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May 9 & 10, 2014 KU Edwards Campus BEST Conference Center 12604 Quivira Overland Park, KS 66213

Sponsored by the University of Kansas Department of and the Lemoine Alumni Society and in association with the Kansas Society of Eye Physicians and Surgeons (KSEPS) DEPARTMENT OF OPHTHALMOLOGY

SCHOOL OF MEDICINE CLINICAL FACULTY

John Sutphin, MD 7400 STATE LINE RD Luther & Ardis Fry Professor and Chairman

PRAIRIE VILLAGE, KS & Anterior Segment

66208 APPOINTMENTS: 913- 588-6600

Miranda Bishara, MD Dirck DeKeyser, OD William Godfrey, MD Mallory Kuchem, OD Cornea/Refractive/ Optometrist Optometrist

Paul Munden, MD Timothy Lindquist, MD J. Robbie Overlease, MD Ajay Singh, MD & Anterior Segment Pediatric Ophthalmology Glaucoma and Vitreous

Jason Sokol, MD Johnny Tang, MD W. Abraham White, MD Thomas J. Whittaker, JD, MD Oculofacial Plastic & Orbital Surg. Retina and Vitreous Comprehensive Neuro-Ophthalmology Kansas EyeCon 2014 ACKNOWLEDGMENTS

We wish to acknowledge and sincerely thank these organizations for exhibiting at both days of this conference:

Akorn Pharmaceuticals Alcon Laboratories, Inc. Carl Zeiss Meditec, Inc. Diopsys, Inc. Ellex IRIDEX Katena Products & IOP Ophthalmics

Regeneron Pharmaceuticals, Inc. And for exhibiting part of this conference:

Sightpath Medical

Allergan Envision University Heidelberg Engineering Midwest Microsurgical Repair, Inc. Kansas EyeCon 2014 Program Overview: This conference is intended to provide ophthalmologists with an educational forum to learn about new developments in the profession and their application to patient care. Covering a cross-section of all sub-specialties, physicians can expect to walk away having heard evidence-based presentations. Target Audience: This program will be of interest to all practicing ophthalmologists. Learning Objectives: Upon completion of the educational activity, participants should be able to: Evaluate and differentiate between current diagnoses, treatments and procedures in order to optimally treat their patients with retinal conditions and complications; Analyze case studies addressing several retinal conditions to effectively proceed with appropriate management; Describe diagnoses and treatments in the management of corneal diseases and Review treatments of in the pediatric population including management of complications and retrospective case reviews. Method of Participation: Statements of credit will be awarded based on the participant's attendance and submission of the activity evaluation form. A statement of credit will be available upon completion of an activity evaluation/claimed credit form that should be turned it at the end of the meeting. If you have questions about this CME activity, please contact AKH Inc. at service @akhealthcare.com. CME Credit Provided by AKH Inc., Advancing Knowledge in Healthcare Physicians: This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint providership of AKH Inc., Advancing Knowledge in Healthcare and the University of Kansas, Department of Ophthalmology and The Lemoine Alumni Society. AKH Inc., Advancing Knowledge in Healthcare is accredited by the ACCME to provide continuing medical education for physicians. AKH Inc., Advancing Knowledge in Healthcare designates this live activity for a maximum of 7.0 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity. FACULTY Adam AufderHeide, MD John F. Doane, MD, FACS Timothy W. Olsen, MD Prairie Village, KS Leawood, KS Atlanta, GA Anna Berry, MD, MPH Alina V. Dumitrescu, MD Ajay Singh, MD Prairie Village, KS Prairie VIllage, KS Prairie Village, KS Michelle Boyce, MD Alan Hromas, MD Erin Stahl, MD Prairie Village, KS Prairie Village, KS Kansas City, MO Emily Broxterman, MD John D. Hunkeler, MD R.C. Andrew Symons, MD, PhD Prairie Village, KS Overland Park, KS Melbourne, Australia Anita Campbell, MD Timothy P. Lindquist, MD W. Abraham White, MD Prairie Village, KS Kansas City, MO Prairie Village, KS Mary Champion, MD Martin A. Mainster, PhD, MD, FRCOphth. Lillian Yang, MD Prairie Village, KS Reno, NV Prairie Village, KS FACULTY DISCLOSURES Name Relationship Commercial Interest Adam AufderHeide, MD N/A Nothing to Disclose Anna Berry, MD, MPH N/A Nothing to Disclose Michelle Boyce, MD N/A Nothing to Disclose Emily Broxterman, MD N/A Nothing to Disclose Anita Campbell, MD N/A Nothing to Disclose Mary Champion, MD N/A Nothing to Disclose John F. Doane, MD, FACS Contracted Research Carl Zeiss Alina V. Dumitrescu, MD N/A Nothing to Disclose Alan Hromas, MD N/A Nothing to Disclose John D. Hunkeler, MD N/A Nothing to Disclose Timothy P. Lindquist, MD N/A Nothing to Disclose Martin A. Mainster, PhD, MD, FRCOphth. N/A Nothing to Disclose Timothy W. Olsen, MD N/A Nothing to Disclose Ajay Singh, MD N/A Nothing to Disclose Erin Stahl, MD Contracted Research Ophtec R.C. Andrew Symons, MD, PhD Contracted Research Novartis Corporation; PAREXEL Stock CSL Limited Other (Clinic Support) Novartis Corporation W. Abraham White, MD N/A Nothing to Disclose Lillian Yang, MD N/A Nothing to Disclose PLANNER DISCLOSURES KUMC/KSEPS Staff and Planners N/A Nothing to Disclose AKH Staff and Planners N/A Nothing to Disclose Commercial Support: There is no commercial support for this activity. Disclosures: It is the policy of AKH Inc. to ensure independence, balance, objectivity, scientific rigor, and integrity in all of its continuing education activities. The author must disclose to the participants any significant relationships with commercial interests whose products or devices may be mentioned in the activity or with the commercial supporter of this continuing education activity. Identified conflicts of interest are resolved by AKH prior to accreditation of the activity and may include any of or combination of the following: attestation to non-commercial content; notification of independent and certified CME/CE expectations; referral to National Author Initiative training; restriction of topic area or content; restriction to discussion of science only; amendment of content to eliminate discussion of device or technique; use of other author for discussion of recommendations; independent review against criteria ensuring evidence support recommendation; moderator review; and peer review. Disclosure of unlabeled use and investigational product - This educational activity may include discussion of uses of agents that are investigational and/or unapproved by the FDA. Please refer to the official prescribing information for each product for discussion of approved indications, contraindications, and warnings. DISCLAIMER - This course is designed solely to provide the healthcare professional with information to assist in his/her practice and professional development and is not to be considered a diagnostic tool to replace professional advice or treatment. The course serves as a general guide to the healthcare professional, and therefore, cannot be considered as giving legal, nursing, medical, or other professional advice in specific cases. AKH Inc. specifically disclaims responsibility for any adverse consequences resulting directly or indirectly from information in the course, for undetected error, or through participant's misunderstanding of the content.

