthalmol 2007; 91(2): 233-236.

Torre A, Stanford M, Curi A, Jaffe GJ, Gomez-Marin JE. Therapy for ocular toxoplasmosis. Ocul Immunol Inflamm 2011; 19(5): 314-320.

Bosch-Driessen LH, Verbraak FD, Suttorp-schulten MSA, Van Ruyven RLJ, Klok AM, Hoyng CB, Rothova A. A prospective, randomized trial of pyrimethamine and azithromycin vs pyrimethamine and sulfadiazine for the treatment of ocular toxoplasmosis. Am J Ophthalmol 2002; 134(1): 34-40.

Lasave AF, Diaz-Llopis M, et al. Intravitreal clindamycin and dexamethasone for zone 1 toxoplasmic retinochoroiditis at twenty-four months. Ophthalmology 2010; 117(9): 1831-1838.

Silveira C, Belfort R, Muccioli C, Holland GN, Victora CG, Horta BL, Nussenblatt RB. The effects of long-term intermittent trimethoprim/sulfamethoxazole treatment on recurrences of toxoplasmic retinochoroiditis. Am J Ophthalmol 2002; 134(1): 41-46.

Mansour AM, Arevalo JF, Fardeau C, Hrisomalos EN, Chan WM, Lai TYY, Kurup SK. Three-year visual and anatomic results of administering intravitreal bevacizumab in inflammatory ocular neovascularization. Can J Ophthalmol 2012; 47(3): 269-274.

Green WR. Toxoplasmosis-associated neovascular lesions treated successfully with ranibizumab and antiparasitic therapy. Arch Ophthalmol 2008; 126(8): 1152-1156.

Wirthlin R, Song A, Song J, Rosenfeld PJ. Verteporfin photodynamic therapy of choroidal neovascularization secondary to ocular toxoplasmosis. Arch Ophthalmol 2006; 124(5): 741-743.

Burnett AJ, Shortt SG, Isaac-Renton J, King A, Werker D, Bowie WR. Multiple cases of acquired toxoplasmosis retinitis presenting in an outbreak. Ophthalmology 1998; 105(6): 10321037.

Balasundaram MB, Andavar R, Palaniswamy M, Venkatapathy N. Outbreak of acquired ocular toxoplasmosis involving 248 patients. Arch Ophthalmol 2010; 128(1): 28-32.

Toxoplasma gondii: An Atypical Presentation of Optic Neuritis — Holdeman et al

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QUESTIONNAIRE Toxoplasma Gondii: An Atypical Presentation of Optic Neuritis Nicky R. Holdeman, OD, MD; Steven Burnham, OD; R. Alejandro Cruz, MD; Rosa A. Tang, MD 1. ❑ ❑ ❑ ❑

Which of the following is the natural host for Toxoplasma Gondii?

Hamster Bird Dog Cat

2. ❑ ❑ ❑ ❑

What percentage of people exposed to T. gondii will develop ocular manifestations?

1% to 3% 3% to 4% 4% to 6% 7% to 9%

Clinical and Refractive Optometry 28:2, 2017

Cats release oocysts in the environment via what means?

Saliva Skin Fecal matter Urine

4. ❑ ❑ ❑ ❑

All of the following describe the patient at first presentation in the Case Report, EXCEPT: Low simple hyperopia in both eyes Unremarkable slit lamp examination Optic nerve edema in the left eye Enlarged blind spot in the right eye

5. ❑ ❑ ❑ ❑

In the Case Report presented, what was the patient’s BCVA in the right eye at hospital Emergency?

6/4.8 (20/16) 6/6 (20/20) 6/30 (20/100) 6/60 (20/200)

6. ❑ ❑ ❑ ❑

In the Case Report presented, when the patient was hospitalized for observation, testing revealed which antibodies?

IgM IgA IgG IgE

7. ❑ ❑ ❑ ❑

What percentage of the population can show positive IgM titers?

5% to 50% 15% to 65% 20% to 70% 30% to 85%

8. ❑ ❑ ❑ ❑

In the Case Report presented, what were the patient’s BCVAs? 6/3.8 (20/12.5) OD 6/6 (20/20) OU 6/7.5 (20/25) OD 6/12 (20/40) OU

9.

What percentage of patients with ocular toxoplasmosis will have permanent visual defects, per automated perimetry?

68% 75% 86% 94%

❑ ❑ ❑ ❑

10. In the Case Report presented, all of the following statements describe the patient’s situation at initial optometric examination, EXCEPT: ❑ He had blurred vision ❑ He had no previous vision abnormalities ❑ He had “Black specks” in the right eye ❑ He had a family history of glaucoma

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Clinical & Refractive Optometry is pleased to present this continuing education (CE) article by Dr. Wess Jordan et al. entitled Spontaneous Malignant Glaucoma in a Phakic Patient. In order to obtain a 1-hour Council of Optometric Practitioner Education (COPE) approved CE credit, please refer to the page 65 for complete instructions.

Spontaneous Malignant Glaucoma in a Phakic Patient Wess Jordan, OD; Lane Fujimoto, OD; Paul Vejabul, OD; Theresa Chong, OD; Michelle Matson, OD

ABSTRACT Background: Malignant glaucoma is a rare form of secondary glaucoma characterized by an axially shallow anterior chamber angle and elevated intraocular pressure despite a patent iridectomy. Case Report: A 71-year-old Caucasian female presented for a cataract evaluation with the complaint of blurred vision in the right eye.

Ocular examination revealed a fixed, mid-dilated right pupil, a shallow right anterior chamber, a closed right anterior chamber angle, and intraocular pressure of 44 mmHg OD and 18 mmHg OS. Despite placement of two patent laser iridotomies OD, the patient's anterior chamber remained shallow and her IOP remained elevated. The patient was consequently diagnosed with malignant glaucoma and referred for trans-pars plana vitrectomy. Due to surgical complications, the patient ended up with a blind, painful right eye necessitating enucleation. Conclusion: Eye care providers need to make the proper referrals and initiate the proper treatment protocols to prevent significant ocular morbidity.

INTRODUCTION Malignant glaucoma, also known as ciliary block glaucoma or aqueous misdirection syndrome, is a condition characterized by high intraocular pressure in the presence of a

W. Jordan â&#x20AC;&#x201D; Veterans Affairs Southern Nevada Healthcare System, Las Vegas, Nevada and Southern California College of Optometry, Fullerton, California; L. Fujimoto; P. Vejabul; T. Chong; M. Matson â&#x20AC;&#x201D; Veterans Affairs Southern Nevada Healthcare System, Las Vegas, Nevada, Southern California College of Optometry, Fullerton, California, and Illinois College of Optometry, Chicago, Illinois Correspondence to: Dr. Wess Jordan, 3880 S. Jones Blvd., Las Vegas, NV 89103; E-mail: WessJordanOD@gmail.com The authors have no financial or other relationships that might lead to a conflict of interest.

This article has been peer reviewed.

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Clinical and Refractive Optometry 28:2, 2017

patent iridectomy/iridotomy, shallowing of the central and peripheral anterior chamber, and absence of choroidal detachment or choroidal effusion.1 Most cases of malignant glaucoma occur following anterior segment surgeries such as glaucoma filtration surgery, cataract surgery, combined filtration and cataract surgery, and surgical peripheral iridectomy.1 Some authors have suggested that a history of recent anterior segment surgery be included in the diagnostic criteria for malignant glaucoma.1 However, several cases have been presented in the literature in which malignant glaucoma occurred spontaneously, in the absence of recent ocular surgery.2-5 The purpose of this report is to present a case of spontaneous malignant glaucoma and to review the diagnosis and treatment of the disease.

CASE REPORT A 71-year-old Caucasian female presented to our clinic for a cataract evaluation complaining of blurred vision in the right eye. Her medical history was significant for type 2 diabetes mellitus; hypertension; hyperlipidemia; osteoporosis; coronary artery disease; chronic ischemic heart disease; and ovarian cysts. Her ocular history was significant for compound hyperopic astigmatism in both eyes (OU), nuclear sclerotic cataracts (NSC) OU, and cortical cataracts (CC) OU. Current medications included metformin and pioglitazone for diabetes, benazapril and furosemide for hypertension, simvastatin for hyperlipidemia, alendronate and calcium for osteoporosis, and aspirin for coronary artery disease.

Best corrected visual acuities were 6/120 (20/400) with pinhole acuity of 6/60 (20/200) in the right eye (OD) and 6/7.5 (20/25) in the left eye (OS). Refractive error was not assessed at this exam, but was found to be +4.50 -1.00 x 078 OD and +4.50 -1.00 x 065 OS at her last exam eight months prior. The right pupil was mid-dilated and unreactive to light, while the left pupil was round and reactive to light. There was no afferent pupillary defect present. Extraocular motilities were unrestricted OU. Confrontation fields were full to finger counting OD, OS. Anterior segment biomicroscopy of the right eye was significant for grade 1 diffuse injection of the conjunctiva, pigment on the corneal endothelium with microcystic edema, and a shallow central and peripheral anterior chamber. The anterior chamber of the left eye was also

Fig. 1 Flattened anterior chamber

shallow centrally and peripherally, but was comparatively less shallow than the right eye. Iris bombe was not present in either eye. Intraocular pressures (IOP) using Goldmann applanation tonometry were 44 mmHg OD and 18 mmHg OS. The crystalline lenses had grade 2 nuclear sclerotic cataracts and grade 1 cortical cataracts OD, and grade 1-2 nuclear sclerotic cataracts and grade 1-2 cortical cataracts OS. Gonioscopy using a Sussman 4-mirror lens revealed a closed anterior chamber angle with no visible structures OD. There were no structures visible in the superior and temporal quadrants OS. In the left eye, the most posterior structure visible in the inferior quadrant was one-half of the trabecular meshwork, and the most posterior structure visible in the nasal quadrant was one/quarter of the trabecular meshwork. Peripheral anterior synechiae was not present in either eye. The posterior segment was within normal limits OD with a cup-to-disk ratio of 0.4. The cup-to-disk ratio was 0.35 OU at the previous exam eight months prior demonstrating symmetry between the two eyes and a relatively stable cup-to-disk ratio OD. An initial diagnosis of acute angle closure glaucoma OD was made and the patient was given 500 mg of acetazolamide and several drops of 0.5% iopidine in office.

