Glaucoma? Maybe Not Look for These Mimics

EyeNetNOVEMBER 2018

Fix That Leaky Bleb! Defuse a Ticking Time Bomb

Today’s Scleral Lenses Options, Pearls, New Algorithms for Tx

CASE STUDY The Surprising Reasons for a Practice’s Poor Profitability

01_Cover_F.indd 1 10/9/18 4:45 PM RHOPRESSA (netarsudil ophthalmic solution) 0.02% Rx Only ®

BRIEF SUMMARY Consult the Full Prescribing Information for complete product information.

INDICATIONS AND USAGE RHOPRESSA® (netarsudil ophthalmic solution) 0.02% is indicated for the reduction of elevated intraocular pressure (IOP) in patients with open-angle glaucoma or ocular hypertension.

DOSAGE AND ADMINISTRATION The recommended dosage is one drop in the affected eye(s) once daily in the evening.

If one dose is missed, treatment should continue with the next dose in the evening. Twice a day dosing is not well tolerated and is not recommended. If RHOPRESSA is to be used concomitantly with other topical ophthalmic drug products to lower IOP, administer each drug product at least 5 minutes apart.

WARNINGS AND PRECAUTIONS Bacterial Keratitis There have been reports of bacterial keratitis associated with the use of multiple-dose containers of topical ophthalmic products. These containers had been previously contaminated by patients who, in most cases, had a concurrent corneal disease or a disruption of the ocular epithelial surface.

Use with Contact Lenses RHOPRESSA contains benzalkonium chloride, which may be absorbed by soft contact lenses. Contact lenses should be removed prior to instillation of RHOPRESSA and may be reinserted 15 minutes following its administration.

ADVERSE REACTIONS Clinical Trials Experience Because clinical studies are conducted under widely varying conditions, adverse reaction rates observed in the clinical studies of a drug cannot be directly compared to rates in the clinical studies of another drug and may not reflect the rates observed in practice.

The most common ocular adverse reaction observed in controlled clinical studies with RHOPRESSA dosed once daily was conjunctival hyperemia which was reported in 53% of patients. Other common (approximately 20%) ocular adverse reactions reported were: corneal verticillata, instillation site pain, and conjunctival hemorrhage. Instillation site erythema, corneal staining, blurred vision, increased lacrimation, erythema of eyelid, and reduced visual acuity were reported in 5-10% of patients.

Corneal Verticillata Corneal verticillata occurred in approximately 20% of the patients in controlled clinical studies. The corneal verticillata seen in RHOPRESSA-treated patients were first noted at 4 weeks of daily dosing. This reaction did not result in any apparent visual functional changes in patients. Most corneal verticillata resolved upon discontinuation of treatment.

USE IN SPECIFIC POPULATIONS Pregnancy There are no available data on RHOPRESSA use in pregnant women to inform any drug associated risk; however, systemic exposure to netarsudil from ocular administration is low. Intravenous administration of netarsudil to pregnant rats and rabbits during organogenesis did not produce adverse embryofetal effects at clinically relevant systemic exposures.

Animal Data Netarsudil administered daily by intravenous injection to rats during organogenesis caused abortions and embryofetal lethality at doses ≥0.3 mg/kg/day (126-fold the plasma exposure

at the recommended human ophthalmic dose [RHOD], based on Cmax). The no-observed-adverse-effect-level (NOAEL) for embryofetal development toxicity was 0.1 mg/kg/day

(40-fold the plasma exposure at the RHOD, based on Cmax).

Netarsudil administered daily by intravenous injection to rabbits during organogenesis caused embryofetal lethality and decreased fetal weight at 5 mg/kg/day (1480-fold the plasma

exposure at the RHOD, based on Cmax). Malformations were observed at ≥3 mg/kg/day (1330-fold the plasma exposure at the RHOD, based on Cmax), including thoracogastroschisis,

umbilical hernia and absent intermediate lung lobe. The NOAEL for embryofetal development toxicity was 0.5 mg/kg/day (214-fold the plasma exposure at the RHOD, based on maxC ).

Lactation There are no data on the presence of RHOPRESSA in human milk, the effects on the breastfed infant, or the effects on milk production. However, systemic exposure to netarsudil following topical ocular administration is low, and it is not known whether measurable levels of netarsudil would be present in maternal milk following topical ocular administration. The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for RHOPRESSA and any potential adverse effects on the breastfed child from RHOPRESSA.

Pediatric Use Safety and effectiveness in pediatric patients below the age of 18 years have not been established.

Geriatric Use No overall differences in safety or effectiveness have been observed between elderly and other adult patients.

NONCLINICAL TOXICOLOGY Carcinogenesis, Mutagenesis, Impairment of Fertility Long-term studies in animals have not been performed to evaluate the carcinogenic potential of netarsudil. Netarsudil was not mutagenic in the Ames test, in the mouse lymphoma test, or in the in vivo rat micronucleus test. Studies to evaluate the effects of netarsudil on male or female fertility in animals have not been performed.

Manufactured for: Aerie Pharmaceuticals, Inc., Irvine, CA 92614, U.S.A.

For more information, go to www.RHOPRESSA.com or call 1-855-AerieRx (1-855-237-4379).

RHOPRESSA is a registered trademark of Aerie Pharmaceuticals, Inc. U.S. Patent Nos.: 8,450,344; 8,394,826; 9,096,569; 9,415,043

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A NEW MECHANISM TO LOWER ELEVATED IOP1,2

› A ROCK inhibitor specifically designed to target the trabecular meshwork to increase aqueous outflow1 › Provides IOP reduction of up to 5 mmHg1

Targeted MOA Once-daily dosing Minimal systemic AEs is different from all other simplifies treatment regimen4 and no contraindications1 FDA-approved IOP-lowering The most common ocular AE observed therapies1-3 in controlled studies with Rhopressa® was conjunctival hyperemia (reported in 53% of patients)1

AE, adverse event; IOP, intraocular pressure; ROCK, rho-associated protein kinase.

TO LEARN MORE, PLEASE VISIT RHOPRESSA.COM

INDICATION of topical ophthalmic products. These containers had RHOPRESSA® (netarsudil ophthalmic solution) 0.02% been inadvertently contaminated by patients who, in most is a Rho kinase inhibitor indicated for the reduction of cases, had a concurrent corneal disease or a disruption elevated intraocular pressure in patients with open-angle of the ocular epithelial surface. glaucoma or ocular hypertension. Adverse Reactions: The most common ocular adverse Dosage and Administration: The recommended dosage is reaction observed in controlled clinical studies with one drop in the affected eye(s) once daily in the evening. RHOPRESSA® dosed once daily was conjunctival hyperemia, IMPORTANT SAFETY INFORMATION reported in 53% of patients. Other common (approximately 20%) adverse reactions were: corneal verticillata, Dosage and Administration: Twice a day dosing is not ® instillation site pain, and conjunctival hemorrhage well tolerated and is not recommended. If RHOPRESSA Instillation site erythema, corneal staining, blurred vision, is to be used concomitantly with other topical ophthalmic increased lacrimation, erythema of eyelid, and reduced drug products to lower IOP, administer each drug product visual acuity were reported in 5-10% of patients. The at least 5 minutes apart. corneal verticillata seen in RHOPRESSA®-treated patients Warnings and Precautions: were first noted at 4 weeks of daily dosing. This reaction did Bacterial Keratitis - There have been reports of bacterial not result in any apparent visual functional changes. Most keratitis associated with the use of multiple-dose containers corneal verticillata resolved upon discontinuation of treatment.

Please see the adjacent page for Brief Summary of Safety Information. For full Prescribing Information, please visit Rhopressa.com.

REFERENCES: 1. Rhopressa [prescribing information]. Irvine, CA: Aerie Pharmaceuticals, Inc; 2017. 2. Serle JB, Katz LJ, McLaurin E, et al; and ROCKET-1 and ROCKET-2 Study Groups. Two phase 3 clinical trials comparing the safety and efficacy of netarsudil to timolol in patients with elevated intraocular pressure.Am J Ophthalmol. 2017;S0002-9394(17)30513-5. 3. US Department of Health and Human Services, Food and Drug Administration Dermatologic and Ophthalmic Drugs Advisory Committee briefing document: NDA 208254. Published October 13, 2017. 4. Bansal R, Tsai J. Compliance/adherence to glaucoma medications—a challenge. J Curr Glaucoma Pract. 2007;1(2):22-25.

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624 RefSol-M5ion-EN.indd 1 10/2/18 10:27 AM 00_Ads_F.indd 4 10/4/18 3:49 PM CONTENTS NOVEMBER 2018 VOLUME 22 • NUMBER 11

FEATURE 40-45 When It’s Not Glaucoma A variety of conditions can produce visual field defects, OCT findings, optic nerve abnormalities, and nerve fiber layer loss that mimic glaucoma. What you need to know.

CLINICAL INSIGHTS 17-19 News in Review Genetics Gene tx for retinitis pigmentosa. Drug Side Effects Review of novel oral anti coagulants and intraocular bleeding. Infectious Disease Lyme disease and the risk of neuroborreliosis. 40 Retina PRP or anti-VEGF for proliferative diabetic retinopathy?

4 21-2 Journal Highlights Key findings from Ophthalmology, Ophthal mology Retina, AJO, JAMA Ophthalmology, and more.

27-32 Clinical Update Cornea Update on scleral lenses: Lens choice, 17 27 clinical pearls, and new treatment algorithms. Glaucoma How to fix a leaky bleb—and why it’s critical to do so. 31 35 35-36 Ophthalmic Pearls Terson Syndrome Timely diagnosis and management of this often-overlooked disease are critical for avoiding permanent visual impairment and supporting neurorehabilitation.

EyeNet® Magazine (ISSN 1097-2986) is published monthly by the American Academy of Ophthalmology , 655 Beach St., San Fran- cisco, CA 94109-1336, as a membership service. Subscription is included in U.S. members’ annual dues. International Member , IMIT, $135 per year. Nonmember in U.S., $150 per year. Nonmember outside U.S., $210 per year. Periodicals Postage Paid at San Francisco, CA, and at additional mailing offices. POSTMASTER: Send address changes to EyeNet, P.O. Box 7424, San Francisco, CA 94120-7424. American Academy of Ophthalmic Executives®, EyeSmart®, EyeWiki®, IRIS® Registry, MIPS QCDR measures, and ONE® Network are trademarks of the American Academy of Ophthalmology®. All other trademarks are the property of their respective owners.

EYENET MAGAZINE • 5

05-06_TOC_F.indd 5 10/9/18 4:47 PM CLINICAL INSIGHTS 37-38 Morning Rounds A Bothersome Bump The 8-year-old’s eyelid was swollen, droopy, and painful. He started taking Augmentin, but it didn’t help.

IN PRACTICE 47-48 Savvy Coder ICD-10 News Overwhelmed by the recent changes to ICD-10? Here are the key points. 37 49-50 Practice Perfect Case Study: Tackling Profitability Problems Academy benchmarking data revealed hidden challenges for this practice.

FROM THE AAO 53-55 Academy Notebook Former Academy president wins Eye Health Hero Award. • Member survey results. • Qualifying for the MIPS hardship exception. 53

VIEWPOINTS 10 Opinion Collaboration in academic research.

13-14 Current Perspective All about trust.

MYSTERY IMAGE 58 Blink What do you see?

COVER ILLUSTRATION © Peter Bollinger 58 xx

® COPYRIGHT © 2018, American Academy of Ophthalmology, Inc.® All rights reserved. No part of this publication may be reproduced without written permission from the publisher. Letters to the editor and other unsolicited material are assumed intended for publication and EyeNet are subject to editorial review, acceptance, and editing. Disclaimer. The ideas and opinions expressed in EyeNet are those of the authors, and do not necessarily reflect any position of the editors, the publisher, or the American Academy of Ophthalmology. Because this publication provides information on the latest developments in ophthalmology, articles may include information on drug or device applications that are not considered community standard, or that reflect indications not included in appr oved FDA labeling. Such ideas are provided as information and education only so that practitioners may be aware of alternative methods of the practice of medicine. Information in this publication should not be considered endorsement, promotion, or in any other way encouragement for the use of any particular procedure, technique, device, or product. EyeNet, its editors, the publisher, or the Academy in no event will be liable for any injury and/or damages arising out of any decision made or action taken or not taken in reliance on information contained herein.

6 • NOVEMBER 2018

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00_Ads_F.indd 7 10/5/18 10:21 AM EDITORIAL BOARD ®

MAGAZINE CATARACT Steven L. Mansberger, MD, MPH Michael F. Chiang, MD Kevin M. Miller, MD, Ronit Nesher, MD Jane C. Edmond, MD David W. Parke II, MD Section Editor Richard K. Parrish II, MD Frank Joseph Martin, MD Editor-in-Chief William R. Barlow, MD Sarwat Salim, MD, FACS Federico G. Velez, MD Ruth D. Williams, MD Kenneth L. Cohen, MD LOW VISION REFRACTIVE SURGERY Chief Medical Editor Kendall E. Donaldson, MD Lylas G. Mogk, MD George O. Waring IV, MD, Dale E. Fajardo, EdD, MBA Jason J. Jones, MD John D. Shepherd, MD Section Editor Publisher Boris Malyugin, MD, PhD Damien Gatinel, MD NEURO-OPHTHALMOLOGY Patty Ames Cathleen M. McCabe, MD Soosan Jacob, FRCS Executive Editor Randall J. Olson, MD Leah Levi, MD, Section Editor A. John Kanellopoulos, MD Marie Jose Tassignon, MD J. Bradley Randleman, MD Carey S. Ballard Kimberly Cockerham, MD, FACS Art Director / COMPREHENSIVE Karolinne M. Rocha, MD Helen V. Danesh-Meyer, MD, PhD, Production Manager Marcony R. Santhiago, MD OPHTHALMOLOGY FRANZCO Preston H. Blomquist, MD, Chris McDonagh, Jean Shaw Prem S. Subramanian, MD, PhD RETINA/VITREOUS Section Editor Senior Editors Julia A. Haller, MD, Sherleen Huang Chen, MD OCULOPLASTICS Section Editor Krista Thomas Evan H. Black, MD, April Y. Maa, MD Neil M. Bressler, MD Assistant Editor / Section Editor Advertising Manager Linda M. Tsai, MD Kimberly A. Drenser, MD, PhD Elizabeth A. Bradley, MD CORNEA AND EXTERNAL Sharon Fekrat, MD Lori Baker-Schena, MBA, EdD; Femida Kherani, MD Leslie Burling; Peggy Denny; DISEASE Mitchell Goff, MD Don O. Kikkawa, MD Miriam Karmel; Mike Mott; Christopher J. Rapuano, MD, Lawrence S. Halperin, MD Linda Roach; Lynda Seminara; Section Editor OPHTHALMIC ONCOLOGY Gregg T. Kokame, MD Annie Stuart; Gabrielle Weiner Kathryn A. Colby, MD, PhD Zélia M. Corrêa, MD, PhD, Andreas K. Lauer, MD Section Editor Contributing Writers Helena Prior Filipe, MD Prithvi Mruthyunjaya, MD, MHS Bennie H. Jeng, MD Dan S. Gombos, MD Kyoko Ohno-Matsui, MD Mark Mrvica, Kelly Miller Stephen D. McLeod, MD Tatyana Milman, MD Andrew P. Schachat, MD M.J. Mrvica Associates, Inc. 2 West Taunton Ave., Sonal S. Tuli, MD OPHTHALMIC PATHOLOGY Ingrid U. Scott, MD, MPH Berlin, NJ 08009 Deepak Paul Edward, MD Gaurav K. Shah, MD GLAUCOMA 856-768-9360 Sanjay G. Asrani, MD, David J. Wilson, MD UVEITIS [email protected] Section Editor OPHTHALMIC Gary N. Holland, MD, Advertising Sales Section Editor Iqbal K. Ahmed, MD PHOTOGRAPHY Lama Al-Aswad, MD, MPH Jason S. Calhoun Muge R. Kesen, MD Ahmad A. Aref, MD, MBA Michael P. Kelly, FOPS H. Nida Sen, MD Anne Louise Coleman, MD, PhD Steven Yeh, MD PEDIATRIC Steven J. Gedde, MD 655 Beach St. OPHTHALMOLOGY Catherine Green, MBChB San Francisco, CA 94109 David A. Plager, MD, 866-561-8558, 415-561-8500 Section Editor aao.org

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Keith D. Carter, MD, FACS CLINICAL EDUCATION Paul B. Ginsburg, PhD ARTICLE REVIEW PROCESS. Articles Louis B. Cantor, MD involving single-source medical and tech- PRESIDENT-ELECT TRUSTEES-AT-LARGE nical news are sent to quoted sources for George A. Williams, MD Michael F. Chiang, MD verification of accuracy prior to publication. SR. SECRETARY FOR Quotes and other information in multisource OPHTHALMIC PRACTICE William S. Clifford, MD articles are subject to confirmation by their PAST PRESIDENT respective sources. The chief medical editor Robert E. Wiggins Jr., MD, MHA Cynthia A. Bradford, MD Sanjay D. Goel, MD and the executive editor review all news and Cynthia Mattox, MD, FACS feature articles and have sole discretion as CHAIR, THE COUNCIL CEO to the acceptance and rejection of material Lynn K. Gordon, MD, PhD William F. Mieler, MD and final authority as to revisions deemed David W. Parke II, MD necessary for publication. Andrew M. Prince, MD VICE CHAIR, THE COUNCIL SR. SECRETARY FOR DISCLOSURE KEY. Financial interests Sarwat Salim, MD, FACS INTERNATIONAL TRUSTEES are indicated by the following abbrevia- ADVOCACY Kgaogelo Edward Legodi, MD tions: Daniel J. Briceland, MD OPHTHALMOLOGY EDITOR C = Consultant/Advisor Lihteh Wu, MD E = Employee Stephen D. McLeod, MD L = Speakers bureau SECRETARY O = Equity owner FOR ANNUAL MEETING CHAIR OF THE FOUNDATION P = Patents/Royalty S = Grant support Maria M. Aaron, MD ADVISORY BOARD Learn more about the Board For definitions of each category, see Christie L. Morse, MD at aao.org/bot. aao.org/eyenet/disclosures.