LEMOINE DISTINGUISHED ALUMNI LECTURERS

LECTURER TITLE DATE

Timothy W. Olsen, MD Rock Chalk Retina Talk: 100 Year KU 5/9/2014 KU SOM MD ‘89

Please join us in congratulating Dr. Timothy W. Olsen, who was selected as the first Lemoine Distinguished Alumni Lecturer, which was established as part of our yearlong Centennial Celebration. Alumni Speakers

John Doane, MD MD: 1990; Residency: 1995

John Hunkeler, MD MD: 1967; Residency: 1973

Timothy Lindquist, MD Residency: 2012

Erin Stahl, MD MD: 2005; Residency: 2009; Fellow: 2011

AGENDA

Kansas EyeCon May 9 – 10, 2014 KU Edwards Campus BEST Conference Center

Friday, May 9, 2014

12:00 p.m. Registration and lunch with exhibitors

Retina Session

1:00 p.m. Welcome: Miranda Bishara, MD

1:05 p.m. Adam AufderHeide, MD, Twenty-Seven Gauge Vitrectomy: Outcomes and Complications

1:15 p.m. R.C. Andrew Symons, MD, PhD, Diagnostic Dilemmas: Differentiating from Optic Neuropathies

1:40 p.m. Ajay Singh, MD, Force Data Measurements During Manual Small Incision Surgery (MSICS)

2:05 p.m. Martin Mainster, MD, Glare’s Causes and Countermeasures: Perception and Misperception

2:30 p.m. Alan Hromas, MD, Experience with the Micropulse Laser for Diabetic

2:40 p.m. R.C. Andrew Symons, MD, PhD, Update on AMD Trials: Recent Lessons about Natural History and Treatment

3:05 p.m. Break

3:35 p.m. Lillian Yang, MD, Retrospective Review of Interventional Thrombolysis for Central Retinal Artery Occlusion at the University of Kansas Medical Center

3:45 p.m. John Hunkeler, MD, Introduction of Dr. Olsen and brief Lemoine history

4:00 p.m. Timothy W. Olsen, MD, Lemoine Distinguished Alumnus Lecturer, Rock Chalk, Retina Talk: 100 Years KU