Several hours later, the IOP measured 30 mmHg OD. The patient was instructed to take 250 mg of acetazolamide twice a day (b.i.d.), and 1 drop of Cosopt b.i.d. OD, and to return to the clinic the next day for follow-up and laser peripheral iridotomy (LPI).

The patient did not return for follow-up the next day, and was subsequently seen two days later by our ophthalmologist for LPI. At this visit, the preoperative IOP was 56 mmHg OD. The LPI procedure was performed, and the postoperative IOP was 61 mmHg OD. The 5 mmHg increase in IOP following the LPI is a relatively common occurrence hypothesized to be either prostaglandin mediated or due to pigment deposition in the trabecular

Fig. 2 Flattened anterior chamber

meshwork.6,7 An additional LPI procedure was performed without successful lowering of the IOP. Malignant glaucoma OD was diagnosed, and a vitreous tap was performed by the ophthalmologist. An amount of 0.25 cc of fluid was removed and the subsequent IOP was 4 mmHg OD. The patient was instructed to take 500 mg of acetazolamide b.i.d., 5% homatropine four times a day (q.i.d.) OD, 1% prednisolone acetate q.i.d. OD, and ciprofloxacin q.i.d. OD, and to return for follow-up the next day. The patient returned three days later with an IOP of 46 mmHg OD. The patient’s anterior chamber was confirmed as being axially shallow and funduscopic examination revealed no evidence of suprachoroidal hemorrhage or choroidal detachment. The patient was referred for a trans-pars plana vitrectomy.

The patient was subsequently seen at another hospital, where the surgeon recommended and implanted an Ahmed valve into the anterior chamber OD. The postoperative IOP was reported at 23 mmHg OD. The patient returned to our clinic one week later with a flat anterior chamber, iridocorneal touch, and with the shunt tube indenting the anterior surface of the lens. The patient’s IOP measured 7 mmHg OD, likely reduced from the previous measurement due to aqueous hyposecretion, leakage of aqueous around the drainage tube entry site, or damage to the valve during implantation.8-10 Figures 1 and 2 show the appearance of the eye upon presentation to our

Spontaneous Malignant Glaucoma in a Phakic Patient — Jordan et al.

59

closure glaucoma. A lack of pain, as in this case, is more common in chronic angle closure glaucoma, although cases of acute angle closure have been presented where pain is absent or minimal.11,12

PATHOGENESIS

Fig. 3 A/B scan ultrasonography demonstrating choroidal effusion

clinic. A referral was made back to the surgeon for an urgent follow-up visit, when she was diagnosed with a choroidal effusion secondary to the large decrease in IOP post-tube implantation. Choroidal drainage was performed and the anterior chamber was re-inflated. Three days later, the patient presented to our clinic again with a flat anterior chamber, iridocorneal touch, and with the shunt tube indenting the anterior lens.

The patientâ&#x20AC;&#x2122;s IOP measured 6 mmHg OD, likely due to continued overfiltration of the Ahmed valve. A/B scan ultrasonography results shown in Figure 3 revealed an inferior choroidal effusion, and the patient was again referred back to the surgeon. Choroidal drainage was performed for the second time along with re-inflation of the anterior chamber. The patient continued follow up with the surgeon including addition surgeries for valve repositioning and cataract extraction. Her visual acuity was documented as light perception OD following these procedures, and due to extreme eye pain, the eye was eventually enucleated.

DISCUSSION Malignant glaucoma was first reported by von Graefe in 1869 as a condition characterized by high intraocular pressure and a flat or shallow anterior chamber in the presence of a patent iridectomy. Malignant glaucoma is found more commonly in patients with small, hyperopic eyes with narrow anterior chamber angles. This is presumably because of the close proximity of the angle, iris, lens, and ciliary body, which comprise the middle segment of the eye.1 Symptoms of malignant glaucoma may range from mild to severe. Patients may complain of pain in the eye, redness of the eye, blurring of vision, or decreased vision. Pain in the eye may be severe enough to induce nausea or vomiting, mimicking acute primary angle

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The exact pathogenesis of malignant glaucoma is not well understood, and further investigations are necessary to better understand this highly complex process. Shaffer hypothesized that aqueous humor is misdirected into the vitreous chamber causing an increase in vitreous pressure and forward movement of the ocular structures.13 However, he did not speculate as to the cause of the initial aqueous misdirection. Epstein et al proposed a second hypothesis where a sustained increase in vitreous volume causes a forward rotation of the anterior hyaloid face into apposition with the posterior ciliary body. This increases the resistance to the anterior flow of aqueous humor leading to a forward displacement of the iris and lens and a shallowing of the anterior chamber.14 Using experimental perfusion studies, Epstein et al showed that increased intraocular pressure causes resistance to aqueous flow through the vitreous. He suggested that dehydration, compression, and decreased permeability of the vitreous may be responsible for this resistance.14 Epstein also observed that malignant glaucoma is more common after surgical iridectomy than after laser iridotomy. He hypothesized that the surgical decompression induced when performing certain anterior segment surgeries is the inciting event to this process in which the anterior hyaloid face is displaced into apposition with the posterior ciliary body, causing aqueous humor to move posterior into the vitreous chamber.1 More recently, Quigley has suggested choroidal expansion as the inciting factor in malignant glaucoma.15,16 According to Quigley, choroidal expansion leads to a higher pressure in the posterior globe than in the anterior chamber causing volume loss from the anterior chamber. From Epstein et al one can conclude that the greater the pressure differential across the vitreous body, the greater the resistance to vitreous fluid conductivity.

Quigley states that further choroidal expansion sets up a cycle of higher pressure gradient across the vitreous and poorer fluid conductivity through the vitreous leading to forward movement of the vitreous and lens.15,16 This forward movement of the vitreous and lens presents as a shallow anterior chamber. Although Quigley sites many conditions in which choroidal expansion occurs, he states that there is currently no instrumentation to accurately measure this phenomenon.15,16

EPIDEMIOLOGY Recent epidemiological studies of malignant glaucoma are lacking. In 1951, Chandler reported a malignant

Table I Differential diagnoses for malignant glaucoma20-23,27,42 Differential

Laterality

Clinical Signs

Symptoms

Malignant Glaucoma

Typically unilateral

Shallow peripheral and central anterior chamber, high intraocular pressure, closed anterior chamber angle.

Red eye, painful eye, blurred vision.

May have nausea and vomiting

Acute Angle Closure Glaucoma

Typically unilateral

Shallow peripheral anterior chamber and relatively deeper central anterior chamber, high intraocular pressure, closed anterior chamber angle, iris bombe.

Red eye, painful eye, blurred vision.

May have nausea and vomiting.

Chronic Angle Closure Glaucoma Typically unilateral

Intermittent signs of: shallow peripheral anterior chamber and relatively deeper central anterior chamber, high intraocular pressure, closed anterior chamber angle, iris bombe, peripheral anterior synechiae, possibly glaucomatous optic nerve and visual field defects

Usually asymptomatic until vision decreased, visual field defects become present, or angle completely closes.

Suprachoroidal Hemorrhage

Typically unilateral

Shallow peripheral and central anterior chamber, high intraocular pressure, closed anterior chamber angle.

Red eye, moderate to severe pain in the eye.

Choroidal Detachment

Typically unilateral

Shallow peripheral and central anterior chamber, low intraocular pressure, closed anterior chamber angle.

No pain in the eye. Vision may be normal to blurred if detachment extends into the macula.

Sulfonamide Induced Secondary Bilateral Angle Closure Due to Topiramate

Shallow peripheral and central anterior chamber, high intraocular pressure, closed anterior chamber angle.

Red eyes, painful eyes, blurred vision.

May have nausea and vomiting.

Plateau iris syndrome

Shallow peripheral anterior chamber and deeper central anterior chamber, high intraocular pressure, closed anterior chamber angle.

Red eye, painful eye, blurred vision.

May have nausea and vomiting.

Typically unilateral

glaucoma rate of 2% to 4% in patients undergoing surgery for acute angle closure.17 The incidence of malignant glaucoma is believed to have decreased since then due to early implementation of medical and surgical treatments and the advancements of these treatments.

CLINICAL SIGNS AND SYMPTOMS Patients with malignant glaucoma will present with an anterior chamber that is shallow both peripherally and centrally, or axially shallow. Intraocular pressure will be elevated and gonioscopy will reveal a closed angle. The patient may complain of a red, painful eye and blurred vision. The patient may also experience nausea and vomiting as with an acute primary angle closure attack.

DIAGNOSING AND TESTING Epstein proposed minimum requirements for a diagnosis of malignant glaucoma including: an axially shallow anterior chamber; elevated intraocular pressure; presence of a patent peripheral iridectomy; and absence of suprachoroidal fluid.1 A careful ocular surgical history is also important to diagnosing malignant glaucoma as it most

commonly follows glaucoma filtration surgery, cataract surgery, combined filtration and cataract surgery, and surgical peripheral iridectomy.1 More recently, ultrasound biomicroscopy has proven useful in distinguishing malignant glaucoma from other similar conditions and in determining the efficacy of various treatments.16,18,19 Table I outlines the differential diagnoses for malignant glaucoma including their typical laterality, clinical signs, and symptoms.