8 • NOVEMBER 2018

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RUTH D. WILLIAMS, MD Collaboration in Academic Research

hen Bob Dylan was awarded the 2016 Nobel disciplinary approach, ophthalmic researchers must recognize Prize in Literature, his fans were astonished and and woo individuals who may not currently be working on Wdelighted. His Nobel acceptance speech (recorded vision research. Neeru finds this task exciting and rewarding, after the ceremony that he didn’t attend) is mostly an ode to but it requires creativity, great communication skills, time, his influences, from Buddy Holly and Appalachian ballads to and investment in relationships. Don Quixote, the Odyssey, and All Quiet on the Western Front, Third, as David pointed out, among others. While Dylan appears to be a singular genius, promotion in academic institutions and the Nobel Prize in Literature is given to 1 person, the is based on individual metrics, Nobel Prize in Physiology or Medicine can be given to which are easier to assess than 3 people (and almost always leaves out a significant contrib- collaborative efforts. Neeru utor). Scientific research is, by nature, collaborative. agreed, although she noted David Calkins, at Vanderbilt Eye Institute in Nashville, a positive shift “toward rec- Tennessee, credits the Human Genome Project as one of the ognizing collaborative efforts, best examples of collaborative research. The project required both in publications and at support from the NIH and private industry, along with the the institutional level, includ- partnership of scientists from nearly a dozen countries with ing promotions.” expertise in genetics, molecular biology, information tech- How to overcome these bar- nology, biochemistry, and biostatistics. At the University riers? Many academic researchers of Toronto, Neeru Gupta believes such collaboration is encourage teamwork. At Vanderbilt, necessary because of the rapid growth of specialized knowl- David aspires to create a culture of col- Ruth D. edge. Her own research into the lymphatic vessels as a new laboration by emphasizing the values Williams, MD target for eye disease draws on the expertise of physiologists, of teamwork, accountability, and data Chief Medical pathologists, physicists, and engineers. And Gary Novack, sharing. And philanthropic and organi- Editor, EyeNet a pharmacologist at the University of California, Davis, zational efforts can promote innovative asserted that basic science research and drug development collaborative projects. For example, the require working together. “You cannot be successful unless Glaucoma Research Foundation (GRF) initiated Catalyst for you realize that you do not know everything.” a Cure. In this program, a team of 4 researchers is selected by Yet academic researchers can be somewhat isolated. This a scientific advisory board and funded to work together on a isn’t a new issue: In 1963, Science published a letter by Bernard specific challenge. GRF’s current team is gearing up to coor- K. Forscher, in which he compared academic research to a dinate innovative research on neuroprotection. And let’s not brickworks.1 Warning of academic isolationism, he suggested forget the IRIS Registry (see page 13), which is the world’s that the brickmaking could become an end unto itself. largest specialty clinical database and can be employed to What are some of the barriers to collaborative research in answer specific questions quickly. ophthalmology? First, most academic scientists must build In looking ahead, Carla imagines that “Ophthalmology an individual extramural funding portfolio. NEI funding can be a leader—as we have been in other aspects of med- for research is a competitive process, and scientists contend icine—to elevate the profession and provide guidelines for for the same too-small pool of money. As Carla Siegfried, at this new perspective of collaboration in research develop- Washington University in St. Louis, said, “If one views the ment, adding value to our scarce research dollars.” funding source as a ‘zero-sum game,’ then the competition 1 Forscher BK. Science. 1963;142(3590):339. may suppress potential valuable collaborations.” Second, since major breakthroughs usually require a multi NEXT MONTH. Successes in increasing NEI funding.

10 • NOVEMBER 2018

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DAVID W. PARKE II, MD All About Trust

n September 20, The New York Times and ProPubli- stripped of patient and physician identifiers when used for ca broke a story about Memorial Sloan Kettering commercial purposes. Further, it can be used for analysis OCancer Center (MSKCC) and its health care data only if it is aggregated with other data to further preclude start-up Paige.AI. They detailed how the start-up company discovery of personal identification. Additionally, the data had exclusive access to MSKCC tissue resources and that 3 can be used only for projects that are scientifically valid and board members and 3 key executives were investors or had consistent with the Academy’s mission. equity stakes. This all came on the heels of an investigation It became obvious that there was commercial appetite into drug industry ties of its chief medical officer (who then for the data. Accordingly, the Academy decided in 2016 to resigned). Questions were also raised about using individual market some IRIS-derived data to pharmaceuti- identifiable information about patients and physicians with- cal and device companies under carefully out their knowledge. MSKCC in response noted the expense controlled circumstances. I won’t go and risk of an enterprise with the potential to change the fu- into great detail as to the review ture of cancer diagnosis. Overall, the report stimulated ques- and control processes, but suffice tions about transparency, optics, and conflicts of interest. it to say that they were drafted The American Academy of Ophthalmology adheres to a by task forces of Academy robust set of policies that govern our approach to financial members, subjected to legal (and nonfinancial) conflicts of interest. These can be found review, and then approved on aao.org and include our internal policies, policies of the by the Board of Trustees. A Accreditation Council for Continuing Medical Education, Research and Analytics Com- and those from the Council of Medical Specialty Societies’ mittee composed of Academy Code for Interactions With Companies (which I helped write). members provided ongoing Their collective objective is not to eliminate all potential con- oversight. flicts, but to create a path for disclosure and management, However, marketing data was not recusal when appropriate, and elimination where necessary. the “core business” of the Academy, and It is meant to give members, and the public, trust in the the Board concluded that the Academy David W. transparency and integrity of the Academy. either had to find a commercialization Parke II, MD partner or develop a commercialization Academy CEO The IRIS Registry arm. At the same time, the need to com- In the aftermath of the MSKCC debacle, I received questions mercialize the data became critical. Early in the development about the relationship between the Academy IRIS (Intelligent of the IRIS Registry, we decided to offer it at no cost as a mem Research in Sight) clinical registry data and Verana Health, ber benefit—which was unusual in the registry space. How- the for-profit company to which the Academy has licensed ever, the IRIS Registry’s operating costs, due to its size and commercialization of IRIS Registry data. Here are the facts. complexity, grew to exceed $5 million per year. This meant that As you are probably aware, health-related data has signifi- to sustain the IRIS Registry, the Academy either had to charge cant potential commercial value. One company in the oncol- its members or find a revenue stream to offset part of that cost. ogy space, Flatiron Health, sold last year for over $2 billion. Several companies have been founded by Academy members Choosing a Partner to monetize various subsets of clinical data. We therefore looked for a partner organization that (at a Each Academy member whose electronic health record minimum) fulfilled the following characteristics: (EHR) system is integrated with the IRIS Registry has signed 1. Expertise in the health data space an agreement that permits data to be used—but only if it is 2. Strong technology team

EYENET MAGAZINE • 13

13-14_Perspective_F.indd 13 10/4/18 2:59 PM 3. Access to substantive investment capital treatment alternatives, illuminating disease natural history in 4. Knowledge of ophthalmology large populations, and examining the impact of comorbidi- 5. Expertise with for-profit/nonprofit partnerships ties and confounding variables on disease progression. 6. Reputation for operational integrity Initial steps. In the year since the relationship was signed The Academy found this partner in DigiSight Technologies, with DigiSight what has happened? First, as mentioned earlier, a Silicon Valley–based company cofounded by ophthalmol- the company was renamed Verana Health. This was intended ogist Mark Blumenkranz. After nearly 6 months of negotia- to signal that the company is now a health care data company tions and constant review by the Board of Trustees, general —not an ophthalmology company. Second, the company has counsel, outside legal counsel, and extramural advisors, the totally restructured itself around the IRIS Registry—its core Board approved licensing commercialization of the IRIS asset. Third, it has received a large infusion of capital from Registry to DigiSight Technologies in early November 2017. some of America’s legendary venture capitalists and com- Here are some of the basic terms of that agreement. The panies. Fourth, it has recruited outstanding leadership and Academy continues to own the IRIS Registry. It licenses beefed up its technology and data science teams. It has had commercialization to DigiSight. The Academy nominates a commercial success in its mission to accelerate health care member to the DigiSight board. DigiSight is bound to the innovation through data insights. same restrictions regarding de-identified and aggregated data Most important for Academy members, nothing has as is the Academy. The Academy has considerable influence happened to cast a shadow on the Academy’s reputation (and at times absolute authority) over many elements of the for independence and integrity. Rather, we are getting kudos commercial transactions. In return, the IRIS Registry’s opin the medical society space for the way we executed this erating costs were reduced by 80%, and there is downstream relationship. Others are trying to emulate us. revenue potential. Overall, the final document runs 70 pages. There were weeks when I spent over 20 hours in document Conflict of Interest review and negotiation. As for potential financial conflicts, these are carefully scruti- Benefits.What do the Academy and its members get out nized. I sit as the Academy’s Board-designated representative of this? First and foremost, it helps to ensure the sustainability on the boards of FIGmd (our registry software vendor) and of the IRIS Registry—the largest specialty society clinical Verana Health. I receive no compensation, stock, or stock op- data registry in all of medicine. The IRIS Registry is trans- tions for my work representing the Academy. And no member forming our profession, and it also provides a Merit-Based of the Academy Board or staff has any financial relationship Incentive Payment System quality reporting system that will with Verana or FIGmd—not even a complimentary T-shirt. save our members an estimated $186 million in avoided penalties next year, when the MIPS payment penalties—based The IRIS Registry: A Growing Asset on last year’s performance—start to take effect. It has led to The IRIS Registry had its fifth birthday and is still growing. numerous peer-reviewed scientific papers with novel clinical Over 18,000 ophthalmologists and their employed optom- findings that promise to enhance the quality and delivery etrists use it. It contains more than 200 million patient of care for our patients. And it is an increasingly important encounter records. Over 50 EHR companies are mapped to vehicle for clinical benchmarking and quality improvement. it. It still has problems, however. EHR updates play havoc The IRIS Registry has received accolades from policymakers, with data mapping. Every year CMS approves new quality public health experts, data analysts, and other medical orga- measures, which must be coded into the IRIS Registry’s soft- nizations as “best of breed.” ware. The data must be continuously and carefully curated to Second, the Academy will likely now be financially able to ensure quality to avoid the “garbage in/garbage out” dilem- continue offering the IRIS Registry at no charge to members. ma. With time, we hope to ingest more data from images, And if it is very successful commercially, IRIS Registry revenue from genetic testing, and from patients themselves. may provide an alternative to dues dollars. Throughout this process, the Academy hopes the IRIS Third, and critically, DigiSight (now renamed Verana Registry will bring more and more value to its members as a Health) makes the data analytic tools and platforms that it tool to benchmark and judge clinical outcomes and processes develops and uses for commercial purposes available at no of care. We anticipate that ophthalmologists will choose to charge to the Academy for our own analytic purposes. employ the IRIS Registry to report their quality outcomes Fourth, the Academy retains full authority over noncom- to CMS, commercial payers, and health systems as means of mercial data projects conducted by members, Academy- avoiding payment penalties, garnering bonuses, and demon- affiliated organizations, or the Academy itself. Commercial strating their value. It will continue to support our advocacy activities do not interfere with these projects. and policy objectives. And we anticipate that it will become Finally, all of ophthalmology retains a high-quality tool a robust source of generating new scientific information that that can provide novel insights into real-world evidence— will change the way we care for patients. what actually happens in the practice of ophthalmology. At all times, the Board of Trustees and senior staff pledge It can be a basis for comparing clinical trials to real-world to oversee the IRIS Registry and Verana Health to protect the practice, performing postmarket surveillance of drugs and reputation of the Academy for integrity, independence, and devices, studying rare diseases, comparing outcomes from trustworthiness. It may be our most important asset.

14 • NOVEMBER 2018

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GENETICS from the normal allele, or it has on its own a toxic gain of function, meaning Potential Gene that this protein may be toxic to the cell Therapy for RP and kill it over time,” said William A. Beltran, DVM, PhD, at the University SCIENTISTS HAVE DEVELOPED A GENE of Pennsylvania in Philadelphia. therapy for the most common form of The scientists concluded that their autosomal-dominant retinitis pigmen- treatment would have to not only tosa (RP) caused by mutations in the prevent the mutant gene already there rhodopsin (RHO) gene—and success- from producing abnormal, toxic rho fully demonstrated that it can prevent dopsin but also provide sufficient nor- retinal degeneration in a canine model, mal rhodopsin protein for the rods to an approach that someday could be survive and function normally. But in harnessed to halt the disease in humans. order to do this, the therapy also would The researchers designed an adeno- need to work against the more than 150 NOVEL STRATEGY. Topographical maps associated viral vector to knock down gene mutations known to cause auto of outer nuclear layer thickness show the expression of existing RHO (both somal-dominant RP. “The challenge how the combined RHO knockdown normal and mutant genes) and replace was to develop a treatment that can and replacement therapy protected them with a normal copy of human address any mutation,” Dr. Beltran said. against severe retinal degeneration in RHO.1 Retinal imaging and electro- Their solution was a dual-purpose a naturally occurring canine model of retinography showed that this approach viral vector: One part was targeted at autosomal-dominant RP (right panels). kept the rod photoreceptors healthy and “knocking down” all endogenous prevented retinal degeneration for at rhodopsin mRNAs, both mutant and successfully treat areas where the retina least 8 months of follow-up. normal, through a technique known is already degenerating. “We’re going to A dual-purpose approach. This as RNA interference. The second part be looking at whether we can intervene strategy differs in key ways from the consisted of normal human RHO that at stages that are clinically relevant— gene augmentation approach that was modified to avoid degradation by and target areas where there are still led to a commercially available gene the knockdown component. rod photoreceptor cells left that can be therapy (Luxturna) for Leber congen- “The rods would not be able to rescued,” Dr. Beltran said. “If we’re able to ital amaurosis (LCA). Because LCA is function, or survive over the long term, show that, that will expand the poten- autosomal recessive, that viral vector if you didn’t bring back a normal wild- tial candidates for gene therapy.” was engineered solely to transduce type copy of RHO. The vector also —Linda Roach the retinal pigment epithelium with delivers a gene that has been modified a single copy of the RPE65 gene to at some very specific sites so that it still 1 Cideciyan AV et al. Proc Natl Acad Sci U S A. produce the missing protein. produces the same amino acid sequence 2018;115(36):E8547-E8556. In autosomal-dominant RP, rod as the rhodopsin protein, but it is not Relevant financial disclosures—Dr. Beltran: photoreceptor cells express rhodopsin knocked down at the RNA level,” said Foundation Fighting Blindness: S; NIH/NEI: proteins from both normal and mutant coauthor Alfred S. Lewin, PhD, at the S; Research to Prevent Blindness: S; The Shaler RHO. “The mutant protein produced University of Florida in Gainesville. Richardson Professorship Endowmment: S; in the rods either interferes negatively What’s next. The researchers will University of Pennsylvania: P. Dr. Lewin: NEI: S;

University of Pennsylvania University with the wild-type protein that comes study whether the viral vector can University of Florida: P.

EYENET MAGAZINE • 17

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00_Ads_F.indd 16 10/4/18 3:49 PM DRUG SIDE EFFECTS of edoxaban and warfarin might explain the difference Bleeding Risk and in bleeding outcomes. “In NOACs the case of edoxaban . . . it can be speculated that the A REVIEW OF OUTCOMES IN MORE direct inhibition of Factor than 100,000 patients suggests that one Xa confers enhanced control of the novel oral anticoagulant medica- over the coagulation cascade; tions (NOACs) on the market might be in contrast, warfarin targets less likely than warfarin to cause ocular factors II, VII, IX, X and hemorrhages. regulatory factors protein Outcomes better with edoxaban. A C, S, and Z, offering poorer network meta-analysis of findings from pharmacodynamic preci- BLEEDING. This image shows a preretinal sub 12 randomized controlled clinical trials sion,” they wrote. hyaloid hemorrhage. found a statistically significant reduced Equivalent outcomes risk of ocular bleeding in anticoagulat- with other NOACs. No statistically sig- tions profiles relative to warfarin,” the ed patients who took edoxaban when nificant reductions in risk were found researchers wrote. compared to warfarin (odds ratio: 0.59, with 3 other available NOACs (rivar- Study limitations. However, the confidence interval 0.34-0.98).1 Tra- oxaban, dabigatran, and apixaban). possible ophthalmic lessons from this ditional meta-analyses, using pooled “There was a nonsignificant trend review study are limited because of pairwise and subgroup comparisons, toward apixaban having more intra deficiencies in the underlying data, also supported this conclusion (both ocular bleeding adverse events com- commented M. Gilbert Grand, MD, of p = 0.02). pared with warfarin, whereas dabiga- The Retina Institute in St. Louis. The authors hypothesized that the tran and rivaroxaban appeared to have No sham control. One difficulty molecular inhibition characteristics similar intraocular bleeding complica- is that there is no sham control, Dr.