5:00 p.m. Session Adjourns

On site reception immediately following

University of Kansas Department of Ophthalmology and The Lemoine Alumni Society Kansas EyeCon May 9 – 10, 2014 Saturday, May 10, 2014

7:00 a.m. Breakfast with exhibitors Pediatrics, and Anterior Segment Section 8:00 a.m. Welcome – Miranda Bishara, MD

8:05 a.m. Anna Berry, MD, Retrospective Chart Review of the use of Imaging and Biopsy in the Diagnosis of Sheath Meningiomas and Orbital Lymphomas at University of Kansas Medical Center

8:15 a.m. Erin Stahl, MD, Use of an -Fixated Anterior Chamber for the Treatment of in Patients with

8:40 a.m. Alina Dumitrescu, MD, A Four Year Retrospective Review of Space Occupying Lesions of the Orbit

8:50 a.m. Timothy Lindquist, MD, Not ALL the Same old, Same old: Advancements in Pediatric Ophthalmology

9:15 a.m. Michelle Boyce, MD, The Incidence of Scleral Lens Associated Infections in the Setting of Ocular Graft versus Host Disease

9:25 a.m. Mary Champion, MD, Outcomes of Treatment: Retinopathy of Prematurity and Bevacizumab

9:35 a.m. Break

10:05 a.m. Emily Broxterman, MD, Bilateral Lateral Rectus Recession versus Unilateral Lateral Rectus Recession for Small Angle

10:15 a.m. John Doane, MD, Small Incision Lenticule Extraction (SMILE) for Simple

10:40 a.m. Anita Campbell, MD, Trabectome Efficacy and Safety: Early Experience

10:50 a.m. W. Abraham White, MD, International Volunteering in Ophthalmology: Opportunities and Challenges

11:15 a.m. John Hunkeler, MD, Soemmering’s Ring Subluxation with Encapsulated Lens Implant

11:40 a.m. John Sutphin, MD, Luther and Ardis Fry Professor and Chairman, Closing Remarks: Future of KU Eye

12:00 p.m. Session Adjourns

University of Kansas Department of Ophthalmology and The Lemoine Alumni Society

ABSTRACTS

 Retrospective non-comparative case study  220 potential cases were identified Adam AufderHeide, MD/PhD between July 2011 and June 2013 by Gregory Fox, MD surgical booking criteria  Exclusion criteria included any patient that underwent 25 gauge vitrectomy instead  Patient’s that started with 27 gauge but converted to 25 gauge were included

 190 patients underwent 27 gauge vitrectomy  114 females, 76 males  93 Right eyes, 97 left eyes  Average age 68 +/- 14 years 3.000

2.500 ERM PreOpLogMAR Pre-Op FinalLogMAR Post-Op PreOp IOP Day 1 IOP Final IOP ReOp

MacHole Ave StDev Snellen Ave StDev Snellen Ave StDev Ave StDev Ave StDev Rate

TRD ERM 102 0.506 0.292 20/63 0.397 0.374 20/50 15 3 14 5 15 3 3% 2.000 VitOpac MacHole 49 0.910 0.579 20/160 0.636 0.574 20/80 16 3 15 6 15 3 8%

Endoph TRD 3 1.247 1.347 20/320 1.091 1.398 20/250 18 4 24 16 15 5 0%

PDR VitOpac 11 0.374 0.333 20/50 0.226 0.303 20/32 14 4 14 2 15 3 18% 1.500 Ret.Lens.Frag Endoph 2 1.699 1.415 20/1000 0.320 0.316 20/50 17 4 26 13 21 13 0%

Vit.Heme. PDR 15 1.664 1.069 20/900 0.573 0.707 20/50 16 3 16 9 17 9 20% Ret.Lens.Fra Lamelar Hole g 1 1.000 20/200 0.301 20/40 29 27 19 0% 1.000 VMT Vit.Heme. 16 2.039 0.914 20/2000 0.626 0.848 20/80 16 4 15 9 18 8 19% Lamelar BRVO Hole 4 0.651 0.288 20/90 1.649 1.329 20/90 12 1 10 5 15 2 75%

RhegRD VMT 10 0.413 0.099 20/50 0.303 0.187 20/40 15 2 14 4 17 6 0% 0.500 Total* BRVO 2 2.600 0.000 20/2000 0.088 0.125 20/25 17 5 13 1 16 3 0%

RhegRD 2 1.631 0.193 20/125 1.000 0.000 20/63 14 3 18 4 17 3 0%

Total* 190 0.753 0.647 20/125 0.493 0.543 20/63 16 3 14 6 15 4 7% 0.000 PreOpLogMAR FinalLogMAR