Malignant glaucoma will initially present as primary angle closure glaucoma with similar signs and symptoms; visual acuity may be reduced, slit lamp biomicroscopy will reveal an axially shallow or flat anterior chamber; gonioscopy will demonstrate a closed angle; tonometry will show elevated intraocular pressure; and funduscopic examination or B-scan ultrasonography will show no elevation of the retina and choroid. Other differential diagnoses include chronic angle closure with extensive peripheral anterior synechiae, plateau iris syndrome, suprachoroidal hemorrhage, choroidal detachment, and sulfonamide-induced secondary angle closure due to topiramate.

Spontaneous Malignant Glaucoma in a Phakic Patient â&#x20AC;&#x201D; Jordan et al.

61

In primary angle closure glaucoma caused by pupillary block, the anterior chamber tends to be shallow peripherally and deeper centrally, whereas in malignant glaucoma, the anterior chamber is shallow both peripherally and centrally. Another important distinction is the presence of iris bombe, which is found in cases of pupillary block angle closure glaucoma, but not in cases of malignant glaucoma.20 The only definitive way to distinguish these two conditions is by performing surgical iridectomy or laser iridotomy.4 After iridectomy or iridotomy, the anterior chamber will deepen and intraocular pressure will normalize in pupillary block angle closure glaucoma, provided there is not extensive peripheral anterior synechiae as could be seen in chronic cases.

The subsequent deepening of the anterior chamber and normalizing of the intraocular pressure would not occur after iridectomy or iridotomy in cases of malignant glaucoma. In diagnosing malignant glaucoma a second iridectomy or iridotomy is recommended to confirm non-resolution of the axially shallow anterior chamber and elevated intraocular pressure.

Chronic angle closure glaucoma with extensive peripheral anterior synechiae may present similarly to malignant glaucoma since both of these conditions would involve a closed angle and elevated IOP which may not respond to peripheral iridectomy or iridotomy. However, iris bombe and progressive and/or advanced optic nerve head cupping would be present in cases of chronic angle closure glaucoma.20 Plateau iris syndrome is another ocular condition which must be considered in cases of acute angle closure glaucoma which do not respond to iridectomy or iridotomy.

Unlike malignant glaucoma, patients with plateau iris syndrome typically present with a deep central anterior chamber.20 In plateau iris syndrome, the ciliary processes and iris are anteriorly displaced causing occlusion of the trabecular meshwork.21,22 The displaced ciliary processes prevent the iris root from falling away from the trabecular meshwork after peripheral iridectomy or iridotomy.21,23 There may also be a relative pupillary block component in some patients with this condition.20 Ultrasound biomicroscopy, when available, can be very useful in distinguishing plateau iris from other causes of angle closure, including malignant glaucoma.

Suprachoroidal hemorrhage usually occurs intraoperatively or postoperatively due to rupture of the short posterior ciliary arteries. The anterior chamber is shallow axially, intraocular pressure will be elevated, and the patient will usually have severe pain. In contrast, choroidal detachment, which also occurs intraoperatively or postoperatively, presents with a peripherally shallow anterior chamber, ocular hypotony, and usually no pain.24 Both suprachoroidal hemorrhage and choroidal detachment may be ruled out by fundus examination and/or

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B-scan ultrasonography. On funduscopy they present as a smooth, bullous, orange-brown elevations of the retina and choroid. In cases in which there is doubt and the absence of suprachoroidal fluid cannot be confirmed, Chandler recommends performing a sclerotomy to drain any possible fluid.25,26 Sulfonamide-induced secondary angle closure due to topiramate use presents bilaterally in the majority of cases.27 The anterior chambers are axially shallow, intraocular pressures are elevated, and the eyes will be painful. Peripheral iridectomy or iridotomy will not deepen the anterior chamber or normalize the IOP. Secondary angle closure usually occurs within the first two weeks of initiating topiramate treatment.27 Therefore, case history is important to ruling out this cause.

TREATMENT The treatment of malignant glaucoma involves both medical and surgical options. Upon presentation of a suspected case of malignant glaucoma, a peripheral iridectomy or iridotomy should be performed to rule out primary angle closure glaucoma. If there is any doubt as to the patency of the iridectomy or iridotomy, a second iridectomy or iridotomy should be performed. In cases where the anterior chamber angle of the fellow eye is also narrow, prophylactic peripheral iridectomy or iridotomy should be performed. Once the iridectomies or iridotomies have been shown to be ineffective in deepening the anterior chamber and relieving the elevated intraocular pressure, medical therapy with cycloplegics and sympathomimetics should be initiated. Simmons and Maestre recommend adding topical beta blockers, oral carbonic anhydrase inhibitors, and oral hyperosmotics to this regimen.24 The cycloplegics move the ciliary body outward, away from the anterior hyaloid face while sympathomimetics tighten the zonules and contract the iris dilator muscle, helping to resist forward pressure from the vitreous.28,29 Topical beta blockers, oral carbonic anhydrase inhibitors, and oral hyperosmotics help to lower the intraocular pressure. Therapy should continue for at least four to five days before being deemed unsuccessful.

During this time period, 50% of cases of malignant glaucoma are successfully resolved.24 With successful treatment, topical beta blockers, oral anhydrase inhibitors, and oral hyperosmotics may be tapered, but cycloplegics should be continued. Cessation of cycloplegics creates a significant risk of return of the malignant glaucoma, necessitating continued therapy or the exploration of other treatment options.1,28 If malignant glaucoma is not resolved with medical treatment, surgical options are required. Laser therapies that are available include argon laser treatment of the ciliary processes and neodymium-doped yttrium aluminum garnet (Nd:YAG) laser anterior hyaloidotomy. Argon laser

shrinkage of the ciliary processes was first proposed

by Herschler, who reported successful treatment of malignant glaucoma in five of six patients.30 He suggested that treating two to four processes visible through an iridectomy causes relief of ciliolenticular block and restores normal flow of aqueous humor anteriorly.30 Nd:YAG laser anterior hyaloidotomy is believed to disrupt the anterior hyaloid face, relieving any iridovitreal block that may be present. Immediate, slight deepening of the anterior chamber has been observed after this procedure, and significant deepening is more commonly observed 12-24 hours later.31 Another surgical option is pars plana vitrectomy with disruption of the anterior hyaloid face.

Vitrectomy alleviates iridovitreal block and restores the normal flow of aqueous humor anteriorly. Byrnes et al reported successful treatment of malignant glaucoma using vitrectomy in 15 of 21 eyes. Of the six cases in which recurrence of malignant glaucoma was found, five of the eyes were phakic and one was pseudophakic.32 It was speculated that when using caution not to damage the crystalline lens and zonules, surgeons are less likely to successfully disrupt the anterior hyaloid face. Because of this, several authors have recommended combined cataract extraction and vitrectomy in phakic patients with malignant glaucoma.32-34

6.

7.

8.

9.

10.

11.

12.

13.

14.

15.

CONCLUSION Malignant glaucoma is an urgent, potentially blinding, rapidly progressing condition which requires a stepwise approach to diagnosis and treatment. The diagnosis of malignant glaucoma can be challenging without prior knowledge of the condition and its features. Multiple treatments are available for this type of glaucoma, and should be approached in a logical sequence. As this case demonstrates, timely diagnosis and appropriate treatment are essential to prevent significant vision loss and blindness. ❏

REFERENCES 1.

2.

3.

4.

5.

16.

17.

18.

19.

20.

Epstein DL. The malignant glaucoma syndromes. In: Epstein DL, ed., with Allingham RR, Schuman JS. Chandler and Grant’s glaucoma. 4th ed. Baltimore: Williams and Wilkins; 1997. p. 285-303.

Fanous S, Brouillette G. Ciliary block glaucoma: malignant glaucoma in the absence of a history of surgery and of miotic therapy. Can J Ophthalmol 1983; 18: 302.

Gonzalez F, Sanchez-Saloria M, Pacheco P. Simultaneous bilateral “malignant glaucoma” attack in a patient with no antecedent eye surgery or miotics. Eur J Ophthalmol 1992; 2(2): 91-93.

Manku MS. Spontaneous bilateral malignant glaucoma.

Aust NZ J Ophthalmol 1985; 13: 249.

Schwartz AL, Anderson DR. “Malignant glaucoma” in an eye with no antecedent operation or miotics. Arch Ophthalmol 1975; 93: 379.

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Taniguchi T, Rho SH, Gotoh Y, Kitazawa Y. Intraocular pressure rise following q-switched neodymium:YAG laser iridotomy. Ophthalmic Laser Therapy 1987; 2(2): 99-104.

Krupin T, Stone R, Cohen B, Kolker A et al. Acute intraocular pressure response to argon laser iridotomy.

Ophthalmology 1985; 92(7): 922-926.

Ayyala R, Zurakowski D, Smith J, Monshizadeh R et al.

A clinical study of the Ahmed glaucoma valve implant in advanced glaucoma. Ophthalmology 1998; 105(10): 1968-1976.

Coleman A, Hill R, Wilson MR, Choplin N et al. Initial clinical experience with the Ahmed glaucoma valve implant. Am J Ophthalmol 1995; 120: 23-31.

Hill R, Pirouzian A, Liaw L. Pathophysiology of and prophylaxis against late Ahmed glaucoma valve occlusion.

Am J Ophthalmol 2000; 129(5): 608-612.

Rosenberg CA, Adams SL. Narrow-angle glaucoma presenting as acute, painless visual impairment. Ann Emerg Med 1991; 20:1020-1022.

Ravits J, Seybold ME. Transient monocular visual loss from narrow angle glaucoma. Arch Neurol 1984; 41: 991-993.

Shaffer RN. The role of vitreous detachment in aphakic and malignant glaucoma. Trans Am Acad Ophthalmol Otolaryngol 1954; 58: 217.

Epstein DL, Hashimoto JM, Anderson PJ, Grant WM. Experimental perfusions through the anterior and vitreous chambers with possible relationships to malignant glaucoma. Am J Ophthalmol 1979; 88: 1078-1086.