INFECTIOUS DISEASE mic symptoms and findings in individuals diagnosed Neuroborreliosis: Add Lyme with neuroborreliosis verified by CSF analysis.” Study specifics. Over a 6-year period, 24 patients Disease to Your Differential who had either been diagnosed with Lyme disease or were strong suspects were referred to the hospital’s OCULAR MANIFESTATIONS OF LYME DISEASE MAY department of ophthalmology. All were tested for Bor be more prevalent than commonly thought, a small relia burgdorferi antibodies no later than 2 days after prospective Swedish study suggests. admission and underwent lumbar puncture no later The study, conducted in western Sweden, found that than 3 days after admission. the majority of patients diagnosed with neuroborreli- Results. Neuroborreliosis was confirmed in 16 osis (typically found in stage 2 of Lyme disease) had patients, while 2 patients were classified as possible ophthalmic signs and symptoms.1 The most frequent cases. Diagnosis was negative in 4 patients and un- findings were blurred vision, diplopia, photophobia, known in the remaining 2. Of the 18 patients classified redness, sixth nerve involvement, and palpebral dias- as definite or possible, 14 (78%) had ophthalmic signs tasis resulting from facial palsy. Moreover, there was a and symptoms. All patients improved except for 1 with positive correlation between signs and symptoms and fulminant papilledema; this patient still had optic disc cerebrospinal fluid (CSF) antibody titres. atrophy and affected visual fields at last follow-up. Study rationale. The research team was motivated A surprise. In contrast with previous studies, the in part by evidence that “ticks are increasing in number researchers found no evidence of either conjunctivitis and becoming more widespread in the northern parts or uveitis, Dr. Grönlund said. The reason for this remains of Europe,” said coauthor Marita A. Grönlund, MD, PhD, unknown. at Sahlgrenska University Hospital in Gothenburg, Take-home message. “For ophthalmologists, there Sweden. might be reason to think twice about neuroborreliosis And although previous studies have documented not only in subjects with facial palsy but also in those the ocular manifestations of Lyme disease, they have with [new-onset] diplopia and/or sixth nerve affection,” been case reports and case studies, she noted. “There- Dr. Grönlund concluded. —Jean Shaw fore, we and our coworkers at the [hospital’s] department of infectious diseases thought that it was of great 1 Škiljić D et al. Acta Ophthalmol. Published online Aug. 26, 2018. importance to further evaluate and follow up ophthal- Relevant financial disclosures—Dr. Grönlund: None. M. Gilbert Grand, MD M. Gilbert Grand,

18 • NOVEMBER 2018

17-19_News_F.indd 18 10/9/18 4:58 PM Grand cautioned. The researchers did RETINA not “calculate how many people get ocular bleeding because of preexisting PRP Preferred for ocular disease, without taking warfarin Some Patients? and the NOACs.” No definition of hemorrhage.In IN THE IDEAL WORLD OF CON- addition, they did not define intra trolled scientific studies, the strategy ocular hemorrhage, Dr. Grand said. of treating proliferative diabetic reti For instance, subconjunctival hemor nopathy (PDR) with intravitreal injec rhages were included in a list of po tions has proved effective and possibly tential intraocular bleeds. “So we don’t superior to panretinal photocoagulation know what type of events they were (PRP). Now researchers report that really analyzing.” in the “real” world, where patients are Need for clarification.The study often lost to follow-up, PRP may be RD RISK. This eye with PDR developed does support the conclusion that spon the better option.1 an RD after being lost to follow-up. taneous ocular hemorrhages occur “Part of the impetus for this study rarely in patients undergoing anti was that we know PRP has long-lasting eyes in the anti-VEGF group had coagulation therapy, Dr. Grand said. effects on stabilizing PDR, but we have tractional retinal detachment (RD) However, it “does not add any data little data on whether anti-VEGF thera after patients returned to care. At the describing the risk of hemorrhage in py has any long-lasting effects once it is return visit, 5 in the anti-VEGF group anticoagulated patients who undergo stopped,” said Jason Hsu, MD, at Wills experienced tractional RD, versus none intraocular surgery,” he said. “So this Eye Hospital in Baltimore. in the PRP group. At the final visit, 10 dataset, while valuable in itself, does Now, data exist. “Our study sug anti-VEGF patients had tractional RD, not provide insights for ophthalmol gests that if there is a period of loss versus 1 PRP patient. However, the ogists who must make decisions as to to follow-up, patients with PDR who incidence of tractional RD was lower in whether to continue or discontinue receive PRP may have better outcomes eyes that received a greater number of anticoagulation at the time of ophthal compared to those who received only anti-VEGF injections prior to being lost mic surgery.” anti-VEGF therapy,” said coauthor An to follow-up. “This may suggest that Fortunately, there are published data thony Obeid, MD, MPH, also at Wills receiving a certain minimum number from earlier research on the safety of Eye Hospital. of injections may have lasting effects on vitreoretinal surgery in patients taking Retrospective cohort. The findings PDR regression,” Dr. Obeid said. warfarin2 or the NOACs,3 Dr. Grand are based on medical records of 59 pa Stick with PRP. The findings are par noted. tients with PDR (76 eyes) who returned ticularly relevant as practice patterns Need for further research. This at various time points for follow-up are shifting toward anti-VEGF mono study also raises an issue worthy of treatment. All of the 59 patients had therapy for eyes with PDR, the authors further examination, Dr. Grand pointed been lost to follow-up for 6 or more said. They assume even greater relevance out. “There are certain ophthalmic months immediately after receiving ei given the “strikingly high” rates of pa diseases, such as exudative age-related ther intravitreal injections (20 patients; tients lost to follow-up,2 said Dr. Hsu. macular degeneration or proliferative 30 eyes) or PRP (39 patients; 46 eyes). “Some clinicians believe that PRP retinopathies, in which patients bleed Findings include the following: may not be necessary or can be delayed spontaneously whether or not they’re • Visual acuity (VA) scores worsened while the patient is actively receiving taking anticoagulants,” he said. “What in anti-VEGF eyes, from 20/54 at the anti-VEGF treatments,” Dr. Hsu added. this study does not determine, and visit before patients were lost to follow- “However, our study suggests that phy what we need to know, is whether an up to 20/187 at the return visit and 20/ sicians may want to proceed with PRP ticoagulation therapy in those patients 166 at the final visit. at an earlier time point given the po increases their risk of hemorrhages.” • In PRP eyes, VA significantly wors tential for poorer outcomes with erratic —Linda Roach ened from 20/53 at the visit before pa follow-up.” (For more on this topic, see tients were lost to follow-up to 20/83 at pg. 23.) —Miriam Karmel 1 Phan K et al. Br J Ophthalmol. Published online the return visit. However, VA improved June 20, 2018. by the final visit to 20/58. 1 Obeid A et al. Ophthalmology. Published online 2 Dayani PN, Grand MG. Arch Ophthalmol. 2006; • There was a significantly greater in Aug. 2, 2018. 124(11):1558-1565. cidence of neovascularization of the iris 2 Obeid A et al. Ophthalmology. 2018;129(9): 3 Grand MG, Walia HS. Retina. 2016;36(2):299- in the anti-VEGF group compared to 1386-1392. 304. the PRP arm at the final visit (4 vs. 0). Relevant financial disclosures—Drs. Hsu and Relevant financial disclosures—Dr. Grand: None. • A significantly greater number of Obeid: None.

Anthony Obeid, MD, MPH Obeid, MD, Anthony See the financial disclosure key, page 8. For full disclosures, including category descriptions, view this News in Review at aao.org/eyenet.

EYENET MAGAZINE • 19

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BC-1960 2017 Partners for Sight Ad FullPage-EyeNet FINAL.indd 1 9/7/18 11:58 AM 00_Ads_F.indd 20 10/4/18 3:49 PM Journal Highlights NEW FINDINGS FROM THE PEER-REVIEWED LITERATURE

Ophthalmology the rates of RNFL loss, after adjusting Baseline Influencers of Vision Selected by Stephen D. McLeod, MD for confounding factors. Outcomes of and Edema in Proliferative DR: interest were the effects of m-wiVD and Ranibizumab Versus PRP Predicting RNFL Thinning in onh-wiVD on rates of RNFL loss. November 2018 Glaucoma The average RNFL thickness at base- November 2018 line was 79.5 ± 14.8 μm, which declined In a post hoc analysis of data from by a mean slope of –1.07 μm per year. randomized multicenter trials, Bressler Moghimi et al. investigated potential In the univariate model, which includ- et al. aimed to identify baseline factors links between thinning of the retinal ed just a predictive factor and time associated with change in visual acuity nerve fiber layer (RNFL) and baseline plus their interaction, each 1% lower (VA) or development of vision-impair- vessel density of the mac- m-wiVD and ing central-involved diabetic macular Volume 125 | Number 11 pp. XXX–XXX ula and optic nerve head Volume 125 | Number 11 | November 2018 onh-wiVD was edema (DME) occurring after treat- Elsevier | ISSN 0161-6420 (ONH). They hypothesized associated with a ment of proliferative diabetic retinop that the degree of vessel 0.11-μm per year athy (PDR) with ranibizumab or pan density may predict RNFL and 0.06-μm per retinal photocoagulation (PRP). thinning of eyes with mild year faster rate The study included 328 eyes that or moderate glaucoma. of RNFL decline, received 2 years of follow-up and 302 Their findings suggest that respectively. A eyes that did not have vision-impairing lower macular and ONH OPHTHALMOLOGY similar relation- central-involved DME at baseline in INSERT ADVERT vessel density are associated ship between low Protocol S of the Diabetic Retinopathy with faster RNFL decline, m-wiVD/onh- Clinical Research Network (DRCR. as measured by spectral- wiVD and faster net). The latter eyes were not required November 2018 domain optical coherence rates of RNFL loss to complete the 2-year visit because the tomography (SD-OCT). was observed with analysis incorporated all available and OPHTHA_v125_i11_COVER.inddFor 1this prospective 24-08-2018 17:46:07 other multivariate censored data for participants without observational study, 83 patients with models. Nevertheless, in this study, the vision-impairing central-involved DME. mild or moderate primary open-angle link between vessel density measure- Treatments were intravitreous glaucoma (132 eyes) received follow-up ments and rate of RNFL loss was weak. ranibizumab (0.5 mg/0.05 mL) or for at least 2 years (average, 27.3 ± 3.36 In univariate and multivariate analyses, PRP. Primary outcome measures were months). Measurements of macular average central corneal thickness pre- change in VA (area under the curve) whole-image vessel density (m-wiVD) dicted faster RNFL decline. and development of vision-impairing and ONH whole-image vessel density Eyes with advanced glaucoma were (20/32 or worse) central-involved DME (onh-wiVD) were acquired at baseline, not included in the study because their during the 2-year period. using OCT angiography. Measurements RNFL is unlikely to undergo rapid After multivariable analysis with of RNFL thickness, minimum rim change. This research offers new insight adjustment for baseline VA and central width, and ganglion cell plus inner for glaucoma management and sup- subfield thickness, no factors were plexiform layer thickness were ob- ports the role of OCT parameters in identified as being relevant to either tained semiannually using SD-OCT. predicting the risk and rate of glau- primary outcome. In the PRP group, Random-effects models were used to coma progression. Macular and ONH worsening VA was more common

ascertain relationships between vessel vessel density may be specific parame- with higher levels of hemoglobin A1c, density parameters at baseline and ters to include in this assessment. greater severity of diabetic retinopathy

EYENET MAGAZINE • 21

21-24_JHI_F.indd 21 10/4/18 3:57 PM (DR), and higher mean arterial pressure. deaths. Pairwise comparisons for ran- surements for each time point, with Vision-impairing central-involved DME ibizumab 0.5 mg (the globally approved and without adjustment for biome- was more likely to occur in the presence dosage for wet AMD) and sham or chanically corrected IOP and central

of high hemoglobin A1c, more severe verteporfin were performed using Cox corneal thickness. DR, and cystoid defects within 500 μm proportional hazard regression and All IOP measurements decreased of the macula center. rates per 100 patient-years. over time. Increased central corneal Overall, VA improved and vision- Hazard ratios (95% confidence thickness was noted at 1 week and 3 impairing central DME was rare with intervals) included 1, indicating no sig months. Although the Corvis biome- ranibizumab in Protocol S. The analysis nificant treatment differences, for all chanical index was elevated at 1 week, suggests that these favorable outcomes endpoints, between ranibizumab 0.5 mg it returned to preoperative status by occur regardless of baseline factors. and sham or verteporfin. Although this 1 month. A decrease in ARTh was However, when PRP is the main treat supports the established risk-benefit observed at 1 week and 1 month; this ment for PDR, patients with poor gly- profile of ranibizumab in patients with parameter returned to its preoperative cemic control or severe DR may be neovascular AMD, the authors noted level by 3 months. DA max and inte- more susceptible to vision-impairing that extrapolating these findings to the grated radius had increased by month 3, central-involved DME and VA loss than real-world population is limited by the and SP A1 had decreased by this time. are those with better glycemic control enrollment criteria of the selected stud- The authors advise caution when or milder DR, even if the DME is treat- ies—and that more data are needed on applying these results to clinical practice. ed with ranibizumab. the systemic safety of anti-VEGF drugs They noted that 1 week following —Summaries by Lynda Seminara in clinical practice. surgery may be too soon to use the —Summary by Jean Shaw Corvis biomechanical index to identify Ophthalmology Retina keratoconus. Selected by Andrew P. Schachat, MD American Journal of Ophthalmology Is It Time to Reclassify Large Vascular Safety Profile of Selected by Richard K. Parrish II, MD Macular Holes? Ranibizumab November 2018 November 2018 Cataract Surgery Alters Corneal Biomechanics and IOP In the Manchester Large Macular Hole Intravitreal anti–vascular endothelial November 2018 Study, Ch’ng et al. looked at anatomic growth factor (VEGF) drugs carry and functional outcomes after vitrec an increased risk of systemic events, Using the updated Corvis ST tonom- tomy for large full-thickness macular including those of a cardiovascular and eter, Hirasawa et al. studied the effects holes (FTMH). They found that stan- cerebrovascular nature. Zarbin et al. of cataract surgery on corneal biome- dard treatment for FTMH is adequate set out to evaluate the vascular safety chanics and intraocular pressure (IOP). for most holes under 650 μm in diam- profile of ranibizumab 0.5 mg relative They noted a decrease in the stiffness eter. to sham treatment, with or without parameter at applanation 1 (SP A1) and This retrospective interventional verteporfin, in patients with neovascu- increases in deformation amplitude study included 258 eyes with idiopathic lar age-related macular degeneration maximum (DA max) and integrated large FTMH (diameter >400 μm) treat- (AMD). In addition, they compared radius, suggesting that the cornea is less ed during a 5-year period. All eyes un- ranibizumab 0.3 mg to sham and 0.3 stiff following cataract surgery. derwent pars plana vitrectomy (PPV), mg to 0.5 mg of ranibizumab. They This prospective, interventional case internal limiting membrane (ILM) found low rates of vascular events in series included 39 patients (39 eyes) peel, gas tamponade, and face-down these patients overall and no clini- with cataract. Measurements with the posturing. The face-down position was cally meaningful differences between Corvis ST tonometer were obtained maintained for 1-5 days. Anatomic and patients treated with ranibizumab and before surgery and at 1 week, 1 month, functional success rates were measured, those treated with sham or verteporfin. and 3 months postoperatively; param- as was the relationship between the size For this study, researchers evaluated eters included DA max, DA ratio max of the macular holes and their closure. data from 7 randomized trials (phases (1 mm and 2 mm), integrated radius, Anatomic closure was achieved in 2-4). The pooled dataset comprised SP A1, Ambrosio relational thickness 90% of eyes. Rates of closure were ≥91% 4,080 patients with wet AMD. Of these, to the horizontal profile (ARTh), for patients with holes <650 μm. This 1,764 patients were treated with ranibi- Corvis biomechanical index, central coincides with the currently accepted zumab 0.3 mg, and 1,854 were treated corneal thickness, noncorrected IOP, success standard of ~90%. Among with ranibizumab 0.5 mg. Relevant and biomechanically corrected IOP. patients with larger FTMH (650 μm safety endpoints included arterial In addition, they measured IOP with to 1,416 μm), the success rate was only thromboembolic events (ATEs), myo- Goldmann applanation tonometry 76%. Maximum sensitivity and speci- cardial infarction (MI), stroke, transient and a noncontact tonometer. The linear ficity were obtained at a cutoff diameter ischemic attacks (TIAs), and vascular mixed model was used to compare mea of ≤630 μm (76.7% sensitivity, 69.2%

22 • NOVEMBER 2018

21-24_JHI_F.indd 22 10/4/18 3:57 PM specificity), yielding a Youden index of spectively, for a cumulative probability • For criteria A, final intraocular pres- 0.46. By 3 months postoperatively, 57% of 22% in the ranibizumab group and sure (IOP) of ≤18 mm Hg with either of eyes had improved ≥0.3 LogMAR 38% in the PRP group (hazard ratio = ≥20% reduction in IOP or reduction of units from preoperative status. 0.4; 95% confidence interval [CI]: 0.3- at least 2 medications. —Summaries by Lynda Seminara 0.7; p < 001). • For criteria B, a final IOP of≤ 15 mm Despite a mean VA of 20/25 in both Hg and either ≥25% reduction in IOP JAMA Ophthalmology groups at 5 years, vitreous hemorrhage or reduction of at least 2 medications. Selected and reviewed by Neil M. occurred in 48% of eyes treated with • For criteria C, a final IOP of≤ 12 Bressler, MD, and Deputy Editors ranibizumab and in 46% of eyes treated mm Hg or less and either ≥30% reduc- with PRP. Vitrectomy was performed in tion in IOP or reduction of at least 2 Five-Year Outcomes of Random- 11% and 19% of eyes, respectively. Both medications. ized Trial Comparing Laser with groups had low rates of iris neovascu Complete success was similarly de- Ranibizumab for PDR larization and neovascular glaucoma, fined with the additional requirement October 2018 although retinal detachment occurred in of no need for glaucoma medication(s). 6% of the ranibizumab group and 15% At 5 years, the qualified success rates Gross et al. compared the efficacy and of the PRP group. Rates of systemic for patients of African descent and those safety of intravitreous ranibizumab and adverse events were comparable. of European descent were as follows: panretinal photocoagulation (PRP) for The authors note that these findings For criteria A, 61% versus 67% (differ- proliferative diabetic retinopathy (PDR) support either anti–vascular endothe- ence, 7.3%, 95% confidence interval through 5 years in a randomized clini- lial growth factor therapy or PRP as [CI], 4.4-10.4); for criteria B, 43% cal trial. They found that visual acuity viable treatments for patients with PDR versus 60% (difference, 17.6%, 95% (VA) was very good for most patients in through at least 5 years and emphasize CI, 15.2-20.0); and for criteria C, 25% both study arms, consistent with 2-year the importance of considering patient- versus 40% (difference, 15.8%, 95% outcomes. Rates of vision-impairing specific factors when selecting a treat- CI, 11.1-20.5). On multivariable Cox diabetic macular edema (DME) were ment, including the patient’s anticipated regression analyses, being of African lower in the ranibizumab group. likelihood of compliance and overall descent was associated with higher fail- This study included patients who health status as well as cost issues. ure rate for criteria B and C for qual- had enrolled in the Diabetic Retinopa- ified success and with all criteria for thy Clinical Research Network (DRCR. Racial Differences in Long-Term complete success. The incidence of bleb net) Protocol S trial by December 2012. Trabeculectomy Outcomes leaks was higher in those of African Eyes had been assigned randomly to October 2018 descent (29 vs. 11 eyes); these patients receive intravitreous ranibizumab also required additional glaucoma (n = 191) or PRP (n = 203). The fre- Evidence indicates that failure after tra- surgeries more often than did those quency of ranibizumab treatment was beculectomy without antimetabolites is of European descent (47 vs. 26 eyes). based on a protocol-specified algo- more common among patients of Af- These results suggest new strate- rithm. The 5-year analysis began in rican descent. Although adjunctive use gies to control wound healing after January 2018. The main outcome was of mitomycin C (MMC) improves the trabeculectomy are needed, and the the mean change in VA; secondary out- likelihood of success, data are lacking for role of nonfiltering glaucoma surgery comes included peripheral visual field patients of African descent who have should be explored in this subpopula- loss, development of vision-impairing undergone trabeculectomy combined tion. (Also see related commentary by DME, and adverse events. with MMC. To identify prognostic Paul Palmberg, MD, PhD, in the same The 5-year visit was completed for indicators of failure, Nguyen et al. issue.) 240 eyes (184 patients), 117 of which compared outcomes of initial trabe- received ranibizumab. The mean culectomy plus MMC between patients Fellow-Eye Treatment of Open- number of treatments over 5 years of African and European descent and Angle Glaucoma: CIGTS Results was 19.2 in the ranibizumab group found that those of African descent October 2018 (with an average of 3 injections each were more likely to experience failure year in years 2, 3, 4, and 5) and a mean after trabeculectomy and bleb leak. Once it’s clear that a patient requires of 5.4 treatments over 5 years in the In their study, 135 eyes from patients unilateral treatment for open-angle PRP group. Mean changes in VA letter of African descent (n = 105) were glaucoma (OAG), it may help to know score were 3.1 and 3.0, respectively, matched to 135 eyes from patients of which traits portend disease progres- for the ranibizumab and PRP groups. European descent (n = 117). Matching sion and need for eventual treatment The mean change in cumulative visual criteria included age (within 5 years), in the fellow eye (FE). In a post hoc field total point score was −330 dB for surgeon, lens status, and follow-up time analysis of data from the Collabo- ranibizumab recipients and −527 dB (within 1 year). rative Initial Glaucoma Treatment for patients with PRP. Vision-impairing Three levels of qualified success were Study (CIGTS), Niziol et al. estimated DME occurred in 27 and 53 eyes, re- defined as follows: the time between initial treatment of