35  1 Immediate post operative hypotony

30 ERM  1 persistent membrane requiring MacHole 25 TRD reoperation VitOpac Endoph 20  1 RD repaired by 25 g vitrectomy PDR Ret.Lens.Frag 15 Vit.Heme. Lamelar Hole VMT 10 BRVO RhegRD 5 Total*

0 PreOp IOP Day 1 IOP Final IOP

 157 patients completed at least 3 months  Mac hole complications: of follow up  2 repeat surgeries for reopened Mac  82 of these initially pseudophakic Holes  At final follow up time, 115 were  1 total RD repaired with 25 gauge pseudophakic  1 partial RD repaired with 25 gauge  Overall, 66 showed progression of their cataract and only 11 showed no progression over the follow up time (range 3 months to 1 year)  1 RD treated with vitrectomy  27 gauge vitrectomy is a safe and  1 small tears treated with laser effective technique for treating certain vitreoretinal pathologies.

 3 repeat (25 gauge) vitrectomies for recurrent Vitreous Heme

 2 RD requiring 25 gauge vitrectomy  1 choroidal hemorrhage requiring drainage

Diagnostic Dilemmas: Differentiating Retinopathies from Optic Neuropathies

R. C. Andrew Symons, MD, PhD

Visual loss with minimal signs… • Anterior Tear film Lens – eg. Refractive changes out of keeping with severity of cataract • Retina • Optic nerve, chiasm and radiations • Cerebral cortices Symptoms • Retinal Photopsia Metamorphopsia/ micropsia/ macropsia • Colour desaturation/ dyschromatopsia Field loss Signs • Retinal Subtle vascular changes Including arteriolar attenuation Subtle pigmentary changes Sometimes pallor may be the chief fundoscopic sign of an occult retinopathy • Optic neuropathy Colour desaturation Colour deficiency Enlarged blind spot RAPD Nerve fibre layer defect Optic disc oedema or pallor Testing • Optical coherence tomography • Electrophysiology Full-field ERG Pattern ERG Multifocal ERG Visually evoked potentials

Symons: Diagnostic Dilemmas: Differentiating Retinopathies from Optic Neuropathies, continued

Retinal diseases that may be difficult to detect on ophthalmoscopy • Posterior uveitis such as birdshot chorioretinopathy • Acute zonal occult outer retinopathy group pathologies • Autoimmune or paraneoplastic retinopathy • Early stages of rod-cone or cone-rod dystrophy • Chronic retinal vascular disease Messages • Inter-disciplinary approach • Careful history taking • Search for subtle signs on examination • Careful analysis of imaging – esp. OCT • Logical approach to electrophysiology

Cases …

Notes:

Force Data Measurements During Manual Small Incision Cataract Surgery (MSICS)

Ajay Singh, MD

Purpose: To measure forces generated during major steps of manual small incision cataract surgery.

Methods: 14 eyes underwent MSICS surgery by the same surgeon. A force-sensing transducer probe attached to surgical instruments was used to measure the force generated during the major steps of the surgery. Forces generated during horizontal, vertical and anterior-posterior maneuvers were recorded. All feedback from the transducer was correlated with video footage of the surgical steps (Fig 1.). Forces were measured during scleral tunneling, paracentesis entry, anterior chamber entry through the scleral tunnel, capsulotomy, lens vectis assisted lens removal and IOL dialing.

Results: Scleral tunneling produced an average force of 48.6g (range 115.8-4.8). Paracentesis incision produced an average force of 23.4g (range 55.6-13.). Anterior chamber entry through the scleral tunnel produced an average force of 22.9g (range 52.0- 2.1). No significant forces were noted during lens capsule dissection; however repositioning forces were recorded as an average force of 23.6g (range 48.0-10.9). Lens vectis assisted removal of the cataractous lens averaged 35.1g (range 66.3-7.8). Sinskey hook assisted IOL dialing produced an average force of 4.7g (range 7.4-1.6).

Conclusions: This unique technique detects significant and variable forces that are produced during an MSICS procedure. The maximum force generated during this surgery is detected during scleral tunnel construction and the minimum force is generated during anterior lens capsule engagement. A careful recording and study of intraoperative forces will be a helpful guide for generating a haptic (tactile) based MSICS surgical simulator.

Glare’s Causes and Countermeasures: Perception and Misperception

Martin Mainster, MD

Glare is a normal response to abnormal illumination, whereas is an abnormal response to normal illumination that’s exaggerated by abnormal illumination. Glare can range from insignificant to incapacitating. Aging and ocular or systemic disease can increase susceptibility to glare and recovery time from it.