Quigley H, Friedman D, Congdon N. Possible mechanisms of primary angle-closure and malignant glaucoma.

J Glaucoma 2003; 12: 167-180.

Quigley H. Angle-closure glaucoma-simpler answers to complex mechanisms: LXVI Edward Jackson Memorial Lecture. Am J Ophthalmol. 2009; 148(5): 657-669.

Chandler PA. Malignant glaucoma. Am J Ophthalmol 1951; 34(7): 993-1000.

Seymenoglu RG, Baser EF. Management of pseudophakic malignant glaucoma and ultrasound biomicroscopic features. Can J Ophthalmol 2009; 44(6): 719-720.

Shen CJ, Chen YY, Sheu SJ. Treatment course of recurrent malignant glaucoma monitoring by ultrasound biomicroscopy: a report of two cases. Kaohsiung J Med Sci 2008; 24(11): 608-613.

Rhee D. Glaucoma: color atlas and synopsis of clinical ophthalmology. New York: McGraw-Hill; 2003.

Ng WT, Morgan W. Mechanisms and treatment of primary angle closure: a review. Clin Experiment Ophthalmol 2011; doi: 10.1111/j.1442-9071.2011.02604.x.

[Epub ahead of print] Pavlin CJ, Ritch R, Foster FS. Ultrasound biomicroscopy in plateau iris syndrome. Am J Ophthalmol 1992; 15: 390-395.

Wand M, Pavlin CJ, Foster FS. Plateau iris syndrome: ultrasound biomicroscopic and histologic study. Ophthalmic Surg 1993; 24: 129-131.

Simmons RJ, Maestre FA. Malignant glaucoma. In: Ritch R, Shields MB, Krupin T, eds. The glaucomas, vol 2. 2nd ed. St. Louis: Mosby-Yearbook; 1996. p. 841-855.

Balakrishnan W, Abraham JE. Chandler’s operation for malignant glaucoma. Arch Ophthalmol 1969; 82: 723.

Spontaneous Malignant Glaucoma in a Phakic Patient — Jordan et al.

63

26. Chandler PA, Simmons RJ, Grant WM. Malignant glaucoma: medical and surgical treatment. Am J Ophthalmol 1968; 66: 496.

27. Fraunfelder FW, Fraunfelder FT, Keates E. Topiramateassociated acute, bilateral, secondary angle-closure glaucoma.

Ophthalmology 2004; 111: 109-111.

28. Chandler, PA, Grant WM. Mydriatic-cycloplegic treatment in malignant glaucoma. Arch Ophthalmol 1962; 68: 353.

29. Coleman DJ. Unified model for accommodative mechanism.

Am J Ophthalmol 1970; 69: 1063.

30. Herschler J. Laser shrinkage of the ciliary processes â&#x20AC;&#x201C; a treatment for malignant (ciliary block) glaucoma.

Ophthalmology 1980; 87: 155.

31. Epstein DL, Steinert RF, Puliafito CA. Neodymium: YAG laser therapy to the anterior hyaloid in aphakic malignant glaucoma. Am J Ophthalmol 1984; 98: 137.

32. Byrnes GA, Leen MM, Wong TP, Benson WE. Vitrectomy for ciliary block (malignant) glaucoma. Ophthalmology 1995; 102: 1308-1311.

33. Sharma A, Sii F, Shah P, Kirkby G. Vitrectomyphacoemulsification-vitrectomy for the management of aqueous misdirection syndromes in phakic eyes.

Ophthalmology 2006; 113: 1968-1973.

34. Tsai JC, Barton KA, Miller MH, Khaw PT et al. Surgical results in malignant glaucoma refractory to medical or laser therapy. Eye 1997; 11: 677-681.

35. Brown RK, Lynch MG, Tearse JE, Nunn RD. NeodymiumYAG vitreous surgery for phakic and pseudophakic malignant glaucoma. Arch Ophthalmol 1986; 104: 1464-1466.

36. Grant, WM. Experimental aqueous perfusion in enucleated human eyes. Arch Ophthalmol 1963; 69: 732.

37. Harbour JW, Rubsamen PE, Palmberg P. Pars plana vitrectomy in the management of phakic and pseudophakic malignant glaucoma. Arch Ophthalmol 1996; 114: 1073-1078.

38. Muqit MM, Menage MJ. Malignant glaucoma after phacoemulsification: treatment with diode laser cyclophotocoagulation. J Cataract Refract Surg 2007; 33(1): 130-132.

39. Ozeki N, Yuki K, Kimura I. Alternative approach to treating malignant glaucoma after trabeculectomy with unplanned zonulectomy. Clin Ophthalmol 2010; 4: 383-385.

40. Ruben S, Tsai J, Hitchings R. Malignant glaucoma and its management. Br J Ophthalmol 1997; 81(2): 163-167.

41. Steinert RF, Epstein DL, Puliafito CA. Surgical vitrectomy for pseudophakic malignant glaucoma. Am J Ophthalmol 1986; 102: 803-804.

42. Ehlers JP, Shah CP, eds. The Wills eye manual: Office and emergency room diagnosis and treatment of eye disease (ed 5). Philadelphia: Lippincott Williams & Wilkins; 2008.

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QUESTIONNAIRE Spontaneous Malignant Glaucoma in a Phakic Patient Wess Jordan, OD; Lane Fujimoto, OD; Paul Vejabul, OD; Theresa Chong, OD; Michelle Matson, OD 1. ❑ ❑ ❑ ❑

In the Case Report presented, what was the patient’s chief initial complaint?

Ocular pain Blurred vision in her right eye Ocular redness Blurred vision in both eyes

2. ❑ ❑ ❑ ❑

All of the following are symptoms of malignant glaucoma, EXCEPT: Ocular redness Decreased vision Headache Lack of pain

Spontaneous Malignant Glaucoma in a Phakic Patient — Jordan et al.

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In the Case Report presented, all of the following describe the examination findings, EXCEPT: Right pupil was unreactive to light Left pupil was reactive to light No afferent pupillary defect Extraocular motility restricted in the right eye

4. ❑ ❑ ❑ ❑

According to Chandler, in what percentage of patients did malignant glaucoma occur following surgery for acute angle closure?

1% 1% to 2% 2% to 4% 5%

5. ❑ ❑ ❑ ❑

All of the following differential diagnoses for malignant glaucoma are typically unilateral, EXCEPT: Acute angle closure glaucoma Chronic angle closure glaucoma Choroidal detachment Sulfonamide-induced secondary angle closure due to topiramate

6. ❑ ❑ ❑ ❑

Which of the following conditions can cause notably severe ocular pain?

Suprachoroidal hemorrhage Malignant glaucoma Choroidal detachment Plateau iris syndrome

7. ❑ ❑ ❑ ❑

High intraocular pressure is present in all of the following, EXCEPT: Choroidal detachment Acute angle closure glaucoma Chronic angle closure glaucoma Plateau iris syndrome

8. ❑ ❑ ❑ ❑

Suprachoroidal hemorrhage is characterized by all of the following, EXCEPT: Elevated intraocular pressure Peripherally shallow anterior chamber Severe ocular pain Axially shallow anterior chamber

9. ❑ ❑ ❑ ❑

Byrnes et al has reported successful treatment of malignant glaucoma using vitrectomy in how many eyes?

7 of 11 eyes 11 of 17 eyes 13 of 20 eyes 15 of 21 eyes

10. ❑ ❑ ❑ ❑

Anterior segment biomicroscopy of the right eye revealed all of the following, EXCEPT: Iris bombe Pigment on the corneal endothelium with microcystic edema Shallow central and peripheral anterior chamber Grade 1 diffuse injection of the conjunctiva

28.2:17

3. ❑ ❑ ❑ ❑

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Clinical and Refractive Optometry 28:2, 2017

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Clinical & Refractive Optometry is pleased to present this continuing education (CE) article

by Dr. Leonid Skorin, Jr. and Dr. Kristopher K. Sherrill entitled Pars Planitis: A Review and Case Report. In order to obtain a 1-hour Council of Optometric Practitioner Education (COPE) approved CE credit, please refer to page 72 for complete instructions.

Pars Planitis: A Review and Case Report Leonid Skorin, Jr. OD, DO, MS, FAAO, FAOCO Kristopher K. Sherrill, OD

ABSTRACT Pars planitis is an idiopathic intermediate uveitis characterized by a vitritis with snow banking and snowballs. The diagnosis of pars planitis is one of exclusion and the many known systemic associations of intermediate uveitis need to be ruled out. It is usually a chronic condition with exacerbations and remissions. Pars planitis has a bimodal peak occurrence in children and young adults.

This is a case report of a young female presenting with mild blurry vision in one eye. An intermediate uveitis was apparent with snowballs but without signs of snow banking. A systemic work-up including blood testing was done. It is important to consider the severity of pars planitis including its effect on vision, especially macular edema in deciding if treatment is necessary.

INTRODUCTION Pars planitis is a specific type of intermediate uveitis that is idiopathic in nature; characterized as a vitritis with inflammatory condensates (snowballs) and pars plana fibrovascular exudation (snow banking).1,2 It is usually bilateral and is usually asymmetric in severity. Pars planitis is a chronic condition that may reoccur for many years. All other potential causes of intermediate uveitis should be ruled out. These include sarcoidosis, multiple sclerosis and Lyme disease.2-4 L. Skorin, Jr. — Consultant, Community Division of Ophthalmology, Mayo Clinic Health System, Albert Lea, MN; Assistant Professor of Ophthalmology, Mayo Clinic College of Medicine, Rochester, MN; K.K. Sherrill — Staff Optometrist, Rocky Mountain Eye Center, Butte, MT. Correspondence to: Dr. Leonid Skorin, Jr., Mayo Clinic Health System, 404 West Fountain Street, Albert Lea, MN 56007; E-mail: Skorin.leonid@mayo.edu The authors have no financial or proprietary interest in any of the material mentioned in this article.