EYENET MAGAZINE • 23

21-24_JHI_F.indd 23 10/9/18 5:00 PM the study eye (SE) and the need for with dupilumab-treated atopic derma- 2 of the drugs restored osmotically treatment of the FE. They found that titis (AD). They found the strongest driven water flux of SLC4A11 mutants. by 7 years after OAG treatment of the predictors to be severe AD at baseline The 5 drugs studied were bromfenac, SE, roughly two-thirds of patients had and the presence of a concomitant diclofenac, flurbiprofen, ketorolac undergone treatment of the FE. atopic disorder. tromethamine, and nepafenac. In CIGTS, 607 participants with From a cohort of 142 patients who HEK293 cells expressing CHED- newly diagnosed OAG in at least 1 eye received dupilumab for AD, conjuncti- and FECD-causing SLC4A11 mutants were assigned randomly to receive vitis occurred in 12 (8.5%). Dupilumab were grown in 96-well dishes, with or topical medication or trabeculectomy. exposure consisted of a 600-mg loading without an NSAID. Except for ketoro- FEs were treated when eligible or at the dose, followed by weekly injection of lac, the tested drug concentrations physician’s discretion. Data were col- 300 mg. AD severity, as measured by were twice the EC50. The amount of lected for up to 11 years. Survival anal- investigator global assessment, was ketorolac was much lower (0.25 μM) ysis was used to estimate the probabili- documented at the start of treatment because concentrations >5 μM are toxic ty of FE treatment over time and to test and at the onset of conjunctivitis. to HEK293 cells. potential baseline and time-dependent At baseline, 9 (75%) of the 12 pa- Using bioluminescence resonance predictors of treatment need. Using tients had severe AD. The mean age at energy transfer (BRET) and confocal linear regression, disease trajectory was conjunctivitis onset was 30 years. The microscopy, the authors tested each calculated as the eye-specific slopes of conjunctivitis occurred at a mean of NSAID’s ability to correct mutant mean deviation (MD) and intraocular 15.8 weeks of treatment (range, 8-41 SLC4A11 cell-surface trafficking. Upon pressure (IOP) over time. In addition, weeks) and was considered severe or treatment, they also tested the ability correlations between SE and FE tra- moderate-to-severe in 4 patients. Dup- of mutant SLC4A11-expressing cells to jectories also were calculated. Main ilumab was stopped in 3 patients, all of mediate water flux, which may mimic outcomes were time to FE treatment whom had severe conjunctivitis. These water flux across the corneal endotheli- and the slopes over time (MD and IOP) 3 patients had severe AD at baseline al cell basolateral membrane. for SEs and FEs. plus at least 1 other atopic condition. BRET assays showed significant Among the FEs, 291 (47.9%) were The 2 patients who discontinued the rescue of SLC4A11 mutants to the cell treated at baseline along with SEs, 123 drug permanently also had a history of surface by 4 of the 5 NSAIDs. Diclofenac (20.3%) were treated eventually, and hay fever. In both of these patients, the and nepafenac were the most effective 193 (31.8%) did not receive treatment. conjunctivitis improved after treatment for moving endoplasmic reticulum The probability of FE treatment for and dupilumab discontinuation, but it –retained missense mutant SLC4A11 to OAG was 0.57 by year 1 and 0.68 by did not resolve fully. the cell surface. In 20 of 30 intracellular- year 7 after SE randomization. Cor- Larger, multicenter studies are retained SLC4A11 mutants, diclofenac relations in IOP slopes were 0.57, 0.24, needed to confirm risk factors and significantly restored cell-surface abun- and 0, respectively. The similarity of determine effective treatment for con- dance. In some cases, diclofenac restored slopes observed for SEs and treated FEs junctivitis. At-risk patients may benefit mutant SLC4A11 water flux activity to implies that SE change is a harbinger from early ophthalmology referral and the level of wild-type SLC4A11. Ketoro- of FE change and, therefore, warrants prophylactic care. lac had no effect on cell-surface abun- close surveillance. Two variables that dance. Of the 3 mutants examined for predict FE intervention are modifiable: Ophthalmic NSAIDs for Corneal cell-surface abundance (L843P, G709E, hypertension and IOP. Proper attention Dystrophy Caused by SLC4A11 and E143K), L843P had the greatest to these factors may reduce the need for Mutation improvement in trafficking. FE treatment. (Also see related commen- Investigative Ophthalmology & Visual This research suggests that topical tary in the same issue by Rohit Varma, Science ophthalmic NSAIDs possess sufficient MD, MPH, and Xuejuan Jiang, PhD.) 2018;59(10):4258-4267 permeability to reach the corneal endo- —Summaries by Lynda Seminara thelium. The authors encourage testing Some mutations of the SLC4A11 gene of diclofenac eyedrops to treat corneal OTHER JOURNALS cause misfolding of the SLC4A11 pro- dystrophy in patients with certain Selected by Deepak P. Edward, MD tein, which may lead to Fuchs endo- SLC4A11 missense mutations. Wide use thelial corneal dystrophy (FECD) or of NSAIDs for FECD or CHED would Conjunctivitis Secondary to congenital hereditary endothelial dys- require robust data from well-designed Dupilumab Treatment of Atopic trophy (CHED). Alka and Casey tested clinical trials in which appropriate Dermatitis 5 ophthalmic nonsteroidal anti-inflam- dosing regimens are established. JAMA Dermatology matory drugs (NSAIDs) for their ability —Summaries by Lynda Seminara Published online Aug. 29, 2018 to correct SLC4A11 folding defects. They found that 4 of the 5 NSAIDs MORE ONLINE. For an Treister et al. set out to pinpoint risk provided significant rescue of SLC4A11 additional summary, see this factors for conjunctivitis among patients mutants to the cell surface. In addition, article at aao.org/eyenet.

24 • NOVEMBER 2018

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00_Ads_F.indd 26 10/4/18 3:49 PM CORNEA CLINICAL UPDATE

Update on Scleral Lenses

hile awareness of the 1A 1B potential beneﬁts of scleral Wlenses—large-diameter rigid gas-permeable lenses—has steadily increased over the past decade, it still lags among ophthalmologists. Although ftting contact lenses is typically managed by optometrists, it is important for ophthalmologists to understand the potential role for scleral lenses, particularly in corneal ectasia and ocular surface disease, noted Sanjay V. Patel, MD, at the Mayo Clinic in Rochester, Minnesota. IMPROVEMENT. This 10-year-old was diagnosed with Stevens-Johnson syndrome Scleral Lenses 101 at age 5. (1A) Six months after being switched from extended-wear soft contacts to Designed to vault over the entire corne- PROSE treatment. (1B) At the 24-month mark after being switched to PROSE, the al surface and rest on the sclera, scleral eye is quiet and regression of pannus is evident. lenses can morph an irregular cornea into a smooth optical surface to correct management of both corneal ectasia and that which occurs following refrac- vision problems caused by keratoconus and ocular surface disease,” he said. tive surgery—and refractive error from and other forms of corneal ectasia. “They’ve helped reduce the need for irregular corneal shapes, including that Furthermore, the space between the surgical intervention and signifcant- occuring after corneal transplantation cornea and the back of the scleral lens ly improved the quality of life for so or linked to scarring. acts as a fluid reservoir, continuously many patients, especially those with Advantages. The main beneft of bathing the cornea. This can provide severe ocular surface disease [e.g., scleral lenses is that they can be designed relief for people with severe ocular Stevens-Johnson syndrome, graft- to accommodate any degree of corneal surface disease and may help the ocular versus-host disease, neurotrophic steepness or irregularity, said Deborah surface to heal. keratitis, exposure keratitis, and neuro- S. Jacobs, MD, at Massachusetts Eye Indications. The primary indications pathic pain] who have symptoms [that and Ear and Harvard Medical School in for scleral lenses are corneal irregular- are] refractory to other therapies.” Boston. They provide better centration ity, ocular surface disease, and severe When Dr. Mian frst started pre- and stability than corneal lenses, and refractive error. scribing scleral lenses, it was only for they are more comfortable because the At the Kellogg Eye Center in Ann dry eye disease. That is still a common conjunctival tissue on which scleral Arbor, Michigan, Shahzad I. Mian, MD, indication, but he is more likely to use lenses rest is less sensitive than corneal has been using scleral lenses for 16 years. them nowadays for corneal ectasia— tissue. “Scleral lenses have transformed the both the ectasia seen with keratoconus Furthermore, in patients who have experienced damage to corneal tissue, scleral lenses do not touch the cornea BY GABRIELLE WEINER, CONTRIBUTING WRITER, INTERVIEWING DEBORAH but rather bathe it continuously in

Deborah S. Jacobs, MD S. Jacobs, Deborah S. JACOBS, MD, SHAHZAD I. MIAN, MD, AND SANJAY V. PATEL, MD. preservative-free saline so that scar

EYENET MAGAZINE • 27

27-29_CU corn_F.indd 27 10/4/18 3:04 PM formation is not exacerbated.1 and that involves an assessment, then tion, like that offered with PROSE, they Drawbacks. Availability of scleral customization, then monitoring,” meet the needs of most patients at a lenses has improved over the last decade, explained Dr. Jacobs, formerly with lower but variable cost. but other obstacles remain, namely cost BostonSight. Delivery and cost. Scleral lenses are and convenience. Fitting and customization. “The ca- sold through a optometric model: The Time. The optometrist who is fitting pability of the fitter in customizing the lab sells them to the optometrist who the lens requires special training, and design and shape of the lens is different fits the lenses. In turn, the optometrist the fitting process is time consuming, for PROSE than it is for the commercial sells them to the patient. Thus, it is up requiring several visits. lenses, for which there are just a few pa- to the optometrist to determine what to Cost. The fitting process is reflected rameters the fitter can adjust and then charge for the lens and what the fitting in the cost, which ranges broadly from communicate to the lab that makes the fees are. $500 per lens plus a fitting fee to an all- lens,” Dr. Jacobs said. In contrast, in In the United States, the estimated inclusive fee of several thousand per PROSE, fitters use a computer-assisted average overall cost per eye (the lens eye, depending on the type of lens and design system that enables them to ma- plus the fitting) runs from $1,000- the fitting process (see below). This is nipulate directly each prosthetic device $5,000, depending on the complexity not covered by many health insurance to the patient’s precise and unique eye of the condition and the technology plans, though some vision care plans shape. required. may cover it all or in part, Dr. Jacobs “The academic centers that became Another option: Scleral variations. noted. PROSE satellites brought on an optom- Modified, smaller rigid gas-permeable Convenience. “Other limiting factors etrist who typically had specialized in lenses are separated by size into mini are debris collection in the reservoir, cornea and contact lens in a ‘residency’ scleral, semiscleral (also called intralim- fogging, and fouling of the front surface year following optometry school. In bal), or corneoscleral types. Respective- of the lens,” Dr. Jacobs said. In addition, that year, optometrists learn to fit spe- ly, their edges rest slightly outside the Dr. Patel pointed out, “Patients may cialty lenses and get comfortable with limbus, slightly inside it, or partly on need to pop them out in the middle of a broader spectrum of corneal disease,” the cornea and partly on the sclera. the day to give them a scrub, then pop said Dr. Jacobs. These smaller lenses are not true them back in. It can be inconvenient for Delivery and cost. PROSE is deliv- scleral lenses and may only be used some patients.” ered through a medical model, with a for corneal irregularity, not for ocu- single fee per eye set by the institution lar surface disease. Definitions and Which Lens Is Best? with all services related to treatment terminology are still evolving, but Dr. At Kellogg Eye Center, Dr. Mian and his wrapped in, Dr. Jacobs said. Jacobs defines true scleral lenses as colleagues have fitted many hundreds The approximate cost for PROSE, having a diameter greater than 18 mm. of patients with sclerals. Initially, they which is sometimes referred to as the In contrast, the diameters of the lenses used only PROSE (Prosthetic Replace- Rolls Royce of scleral lenses, is on the mentioned above range from 13-17 mm. ment of the Ocular Surface Ecosystem) order of $5,000-$7,000 per eye for the and sent patients to Boston, where entire process. Updated Treatment Algorithms PROSE was developed by BostonSight Commercially available scleral New paradigm for keratoconus. It used in the early 1990s. About 10 years ago, lenses. At Mayo, Dr. Patel and his col- to be that treatment of keratoconus Kellogg became one of the early satellite leagues popularized fitting lenses from consisted of glasses or soft lenses for clinics for PROSE treatment. a diagnostic set. “It is cheaper for the mild stages, rigid gas-permeable corneal Since then, “there has been an patients, and most eyes—not all—can contact lenses for moderate stages, and explosion of different types of scleral be fit that way,” he said. corneal transplantation for severe cases. lenses, and our contact lens special- Fitting and customization. As with The indication for surgery was contact ists fit both PROSE and commercially PROSE, the goals of fitting are that the lens failure. available lenses,” Dr. Mian said. “Often lens is completely supported by the But the advent of specialty lenses, PROSE is still the preferred lens for the sclera, achieves complete clearance over including custom soft lenses, better hy- most severe patients, particularly the the cornea and limbus, and achieves an brid lenses, and now scleral lenses and severe dry eye patients. That said, many even bearing zone on the sclera to avoid PROSE treatment, has extended the patients can be fit successfully with the compression of blood vessels. The fitter limit of what can be accomplished with other types of sclerals,” he added. uses the diagnostic set of trial lenses contact lenses. “A case [of keratoconus] PROSE. “PROSE is really a treatment to determine the correct sagittal depth is not a ‘contact lens failure’ without approach more than a piece of plastic. and then does overrefraction to obtain a trial of specialty lens,” Dr. Jacobs Yes, it produces a large-diameter gas- lens power. The back surface periph- emphasized. permeable lens that the patient wears eral curve system can be modified to “Many patients who would have on a daily basis, but the endpoint of provide the best fit. come to surgery a decade or 2 ago can the whole design, fit, and customiza- Although commercially available retain good vision for life with specialty tion [process] is prosthetic function, sclerals can’t offer complete customiza- lenses and particularly scleral lenses,”

28 • NOVEMBER 2018

27-29_CU corn_F.indd 28 10/9/18 5:01 PM said Dr. Jacobs. “The surgeons who years old.” more expensive.” have access to scleral lenses for their With regard to the other end of Concerns over long-term use. In patients report anecdotally that their the age spectrum, he added, “It’s also order to consider using scleral lenses rate of corneal transplant for ectasia feasible to fit patients into their 80s or for vision correction, not for disease, and astigmatism has gone way down.” 90s, as long as they can take care of the ophthalmologists need to know more Indeed, Drs. Patel and Mian confirmed lenses.” about potential long-term side effects. a significant reduction in surgery rates Work with the optometrist. When Although the lenses sit on the sclera, in their practices. And in a Belgian scleral lenses are used for ocular surface they come very close to the limbus. “If study published earlier this year, 40 of disease, the comprehensive ophthal- scleral lenses damage the limbus, it can the 51 eyes with severe keratoconus mologist or cornea specialist will need cause big problems long term. We have that would have undergone transplant to collaborate on an ongoing basis with no clinical evidence of that happening, surgery were successfully treated with the optometrist, because the underlying but we worry about it,” said Dr. Mian. long-term scleral lens wear, reducing disease that will need to be monitored Possible exception: Athletes with the indication for keratoplasty by more for neovascularization or potential in- astigmatism. One exception to phy- than half.1 fection continues to exist, Dr. Patel said. sician reluctance to prescribe might Management of ocular surface dis- “Supplemental treatments are still be the use of minisclerals for serious ease. Scleral lenses are usually used lat- needed, especially when the patient athletes with astigmatism. er in the disease course. One exception, takes the lenses out. They’ll still use Take a professional baseball player, according to Dr. Mian, is patients with lubricants; they’ll often still use topical for example. “Soft lenses don’t correct severe ocular surface disease associated steroids; they might still use serum sharply enough, glasses are a problem with graft-versus-host disease. “Those tears,” he said. For corneal ectasia, the if you’re diving after a line drive, and patients often progress quickly to the optometrist can often handle the case corneal lenses move around and can get very severe stage and typically don’t alone, but the lines of communication dirt and grit under them,” Dr. Jacobs respond to other therapies, so we will should be kept open if a complication said. “For serious baseball players with move to scleral lenses faster.” arises, Dr. Patel noted. ordinary astigmatism, a miniscleral In all cases, over-the-counter lubri- Observe your patients with their would be easy to fit and would correct cants are started immediately, then top- lenses on. Dr. Jacobs encouraged all them well.” To address concerns regard- ical medication (e.g., cyclosporine or a ophthalmologists to have a look at their ing long-term use, the athlete could topical steroid) and punctal occlusion patients with their scleral lenses on wear the miniscleral lenses just for are considered. The next option may so that they can gain an appreciation games and practices. be serum tears. Dr. Mian then turns to for fit and physiologic function. “In But can an athlete—or anyone else PROSE treatment before considering contemporary practice, where there is —wear minisclerals for 12-14 hours a amniotic membrane contact lenses like so much emphasis on volume, the stan- day over many years? “The miniscleral Prokera (Bio-Tissue). Tarsorrhaphy and dard is that the staff measures vision in world is working on answering that conjunctival flap come after that. lenses, then takes them out to proceed question,” Dr. Jacobs said. with the rest of the eye exam prior to Clinical Pearls the physician seeing the patient,” Dr. 1 Koppen C et al. Am J Ophthalmol. 2018;185:43- Especially in patients who have ocular Jacobs said. “But even if not trained in 47. surface disease, Dr. Patel urged oph- fitting, the ophthalmologist can gain a thalmologists to think about a scleral better understanding of scleral lenses Dr. Jacobs is associate professor of ophthalmolo- lens as an alternative to tarsorrhaphy when examining the eyes with them on gy at Harvard Medical School. She is also faculty or jumping to amniotic membrane or and then off.” on the Cornea Service and director of the Ocular keratoplasty. “Think of scleral lenses Surface Imaging Center at Massachusetts’ Eye and seek them out,” he said. Lowering the Clinical Threshold and Ear in Boston. Relevant financial disclosures: Don’t count out the young—or the Because severe refractive error is an BostonSight: E (through February 2018). old. Dr. Mian reported that some of his indication for scleral lenses, what’s Dr. Mian is professor of ophthalmology and youngest patients have greatly benefit- stopping clinicians from using them visual sciences, the Terry J. Bergstrom Collegiate ted from scleral lenses. These children for more typical refractive errors? Professor for Resident Education in Ophthalmol- had severe dry eye or neurotrophic No added value. Soft lenses remain ogy and Visual Sciences, and associate chair for disease (from herpetic infection) and the primary way to correct refractive education at the University of Michigan’s Kellogg were unable to heal the surface of their error because most patients can tolerate Eye Center in Ann Arbor, Mich. Relevant financial eye on their own. “Despite multiple them, they’re easy to use, and there’s disclosures: None. other treatments that hadn’t worked, less maintenance. “Convenience is the Dr. Patel is professor and chair of ophthalmology scleral lenses made a big difference in No. 1 factor,” said Dr. Mian, “and scler- at the Mayo Clinic in Rochester, Minn. Relevant improving the health of their eyes and als are inconvenient. Using them solely financial disclosures: None. maintaining vision in their eyes,” said for refractive error doesn’t add more See the disclosure key, page 8. For full disclosures, Dr. Mian. “My youngest patient was 2 value to the average patient and is far view this article at aao.org/eyenet.