The brain receives information from at least three different types of retinal photoreceptors. Rod and cone photoreceptors in the outer retina send most of the photic data needed for conscious vision to visual brain centers. Retinal ganglion photoreceptors in the inner retina send most of the photic data needed for unconscious biological and behavioral control to the suprachiasmatic nuclei and other nonvisual brain centers.

The four primary categories of glare are disability, discomfort, dazzling and scotomatic (photostress, flashblindness) glare. Disability glare (physiological glare) is caused by intraocular light scattering (straylight) that reduces the contrast of retinal images by spreading a veiling luminance across them. In common environments, glare and target illumination have the same or similar spectra. Colored spectacle or intraocular lens filters attenuate both light sources equivalently, so filters cannot decrease disability glare because they do not increase retinal image contrast. The practical effects of disability glare from intraocular light scattering are more severe when extraocular light scattering (such as from a dirty windshield, fog, etc.) reduces visual target contrast. Discomfort glare (psychological glare) is caused by illumination that is too intense or variable for someone in a particular situation. It produces annoyance and aversion but may not reduce visual performance. Dazzling glare causes annoyance, squinting, aversion and visual disability when bright light is spread across the retina in brilliant environments. It is an extreme form of discomfort glare associated with . Scotomatic glare (photostress, flashblindness) causes extreme photopigment bleaching and afterimages when the macula is overwhelmed by excessive focal light exposure.

Conclusion: Progress in understanding and managing glare has been hampered by its complex, multidisciplinary nature and limited interdisciplinary communication. The optical origins of discomfort glare are well understood. Neurophysiological research is clarifying how discomfort and dazzling glare depend on visual and non-visual photoreception as well as nociceptive brain pathways involving the trigeminal ganglion and thalamus. Colored or neutral density filters can decrease retinal illuminance in brilliant daytime environments and thus discomfort and dazzling glare. They cannot decrease disability glare in ordinary environments. There are no effective nighttime personal countermeasures currently available for reducing headlight glare from oncoming traffic at night on undivided roadways. Minimizing extraocular light scattering that reduces visual target contrast can potentially improve drivers’ daytime or nighttime visual performance.

Mainster: Glare’s Causes and Countermeasures: Perception and Misperception, continued

References 1. Mainster MA, Turner PL. Glare’s causes, consequences and clinical challenges after a century of ophthalmic study. Am J Ophthalmol 2012; 53:587-93. 2. Parsons JH. Glare, its causes and effects. Illuminating Engineer, London 1910; 3:99-103. 3. Duke-Elder S. Sir John Herbert Parsons, 1868-1957. Br J Ophthalmol 1957; 41:705-8. 4. Mainster MA, Timberlake GT. Why HID headlights bother older drivers. Br J Ophthalmol 2003; 87:113-117. 5. Turner PL, Van Someren EJW, Mainster MA. The role of environmental light in sleep and health: effects of ocular aging and cataract surgery. Sleep Medicine Reviews 2010; 14:269-80. 6. Noseda R, Kainz V, Jakubowski M., et al. A neural mechanism for the exacerbation of headache by light. Nature Neurosciences 2010; 13:239-45. 7. Noseda R, Burstein R. Advances in understanding the mechanisms of migraine-type photophobia. Curr Opin Neurol 2011; 24:197-202. 8. Coppens JE, Franssen L, van den Berg TJ. Wavelength dependence of intraocular straylight. Exp Eye Res 2006; 82:688-92. 9. Gegenfurtner KR, Mayser H, Sharpe LT. Seeing movement in the dark. Nature 1999; 398:475-6. 10. Steen R, Whitaker D, Elliott DB, Wild JM. Effect of filters on disability glare. Ophthalmic Physiol Opt 1993; 13:371-6. 11. Vos JJ. Reflections on glare. Lighting Res Technol 2003; 35:163-176.10. 12. Owsley C, McGwin G, Jr. Vision and driving. Vision Res 2010; 50:2348-61

Notes:

Experience with the Micropulse Laser for Diabetic Macular Edema

Alan Hromas, MD, Resident Class of 2014 Primary Supervisor: Johnny Tang, MD.

Introduction: Diabetic macular edema is a complication of which may result in decreased visual acuity.1 Treatment of diabetic macular edema has historically involved focal or grid laser,2 and more recently, intravitreal corticosteroids and anti-VEGF medications.3 Micropulse laser has emerged more recently as a treatment option for diabetic macular edema.4 Though the technology is not yet widely available, MicroPulse treatment is believed to be effective for diabetic macular edema and capable of producing lasting benefit without appreciable permanent tissue damage.5

Methods: We present a retrospective, non-comparative series of patients treated with MicroPulse laser for diabetic macular edema. The group presented represents our initial experience with this treatment modality. Each of the patients presented were being actively treated with monthly intravitreal anti-VEGF drugs when MicroPulse laser was incorporated into their management. We report data on their visual acuity and their macular thickness as measured by optical coherence tomography, both in the months prior to, and the months following MicroPulse laser treatment. We furthermore provide a review of the available data regarding the effectiveness or MicroPulse treatment.