This article has been peer reviewed.

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Pars planitis usually occurs in children and young adults.2,4 A decrease in vision and an increase in floaters may be the only symptoms. Pars planitis has an increased incidence in patients with multiple sclerosis and may be associated with smoking.1,5 Treatment of pars planitis is not indicated if vision is 6/12 (20/40) or better and there is no evidence of macular edema.1,3,4 If treatment is warranted, sub-tenon’s injections, intravitreal steroid injections or systemic steroids should be prescribed. The patient’s visual outcome is usually good with studies showing a 6/12 (20/40) or better acuity in 75% of patients at ten year follow-up.5,6

CASE REPORT A 16-year-old Caucasian female reported to the clinic for a complete eye exam with concern of blurry vision in her right eye that has persisted for about one month. She had no previous history of vision correction. She reported floaters present in her vision for the same time period with no flashes of light, pain or irritation. There was no history of trauma, headaches or tick bites. She was allergic to amoxicillin and had a history of irritable bowel syndrome. There was no significant ocular or family history. She reported that she did not smoke, though was around second hand smoke from her father. She did not have pain on eye movement, double vision, oscillopsia, numbness or weakness in one or more limbs. She denied any tingling, pain or lack of coordination.

Uncorrected visual acuity was 6/7.5-2 (20/25-2) right eye and 6/6 (20/20) left eye at both distance and near.

Pupils were equal, round, reactive to light with no afferent pupillary defect. Extraocular motilities and confrontation visual fields were grossly full. Color vision was 14 out of 14 plates using Ishihara and stereo acuity was measured as 100 arc seconds. Subjective refraction was plano +0.25 x 166 right eye and +0.25 sphere left eye yielding acuities of 6/6 (20/20) right eye and 6/6 (20/20) left eye. Slit lamp exam was unremarkable in both right and left eyes. There was no iritis. Intraocular pressure was 13 mmHg right eye and left eye using ICARE tonometry. Dilated fundus exam revealed well perfused optic nerve heads and distinct margins in both eyes. The macula was flat with no edema in the right and left eye though the view was limited in the right eye secondary to debris in the vitreous.

Fig. 1 Classic snowball appearance, right eye.

Fig. 2 High magnification of classic snowball appearance, right eye.

The peripheral retina was flat with no holes and no tears.

Inflammatory condensates were present in the right eye greater than the left eye, giving the classic snowball appearance (Figs. 1, 2). Snow banking was not visualized in either eye. Vitreal cells were present in the right eye causing the limited view. The vitreous was clear in the left eye. An optical coherence tomography (OCT) macular cube 512x128 was taken showing no macular edema in either eye.

A diagnosis of vitritis with no macular edema and suspected pars planitis was made in both eyes. A complete blood count (CBC) with differential, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), syphilis serology (FTA-ABS), Lyme titer, angiotensin converting enzyme (ACE), antinuclear antibody test (ANA), Human Leukocyte Antigen (HLA) B27, classical antineutrophil cytoplasmic antibodies (c-ANCA), protoplasmic-staining antineutrophil cytoplasmic antibodies (p-ANCA) and a chest radiograph were ordered.

All blood test results were negative. The chest radiograph showed tiny calcified granulomas especially in the right lung with no active infection or inflammation.

Radiology reported that it resembled calcifications from an old, inactive histoplasmosis infection. A diagnosis of pars planitis with no macular edema and minimal visual acuity involvement was confirmed. The patient and family decided that no treatment would be started at this time due to minimal visual acuity change, no macular edema and the potential for harmful side effects inherent with steroid treatment.

The patient reported back to the clinic two weeks later with concerns of decreasing vision and increased floaters in the right eye. The vision loss was limiting her performance in school and athletics. She reported no other symptoms.

Uncorrected visual acuities were 6/12 (20/40) in the right eye and 6/6 (20/20) in the left eye. Refractive error correction had no improvement on right eye acuity. A dilated fundus exam of the right eye revealed numerous snowballs and an increase in vitreal cells over the macular area since the patient’s last visit. Macular edema, snow banking and peripheral vasculitis was still not evident in either eye.

The decrease in vision was attributed to the increase in vitritis. The patient and family decided to pursue treatment now that it was affecting her vision. She was referred to a retina specialist for consultation. At this consultation uncorrected visual acuities were similar to the previous visit. Dilated fundus exam was also similar though trace snow banking was seen this time in the right eye. OCT findings had not changed and showed no macular edema. Fluorescein angiography revealed perivascular leakage in the peripheral retina in both the right (Fig. 3) and left eye (Fig. 4). A posterior sub-tenon’s triamcinolone (Kenalog 40 mg/mL) injection was given to the right eye. The patient was asked to follow up for an intra-ocular pressure and OCT exam in four to six weeks.

DISCUSSION Pars planitis, an idiopathic intermediate uveitis, is characterized by a vitritis with inflammatory condensates

Pars Planitis: A Review and Case Report — Skorin, Sherrill

69

Fig. 3 Fluorescein angiography showing perivascular leakage of the temporal peripheral arcades in the right eye.

Fig. 4 Fluorescein angiography showing perivascular leakage of the inferior temporal peripheral arcade in the left eye.

(snowballs) and pars plana fibrovascular exudation (snow banking).1,2 It is usually bilateral (75% to 90%).1,2 Pars planitis is a chronic condition and may reoccur for many years. The diagnosis of pars planitis is one of exclusion with all other potential causes of intermediate uveitis being ruled out. Systemic associations of intermediate uveitis include sarcoidosis, multiple sclerosis (MS) and Lyme disease.2-4 Lesser associations include Behcetâ&#x20AC;&#x2122;s disease, syphilis, amyloidosis, familial exudative vitreoretinopathy, toxocariasis, toxoplasmosis, candidiasis, Ealesâ&#x20AC;&#x2122; disease, Vogt-Koyanagi-Harada syndrome, inflammatory bowel disease, cat-scratch disease, retinoblastoma and ocular lymphoma.2-4 Pars planitis usually occurs in children (5 to 15 years old) and young adults (20 to 40 years old) with the average age of 23 to 28 years old.2,4 It rarely occurs after 40 years of age.1 It occurs equally in males and females but is less common in blacks and Asians.2 The most common presenting symptom is decreased vision and a new onset of floaters.1 Pain, photophobia and redness are minimal or usually absent.4 Mild pars planitis may go undetected as the patient may have no symptoms. In a retrospective study, 65.9% of patients diagnosed with pars planitis had better or equal to 6/12 (20/40) acuity in the worse eye upon presentation with 90.9% having better than 6/12 (20/40) acuity at resolution.5 Peripheral retinal snowballs and/or snow banking is always present in pars planitis and may also present with anterior vitreous cells, peripheral retinal periphlebitis and an anterior chamber reaction.4 The most common complications of pars

planitis include cystoid macular edema (most common cause of visual loss), cataracts (from inflammation or steroid treatment) and epiretinal membrane formation.1,4 Posterior vitreous detachment, neovascularization, vitreous hemorrhage, glaucoma and retinal detachment may occur in severe cases.2,7 Clinical work up for suspected pars planitis includes a complete history and review of systems with attention to diseases, infections, skin rashes, eye trauma, tick bites and time spent around chickens or having lived in endemic areas such as the Ohio-Mississippi River valley.3 Complete ocular examination with anterior segment evaluation, intraocular pressure and indirect ophthalmoscopy and scleral depression of the ora serrata should be performed to look for snow banking. OCT or fluorescein angiography is useful in determining if macular edema is present and will aid in treatment plans.4 Additional testing to be considered includes CBC with differential, syphilis serology (FTA-ABS and RPR or VDRL), ACE, purified protein derivative, chest radiograph and Lyme titers.2-4,7 If additional findings suggest MS, an MRI with fat suppression of the brain and orbits, with and without gadolinium, should be considered especially if the patient is over the age of 25.8 Pars planitis has a higher prevalence with MS (3% to 27%) versus the general population (0.02% to 0.08%).5, 6 Immunogenic studies have shown that the predisposition of MS and pars planitis may be associated with the HLA-DR15 allele.9 The most common ocular manifestations with MS are optic neuritis, diplopia, nystagmus and

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Clinical and Refractive Optometry 28:2, 2017

uveitis. Patient symptoms may include vision loss, pain on eye movement, double vision, oscillopsia, numbness or weakness in one or more limbs, tingling sensation or pain.

The patient may also experience electric-shock sensations that occur with certain head movements (Lhermitte’s sign), tremor, lack of coordination, fatigue, dizziness and a visual heat intolerance (Uhtoff’s sign). If a patient presents with pars planitis these symptoms should be discussed with the patient and if they have neurologic signs of MS a referral to a neurologist is warranted. If MS is diagnosed, the term intermediate uveitis should be applied.5 A high correlation between pars planitis and smoking has also been noted. In one study 52% of the patients with pars planitis were smokers compared to the national prevalence of smoking at 23%.1 There is no data specific to pars planitis and second hand smoke. At this time no direct cause has been shown to link smoking and pars planitis. This possible link should be conveyed to the patient and a recommendation given to quit smoking for the ocular and systemic benefits.