EYENET MAGAZINE • 29

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00_Ads_F.indd 30 10/9/18 12:23 PM GLAUCOMA CLINICAL UPDATE

A Ticking Time Bomb: How to Fix a Leaking Bleb

leaking filtration bleb is a conjunctival tissue itself. “Early-onset 1 common complication of bleb leakage occurs in the immediate Atrabeculectomy that can occur period following surgery,” said Sunita days, months, or years after the initial Radhakrishnan, MD, at the Glauco- surgery. It’s also a vision-threatening ma Center of San Francisco. “There danger that shouldn’t be ignored. might be a leak at the incision site due The bottom line: There should be to incomplete conjunctival closure or no delay when addressing any type of wound dehiscence, for example. Or the bleb leak, said Neeru Gupta, MD, PhD, surgeon might have created an inad- MBA, at the University of Toronto. “If vertent opening in the conjunctival the leak does not seal, the patient will tissue.” The area where the bleb is most have fluid leaving the eye, which can elevated can also develop a leak due to drop the pressure and lead to a whole drying or microtrauma from repeated host of complications.” Moreover, she blinking. pointed out, “the leaking eye has no Late-onset bleb leakage, on the other barrier to the outside world and, sud- hand, is typically the result of thin bleb denly, it’s open season for pathogens.” tissue. “The biggest reason a patient ENDOPHTHALMITIS. Three years after Thus, “It’s really important to see each would develop this type of avascular undergoing trabeculectomy, this patient leaking bleb as a ticking time bomb,” tissue is the use of antifibrotics,” Dr. presented with pain, redness, and loss Dr. Gupta said. “Yes, the bleb surgery Radhakrishnan said. “Mitomycin C of vision in the left eye. can be technically challenging, but the and 5-fluorouracil are commonly used general ophthalmologist has a critical- as adjunctive treatment in filtering untreated, the leak can lead to hypoto- ly important role to play. Although a surgery to help prevent fibrosis and ny, which can result in a shallow or flat glaucoma specialist may be involved in scarring. Although this can increase chamber, peripheral anterior synechiae,

. aao.org/eyenet fixing the [actual] leak, it’s imperative the survival of a filtering bleb, it can hypotony maculopathy, choroidal ef- that every ophthalmologist knows how also result in more fragile tissue that is fusion, corneal striae, or even bleeding to read the signs of what can be a very prone to leakage in the future.” and surgical failure,” Dr. Robin said. sight-threatening condition.” Risk of infection. “Bleb-related in- Danger, Danger fection is a very dangerous and volatile A Faulty Filter Regardless of when the leakage occurs, situation if not managed promptly,” Dr. The conjunctival tissue of a bleb can “A leaking bleb is not a minor issue,” Gupta emphasized. “An open barrier in become thinned and cystic due to the said Alan L. Robin, MD, at the Uni- the ocular surface exposes the eye to constant flow of aqueous, and the thin- versity of Michigan in Ann Arbor and any number of pathogens and micro- nest and most avascular areas are most John Hopkins University in Baltimore. organisms, so there’s a serious risk of susceptible to developing a leak. “Early detection and management can intraocular infection. This can range This leakage can result from the sur- help prevent serious complications.” from blebitis, an infection in or around gical technique and/or the nature of the Potential complications. “If left the bleb without vitreous involvement, to endophthalmitis [Fig. 1].” Signs of trouble. The surgeon can BY MIKE MOTT, CONTRIBUTING WRITER, INTERVIEWING NEERU GUPTA, usually monitor for early-onset bleb

The American Society of Retina Specialists. For full credit, see this article at see this article at full credit, For Specialists. American Society of Retina The MD, PHD, MBA, SUNITA RADHAKRISHNAN, MD, AND ALAN L. ROBIN, MD. leaks during surgery or in the immedi-

EYENET MAGAZINE • 31

31-32_CU glau_F.indd 31 10/9/18 7:30 PM ate postoperative period. for bleb leak repair, the common goal is disturbing the preexisting aqueous After this point—and during nor- in the various surgical techniques is flow. “Once you’ve repaired a leak, the mal follow-up—common signs of bleb to cover the filtration site with healthy conjunctiva is now thicker, so the leakage include a newly tearing eye, a conjunctiva. The unhealthy bleb tissue pressure-lowering efficacy of the noticeable change in vision, redness, or is usually denuded or excised. original trabeculectomy can decrease. a drop in intraocular pressure (IOP) “Conjunctival advancement is 1 You might see an early postoperative from baseline. “If you suspect a patient technique of bleb repair that entails pressure spike from closing the bleb might have a leak, perform a Seidel test covering the leaking bleb with a new leak, or a slow rise in pressure over time at the slit lamp to confirm,” said Dr. flap of healthy conjunctival tissue that as the bleb function slowly decreases. In Gupta. “After painting the conjunctiva is advanced from the region posterior our study, for example, 9% of patients with a fluorescein strip under cobalt to the bleb,” said Dr. Radhakrishnan. required additional glaucoma surgery blue light, you’ll see the leak in the “I typically remove the tissue that is anywhere from 2 months to 7 years form of a greenish fluid escaping from leaking or ischemic. If there is not following bleb revision.” behind the brown stain.” enough healthy conjunctiva for this Aside from glaucoma control, there Referral. “The bleb should be ex- approach, then a free conjunctival are a few other—and less common— amined at every visit,” Dr. Robin said. autograft from either the same eye complications to be mindful of, said “If a leak is present, given the potential or the fellow eye can be used.” Dr. Gupta. These include hypertropia, serious sequelae, it should be addressed To test the bond after tacking down ptosis, and dysesthesia. “When we’re immediately.” the advancement, Dr. Robin will inject performing advancement surgery, balanced salt solution into the anterior we are tugging on tissue from behind Stopping the Flow chamber to check for any leakage and the upper lid close to the muscle and Bleb leaks can resolve spontaneously, then confirm the result with a fluores- pulling it forward. This can result in but if they don’t, what’s the fix? Treat- cein test. muscle misalignment and drooping of ment depends largely on your patient’s Learning curve. “Repairing a bleb the eyelid. And because the advanced needs and how they present. leak surgically is not a particularly easy conjunctiva might not sit flat at the Medical treatment. “Conservative fix,” said Dr. Gupta. “Suturing through limbus, the patient may experience eye management is my first-line approach,” an avascular bleb that is already thin discomfort when blinking.” said Dr. Radhakrishnan, “especially if and friable—even with a fine suture Risk of another leak. Of course, the the leak is small with no infection, the like 10-0 nylon—can be challenging. patient might also develop another visual acuity and pressure are stable, The tissue may be as delicate as wet tis- leak, she added. “There might also be and the patient has no past history of sue paper, and punching holes in it can something about the quality of the bleb-related infection. Initially, I’ll use be like operating on Saran Wrap: One patient’s conjunctiva that predisposes it aqueous suppressants alongside pro- hole creates another even larger one.” to leakage, and, voila, after plugging 1 phylactic antibiotics to protect against Because of these difficulties, oph- hole, you’ve got another, and you have infection. As long as the eye is stable thalmologists are continually searching to treat all over again.” and the bleb leak is decreasing, this ap- for innovative ways to achieve the proach can be followed until complete same results. “The patient might have Dr. Gupta is professor of ophthalmology and resolution in many cases.” unhealthy conjunctiva lacking useable chief of the Glaucoma Service at the University of Other conservative approaches tissue,” said Dr. Gupta. “For example, Toronto, professor at the university’s Dalla Lana include: there might be too much scarring to School of Public Health, and the Dorothy Pitts • Direct pressure patching perform a successful bleb revision. To Chair of Ophthalmology and Vision Science at St. • Bandage contact lens fill these gaps, ophthalmologists have Michaels’ Hospital in Toronto. Relevant financial • Collagen shields experimented with different patch graft disclosures: None. • Autologous blood injections materials other than conjunctiva, such Dr. Radhakrishnan is a glaucoma specialist at the • Compression sutures as corneal tissue, amniotic membranes, Glaucoma Center of San Francisco and research • Cyanoacrylate glue mucosa from inside the cheek, and even director of the Glaucoma Research and Educa- Surgical treatment. “There’s no fascia. It’s a struggle to manage aggres tion Group in San Francisco. Relevant financial hard-and-fast rule as to when you need sive bleb leakages, so we’re always look disclosures: None. to fix a bleb leak surgically,” Dr. Robin ing for better ways.” Dr. Robin is professor of ophthalmology at the said. “But if the leak is not responding University of Michigan in Ann Arbor, Mich., as- to initial management—or is brisk Postoperative Complications sociate professor of ophthalmology at the Wilmer enough to cause corneal decompensa- Although positive outcomes are typi- Eye Institute in Baltimore, and associate pro- tion—or if the patient has experienced cally high for bleb repair, the clinician fessor of international health at Johns Hopkins repeated episodes of bleb-related should expect that a few patients will Bloomberg School of Public Health in Baltimore. infection, definitive treatment always develop complications, Dr. Radhakrish- Relevant financial disclosures: None. requires surgical repair.” nan said. For full disclosures, see this article at aao.org/ Although there is no gold standard The primary concern, she noted, eyenet.

32 • NOVEMBER 2018

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00_Ads_F.indd 34 10/9/18 11:58 AM RETINA OPHTHALMIC PEARLS

Terson Syndrome: Don’t Let It Go Unrecognized

erson syndrome (TS) is the presence of any intraocular 1A 1B Themorrhage, including vitreous, subhyaloid, intraretinal, or subretinal bleeding, in patients with intracranial hemorrhage or traumatic brain injury. The term originally referred only to vitreous hemorrhage in the setting of a subarachnoid hemorrhage (SAH), but the definition has since been expanded. Incidence. In part due to this change in diagnostic criteria, estimates of the incidence of TS vary. In a systematic re- FUNDUS FINDINGS. Fundi of a 48-year-old woman who had an anterior communi- view of SAH patients, McCarron et al. cating cerebral artery aneurysm, diffuse subarachnoid hemorrhage, and left inferior reported a 13% incidence of TS among frontal cerebral hematoma. (1A) Right eye, showing intraretinal hemorrhages nasal patients evaluated prospectively.1 Czor- and superior to the disc. (1B) Left eye, showing a large area of preretinal hem- lich et al. reported an incidence of 19% orrhage overlying the macula, subretinal hemorrhage along the superior arcade, in patients with SAH, 9% in those with and intraretinal hemorrhage nasal to the disc. (Images, taken with a 20 D lens and intracerebral hemorrhages, and 3% in iPhone 7 camera with flash, are inverted vertically and horizontally.) patients with traumatic brain injury.2 The incidence of TS is significantly relatively high incidence of TS in pa- al. reported an average of 5.2 months higher in patients with greater impair- tients with SAH, the syndrome remains between the time that a TS patient first ment in consciousness (as indicated by underdiagnosed. One reason may be complained of ocular symptoms and a low Glasgow Coma Scale) or more se- that the patients who are most likely when an ophthalmology consultation vere subarachnoid hemorrhage (based to have TS are also more likely to be occurred.5 This delay in diagnosis can on either a high Hunt and Hess grade neurologically impaired and, therefore, lead to permanent visual impairment or a high Fisher grade).2 TS is more limited in their ability to verbalize their and impede neurorehabilitation efforts. likely to occur in patients who have had ocular complaints.2 In addition, given prolonged episodes of unconsciousness the neurological acuity and severity of Pathophysiology or elevated intracranial pressure3; and these patients’ conditions, an ocular The pathophysiology of TS is debat- some, but not all, studies have observed examination may not be performed able, and multiple mechanisms have a stronger association with anteriorly until other, more emergent, interven- been proposed. The leading theory located aneurysms.4 Although TS usually tions have been undertaken. suggests that an increase in intracra- develops within hours of the neurolog- Thus, referral of patients with TS nial pressure causes a rapid efflux of ical event, it can occur days or weeks to ophthalmology is often delayed. cerebrospinal fluid or hemorrhage via later.2 In a review of TS patients who later the optic nerve sheath into the orbit. Delayed diagnoses. Despite the underwent vitrectomy, Gnanaraj et This, in turn, compresses the central retinal vein, obstructing venous outflow and subsequently rupturing the smaller BY MEGAN A. ROWLANDS, MD, MPH, BING CHIU, MD, AND JOEL S. SCHUMAN, retinal venules.6

Bing Chiu, MD MD. EDITED BY INGRID U. SCOTT, MD, MPH, AND SHARON FEKRAT, MD. Another theory proposes that an

EYENET MAGAZINE • 35

35-36_Pearls_F.indd 35 10/9/18 10:39 PM acute elevation in intracranial pressure early as 1 week after onset.9 Thus, it is important for TS be recog can increase orbital venous pressure, Imaging. It can be challenging to nized early, not only for its prognostic leading to backflow of blood through identify this pathology by funduscopy significance and impact on patients’ the retinal veins.7 A third theory sug if the view is obscured by vitreous hem neurorehabilitation efforts, but also gests that the intraocular hemorrhages orrhage; in such cases, other modalities, because its complications may lead to may be the result of direct extension such as B-scan ultrasonography, should permanent vision loss if left untreated. of blood from the subarachnoid space be considered to aid in the diagnosis. itself via the optic nerve sheath.8 In a study of patients with SAH, B-scan Key Points Although the exact etiology remains ultrasonography was 100% sensitive • When medically stable, patients unknown, an association with intra and specific for identifying vitreous with intracerebral and subarachnoid cranial pressure has been supported by or preretinal hemorrhages and 44% hemorrhages should receive a prompt Czorlich et al., who found that patients sensitive for identifying intraretinal funduscopic exam to evaluate for TS. with TS were more likely to have had hemorrhages. Head CT scans, in con • B-scan ultrasonography or CT head periods of elevated intracranial pres trast, were 60% sensitive for detecting scan can be used as to screen for TS. sure greater than 25 mm Hg.3 preretinal hemorrhages and 32% sen • Early vitrectomy should be consid sitive for any intraocular hemorrhage.4 ered in patients with severe vision loss Symptoms Thus, these modalities may be viable or bilateral hemorrhages and in young The symptoms reported by TS patients screening options. children at risk for amblyopia. can vary widely, depending on the de • The presence of TS is associated gree and location of the hemorrhage as Management and Outcomes with a worse neurological prognosis well as the individual’s neurological sta Ophthalmologic. The ophthalmic prog and higher risk of mortality. tus. Many patients, especially those who nosis for TS patients is quite good. are most neurologically compromised, Many intraocular hemorrhages resolve 1 McCarron MO et al. J Neurol Neurosurg Psychi- may not be able to perceive or com spontaneously over several months. atry. 2004;75(3):491-493. municate ocular complaints. In such For those that do not, vitrectomy has 2 Czorlich P et al. Neurosurg Rev. 2015;38(1):129- cases, prompt diagnosis depends on the been successful in improving visual 136. primary physician’s first being aware outcomes.5,10 3 Czorlich P et al. J Clin Neurosci. 2016;33:182-186. of the possibility of Terson syndrome In a series of 25 TS eyes undergoing 4 Bauerle J et al. J Neuroimaging. 2016;26(2):247- and then arranging for evaluation by an vitrectomy, 88% of eyes achieved 20/30 252. ophthalmologist. Patients who are able vision or better.5 A study of 44 vitrecto 5 Gnanaraj L et al. Retina. 2000;20(4):374-377. to describe their symptoms typically mized TS eyes found that patients who 6 Manschot WA. Am J Ophthalmol. 1954;38(4): report an acute decrease in vision in 1 underwent early vitrectomy (within 90 501-505. or both eyes in the setting of a recent days of vitreous hemorrhage) achieved 7 Weingeist TA et al. Ophthalmology. 1986;93(11): severe headache or head trauma. better visual outcomes than those who 1435-1442. were operated on after 3 months.11 8 Gress DR et al. J Neuroradiol. 2013;40(4):312- Diagnosis Thus, many experts advocate for early 314. Given the notable incidence of TS in vitrectomy, especially in cases of bi 9 Ko F, Knox DL. Ophthalmology. 2010;117(7): patients with intracranial hemorrhage, lateral vitreous hemorrhages, dense 1423-1429.e1422. SAH, or traumatic brain injury—espe unilateral hemorrhage, or hemorrhages 10 Schultz PN et al. Ophthalmology. 1991;98(12): cially in patients with a loss of con in young children.5,7,9,10 1814-1819. sciousness or low initial Glasgow Coma The other types of retinal pathology 11 Garweg JG, Koerner F. Acta Ophthalmol. 2009; Scale—screening for TS is important associated with TS, including retinal 87(2):222-226. once the patient is medically stable.2,3 detachment and macular holes, can also Fundus examination. Funduscopy have profound effects on vision if not Dr. Rowlands and Dr. Chiu are residents in is the gold standard for detecting and promptly recognized and treated.9 ophthalmology at NYU Langone Health, in New diagnosing TS. Hemorrhages involving Neurological and systemic. In terms York. Dr. Schuman is professor and chairman of multiple intraocular layers may be seen; of neurological and survival outcomes, ophthalmology, and professor of neuroscience they can manifest as a “double ring” the prognosis for these patients is and physiology at NYU Langone Health, and sign, in which blood is present below notably poor. In a systematic review of professor of electrical and computer engineering the internal limiting membrane and outcomes in SAH, patients with TS had at NYU Tandon School of Engineering. Relevant posterior hyaloid.9 Patients may also a risk of mortality almost 5 times higher financial disclosures: None. have a loss of the red reflex. than that of patients with SAH alone For full disclosures, view this article at aao.org/ TS has been linked with develop (50% vs. 11%, respectively, among pa eyenet. ment of macular holes, epiretinal tients studied prospectively).1 Further membranes, retinal folds, proliferative more, TS patients who do survive have MORE ONLINE. See this article vitreoretinopathy, retinal detachment, significantly lower Glasgow scales at 3 at aao.org/eyenet for brain and optic nerve sheath hemorrhage as months than SAH patients without TS.2 imaging of the patient in Fig. 1.