Results/Conclusion: Our experience with subthreshold MicroPulse diode laser has found the treatment to be well-tolerated with no appreciable tissue damage. In our patient population, treatment appears to have been more beneficial in those patients who had not undergone prior treatment for DME. Patients with DME that had displayed minimal response to intravitreal anti- VEGF generally did not appear to have significant added benefit from the addition of MicroPulse laser.

1 Klein R, Klein BE, Moss SE, et al. The Wisconsin Epidemiologic Study of Diabetic Retinopathy. XIV. Ten- year Incidence and Progression of Diabetic Retinopathy. Arch Ophthalmol. 1994;112:1217–1228. 2 Early Treatment Diabetic Retinopathy Study Research Group. Photocoagulation for Diabetic Macular Edema. Early Treatment Diabetic Retinopathy Study Report Number 1. Arch Ophthalmol. 1985;103: 1796–1806. 3 Mitchell, Paul et al. Management Paradigms for Diabetic Macular Edema. American Journal of Ophthalmology , Volume 157 , Issue 3 , 505 - 513.e8. 4 Sivaprasad S. Micropulsed Diode Laser Therapy: Evolution and Clinical Applications. Survey of Ophthalmology. 2010-11;55:516-30. 5 Othman IS. Subthreshold Diode-laser Micropulse Photocoagulation as a Primary and Secondary Line of Treatment in Management of Diabetic Macular Edema. Clinical ophthalmology (Auckland, N.Z.). 2014;8:653-9. Update on AMD Trials: Recent Lessons about Natural History and Treatment

R. C. Andrew Symons, MD, PhD

Trials comparing bevaicuzmab and ranibizumab - CATT - IVAN - GEFAL - MANTA Trials comparing ranibizumab and aflibercept - VIEW - Small trials in non-responders and for pigment epithelial detachments

Outcomes Visual acuity – similar in all Anatomical outcomes – slightly better for ranibizumab than bevacizumab Systemic Safety – mild superiority of ranibizumab compared with bevacizumab; mild superiority of monthly compared with prn Number of injections – slightly fewer injections probably required with ranibizumab than bevacizumab

One year predictors of visual acuity in CATT • Older age • Worse baseline visual acuity • Larger CNV area • Predominantly or minimally classic lesion • Thicker total thickness at fovea • Presence of RPE elevation on OCT

Predictors for less visual acuity improvement • Older age • • Larger CNV area • AbsenceBaseline ofVA RAP ≥ 20/40 lesion • RPE elevation on OCT

Other lesion outcomes, and their risks: Scar • Classic neovascularization • Thicker retina • More fluid or material under the fovea

Geographic atrophy • Older age • Ranibizumab use • Monthly injection regimen • Poor baseline VA • Foveal intraretinal fluid

Bilateral Lateral Rectus Recession versus Unilateral Lateral Rectus Recession for Small Angle Exotropia

Emily Broxterman, MD, Resident Class of 2015 Primary Supervisor: Michelle Ariss, MD

Introduction: There are many causes for pediatric which result in vertical, torsional or horizontal deviations. Moderate to large angle comitant horizontal strabismus (>25 prism diopters) is typically treated with bilateral rectus muscle recessions or unilateral rectus recession with resection of the antagonist rectus muscle. Small angle horizontal deviations (< 25 prism diopters) are treated with bilateral or unilateral rectus muscle recessions. While recent studies have shown that unilateral rectus muscle recession is safe and effective with predictable outcomes, the procedure has been avoided by many physicians due to concern for undercorrection and/or incomitant motility post operatively.

Study Objectives: 1. To evaluate the efficacy and predictability of unilateral versus bilateral lateral rectus recession for treatment of small angle exotropia of subjects who underwent unilateral or bilateral lateral rectus recession at Children’s Mercy Hospitals between 8/1/2009 and 8/1/2010. 2. To determine if the primary objective outcomes vary based on the presence of intermittent versus constant exotropia

Methods: We propose a single-center, retrospective chart review of 193 patients with a small- angle deviation (less than 25 prism diopter) who underwent unilateral or bilateral lateral rectus recession at Children’s Mercy Hospitals between 08/01/2009 and 08/01/2010 and have follow- up records available between 08/01/2009 and 08/01/2013.