TREATMENT Treatment of pars planitis is usually not indicated if visual acuity is 6/12 (20/40) or better and there are no signs of macular edema.1,3,4 Treatment with systemic steroids should be weighed in each case due to potential significant side effects which include cataracts, glaucoma and various systemic complications. Patients with good initial visual acuity may, however, be the most likely to benefit from the aggressive treatment of inflammation to reduce the development of secondary ocular complications.5 The treatment regimen includes topical steroids (prednisolone acetate 1% qh-q6h) and a cycloplegic agent for any anterior chamber reaction. A posterior sub-tenon’s injection of triamcinolone (40 mg/mL) or methylprednisolone acetate (40 mg/mL) is given when macular edema is present or if severe posterior inflammation is present.3-5 Injections are administered every two to eight weeks until there is resolution of the macular edema or the vision and macular edema are no longer improving.3,4 Oral non-steroidal anti-inflammatory drugs (NSAIDS) have been used in conjunction with corticosteroid injections for maintenance therapy.5 Topical NSAIDS may be added in patients with macular edema.3 Oral steroids (prednisone 40 to 60 mg daily) may be used for four to six weeks if injections are not possible or in severe bilateral cases.2,3,5 A slow taper based on response is recommended.

Trans-scleral cryotherapy to the peripheral retina is useful for peripheral retinal neovascularization but may cause retinal tears and retinal detachments.2,4,5 Pars plana vitrectomy may be warranted for removal of vitreous debris and to reduce vitreal traction.2-5 Immunosuppressive agents (methotrexate, azathioprine, chlorambucil, cyclophosphamide and cyclosporine A) should be considered for difficult cases.2,3 The patient should be free of inflammation for three months prior to cataract removal if cataracts develop from the inflammation or steroid treatment.3,4

PROGNOSIS Most patients’ visual outcome is good with studies showing 6/12 (20/40) or better acuity in 75% of patients at ten year follow-up and 90% maintained best corrected visual acuity of at least 6/12 (20/40) in the better eye.5,6 The presence of macular edema is usually the determining factor in final visual acuity. MRI evaluation of the brain for patients over 25 years of age with pars planitis is prudent for early detection of MS. Due to the chronic nature of pars planitis, patients should be re-evaluated every three to six months. ❏

REFERENCES 1.

2.

3.

4.

5.

6.

7.

8.

9.

Donaldson MJ, Pulido JS, Herman DC, et al. Pars planitis: a 20-year study of incidence, clinical features, and outcomes. Am Ophthalmol 2007; 144: 812-817.

Kaiser PK, Friedman NJ, Pineda R. The Massachusetts Eye and Ear Infirmary Illustrated Manual of Ophthalmology, 2nd ed. Saunders, 2004: 358-360.

Ehlers JP, Shah CP. The Wills Eye Manual, 5th ed.

Lippincott Williams & Wilkins, 2008: 339-340.

Onofrey BE, Skorin Jr. L, Holdeman NR. Ocular Therapeutics Handbook: A Clinical Manual, 3rd ed.

Lippincott Williams & Wilkins, 2011: 250-252.

Prieto JF, Dios E, Gutierrez JM, et al. Pars planitis: epidemiology, treatment, and association with multiple sclerosis. Ocular Immunology Inflammation 2001; 9: 93-102.

Vidovic T, Cerovski B, Jukic T. The appearance of pars planitis in multiple sclerosis. Coll Antropol 2005; 29: 203-206.

Kanski JJ. Clinical Ophthalmology: A Systematic Approach, 6th ed. Butterworth Heinemann Elsevier, 2007: 456-459.

Ellis BD, Hogg JP. Neuroimaging for the General Ophthalmologist. Focal Points. Am Academy of Ophthalmology, 1998; 16: 1-8.

Raja SC, Jabs DA, Dunn JP, et al. Pars planitis: clinical features and class II HLA associations. Ophthalmology 1999; 106: 594-599.

Pars Planitis: A Review and Case Report — Skorin, Sherrill

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CE CREDIT

This course is valid for 1 hour of COPE-approved CE credit, to be taken no later than May 1, 2020.

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IDENTIFICATION Name: First______________________________ Last___________________________________ Address:________________________________________________________________________ Number

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QUESTIONNAIRE Pars Planitis: A Review and Case Report Leonid Skorin Jr., OD, DO, FAAO, FAOCO; Kristopher K. Sherill, OD 1. ❑ ❑ ❑ ❑

Which of the following profiles best characterizes a patient with pars planitis?

5-year-old girl 35-year-old woman 50-year-old man 65-year-old woman

2. ❑ ❑ ❑ ❑

All of the following statements about par planitis are true, EXCEPT: It usually occurs in children and young adults It rarely occurs after age 40 It is usually accompanied by pain It is usually bilateral

Clinical and Refractive Optometry 28:2, 2017

COPE-APPROVED CE CREDIT APPLICATION FORM

All of the following statements about par planitis are true, EXCEPT: It has an increased incidence in patients with multiple sclerosis It may be associated with smoking Decreased visual acuity may be a symptom It occurs predominantly in males

4. ❑ ❑ ❑ ❑

What is the visual acuity of the majority of patients with pars planitis at ten-year follow-up?

6/6 (20/20) 6/7.5 (20/25) 6/12 (20/40) 6/15 (20/50)

5. ❑ ❑ ❑ ❑

In the Case Report presented, the patient first presented with all of the following symptoms, EXCEPT: Blurry vision in her right eye Pain Floaters No reported double vision

6. ❑ ❑ ❑ ❑

All of the following describe the patient in the Case Report presented, EXCEPT: Intraocular pressure was 13 mmHg in both eyes Slit lamp examination was unremarkable Iritis in the right eye Dilated fundus examination showed distinct margins in both eyes

7. ❑ ❑ ❑ ❑

All of the following are systemic associations of intermediate uveitis, EXCEPT: Lupus erythematosus Lyme disease Multiple sclerosis Sarcoidosis

8. ❑ ❑ ❑ ❑

How was the patient in this Case Report treated?

Watchful waiting Topical steroids Oral steroids Corticosteroid injections

9. ❑ ❑ ❑ ❑

All of the following are symptoms of multiple sclerosis, EXCEPT: Tingling sensation Pain Vertigo Double vision

10. ❑ ❑ ❑ ❑

How often should patients with pars planitis be re-evaluated?

Every six weeks Every three to six months Every six months Every nine months

28:2, 17

3. ❑ ❑ ❑ ❑

Pars Planitis: A Review and Case Report — Skorin, Sherrill

73

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Clinical & Refractive Optometry is pleased to present this continuing education (CE) article by Dr. Meggie N. Nguyen entitled A Case of Resolving Charles Bonnet Syndrome Following Treatment of Intraocular Posterior Capsular Opacification. In order to obtain a 1-hour Council of Optometric Practitioner Education (COPE) approved CE credit, please refer to page 78 for complete instructions.

A Case of Resolving Charles Bonnet Syndrome Following Treatment of Intraocular Posterior Capsular Opacification Meggie N. Nguyen, OD, MPH

ABSTRACT Charles Bonnet syndrome (CBS) is a phenomena in which visual hallucinations are perceived in patients with ocular pathology and/or damage along the visual pathway, absent of other conditions. Patients with CBS are fully aware that the hallucinations are not real, and may find them to be pleasant. CBS is not well documented nor reported in the literature due to varying criteria for diagnosis, in addition to diagnoses made by different disciplines in medicine. CBS is a diagnosis of exclusion, and a thorough investigation is necessary to rule out other etiologies of visual hallucinations. From literature review, this is the first reported case of CBS secondary to a posterior capsular opacification after cataract surgery and some resolution after treatment.

INTRODUCTION Charles Bonnet syndrome (CBS) is a condition in which visual hallucinations are present secondary to damage or obstruction along the visual pathway. Key features of patients with CBS are intact cognitive function, awareness that the hallucinations are not real, other sensory modalities are not affected, and absent of other neurological, psychiatric, metabolic, or toxic disorders.1-8 Many of these patients with CBS find the hallucinations pleasant although bizarre due to their novel nature, and may be unaware that the hallucinations they experience is a function of their visual loss.1-6 In CBS, hallucinations can range from simple forms, e.g., photopsias, shapes and patterns, to complex forms including clear, vivid faces, and objects.1-5,9 M.N. Nguyen — Staff Optometrist, United States Air Force, Wright Patterson AFB (WPAFB) OH, 88th Medical Group, WPAFB, OH Correspondence to: Dr. Meggie N. Nguyen, Wright-Patterson Air Force Base, 4881 Sugar Maple Drive, WPAFB, OH 45433, E-mail: meggienguyen@gmail.com The author has no financial or proprietary interest in any material or method mentioned in this article.

This article has been peer reviewed.

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The prevalence of CBS is variable in the literature, as there is no uniform definition, and diagnosis is made by different disciplines in medicine.1-4 Patients may be reluctant to disclose their experience of hallucinations from fear of being labeled as mentally unstable, not taken seriously, and unaware that the condition could coexist with ocular pathology.1,3-4,10 Underlying etiologies include retinal and macular disease, cataracts, optic nerve disease, cerebrovascular disorders, corneal opacities and keratopathies, nystagmus, amblyopia, and visual field defects.1,4 Other etiologies can be secondary to ophthalmic procedures, including intravitreal injections of bevacizumab and laser photocoagulation.4,11-12 From literature review, this is the first reported case of CBS secondary to a posterior capsular opacity (PCO) after cataract surgery, and some resolution after treatment with neodymium-doped yttrium aluminum garnet (Nd:YAG) laser treatment.

CASE REPORT A 74-year-old Native American female presented with visual disturbances of seeing “people moving side to side” and “people watching her in bed constantly”. She also denoted minstrels and animals, particularly a monkey that moved around in her vision, all when looking out of a specific window in her home; this particular window had animal print curtains. The patient was able to describe this in vivid detail and knew the visual disturbances were not real.

Likewise, the patient also sought mental health consultation for reassurance. Medical history included diabetes mellitus type 2, hypertension, allergic rhinitis, and onychomycosis.