36 • NOVEMBER 2018

35-36_Pearls_F.indd 36 10/10/18 10:36 AM WHAT’S YOUR DIAGNOSIS? MORNING ROUNDS

A Bothersome Bump

ne morning, Mark Mario* elevation, and he demonstrated ptosis 1 woke up with a tender, swollen with an associated superior visual field Oleft eyelid. The 8-year-old had defect to confrontation. a history of sinus infections but other Eyelid eversion was difficult due to wise had been in good health, with pain and mass effect; however, the pal- no history of trauma or recent illness. pebral conjunctiva appeared normal. After several days of worsening swelling Mark had mild hypoglobus but no and pain, Mark’s mother sought help. axial proptosis. He had symmetric At the pediatrician’s office.When sensation in the V1-V3 distribution Mark presented at the pediatrician’s on both sides. His slit-lamp exam was office, he was afebrile and, overall, normal in both eyes, and he had a seemed well—except for his left eyelid, normal fundus exam without evidence which was swollen, droopy, and painful. of retrobulbar mass effect. WHEN WE FIRST SAW MARK. His left The pediatrician found the left eyelid eyelid was swollen, and we noted trace tender to touch and was concerned that Our Differential Diagnosis ptosis and hypoglobus. he might have early preseptal cellulitis, It was apparent to us that this lesion so she prescribed a 2-week course of was not a preseptal or orbital cellulitis, We Send Mark to the ED Augmentin (amoxicillin with clavula- as even an abscess would not cause With a high level of concern, we nate). When Mark’s condition did not such a very firm lesion in the anterior immediately sent our patient to the improve after 1 week, she referred him orbit, and Mark seemed too well overall emergency department for radiologic to us. to have an aggressive orbital infection. studies and a systemic investigation for A rapidly appearing orbital lesion a potential malignancy. What We Saw in a child always gives the ophthalmol- A computed tomography (CT) On examination, Mark’s best-corrected ogist a sense of fear, with rhabdomyo- scan showed an enhancing soft-tissue visual acuity was 20/20 in the right eye sarcoma, metastatic neuroblastoma, mass centered in the left frontal bone and 20/25 in the left. There was no af- osteosarcoma, and leukemia jumping and left orbital roof with erosion into ferent pupillary defect in either eye, and quickly to mind. However, we didn’t see the frontal bone (Figs. 2A and 2B). his color vision was full. His intraocular the proptosis typical of rhabdomyo- Same-day magnetic resonance imaging pressure was 16 mm Hg in the right eye sarcoma, nor the typical ecchymoses of (MRI) demonstrated a mass effect on and 13 mm Hg in the left. orbital neuroblastoma. Lymphangioma the dura and left frontal lobe (Fig. 2C). Most remarkable was the fullness seemed possible given the rapid onset, A thorough systemic lab workup was of his left eyelid (Fig. 1). On palpation, but there was no antecedent upper unremarkable except for an elevated he had a firm, nonmobile, approxi- respiratory infection. An eosinophilic eosinophil count. mately 2-cm mass of the left anterior granuloma seemed possible especially Otolaryngology performed an image- orbit. It was contiguous with the left given the bony pain, although it is less guided biopsy of the lesion through a superior orbital rim and was tender to common in children than the previously left medial supraorbital incision; a soft- light touch. The mass limited his eyelid mentioned orbital neoplasms. tissue mass that appeared granulomatous was noted intraoperatively. Frozen tissue analysis revealed mul- BY SAILAJA BONDALAPATI, MD, SARA GRACE, MD, AND KENNETH COHEN, tiple eosinophilic granules and dendrit-

Sara Grace, MD Grace, Sara MD. EDITED BY STEVEN J. GEDDE, MD. ic cells positive for the CD1a and S100

EYENET MAGAZINE • 37

37-38_AMR_F.indd 37 10/4/18 3:08 PM markers and the BRAF V600E mutation 2A 2B 2C (Fig. 3), confirming that Mark had Langerhans cell histiocytosis (LCH), formerly known as histiocytosis x.

Discussion LCH is a rare medical condition of un- clear etiology that commonly presents 3A 3B 3C with a triad of exophthalmos, diabetes insipidus, and solitary bone lesions (eosinophilic granuloma). Other variants reported in the literature are Hand- Schüller-Christian disease and, if infants present with severely disseminated IMAGING AND SOFT TISSUE ANALYSIS. CT scan shows (2A,B) soft tissue mass. disease, Letterer-Siwe disease. MRI (2C) demonstrates erosion of the orbital roof. There was positive staining for In 1893, Alfred Hand initially mis- (3A) CD1a, (3B) S100, and (3C) BRAF mutation. diagnosed a young child with polyuria, exophthalmos, and skull lesions as modalities are reported in the litera- palsy. Every 4 months, he is checked tuberculosis.1 Later—in collaboration ture, including observation, surgical by the oncology service to assess for with Artur Schüller and Henry Chris- resection, localized high-dose steroids, local recurrence, with skull plain films, tian—Hand coined the term histiocy- radiation, and chemotherapy, which and he undergoes urinalysis to rule out tosis for this medical condition, which are tailored depending on the organ diabetes insipidus. has characteristics of multiple skeletal involvement and medical conditions of lesions, pituitary infiltration, and ex- the patient.4 The multitude of treat- Conclusion ophthalmos. ments reflects the spectrum of disease LCH is a rare neoplasm in children but Incidence. In the United States, the presentations—although the treatment should be considered in patients who incidence is rare (5-6 cases per million of choice for isolated lesions is usually present with orbital lesions, especially if per year); most patients are between 5 excision or local radiation therapy to they are experiencing bone pain. and 15 years old. The incidence in males limit systemic morbidity. Bony erosion on imaging is a hall- is 2 to 3 times greater than in females.2 Prognosis. The prognosis is excel- mark of the disease, although it may Presentation. Patients usually see a lent, with 1 study reporting a 10-year be seen in other orbital lesions, both pediatrician or an orthopedist for bone survival rate of 100%, with low rates malignant and benign. pain before the diagnosis is made. Dis- of recurrence for monostotic disease Surgical biopsy is needed for defin- ease presentation is variable, with bone and 71% recurrence for multiorgan itive diagnosis; once confirmed, a find- being the most commonly affected disease.5 ing of eosinophilic granuloma should organ in up to 80% of cases.2 Skin is prompt complete systemic evaluation the next most frequent site of involve Mark’s Treatment for other sites of disease. ment, followed by pituitary, liver, spleen, Following the orbital biopsy, a full-body lungs, and lymph nodes. positron emission tomography (PET) * Patient name is fictitious. Neuroendocrinopathies related to scan was performed to search for other hormonal deficiencies (such as polyuria sites of involvement. This was negative, 1 Badalian-Very G et al. Annu Rev Pathol. 2013; from diabetes insipidus, growth failure, and our neurosurgery colleagues 8:1-20. and gonadotropin disturbances) are recommended surgical excision and 2 Khatami et al. Pakistan Journal Radiology. also reported due to both anterior and reconstruction to decompress his orbit 2010;20(3):114-120. posterior pituitary involvement.3 and reconstruct the orbital bar and roof. 3 Haupt R et al. Pediatr Blood Cancer. 2013;60(2): Evaluation and diagnosis. A thor- Two weeks following Mark’s initial 175-184. ough physical exam is essential. This presentation to the ophthalmology 4 DiCaprio MR et al. J Am Acad Orthop Surg. should include inspection of skin and clinic, he underwent left eyebrow crani- 2014;22(10):643-652. mucous membranes. Laboratory stud- otomy with resection of the tumor. The 5 Maria Postini A et al. J Pediatr Hematol Oncol. ies should include a complete blood surgery was successful at eliminating 2012;34(5):353-358. count and basic chemistry, urinalysis, the mass effect, and his pain, ptosis, and inflammatory markers, thyroid, and extraocular motility restriction have Dr. Cohen is a comprehensive ophthalmologist, coagulation studies. Radiological stud- resolved (Fig. 4, available online at aao. Dr. Grace is a pediatric ophthalmologist, and Dr. ies delineate bone and tissue involve- org/eyenet). Bondalapati is an ophthalmology resident; all 3 ment, and histopathological analysis is Mark continues to demonstrate a are at The Kittner Eye Center at The University required for confirmative diagnosis. postsurgical hypoesthesia in the left V1 of North Carolina Hospitals in Chapel Hill, N.C.

Treatment. Multiple treatment distribution as well as a left frontalis Financial disclosures: None. MD Sailaja Bondalapati,

38 • NOVEMBER 2018

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40-45_Feature_F.indd 40 10/9/18 7:40 PM When It’s Not Glaucoma

A variety of conditions can produce visual field defects, OCT findings, optic nerve abnormalities, and nerve fiber layer loss that mimic glaucoma

By Annie Stuart, Contributing Writer

OW OFTEN ARE PATIENTS MISDIAGNOSED WITH GLAUCOMA? “It happens more frequently than you might think,” said Steven D. Vold, HMD, at Vold Vision in Fayetteville, Arkansas. Kimberly Cockerham, MD, FACS, who practices in Stockton, California, agreed. “This is not something I see once in a blue moon. It is fairly common to see a patient who is on glaucoma drops and may not need them.” Whether it’s glaucoma, an intracranial problem (such as pituitary adenoma, meningioma, or carotid or ophthalmic artery aneurysm), or an orbital problem (such as thyroid eye disease or an orbital tumor), certain cases can be a complex challenge for even the most experienced observer. But finding your way through the challenge is essential, as a misdiagnosis may lead to unnecessary testing and treatment. Even worse, it may seriously threaten the patient’s health or vision. Four experts offer guidance for sorting out the differences.

The History Patient histories can offer clues to suggest there’s something other than glaucoma at play. Make sure these clues don’t go unnoticed, said Dr. Cockerham. Among her most baffling cases was a recent referral—a patient who was diagnosed as a “glau coma suspect” decades ago and had been on eyedrops ever since. Listen for clues. “He was a good historian,” she said, “but nobody had listened to him.” The patient described being hospitalized after a severe motor vehicle acci dent that resulted in a brain abscess. He recalled losing his visual field immediately after the accident and could provide specific details about which areas of his visual field were lost. He had had a completely stable visual field abnormality and optical coherence tomography (OCT) test results for years. Consider age. Consider the patient’s age when taking the history and think about potential causes other than glaucoma, said Dr. Cockerham. “In a young patient, the cause is more likely hereditary, post-traumatic, inflammatory, or infectious. In middle age, compressive conditions and vascular events can occur. In older patients, giant cell arteritis can cause posterior ischemia that results in

EYENET MAGAZINE • 41

40-45_Feature_F.indd 41 10/9/18 7:40 PM Nonglaucomatous problems that look like glau • Vision loss that came on with a severe headache coma can be asymptomatic. However, 1 common • Double vision clue is sudden vision loss, which is typical of • Temporary graying or blacking out ischemic optic neuropathies, but not of glauco • Orbital ache or pain ma, said Dr. Vold. In contrast, compressive optic Neurologic symptoms. Ask whether patients neuropathy tends to progress more gradually, have experienced any of the following neurologic confounding the diagnosis. symptoms or problems: Watch symptoms, signs. Other symptoms and • Previous brain trauma or brain problem signs can help you begin to piece together the • Numbness, weakness, or tingling puzzle. The key is asking the right questions about • Headaches, especially those that awaken them vision, as well as asking probing questions about in the morning neurologic symptoms, said Prem S. Subramanian, • A loss of libido MD, PhD, at the University of Colorado Health/ Signs. Although optic disc pallor is a hallmark Sue Anschutz-Rodgers Eye Center in Aurora, of a nonglaucomatous condition, said Dr. Subra Colorado. “For example, loss of libido is a cardinal manian, look for other signs like these as well: sign of some pituitary tumors in men, but patients • Proptosis, droopy eyelid, or facial asymmetry often won’t volunteer this information.” • Loss of central visual acuity without a loss of Ocular symptoms. Ask patients whether they peripheral vision have experienced any of the following symptoms: • Central scotoma or visual field that respects the • Sudden or quickly progressing vision loss vertical meridian • Vision that’s different in only 1 eye • Optic nerve pallor • Lack of color vision in 1 eye (red desaturation) • Optic nerves that are symmetric in appearance • Vision loss with eye movement to each other, but 1 visual field is very different

GLAUCOMA PLUS A Case of “Ticks and Fleas on the Same Dog”

A 70-year-old woman was her primary care physi- 1 referred to Dr. Levi’s clinic with cian apparently contin- chronic visual loss. Her medi- ued to refill the drops. cal history included hyperten- Over the next 2-3 years, sion, obstructive sleep apnea, she gradually lost vision well-controlled diabetes, and in the left eye. In 2014 breast cancer that was treated she began to notice in 1999 and was in remission. visual changes in the Her ocular history included right eye and returned laser and cryotherapy in each to the retina specialist who was –11.61, and no responses eye in the 1990s for retinal had seen her in the 1990s. in the left (Fig. 1). There was a holes due to lattice degen- In July 2014, her IOP was left relative afferent pupillary eration. She had cataract 24 mm Hg in each eye. She defect. There was no clinical extraction in her right eye was placed on brinzolamide/ evidence of Horner syndrome. in 2005 and in her left eye in brimonidine drops and was Extraocular movements were 2011. referred for neuro-ophthalmo- full. Trigeminal nerve function, The patient began to notice logical evaluation. including corneal sensation, a cloud in the vision of her left On initial neuro-ophthalmo- was symmetric and normal. eye in 2010. This progressed logical evaluation in August IOP was 14 mm Hg in each over several months. She was 2014, acuity was 20/30 in eye. There was 0.8 cupping told by a glaucoma specialist the right eye and bare light of the right disc with pallor of that she had normal-tension perception in the left. Har- the remaining neuroretinal rim. glaucoma that was worse in dy Rand and Rittler (HRR) The left disc was completely the left eye than the right, color plates was 3/6 in the cupped. Because of the pallor and he started her on latano- right eye. Visual field test- of the neuroretinal rim in both prost. ing showed a dense superior eyes, an MRI scan was done; She was then lost to oph- arcuate defect in the right it showed a large sellar mass thalmological follow-up but eye and the mean deviation with suprasellar extension and

42 • NOVEMBER 2018

40-45_Feature_F.indd 42 10/11/18 6:47 PM • Unilateral or very asymmetric damage movement problems,” she said. • Afferent pupillary defect (APD) “A patient with orbital problems may not be • Color desaturation able to completely move his or her eyes in all • Conjunctival injection or chemosis directions,” added Dr. Cockerham. “Delegating the pupil and motility testing to your technician Ophthalmic Exam: Keep an Open Mind can be a problem.” Above all, be suspicious, said Dr. Cockerham. Testing intraocular pressure (IOP) is obviously “Once a person gets a label of glaucoma, it often important, said Dr. Vold, and if there are concerns doesn’t get challenged, even when the patient ends about optic nerve head disease, additional visual up with a different doctor. The most suspicious fields may be needed. “A thorough vascular eval- diagnosis is unilateral normal-pressure glaucoma uation by an internist may be necessary to rule with an afferent pupillary defect. This is never the out uncontrolled diabetes or hypertension, and a correct diagnosis.” fluorescein angiogram [may be needed] to spot If a patient says they have glaucoma, make sure a previous retinal injury from an old vein occlu- you agree, said Dr. Subramanian. “If something sion,” he said. ‘smells funny’ or doesn’t quite fit, don’t be afraid Look—with the light on. “The most confusing to question another ophthalmologist’s diagnosis.” patient of all is one with a family history of glau- A comprehensive ophthalmic exam. To con- coma, no history of brain issues, and no symp- firm or rule out a diagnosis of glaucoma, Leah toms whatsoever,” said Dr. Cockerham. If you Levi, MD, at Scripps Health in San Diego, con- suspect an abnormality, she said, turn on the light ducts a comprehensive ophthalmic exam. to see the patient’s eyes and face more clearly. “This includes checking acuity, color vision, “A lot of eye specialists work in dim rooms, pupils, and visual fields, and looking for eye going from slit lamp to slit lamp,” she said. “We

2 noma. The re- 3 sulting decom- pression of the anterior visual pathways led to improvement of the color vision to 4.5/6 in the right eye. Visual left cavernous sinus invasion field testing in the right eye sion (Fig. 3). The left eye did (Fig. 2). improved; the mean deviation not improve. The patient’s last In September 2014 the in the right eye improved to examination in June 2018 was patient underwent subtotal –4.43. In addition, a superior stable as was her MRI scan. resection of the mass, which vertical step was revealed re- She has continued to use the proved to be a pituitary ade- flecting the chiasmal compres- drops in her right eye.

TAKE-HOME LESSONS • More visual field loss than the anterior visual pathways • Patients with glaucoma expected. This patient had can produce cupping that is need to be followed by an more visual field loss than similar to glaucoma, but in ophthalmologist. This patient expected for the degree of these patients the remaining with glaucoma was lost to cupping as well as faster neuroretinal rim will show ophthalmological follow-up progression of visual loss than pallor. The pallor in this case for about 3 years while she expected for glaucoma, sug- indicated that the patient had progressively lost vision, but gesting a nonglaucomatous a chronic nonglaucomatous her primary care physician condition. optic neuropathy in addition continued to prescribe her • Pay attention to pallor. to glaucoma. An MRI scan was glaucoma drops. Uncommonly, compression of therefore indicated.