The following data are used as inclusion criteria for study participants: • Age 3 to < 11 years • Small angle exotropia (manifest deviation) • Presence of a deviation between 15 and 25 prism diopters • Subjects who underwent unilateral or bilateral lateral rectus recession strabismus surgery at CMH or CMH South between 08/01/2009 and 08/01/2010 • Follow-up records available between 08/01/2009 and 08/01/2013

The following data are used as exclusion criteria for study participants: • Lack of post-operative measurement data • • Previous surgery on lateral rectus muscles • CombinedVisual acuity surgery difference on vertical of ≥ 2 linesrectus between muscles each or oblique eye preoperatively muscles at time of lateral rectus recession

Broxterman: Bilateral Lateral Rectus Recession versus Unilateral Lateral Rectus Recession for Small Angle Exotropia, continued

Each patient was reviewed for the following pre-operative data: age at the time of surgery, gender, type of surgery, amount of lateral rectus recession (mm), ocular alignment, visual acuity, stereoacuity and refraction. Each patient was then reviewed for the following post-operative data: surgical complications, need for re-operation, type of surgical procedure and post-operative alignment at 1 week, 6 weeks, < 6 months, <1.5 years, < 2 years, < 2.5 years and < 3 years. All research data was collected using subject confidentiality and analyzed with the assistance of the statistics department at Children’s Mercy Hospital.

References: 1. PEDIG Protocol IXT1 - A Randomized Trial of Bilateral Lateral Rectus Recession versus Unilateral Lateral Rectus Recession with Medial Rectus Resection for Intermittent Exotropia, Version 2.0, April 30, 2010. http://publicfiles.jaeb.org/pedig/protocol/IXT1Protocol_v2.pdf 2. Wang L, Nelson L. One Muscle Strabismus Surgery. Current Opinion in Ophthalmology 2010;21:335-340. 3. Olitsky SE. Early and Late Postoperative Alignment Following Unilateral Lateral Rectus Recession for Intermittent Exotropia. J Pediatr Ophthalmol Strabismus 1998 May- June;35(3):146-8. 4. Kim H, Kim D, Choi D. Long-term Outcomes of Unilateral Lateral Rectus Recession versus Recess-Resect for Intermittent Exotropia of 20-25 Prism Diopters. BMC Ophthalmology April 2014;14:46

Notes

Rock Chalk, Retina Talk: 100 Years KU

Timothy W. Olsen, MD, Lemoine Distinguished Alumnus Lecturer

1. Recognition of Albert N. Lemoine, MD and his contributions to Ophthalmology, the Community, and the University of Kansas

2. Evidence Based Care a. AAO’s Preferred Practice Patterns i. ONE Network 1. http://one.aao.org/CE/PracticeGuidelines/PPP.aspx b. The Cochrane Collaboration i. Best Evidence for Healthcare ii. Multinational Organization iii. Structured Evidence Reviews c. SIGN i. Scottish Intercollegiate Guidelines Network ii. Assess the Evidence based on a Judgment of Importance iii. Balance complexity of data with Clarity to the users d. GRADE i. Grading of Recommendations Assessment, Development and Evaluation ii. Summary of quality of data/evidence iii. Balance benefits and potential harm iv. Balance net benefits and costs 3. Case Examples a. Pediatric Retinal Disorders b. Adult Inherited Disorders c. Acquired Retinal Disorders d. Selected Intraocular Neoplasia e. Age Related i. A risk assessment paradigm f. Diabetic Retinopathy

4. Each Category will have an audience based response a. Confidential answers (no one sees who votes) b. Compare your answers to the current literature and evidence based options

5. Summary and Conclusion

Retrospective Chart Review of the Use of Imaging and Biopsy in the Diagnosis of Optic Nerve Sheath Meningiomas and Orbital Lymphomas at University of Kansas Medical Center

Anna Berry, MD, Resident Class of 2016 Primary Supervisor: Jason Sokol, MD

Optic nerve sheath meningiomas (ONSM) constitute approximately 2% of all orbital tumors and classically present with progressive painless vision loss and proptosis. Both MRI and CT characteristically show enhancing diffuse tubular enlargement of the optic nerve. Histologically, the OSNM encases the optic nerve and displays whirled tumor cells.

Orbital lymphomas are the second most common orbital cancer with majority being non-Hodgkin B cells lymphoma. Orbital lymphomas typically present with a gradually progressive painless orbital mass and imaging displays a puttylike molding of the lesion to the surrounding structures. Immunohistochemistry demonstrates immunopositivity for CD19 and CD20 in B cell lymphomas.

This presentation will review optic nerve sheath meningiomas and orbital lymphomas seen by Dr. Jason Sokol at KUMC from 2010 to 2014 with emphasis on the correlation of imaging and biopsy results.