Family medical history was unknown. Patient was fully alert with cognitive function. Source of information was from the patient herself, who was able to answer all questions quickly and thoroughly. Patient was taking the following medications: econzaole nitrate 1% cream and carboxymethylcellulose sodium 1% ophthalmic. Best-corrected visual acuities (BCVA) were 6/21 (20/70) in the right eye (OD) and 6/9 (20/30) in the left eye (OS). Ocular examination was remarkable for pinguecula and arcus in both eyes (OU) and posterior chamber intraocular lens (PCIOL) OU, in addition to a grade 1+ PCO OD. Dilated fundus examination was only remarkable for posterior vitreous detachment with floaters OU. Patient was referred for Nd:YAG laser treatment.

Upon follow-up 3 months later, the patient denoted that her visual disturbances lingered, however being “smaller than ‘normal size’.” The patient also refrained from looking out of the window to mitigate seeing the visual disturbances. Since treatment, ocular examination was remarkable for BCVA of 6/6 (20/20) OD, 6/6 (20/20) OS, a cleared PCO OD and dry eye syndrome (DES) OU. Artificial lubricants were given to treat DES.Management for the visual disturbances was to monitor, reassurance that they were not real, and intervention to be considered if they became threatening. Since then, there have been no changes in medical history, medication, visual symptoms nor ocular findings.

DISCUSSION CBS was first described in the 18th century by Swiss scientist Charles Bonnet documenting his grandfather’s visual hallucinations in a published essay. In 1967, another Swiss scientist, George de Morsier, known for his contributions to neurodevelopmental disorders, coined the term Charles Bonnet Syndrome.10 Because there is no universal definition for CBS, the prevalence varies among disciplines and studies, ranging from 0.4-60% of patients with visual impairment.1-5 CBS can occur at any age,1,5,13 however, it is associated with older age secondary to increased incidence of eye pathology.1,3,5 There appears to be no predilection for gender, however the majority of studies indicate a higher occurrence in women; this could be attributed to longer lifespan and thus an increased percentage of visual impairment.2-3,9 The frequency and risk for CBS increases with decreased visual acuities; reports of patients with visual field defects and BCVA ranging from 6/6 (20/20) to 6/12 (20/40) have also experienced CBS.14-15 Hallucinations in patient with CBS can range from simple to complex vivid images. It is also important to note that these hallucinations do not affect other sensory modalities.1-2,4,5,14 They can be colored or black and white; motionless or animated; magnified or minified; and localized at varying distances in external space to the patient.2-3,5-7,16 Burke (2002) denoted that simple hallucinations can convert to complex hallucinations and vice versa. Typically, patients who exhibit complex hallucinations have a more creative personality.18 Such complex hallucinations do not have significance to the patient, nor emotional impact, nor are the faces known by the patient.4,16 It is not clear whether the hallucinations are from earlier perceptions or are newly created.6 The complexity of the hallucinations is not well predicted, as it has not been correlated to the degree, severity, and progression of loss of visual function.2-3,6,10,18 Typically, the hallucination is repeated in each episode, however some experience different hallucinations per episode.2-3,5 Changes to visual acuity, including rate of

vision loss, have been suggested to be a predisposing factor for CBS than the actual nature of ocular pathology or the severity of visual impairment.4,8 Visual hallucinations occur more often when vision loss is sudden, when both eyes are affected, and when there is profound vision loss.3-5 Cohen et al proposed that visual hallucinations occur from dynamic changes in vision even from improvement with treatment intervention, and not from the level of visual acuity itself.11 Hallucinations secondary to CBS can begin gradually or suddenly in a matter of hours to days or months from vision loss.5-7 It is important to understand the risk factors that can cause these hallucinations.3-4 These include illumination (typically dim lighting), other sensory deprivation (e.g., hearing loss), mental state (e.g., drowsiness, fatigue, stress) and social and/or physical isolation.2-4,16,18 The duration of hallucinations can be from seconds to minutes at a time; however, may also persist for hours.2-5 Frequency of the hallucinations can be episodic (lasting days to months and disappear), periodic (appearing sometimes), or continuous (everyday).2-3,5-6,19 Most hallucinations last approximately 18 months from the time of onset.3 The underlying mechanism of visual hallucinations associated with Charles-Bonnet Syndrome is not fully understood, although several theories exist.The most popular accepted theories in the literature include deafferentation theory or release phenomena and perceptual release.1-4 Deafferentation is the loss or interruption of sensory input, often caused by damage to sensory nerve fibers.3,4,20 The deafferented nerves, via biomolecular changes, become hyperexcitable, and thus visual hallucinations are released.3-4,17 Hyperexcitability is caused by either an increased amount of neurotransmitters released in the presynaptic neuron or an increase in number and/or sensitivity of the receptors in the postsynaptic membrane due to prolonged inactivity.17 Thus, damage of the neurons along the visual pathway causes the rise of spontaneous, endogenous cortical neuronal firing, leading to visual hallucinations.2,4,5 This is often compared to phantompain and phantom-limb syndromes.4,7 Deafferentation, however, does not have to occur secondary to pathology; sensory deprivation from e.g., blindfolding, can also cause increased cortical firing, leading to visual hallucinations.2,21 This is also demonstrated in case studies involving ocular patching after oculoplastics procedures.15,22 The perceptual release theory is due to an interruption or elimination of sensory nerve impulses, which interferes with the normal circuitry, leading to a disinhibition of areas in the visual association cortex that are normally inhibited, contributing to a release of visual hallucinations.4,5,9,21 It is thought that such images formed derive from the subconscious to the conscious;3,5 however, this mechanism loses its sustainability over time.18

A Case of Resolving Charles Bonnet Syndrome — Nguyen

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Neurobiological studies involving CBS have provided some insight into which parts of the brain are stimulated based on the type of hallucination experienced. In previous neuroimaging studies, functional MRI indicated signaling in the ventral occipital lobe during complex hallucinations.3,23 More specifically, facial hallucinations stimulated the superior temporal sulcus and the ventral occipital lobe around the fusiform gyrus.21,23 Activity in the fusiform gyrus was found during color hallucinations, corresponding to the color center (V4). Activity for black and white hallucinations was found outside of this region, however.21 In other studies involving single photon emission computed tomography, hyperfusion was found in the striatum, thalamus and lateral temporal cortex.3,4,21,24 Vision loss thus caused a cortical compensation in these respective areas and contributes to a rise in visual hallucinations.24

CONCLUSION Patients with CBS typically react positively to their hallucinations, as they are usually pleasant in nature, although they can sometimes cause anxiety.18,25 Other visual hallucinations can arise from a variety of neurological, psychiatric, metabolic, and toxic etiologies.1,2,4,6 Despite the fact that CBS can occur at any age, there is a higher predilection with geriatric patients, who have higher comorbidities, and thus it may be difficult to discriminate.1 CBS requires a diagnosis of exclusion, and thus a multidisciplinary approach is key to rule out other causes of visual hallucinations.1,3,4 A physical examination, medication/illicit drug use review, and blood workup with the primary care provider are necessary to rule out any metabolic or toxic etiologies.1,2,4 A baseline exam and imaging with neurology should also be included to rule out any neurological etiologies.3 A mental health evaluation for cognitive function and psychiatric disorders is also warranted.1,2,4 In addition, a comprehensive eye examination with dilation, cranial nerve testing and threshold visual field to rule out other ocular related pathologies that may contribute to symptoms of hallucinations is needed, including vitreo-retinal traction and retinal detachments.1,3,4 Management from an eye care provider standpoint includes maximizing vision with correction and low vision rehabilitation. By improving vision, it is possible to reduce the frequency of visual hallucinations.1-4,20 Maximizing vision is not only limited to ophthalmic devices such as spectacles, contact lenses, prisms and optical aids; other factors include increasing illumination, decreasing glare and increasing peripheral vision (e.g., prism).2-5 Other non-ophthalmological management strategies include rapid and frequent blinking, closing the eyes, walking toward or away from the hallucination, diversionary activities, and increasing the patientâ&#x20AC;&#x2122;s quality of social interactions.3-5,7,16,19 Ophthalmological procedures to treat

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the underlying ocular pathology such as cataract extraction, intravitreal injections and lasers should also be warranted.1-2 In this case, Nd: YAG laser was used to clear the PCO in the patient to help resolve the hallucinations.

Above all, patient education by reassuring the patient that the condition is benign is crucial in management.1-4 Should the above interventions be ineffective, it is important to refer to psychiatry for possible pharmacotherapy if the hallucinations cause the patient distress and impair their quality of life. There is no established treatment regimen for CBS due to the lack of large-scale, double-blind, placebo-controlled randomized clinical trial studies; CBS is often reported in small-scale studies and case reports and is therefore hard to generalize.1,3 Pharmacotherapy should be based on the patientâ&#x20AC;&#x2122;s needs and currently consists of anti-psychotics, anti-convulsants, and cholinesterase inhibitors.1,2,4-6,20 In the literature these have provided some alleviation of visual hallucinations; however, the results show little success.1,2,5 Overall, patient education is key to the management of CBS. Reassurance that the hallucinations experienced are benign is important to alleviate concerns of psychiatric conditions. There is a greater need for awareness of the condition for proper diagnosis and management; recognizing symptoms allows health care providers to disseminate appropriate information to patients, their families and caregivers.1 Eye care providers play a crucial role in providing patient education that CBS could be a feature secondary to visual impairment, thus alleviating initial confusion.1,4 Eye care providers can also coordinate with other health care providers to rule out other possible and more serious etiologies of visual hallucinations for effective patient management.4 It is also necessary to continue to remain vigilant in care, as elderly patients are vulnerable to many disorders that can cause visual hallucinations, in order to provide appropriate managed care.2

REFERENCES 1.

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Pang, L. Hallucinations experienced by visually impaired: Charles Bonnet syndrome. Optom Vis Sci 2016; 93(12): 1466-1478.

Zerilli-Zavgorodni T, Bisighini S. Charles Bonnet syndrome: comprehensive review providing an optometric approach to diagnosis and management. Optom Vis Perf 2014; 2(1):26-38.

Yacoub R, Ferucci S. Charles Bonnet syndrome.