EYENET MAGAZINE • 43

40-45_Feature_F.indd 43 10/10/18 10:37 AM need to look at these patients in a fully lighted fields of both eyes together, said Dr. Subramanian. room to see if there is asymmetry of the face or “If you don’t look at them side by side, you may globe position or evidence of bilateral involve- miss a homonymous visual field defect or even ment, like thyroid eye disease.” Other signs to a bitemporal hemianopia. Your brain may fail to watch for? “In a patient with a meningioma, for recognize the pattern if you don’t have both visual example, the temporal aspect of the face overlying fields sitting in front of you at the same time.” the meningioma may get bigger,” she said, “and a Fundus exam. “Over time, we’ve evolved to carotid-cavernous sinus fistula will cause a charac- the point where people equate optic disc cupping teristic dilation of the vessels on the surface of the to glaucoma,” said Dr. Cockerham. “But it is just eye, and eyelid swelling and proptosis.” 1 of many optic nerve processes that can cause Palpate and measure. If you suspect an orbital cupping.” If the neuroretinal rim has pallor, it’s problem, checking resistance to retropulsion can definitely a red flag that you are not simply deal- be helpful in detecting a mass or enlarged mus- ing with glaucoma, said Dr. Levi. “With glaucoma, cles behind the eye, said Dr. Cockerham. This is you may have cupping, but the actual surrounding particularly helpful in Asian patients who do not rim is normal in color and looks healthy.” Spotting become proptotic like other ethnicities. Dr. Levi optic disc pallor is key to preventing a misdiagno- also recommends measuring whether 1 eye is sis, agreed Dr. Subramanian. more proptotic than the other by using exophthal- “In addition, with ischemic optic neuropathy, mometry, if available. Taking a photo from above crowded or hypoplastic nerves are more com- can also be helpful, said Dr. Cockerham. mon,” said Dr. Vold. Visual fields.Any ischemic optic neuropathy OCT. Because optic nerve fiber changes are can produce visual field defects similar to those not specific to glaucoma, OCT won’t be defini- seen in glaucoma, said Dr. Subramanian. Although tive in differentiating it from nonglaucomatous certain patterns may raise glaucoma red flags, problems, said Dr. Levi, but an OCT scan may be added Dr. Cockerham, visual field defects in a helpful as a baseline for future follow-up. patient with a tumor and another with true glau- “Because OCT is structural, however, it can coma can be indistinguishable. “There’s nothing provide a very clean delineation along a particu- that’s pathognomonic.” lar anatomic boundary,” said Dr. Subramanian. In addition, she said, digital perimetry is less “That helps you to say, ‘I’m seeing damage here clear than manual visual fields are in respecting in a more diffuse pattern rather than the typical the vertical meridian and in isolating a cecocentral superior and inferior loss, and that makes me con- scotoma. “There’s noise in the signal of automated cerned this is something other than glaucoma.’”1 visual fields,” said Dr. Cockerham. “The Hum- With glaucoma, said Dr. Vold, you’ll typically phrey visual field SITA testing, for example, fills in see inferior rim retinal nerve fiber layer loss before the information in between stimulus points, and you see it anywhere else. “This area is usually this can mute neurologic visual field patterns that affected first, then superior next, nasal third, and are more easily seen when a skilled technician has temporal last,” he said. carefully plotted the Goldmann visual field.” Even though certain patterns may be generally Still, automated visual fields can offer clues. typical for glaucoma, they are not diagnostic, said For example, central loss is indicative of retina or Dr. Levi. For instance, if there is a tumor com- optic nerve maladies, as opposed to glaucoma, pressing the optic nerve from below, you will also said Dr. Vold. And in normal-tension glaucoma, get inferior RNFL thinning—so this finding is not patients usually don’t have visual acuity loss until specific to glaucoma and can’t be interpreted in later in the disease. isolation of the rest of the clinical picture. “Con- Whenever possible, it helps to look at visual versely, certain patterns are very atypical for glaucoma and should raise alarm bells.” These patterns include MEETINGS ON DEMAND AAO Meetings on Demand segmental RNFL thinning allows you to view the Glaucoma Subspecialty Day program due to a loss of signal caused alone or as part of a complete package of all 8 Subspecialty by media opacities, or sec- Day meetings, the AAOE program, and highlights from AAO toral peripapillary decrease 2018. The latter includes a total of nearly 200 hours and 1,000 in RNFL due to branch presentations, inclusive of both glaucoma and neuro-ophthal- retinal vein occlusion. mology symposia and original pa- In all patients but espe- per presentations. To learn more, cially those under age 40, Dr. visit aao.org/ondemand. Subramanian also checks the source images for optic disc

44 • NOVEMBER 2018

40-45_Feature_F.indd 44 10/9/18 7:40 PM drusen, which can mimic glaucomatous defects. some glaucoma specialists—are patients who are Specialized imaging. A variety of red flags losing visual fields despite what seems to be good might warrant specialized imaging. Asymmetry control of their IOP. “Much of the time, patients may be one, said Dr. Cockerham, because glau- referred to me do have glaucoma, however, and I coma does not tend to be an asymmetric process. can ascertain that by careful review of their clini- The following red flags indicate a nonglaucoma- cal findings without getting a scan,” she said. tous problem is to blame, rather than glaucoma: If needed, imaging may involve magnetic res- • The patient has unilateral normal-pressure onance imaging (MRI) or a magnetic resonance glaucoma with an APD, especially if the APD is angiogram or computed tomography (CT) angi- more than a subtle one. ography. “If you have a high degree of suspicion • The patient has chronic open-angle glaucoma and don’t feel comfortable reviewing these scans,” with an APD, especially if it’s more than subtle. said Dr. Cockerham, “consider referring them to a • The optic nerve is more pale than cupped. neuro-ophthalmologist.” • Visual field loss is progressing more rapidly Dr. Cockerham cited the case of a patient than expected for glaucoma. where this didn’t happen. The patient had been • Visual field loss is progressing despite normal seen by 5 previous eye care providers, but over the IOP or IOP that’s under control. course of 6 months she lost vision in the involved • Severity of cupping doesn’t match the visual eye to no light perception. In this patient, the field defect. noncontrast CT scan of the brain was done in • The OCT of the optic nerve and macula does an emergency department and had been read as not correlate with the visual fields. normal, but an apical mass was visible on 1 digital • The visual fields or macular ganglion cell OCT slice. An MRI with gadolinium revealed a large have a vertical feel to them (homonymous pattern/ orbital apex mass that was found to be steroid- bitemporal/junctional). responsive, but there was no return of vision. • There are signs or symptoms of other nerve 1 Gupta PK et al. Open Neurol J. 2011;5:1-7. involvement, such as double vision or a droopy eyelid. Signs and symptoms in synch? Another way MEET THE EXPERTS to suss out nonglaucoma entities: “When making Kimberly Cockerham, MD, FACS your assessment, don’t rely too heavily on any Orbit-plastics-neuro-ophthal- single particular piece of data and ignore others,” mology specialist in private said Dr. Subramanian. Symptoms and signs need practice in Lodi, Modesto, and to align, emphasized Dr. Vold. Stockton, Calif.; Adjunct Associate For example, it’s important to take note when Clinical Professor at Stanford University, Palo a patient has an elevated IOP and some degree Alto, Calif. Relevant financial disclosures: None. of vision loss—whether central visual acuity or Leah Levi, MD Clinical Professor a visual field abnormality—but the appearance Emerita at the University of Cal- of the optic disc doesn’t quite match, said Dr. ifornia, San Diego; and director Subramanian. of neuro-ophthalmology at the Or, in a patient with a potential pituitary tumor Scripps Clinic Division of Oph- or other compressive lesion of the optic nerve or thalmology in San Diego. Relevant retrochiasmal visual pathway, comparing right financial disclosures: None. and left eyes may reveal clues. “Analyzing the mac- Prem S. Subramanian, MD, PhD ular ganglion cell complex, you may see a pattern Professor of ophthalmology, of ganglion cell loss that matches the visual field neurology, and neurosurgery defect and can really demonstrate a homonymous and division head of neuro-oph- or bitemporal defect,” he said. Many glaucoma thalmology at the University of specialists do not look at this testing and may Colorado Health/Sue Anschutz-Rodgers miss that the problem is retrochiasmal, added Dr. Eye Center, Aurora, Colo. Relevant financial Cockerham. disclosures: None. Refer to a Neuro-Ophthalmologist Steven D. Vold, MD Glaucoma If the clinical picture is not consistent with the and cataract specialist at Vold degree of “glaucoma” you are seeing, it may be Vision in Fayetteville, Ark. Rele- time to refer to a neuro-ophthalmologist, said Dr. vant financial disclosures: None. Levi. What results in most referrals—and is most For full disclosures, find this article troubling for many general ophthalmologists and at aao.org/eyenet.

EYENET MAGAZINE • 45

40-45_Feature_F.indd 45 10/9/18 7:40 PM STATE ADVOCACY

I believe in high surgical standards. I support the Surgical Scope Fund. I protect sight.

When surgery-by-surgeons is threatened nationwide, the Academy’s Surgical Scope Fund provides states with the means to counter organized optometry’s dangerous agenda, unscrupulous tactics and limitless resources.

To learn more and make your Surgical Scope Fund contribution, visit one of our AAO 2018 booths in Chicago:

• Lakeside Lobby (Oct. 26 – 27)

• Grand Concourse (Oct. 26 – 29)

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Or contribute online at aao.org/ssf.

Your Surgical Scope Fund contribution is confidential.

ALAN L. WAGNER, MD, FACS, FICS HAMPTON ROADS, VA

00_Ads_F.indd 46 10/4/18 3:49 PM CODING & REIMBURSEMENT SAVVY CODER

How the Oct. 1 Changes to ICD-10 Are Impacting Ophthalmologists

n Oct. 1, CMS implemented hundreds of changes to the Don’t Miss Out on These ICD-10 Resources OICD-10 codes. The updates that are most likely to impact ophthal- Make sure you’re up to speed on this year’s ICD-10 updates, which went into mology include 26 deleted diagnoses, effect on Oct. 1. 6 description changes for the epiphora Get the Academy’s free ICD-10 materials. Visit aao.org/icd10 and make family, and 120 new ICD-10 codes. This sure you have the latest versions of the subspecialty-specific guides to ICD-10 article highlights the key changes. codes, as well as the latest versions of the decision trees. The latter will help As payers update their CPT-to- you identify the correct ICD-10 code for specific conditions, such as blephari- ICD-10 linkage, be sure you also tis or lagophthalmos. update your practice management Get the updated local coverage determinations (LCDs). Visit aao.org/lcds software and electronic health record to find the LCDs that apply to you. (EHR) systems. Buy ICD-10-CM for Ophthalmology: The Complete Reference. This reference lists all of ophthalmology’s new and updated codes. It is available as a Deleted Codes book or as an online subscription (aao.org/codingproducts). Deleted eyelid codes. The codes listed Get coding news updates. Go to aao.org/practice-management/news for below were deleted and replaced with coding updates, regulatory news, coding top-10s, and “Ask the Expert” re- codes that have greater specificity. sponses to common—and not-so-common—coding queries. • C43.11 Malignant melanoma of right Got questions? When ophthalmology practices have a coding conundrum, eyelid they can request help via email ([email protected] or [email protected]), at their • C43.12 Malignant melanoma of left state’s Codequest event (aao.org/codequest), and on the AAOE’s eTalk list- eyelid serv (aao.org/practice-management/listserv). • The C44- family of codes representing Other and unspecified malignant neoplasm of skin Deleted codes for postprocedure Changes to the Epiphora Codes • The D03- family of codes represent- infections have more detailed replace- Description changes impact the epiph- ing Melanoma in situ ments. The T81.4- family of codes ora diagnosis family. In the listings • The D04- family of codes represent- representing Infection following a pro- below, underlining and strikethroughs ing Carcinoma in situ of skin cedure have been deleted and replaced are used to indicate new and deleted Replacement eyelid codes. The with codes that indicate whether it is an text, respectively. replacement codes don’t just indicate initial encounter, a subsequent encoun- • H04.201 Unspecified epiphora right whether the diagnosis applies to the ter, or a sequela. side lacrimal gland left or right eyelid; they also indicate A catch-all code is replaced. The • H04.202 Unspecified epiphora left whether it is the upper or lower eyelid. Oct. 1 changes also delete H57.8 Other side lacrimal gland Example: C43.11 is replaced with specified disorders of eye and adnexa, • H04.203 Unspecified epiphora, bilat- C43.111 and C43.112, which represent but they add H57.89, giving it the same eral lacrimal gland the upper and lower eyelid, respectively. generic catch-all descriptor. • H04.221 Epiphora due to insufficient drainage, right side lacrimal gland • H04.222 Epiphora due to insufficient BY SUE VICCHRILLI, COT, OCS, OCSR, ACADEMY DIRECTOR OF CODING drainage, left side lacrimal gland AND REIMBURSEMENT. • H04.223 Epiphora due to insufficient

EYENET MAGAZINE • 47

47-48_Coder_F.indd 47 10/11/18 6:48 PM drainage, bilateral lacrimal glands • H02.886 MGD of left eye, unspecified Warning. Do not use these 2 codes: eyelid • H04.209 Unspecified epiphora, un- • H02.889 MGD of unspecified eye, specified side lacrimal gland unspecified eyelid • H04.229 Epiphora due to insufficient Although H02.883, H02.886, and drainage, unspecified side lacrimal gland H02.889 are legitimate ICD-10 codes, While these 2 side-unspecific codes their lack of specificity will cause payers are legitimate ICD-10 codes, they are to deny your claim. not payable, and your claim will be denied if you report them. 3 Tips for ICD-10 Coding When linking CPT codes to ICD-10 The ABCs of Eyelid Laterality codes, remember laterality. When the A welcome change but a new adjust- CPT code requires modifiers –RT or ment occurs with the lagophthalmos –LT (to indicate the right and left eye, (H02-) family of codes. Previously, respectively) and the ICD-10 code has you needed 1 code for the upper eye laterality, the CPT code that has –RT lid and a second for the lower eyelid to should be linked to the ICD-10 code indicate that lagophthalmos had been for the right eye and the CPT code with diagnosed in both. –LT linked to the ICD-10 code for the Now, when used in the sixth posi- left eye. If you instead report a bilateral tion of those codes, A, B, and C repre- ICD-10 code, the claim will probably sent both the upper and lower eyelids be denied. of the right eye, the left eye, and both Example: Coding for complex eyes, respectively. cataract surgery in the right eye. CPT Example: code 66982–RT is linked with H25.11 • H02.21A Cicatricial lagophthalmos Age-related nuclear cataract, right eye, right eye, upper and lower eyelids which indicates the type of cataract. It • H02.21B Cicatricial lagophthalmos is also linked with H27.111 Subluxation left eye, upper and lower eyelids of lens, right eye when the operative • H02.21C Cicatricial lagophthalmos, report indicates the intraocular lens bilateral, upper and lower eyelids was supported by using permanent in- OPHTHALMOLOGY Caveat. Some families of codes— traocular sutures or a capsular support JOB CENTER such as the meibomian gland dysfunc ring was employed. tion family (see below) and the bleph- Did you inadvertently bill for aritis family (H01-)—don’t have a cataract surgery twice in the same Find the bilateral code. eye? If over the past 12 months you Example: erroneously reported cataract surgery Right Fit • H01.01A Ulcerative blepharitis right twice in the same eye, you can correct eye, upper and lower eyelids that error over the phone—and avoid a Fast • H01.01B Ulcerative blepharitis left data-driven recovery audit—by calling eye, upper and lower eyelids the Medicare Administrative Con- Choose the only tractor for your state. You only get 1 job site that matters. Meibomian Gland Dysfunction opportunity to make this correction, so The new ICD-10 codes include the make sure you remember to correct the following codes for meibomian gland ICD-10 code, too. Start your search today: dysfunction (MGD): Payers typically don’t pay for se- aao.org/jobcenter • H02.881 MGD right upper eyelid quelae. Diagnosis codes for injury or • H02.882 MGD right lower eyelid trauma use an A, D, or S as the seventh • H02.884 MGD left upper eyelid character to indicate initial encounter, • H02.885 MGD left lower eyelid subsequent encounter, or sequela, • H02.88A MGD right eye, upper and respectively (e.g., S05.01XS Injury lower eyelids of conjunctiva and corneal abrasion • H02.88B MGD left eye, upper and without foreign body, right eye, sequela). lower eyelids Other than workers’ compensation, Warning. Do not use these 3 codes: most federal and commercial payers • H02.883 MGD of right eye, unspeci- consider sequela a noncovered diagno- fied eyelid sis and would deny the claim.

48 • NOVEMBER 2018

47-48_Coder_F.indd 48 10/11/18 6:48 PM BUSINESS OPERATIONS & FINANCE PRACTICE PERFECT

Case Study: How an Ophthalmic Practice Tackled Its Profitability Problem

lthough ophthalmologists collect ample patient data before Academy/AAOE Benchmarking Resources Amaking a diagnosis, they often lack the resources to collect enough The American Academy of Ophthalmic Executives (AAOE), the practice man- business data before making key agement arm of the Academy, sponsors 2 programs that allow you to track decisions about their practice. In the your business data against benchmarks from other ophthalmology practices. case study below, a physician owner of The Academetrics Benchmarking Tool allows you to compare your finan- a practice, Dr. Conch,* thought, “Our cial and patient flow data with benchmarks calculated from participating employee costs are too high—we need ophthalmology practices. to cut those somehow.” If she had acted The Academetrics Ophthalmic Salary Survey, developed in conjunction on that 1 perception, she might have with the Outpatient Ophthalmic Surgical Society, helps you see how your staff terminated staff, cut employee bene- salary rates compare with other practices. fits, or reduced pay rates—responses Both resources are free to those practices that participate by adding their that could have damaged her practice. information to the confidential database any time throughout the year. Instead, Dr. Conch and her practice For more information, visit aao.org/benchmarking where you will find manager, Mr. Duenas,* gathered facts details about and instructions for using both the Academetrics Benchmarking before acting. They used free resources Tool and Ophthalmic Salary Survey. from the Academy—the Academetrics Benchmarking Tool and Academetrics Ophthalmic Salary Survey—which provided critical context that helped High staff costs?Concerned that the quently, further reduction in revenues. pinpoint the underlying issues. practice is spending too much revenue As a new manager, Mr. Duenas realizes on staff members, Dr. Conch approaches that such a path is not conducive to Identifying the Problem her new practice manager, Mr. Duenas, keeping his job either! Dr. Conch is the managing partner of about reducing that cost. a practice with 5 full-time comprehen- The practice administrator’s dilem- Accessing Helpful Resources sive ophthalmologists and 25 full-time ma. Mr. Duenas is alarmed because he To better understand the practice’s support staff. knows that the most obvious solutions financial situation, Mr. Duenas goes to Revenue summary. Revenues for the could harm the practice. Specifically, aao.org/benchmarking and registers for practice in 2017 (not including optical) cutting staff wages or benefits might both the Academetrics Benchmarking were $3.5 million, $1 million of which cause staff turnover, which tends to in- Tool and Ophthalmic Salary Survey was paid to the employees as wages. crease costs as new employees struggle (see “Academy/AAOE Benchmarking Over the course of the year, the doctors to learn the operations of the practice. Resources,” above). First, he enters into had 17,500 patient encounters in their Alternatively, laying off staff could the 2 surveys his practice’s 2017 data, office, including all of the Evaluation & hamper the practice’s ability to provide including numbers from the profit- Management and Eye codes that were good service to patients, likely resulting and-loss statement, payroll, and billing billed and all postoperative exams. in attrition of patients, and, conse- reports. After entering all relevant information, he compares his data to the aggregated numbers in the databas- BY DEREK PREECE, MBA, AND DIXON DAVIS, MHSA, PRINCIPALS AND es. Here, he begins to identify the real CONSULTANTS AT BSM CONSULTING. challenges for the practice.