Optometry 2011; 82:421-427.

Kester EM. Charles Bonnet syndrome: case presentation and literature review. Optometry 2009; 80:360-366.

Menon GJ, Rahman I, Menon SJ, Dutton GN. Complex visual hallucinations in the visually impaired: the Charles Bonnet syndrome. Surv Ophthalmol 2003; 48(1): 58-72.

Lerario A, Ciammola A, Poletti B, et al. Charlese Bonnet syndrome: two case reports and review of the literature.

J Neurol 2013; 260: 1180-1186.

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Schultz G, Needham W, Taylor R, et al. Properties of complex visual hallucinations associated with deficits in vision. Perception 1996; 25: 715-726.

Shiraishi Y, Terao T, Ibi K, et al. Charles Bonnet syndrome and visual acuity. Eur Arch Psychiatry Clin Neurosci 2004; 254(6): 362-364.

Crumbbliss KE, Taussig MJ, Jay WM. Vision rehabilitation and Charles Bonnet syndrome. Semin Ophthalmol 2008; 23: 121-126. de Morsier G. The Charles Bonnet syndrome: visual hallucinations in the aged without mental deficiency.

Ann Med Psychol (Paris) 1967; 2(5): 678-702.

Cohen SY, Bulik A, Tadayoni R, Quentel G. Visual hallucination and Charles Bonnet syndrome after photodynamic therapy for age related macular degeneration. Br J Ophthalmol 2003; 87: 977-979.

Cohen SY, Safran AB, Tadayoni R, et al. Visual hallucinations immediately after macular photocoagulation. Am J Ophthalmol 2000; 129(6): 815-816.

Schwartz TL, Vahgei L. Charles Bonney syndrome in children. J AAPOS 1998; 2(5): 310-313.

Madill SA, Ffytche DH. Charles Bonnet syndrome in patients with glaucoma and good acuity. Br J Ophthalmol 2005; 89(6):785-786.

Santos-Bueso E, Serrador-Garcia M, Saenz-Frances F, Garcia-Sanchez J. Charles Bonnet syndrome in patient with impaired visual field and good visual acuity. Neurologia 2016; 31(3): 208-209.

Teunisse RJ, Cruysberg JR, Hoefnagels WH, et al. Visual halluinations in psychologically normal people: Charles Bonnetâ&#x20AC;&#x2122;s syndrome. Lancet 1996; 347(9004): 794-797.

17. Burke W. The neural basis of Charles Bonnet hallucinations: a hypothesis. J Neurol Neurosurg Pyschiatry 2002; 73: 535-554.

18. Abbott EJ, Connor GB, Artes PH, Abadi RV. Visual loss and visual hallucinations in patients with age-related macular degeneration (Charles Bonnet syndrome). Invest Ophthalmol Vis Sci 2007; 48(3): 1416-1423.

19. Vukicevic M, Fitzmaurice K. Butterflies and black lacy patterns: the prevalence and characteristics of Charles Bonnet hallucinations in an Australian population. Clin Exp Ophthalmol 2008; 36(7): 659-665.

20. Rovner BW. The Charles Bonnet syndrome: a review of recent research. Curr Opin Ophthalmol 2006; 17(3): 275-277.

21. Kazui H, Ishii R, Yoshida T, et al. Neuroimaging studies in patients with Charles Bonnet syndrome. Psychogeriatrics 2009; 9(2): 77-84.

22. Khadavi NM, Lew H, Goldberg RA, Mancini R. A case of acute reversible Charles Bonnet syndrome following unilateral eye patch placement. Ophthal Plast Reconstr Surg 2010; 26(4): 302-304.

23. Santhouse AM, Howard RJ, Ffytche DH. Visual hallucinatory syndromes and the anatomy of the visual brain. Brain 2000; 123: 2055-2064.

24. Adachi N, Watanabe T, Matsuda H, Onuma T. Hyperfusion in the lateral temporal cortex, the striatum and the thalamus during complex visual hallucinations: single photon emission computed tomography findings in patients with Charles Bonnet syndrome. Psychiatry Clin Neurosi 2000; 54(2): 157-162.

25. Schadlu AP, Schadlu R, Shepherd JB 3rd. Charles Bonnet syndrome: a review. Curr Opin Ophthalmol 2009; 20(3): 219-222.

Call for Papers Clinical & Refractive Optometry welcomes original articles based on clinical or basic research, case reports, and review papers related to the practice of optometry.

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QUESTIONNAIRE A Case of Resolving Charles Bonnet Syndrome Following Treatment of Intraocular Posterior Capsular Opacification 1. ❑ ❑ ❑ ❑

Meggie N. Nguyen, OD All of the following are key features of patients with Charles Bonnet Syndrome (CBS), EXCEPT: Insistence that the hallucinations are real They have intact cognitive function Other sensory modalities are not affected Absence of other toxic disorders

2. ❑ ❑ ❑ ❑

Underlying etiologies for CBS include all of the following, EXCEPT: Retinal and macular disease History of the condition in one or both parents Cataracts Optic nerve disease

Clinical and Refractive Optometry 28:2, 2017

❑ ❑ ❑ ❑

In the Case Report presented, what was the patient’s best-corrected visual acuity (BCVA) in the right eye at initial presentation?

20/20 (6/6) 20/32 (6/9.5) 20/50 (6/15) 20/70 (6/21)

4. ❑ ❑ ❑ ❑

All of the following statements about CBS are true, EXCEPT: There appears to be no predilection for gender The frequency increases with decreased visual acuities There is a higher occurrence in men There is a higher occurrence in women

5. ❑ ❑ ❑ ❑

All of the following are risk factors for CBS, EXCEPT: Hormonal changes Mental state Increasing age Certain types of ocular surgery

6. ❑ ❑ ❑ ❑

Which of the following statements about visual hallucinations is not true?

They occur more often when visual loss is sudden They occur more often when both eyes are affected They occur more often when one eye is affected They occur more often when there is profound vision loss

7. ❑ ❑ ❑ ❑

All of the following factors may cause CBS, EXCEPT: Drowsiness Dim lighting Fatigue Extended periods of near work on the computer

8. ❑ ❑ ❑ ❑

Treatment for CBS may include all of the following, EXCEPT: Anti-epileptic medications Anti-psychotic medications Cholinesterase inhibitors Anti-convulsants

9. ❑ ❑ ❑ ❑

In the Case Report presented, what were the patient’s BCVAs since treatment?

20/16 (6/4.8) OD, 20/32 (6/9.5) OD 20/20 (6/6) OD, 20/20 (6/6) OS 20/32 (6/9.5) OD, 20/20 (6/6) OS 20/40 (6/12) OD, 20/20 (6/6) OS

10. ❑ ❑ ❑ ❑

Patients who exhibit complex hallucinations tend to have what type of personality?

Assertive Shy Creative Scientific

28.2:17

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3.

A Case of Resolving Charles Bonnet Syndrome — Nguyen

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FYidoctors Acquires Specs Optometry, City Focus Optometry and Gole Eyeworks FYidoctors, a private, optometristowned and operated eye care company based in Calgary, Alberta, has announced that Specs Optometry, City Focus Optometry and Gole Eyeworks have become part of the FYidoctors group.

As part of the Specs Optometry transaction, Drs. Ali Moradian, Andrew Denson, Marie-Josee Laflamme, Patrick O’Brien and Karen Pinchak of Specs have “joined the FYidoctors family,” according to a recent announcement.

Specs Optometry is a two-location practice that has served the communities of Airdrie and Carstairs, Alberta, for more than 20 years.

Dr. Alan Ulsifer, president and chief executive of FYidoctors, noted that earlier in his career he practiced in the current Specs Optometry location in Airdrie. “It is amazing how these communities have grown and how successful these two clinics have become,” he added. “These are entrepreneurial doctors who have made a name for themselves in the community and I am very proud to have them on our team.”

In a separate acquisition, FYidoctors added City Focus Optometry and its staff of Dr. Laurie Dodds, Dr. Jill Bridges, Dr. Annie Drouin and Dr. Lori Lukey. City Focus is a long-standing and reputable practice located in downtown Calgary, according to FYidoctors.

Bridges, the founding doctor of City Focus, works closely with the Alberta Children’s Hospital in customizing contact lenses specifically for infant and toddler patients, the announcement noted.

A third deal, dating to late |2016, saw FYidoctors acquire Gole Eyeworks, a long-standing practice with two locations serving the communities of Newmarket and Arthur, Ontario. Drs. Robert Gole, Najma Uddin, Michael Hinch and their staff subsequently joined FYidoctors.

Clinical and Refractive Optometry 28:2, 2017

Transitions Optical Launches New, User-Friendly Website as Resource for Canadian ECPs Transitions Optical has unveiled a new, streamlined website for Canadian eye care professionals (ECPs), giving them instant access to recent product education resources and Transitions® brand assets for their practices.

The new website can be found by visiting Transitions.com and clicking on the Transitions PRO tab.

While on the site, eye care professionals can find downloadable information and assets within the following four categories: • Educational Tools: Product Knowledge – The latest information on the Transitions® family of products, including self-guided trainings, Tech Talk videos, product brochures and FAQs • Marketing Assets – Various resources for integrating the Transitions brand into a practice’s marketing and social media efforts, including product logos, images, infographics, and instructions for ordering point-of-sale materials • Blue Light – A wealth of information on how to protect patient eyes from harmful blue light, including videos, a study guide, a white paper and tech notes • White Papers – Top-notch research about photochromic technology, serving culturally diverse groups, fostering healthy sight and harmful blue light “This new website for Canadian professionals is easier to navigate because we’re immediately directing visitors to the resources they look to Transitions Optical for: product education and marketing resources,” said Patience Cook, director North America Marketing, Transitions Optical.

Eye care professionals can visit the new website at Transitions.com and take advantage of the resources available.