EYENET MAGAZINE • 49

49-50_PP_F.indd 49 10/9/18 7:46 PM Getting Specific Feedback practice had 17,500 encounters (an av- Coming in the next Staffing ratios.Mr. Duenas first looks erage of 3,500 per doctor) for the year, ® at the staff payroll ratio (employee which is also below the 25th percentile. payroll divided by total collected Per patient collections are subpar. revenue) and finds that it is 28.5%, In addition, the doctors’ average col- which is high—in the 75th percen- lections per encounter is $200, which is tile for ophthalmology practices that less than that of a typical comprehen- Feature submitted approved data to the survey. sive ophthalmologist. So, his doctors A Look Ahead Experts The practice’s total collections per full- are not seeing as many patients per year talk about developments time employee is only $140,000, which as their peers, and they are collecting is very low—barely above the 25th less cash per patient. The result: a signif- from 2018 that may shape percentile on the Academetrics Bench- icant shortfall in revenues. the field over the next 5 marking Tool. “So maybe Dr. Conch Taking action. His next tasks are years. See what they say is correct that we spend too much on clear. First, he needs to help the doctors about their subspecialties: staff,” he thinks. increase the number of patients they glaucoma, low vision, ocular Investigating further, he sees that see each year. Then, to see if he can help oncology, and refractive his practice’s ratio of staff members them raise per-patient collections, he surgery. to doctors is 5.0 (25 staff members plans to review CPT codes that have divided by 5 doctors). Interestingly, he been used to bill patient encounters to Clinical Update finds that figure to be low—less than see if “downcoding” is responsible for Cornea Pain without stain: the median figure from the survey and below average per-patient collections. How to diagnose and treat just above the 25th percentile. “So, if we Improving the practice’s revenues neuropathic pain. don’t have too many staff members but will decrease the staff payroll ratio and Retina Some macular holes our payroll ratio is high, are we paying raise collections per full-time employee, are more challenging than employees too much?” he wonders. plus help the doctors increase earnings. most. Experts discuss Salaries. Turning to the Academet- Looking further. Mr. Duenas realizes specific approaches. rics Ophthalmic Salary Survey, Mr. that he can examine other ratios to learn Duenas looks at the pay rates for each more about the practice’s situation. For Pearls of his staff members and finds that— example, he can compare the number although some are paid a little above of patient encounters per front desk and Rhegmatogenous Retinal the median for their positions and oth- back office staff to indicate whether he Detachment Recognize risk factors and clinical ers are paid a little below—overall, his is under- or overstaffed in those areas. features, and review exam employees are compensated within the In addition, he can check accounts essentials and management normal range for their specific jobs. receivable ratios to determine whether strategies. Thinking through what he has the practice is collecting money effec- learned so far, Mr. Duenas realizes tively, and he can check optical shop Practice Perfect that the 2 ratios he is most concerned ratios to evaluate how well that opera- about—the staff payroll ratio (28.5%) tion is doing. Boost Patient Satisfaction and collections per employee ratio Taking a strategic approach ($140,000)—share practice collections Compare Your Practice to improving your patients’ as a common factor. Going back This case is just 1 example of how the experience will benefit your through the benchmarking survey Academetrics Benchmarking Tool and practice in many ways. reports, he checks how the practice’s Ophthalmic Salary Survey can tell a collections stack up against those of story about the inner workings of a other practices. practice. These benchmarks provide For Your Convenience valuable information for making ap- These stories also will be Problems With Productivity propriate, disciplined decisions to both available online at Upon comparing his practice’s collec- improve your practice and inoculate aao.org/eyenet. tions data against benchmarking data, it against poor choices made in the he discovers a few startling facts. absence of good, comparative data. FOR ADVERTISING INFORMATION Revenue seems low. His 5 comprehensive ophthalmologists produce $3.5 *Doctor and administrator names are fictitious. Mark Mrvica or Kelly Miller million per year in collections, an av- M. J. Mrvica Associates Inc. erage of $700,000 per full-time doctor, Mr. Preece and Mr. Davis are principals and 856-768-9360 which is well below the 25th percentile consultants with BSM Consulting, headquartered [email protected] for other practices. Wondering why in Incline Village, Nev. Financial disclosures: his numbers are low, he notes that the None.

50 • NOVEMBER 2018

49-50_PP_F.indd 50 10/9/18 7:46 PM OPD-SCAN III Integrated Wavefront Aberrometer/Corneal Analyzer

The OPD-Scan III provides integrated wavefront aberrometry and corneal assessment with topography, pupillometry, autorefraction and keratometry. In just 10 seconds, per eye, over 20 diagnostic metrics are integrated.

Corneal ASSESSMENT “Ocular surface disease and dry eye problems are critically important to identify prior to cataract surgery. I use the placido disc map of the OPD III to illustrate tear fi lm instability: rings that are not perfectly round, are wobbly, irregular, or broken, signify an unhealthy surface. I show the warped black and white ring images to my patients to let them know they have a pre-existing disease called dry eye syndrome which is chronic and progressive in nature. I emphasize that dry eye disease needs ongoing treatment whereas cataracts are removed by surgery and will not recur. I distinctly want patients to know that cataract surgery did not give them their dry eye condition. Patients, for the fi rst time, are able to better Cynthia Matossian, MD understand their ocular surface condition by seeing the irregularities of their tear fi lm.” Matossian Eye Associates Hopewell, New Jersey

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00_Ads_F.indd 52 10/9/18 12:20 PM Academy Notebook NEWS ● TIPS ● RESOURCES

WHAT’S HAPPENING U.S. practicing ophthalmologist members regard the Academy as an or- Dr. Tuck Named Eye Health ganization that offers year-round value, Hero Award Recipient is forward-thinking, and understands During the Global Welcome lunch at their professional needs. AAO 2018, the American Institute for • 86% believe the Academy helps them the Prevention of Blindness presented to be better ophthalmologists. Kenneth D. Tuck, MD, former Academy • 90% see the Academy as the leading President, with the Eye Health Hero source for reliable ophthalmic informa- Award. Dr. Tuck was recognized for EYE HEALTH HERO. Dr. Tuck with his tion and education. spearheading an international mentor- rotary guest from Haiti, Regine Edouard, • 89% consider the Academy as the ing program when he was President. MD, and his wife, Sarah Tuck. leading legislative and regulatory advo- Since its start in 2000, the Rotary cate for ophthalmologists. Club Host Project has provided 122 other cultures and ways of practicing Members say that the most critical young ophthalmologists from 57 de- ophthalmology. I am so excited that we issues affecting ophthalmology are veloping countries the opportunity to are building a global network of young reimbursement, optometry scope of experience the innovative educational leaders in ophthalmology who reach practice, and legislation/regulation. programs and technology available at out and strengthen eye care in their These concerns remain unchanged the Academy’s annual meeting. Devel- own countries and, indeed, the world.” from the 2015 survey findings. Mem- oped through the Academy’s Host an To learn more about the program bers reinforce the need for the Academy Ophthalmologist Program and with and volunteering, visit aao.org/inter to continue to champion fair physician the help of Rotary Clubs, Dr. Tuck has national/outreach/programs. reimbursement, stop the expansion of urged Rotarian ophthalmologists in the optometrists’ scope of practice, and United States to host a young interna- 2018 Member Engagement advocate for regulatory relief. Most tional ophthalmologist at their practice Survey Results rate the effectiveness of the Academy’s the week before the annual meeting. The Academy relies on data to develop advocacy efforts as excellent or very This allows guests time to acclimatize and enhance programs and services good. Eighty-nine percent feel the and see U.S. practices before attending that are responsive and relevant to mem- Academy provides a collective voice for the Academy’s annual meeting, where ber needs. Direct feedback from mem- the profession, and 63% feel the Acad- they can access all courses, seminars, bers through surveys and focus groups emy is either very good or excellent at exhibits, and social events. allows us to both measure awareness positively affecting federal regulatory After stepping down as head of and value of Academy member benefits and/or legislative issues important to the Rotary Club last year, Dr. Tuck and services and to determine the key ophthalmology. reflected, “We’re building relationships drivers of member engagement and International practicing ophthal- and exposing these young doctors to value perceptions. mologist members see the Academy as Recently, the Academy enlisted Loyal- a leader in ophthalmic education. The ty Research Center to conduct the 2018 annual meeting, Ophthalmology journal, Member Engagement Survey. Results EyeNet Magazine, and the Ophthalmic are based on 1,165 responses collected News and Education (ONE) Network from a representative sample of the all receive best-in-class ratings. Most Academy’s membership. rank the annual meeting as the best

EYENET MAGAZINE • 53

53-55_Note_F.indd 53 10/9/18 7:51 PM ophthalmology conference in the world Anterior Segment of someone special. —with 96% rating the annual meeting • Retina/Vitreous/Uveitis/Oncology/ To donate, visit aao.org/foundation/ as excellent, very good, or good. Pathology giving-options. • Glaucoma/Neuro-Ophthalmology TAKE NOTICE • Pediatrics/Strabismus/Oculoplastics/ ACADEMY RESOURCES Orbit MIPS: Applying for the EHR To enter, use the gray “Enroll in Res- Evidence-Based Solutions Hardship Exception? idents and Fellows Contest” submission for Your Practice In the Merit-Based Incentive Payment button at the top of any EyeWiki article Starting in 2019, each issue of Focal System (MIPS), the electronic health related to your entry. The submission Points will tackle important new record (EHR)–based performance cate- deadline is Dec. 1, 2018. Mul- research and

VOLUME XXXVII • NUMBER 1 • JAN 2019 gory is called promoting interoperabil- tiple entries are allowed. See (MODULE 1 OF 3) provide practical ity (PI). It is 1 of 4 MIPS performance www.eyewiki.org/Residents_ tips on how to ef-

FOCAL ® categories and contributes up to 25 and_Fellows for details. POINTS ficiently integrate points to your MIPS final score (0-100 For international doctors Jody Abrams, MD new treatments points). Typically, if you were to report interested in contributing to and methods no PI measures, your PI score would EyeWiki, visit www.eyewiki. into your practice. be zero and your maximum MIPS final org/International_Ophthal Topics for 2019 score would be 75 points. mologists for an international include “Pain in

The significant hardship exception. contest running through May Pain in the “Normal” Eye the ‘Normal Eye,’” You can apply to be exempted from the 2019. “Gene and Stem PI performance category if you are fac- Cell Therapy ing a significant hardship, such as insuf- Submit Your Research BC-2555 2019 Focal Points Redesign_cover_Jan19.indd 1 9/5/18 12:36 PM for Retinal Disor- ficient internet connectivity or extreme to Ophthalmology Glaucoma ders,” “Corneal Endothelial Surgery,” and uncontrollable circumstances. This summer, the Academy and the and “Optical Coherence Tomography If the Centers for Medicare & Medi- American Glaucoma Society collab- in Glaucoma Progression and Diag- caid Services (CMS) accepts your ap orated in launching Ophthalmology nosis.” plication for a hardship exception, PI’s Glaucoma. Subscribe to Focal Points Digital to contribution to your final score will This new journal provides an oppor- get a new issue every month, plus access be reweighted to zero, and the quality tunity to disseminate your glaucoma to the digital archive. Print subscribers performance category’s contribution research directly to those who find it get 12 print issues, plus all the benefits will be reweighted upward; thus you most relevant. Joining the ranks of the of Focal Points Digital. Visit aao.org/ could still earn the maximum MIPS Academy’s esteemed Ophthalmology focalpoints. final score of 100 points despite not and Ophthalmology Retina, Ophthal- reporting any PI measures. mology Glaucoma provides readers with Order 2019 Coding Tools to New for 2018: Special consideration innovative, peer-reviewed works. Maximize Reimbursements given to small practices. If small prac- Submit your research at https:// Your coding tools for 2019 are now tices can demonstrate that obtaining www.evise.com/profile/#/OGLA/login. available. Whether you’re a beginner or and maintaining certified EHR technol- Subscribe at www.ophthalmology experienced coder, whether you work at ogy would cause undue hardship, CMS glaucoma.org. a comprehensive or subspecialty-specif- may grant them a PI hardship exception. ic practice, the Academy has the cod- Submit your application by Dec. 31, Remember the Foundation ing references and training guides to 2018. For guidance on submitting this on Giving Tuesday ensure you’re appropriately reimbursed application, see aao.org/medicare/ad After the holiday shopping rush on for the services you provide. vancing-care-information-exceptions. Black Friday and Cyber Monday, kick Browse the offerings at aao.org/ off your year-end charitable giving on codingproducts. Enter the EyeWiki Contest Giving Tuesday, Nov. 27. Entering its U.S. residents and fellows, November sixth year, this global day of philanthro- New Coding Audit Success is your last opportunity to submit an py encourages donating to initiatives Toolkit Available original EyeWiki article for the chance that are important to you. Stay compliant with payer requirements to win an all-expenses-paid trip in This year, consider supporting and proactively navigate the audit pro- April 2019 to the Academy’s Mid-Year Academy programs including the cess using the Academy’s new Coding Forum in Washington, DC. Submis- Ophthalmic News and Education Audit Success Toolkit (#0120444V). sions, which will be judged based on (ONE) Network, EyeCare America, This downloadable PDF includes valu- quality of work, should pertain to global outreach, and the Museum of able checklists and helpful guidelines 1 of the following categories: Vision campaign. Your tax-deductible you can use daily. • Cataract/Refractive Surgery/Cornea/ gift can be made in honor or memory Order today at aao.org/audit-toolkit.

54 • NOVEMBER 2018

53-55_Note_F.indd 54 10/4/18 3:14 PM New Business Handbook for D.C. REPORT Retina Practices Strategically Grow Your Retina Practice Academy Promotes (#0121003V), the first of 3 handbooks in the new Profitable Retina Practice Veteran Eye Care series, reveals the essentials for growing For veterans, the risk of eye injury is a retina practice and provides real-life much higher than for the general U.S. case studies to guide implementation. population. Order today at aao.org/business- Advocacy. Committed to ensuring of-retina. that veterans and active-duty service MEETING MATTERS members receive the highest quality eye care, the Academy’s recent advo- SERVING THOSE WHO SERVED cacy efforts have focused on a wide AAO 2019 in San Francisco OUR COUNTRY. Dr. Melicher range of issues, including the following: AAO 2019 will take place Oct. 12-15, Larson performs an eye exam on • maintaining a VA directive, which preceded by Subspecialty Day, Oct. a veteran during the American mandates that only ophthalmologists 11-12, at The Moscone Center in San Legion National Convention. will perform laser surgery in U.S. De- Francisco. Be inspired at the world’s partment of Veterans Affairs (VA) medical facilities; largest and most comprehensive oph- • promoting the VA’s new Technology-Based Eye Care Services program, thalmic meeting, offering hundreds of which is expanding veterans’ access to basic eye care services; courses and sessions on topics ranging • supporting the joint Department of Defense/VA Vision Center for from cataract complications to artificial Excellence, which was created to improve the care of American military intelligence in ophthalmology. personnel and veterans affected by combat eye trauma; and For more information, visit aao.org/ • advocating for increased funding for research related to combat-relat- 2019. ed vision trauma. In addition to its advocacy work with the federal gov- 2019 Abstract Deadlines Volunteering. ernment, the Academy also engages in hands-on acts of service to aid Want to create content for AAO 2019 veterans. in San Francisco? Submit your ideas As part of this service, each year the Academy collaborates with a for an instruction course or new Skills state ophthalmology society at the National Convention of the American Transfer lab. Abstracts will be accepted Legion, the largest U.S. wartime veterans service organization. This year, from Dec. 13, 2018, through Jan. 8, the Academy joined forces with the Minnesota Academy of Ophthal- 2019. mology (MAO) to provide glaucoma screenings for veterans during the To submit, visit aao.org/presenter- convention from Aug. 24-30 in Minneapolis. central. Over the course of 3 days, Minnesota ophthalmologists screened more than 140 veterans for glaucoma and other eye diseases. MAO volunteers Claim CME for AAO 2018 identified several glaucoma suspects and assisted 1 veteran with a vitre- AAO 2018 and Subspecialty Day ous hemorrhage who needed immediate surgery. registrants whose attendance was “This [was] a special opportunity for the MAO to honor our nation’s verified onsite in Chicago received an service members at their 100th national convention and provide them email with a link and instructions for with a valuable public service,” said Jill S. Melicher Larson, MD, President claiming Continuing Medical Educa- of the MAO and participant in the screening event. tion (CME) credits online. Starting Next year, the Academy will continue this public service tradition and Thursday, Dec. 13, attendees can claim reach out to the Indiana Academy of Ophthalmology to solicit volunteers credits (if they did not already do so for the American Legion’s 101st convention in Indianapolis. at the meeting) and obtain transcripts that include AAO 2018/Subspecialty Day credits at aao.org/cme-central. component of AAO 2018. View 13 Want to Own Content From The Academy transcript will not list archived sessions from Chicago (ap- AAO 2018? individual course attendance, only proximately 20 hours of educational Enjoy AAO 2018 all year. Meetings overall credits claimed for educational content) through Jan. 31, 2019. Access on Demand offers 8 Subspecialty Day activities. the Virtual Meeting with your Academy meetings or the AAOE Program; or save For more information, visit aao.org/ login and password. The AAO 2018 and buy the complete package, which annual-meeting/cme. Virtual Meeting cannot be reported for includes AAO 2018 highlights—nearly CME credit. 200 hours and 1,000 presentations. View the Virtual Meeting For more information, visit aao.org/ To learn more, visit aao.org/on The Virtual Meeting is a free online virtual-meeting. demand.

EYENET MAGAZINE • 55

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INTRODUCING Ophthalmology ® Retina

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EyeNet00_Ads_F.indd 57 and JRS ad: 8.125x10.875” 10/11/18 2:11 PM MYSTERY IMAGE BLINK Priya Bajgai, MBBS, Advanced Eye Centre, Post Graduate Institute of Medical Education of Medical Education Institute Graduate Post Centre, Eye Bajgai, MBBS, Advanced Priya Chandigarh, India. and Research,

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LAST MONTH’S BLINK Pigmented Keratic Precipitates in Herpes Simplex Virus Anterior Uveitis

27-year-old man presented, 1 2 complaining of decreased Avision in his right eye for 2 weeks. In that eye, best-corrected visual acuity (BCVA) was 20/80 and intraocular pressure (IOP) was 34 mm Hg. The slit-lamp examination showed 2+ cells and flare, along with pigmented keratic precipitates (KPs) in the lower half of the cornea (Fig. 1). In the left eye, BCVA was 20/25, IOP was 12 mm Hg, and the anterior chamber was clear. Polymerase chain reaction analysis of aqueous (though a rare association with postoperative en- humor from the right eye was positive for herpes dophthalmitis caused by Propionibacterium acnes simplex virus (HSV) DNA. The patient received has been reported). Our patient exhibited all of topical corticosteroids, cycloplegic and antiglau- these signs. The authors are following up on this coma drugs, and oral acyclovir. Three weeks later, case semiannually for uveitis flare-ups. the KPs resolved completely and the eye was qui- escent (Fig. 2). BCVA at 5 months was 20/20 and WRITTEN BY ANIRUDDHA K. AGARWAL, MBBS, IOP was 12 mm Hg. AMOD K. GUPTA, MBBS, REEMA BANSAL, MD, MBBS. In addition to high IOP, granulomatous KPs, PHOTOS BY ARUN KAPIL. ADVANCED EYE CENTRE, and sectoral iris atrophy, pigmented KPs are POST GRADUATE INSTITUTE OF MEDICAL EDUCA- characteristic of HSV-associated anterior uveitis TION AND RESEARCH, CHANDIGARH, INDIA.

58 • NOVEMBER 2018

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