Ectopia lentis in Marfan Ophthalmic Findings Syndrome • Ectopia Lentis (60%) – Myopia Erin D. Stahl, MD –Astigmatism – Assistant Clinical Professor University of Kansas, Department of Ophthalmology • Cataract Assistant Professor • Glaucoma University of Missouri, Kansas City, Department of Ophthalmology •

© The Children's Mercy Hospital, 2014. 03/14 4 © The Children's Mercy Hospital, 2014. 03/14

Marfan Syndrome Management

• Mild dislocation with good BCVA • Incidence of 1 in 5,000 – Treat with glasses or contact lenses • Autosomal dominant • Moderate to severe dislocation with good BCVA • 25% of cases are new – Consider quality of vision mutations – Refractive correction versus surgery • Mutation in FNB-1 gene • Moderate to severe dislocation with poor BCVA causing abnormal fibrillin – Depending on age, consider amblyopia – treat accordingly • Abnormal fibrillin causes – Consider lensectomy connective tissue problems

2 © The Children's Mercy Hospital, 2014. 03/14 5 © The Children's Mercy Hospital, 2014. 03/14

Clinical Features Lensectomy

• Aortic dilation with possibility for • Approach rupture or dissection – Pars plana • Ectopia lentis – Limbus • Mitral valve prolapse • Bag management • Arachnodactyly • Scoliosis – Secure bag with hooks, CTR, suture, none • Pectus excavatum • IOL? • Spontaneous pneumothorax – Aphakia, IOL in the bag, scleral fixated IOL, iris fixated IOL

3 © The Children's Mercy Hospital, 2014. 03/14 6 © The Children's Mercy Hospital, 2014. 03/14 My Preference My Preference

• Surgical positioning so my right hand can • In young children (2-12 years) approach axis of dislocation – Aphakia management with glasses or CTL • 2 MVR paracentesis wounds • Well tolerated • Iris hooks as needed • Easy to change rx with eye growth • MVR into lens at equator (PRAY) • Continue as long as glasses/CTL well tolerated • Careful aspiration of lens material, bag and zonules with the vitrector

7 © The Children's Mercy Hospital, 2014. 03/14 10 © The Children's Mercy Hospital, 2014. 03/14

Surgical video My Preference

• In older children (12-21 years) – CTL at 1-2 weeks after surgery – Offer Artisan iris claw lens to be implanted 4-6 weeks after initial surgery

8 © The Children's Mercy Hospital, 2014. 03/14 11 © The Children's Mercy Hospital, 2014. 03/14

Refractive Management Atrisan Aphakic Lens

• Aphakic glasses • Aphakic contact lenses • Primary or secondary IOL –Bag – Sutured (Iris or Sulcus) – Iris claw lens

9 © The Children's Mercy Hospital, 2014. 03/14 12 © The Children's Mercy Hospital, 2014. 03/14 Artisan Aphakia Lens Risks

• Endothelial cell loss • Currently used in an FDA trial for pediatric and adult aphakic patients • Dislocated lens – 5 pediatric sites in the US • ovalization • No capsular support • Wound leak • Good endothelial cell counts • Infection • Adequate iris tissue for enclavation • Retinal detachment

13 © The Children's Mercy Hospital, 2014. 03/14 16 © The Children's Mercy Hospital, 2014. 03/14

Artisan Aphakia Lens Conclusion

• Surgical Approach • causes to ectopia lentis in 60% of patients – 5.7mm superior scleral tunnel incision • Ectopia lentis can cause extreme refractive – Place lens on iris surface error and amblyopia in children – Use special tools to enclavate haptics • Lensectomy with or without secondary IOL is – Surgical PI safe and effective • Implanting the Artisan iris-claw lens is a good option in older patients

14 © The Children's Mercy Hospital, 2014. 03/14 17 © The Children's Mercy Hospital, 2014. 03/14

Surgical video

15 © The Children's Mercy Hospital, 2014. 03/14

A Four Year Retrospective Review of Space Occupying Lesions of the Orbit

Alina Dumitrescu, MD, Resident Class of 2015 Primary Supervisor: Jason Sokol, MD

Background: A wide variety of processes can produce space-occupying lesions in and around the orbit. These include benign neoplasms, malignant neoplasms (primary or metastatic), vascular lesions, inflammatory disease, congenital lesions and infection, among other causes. These lesions can arise from any structure within the orbit including the and adnexa, the lacrimal gland, orbital fat, muscle or vascular structure.

The purpose of this study is to determine the demographics, the frequency, the distribution according to diagnosis, the recurrence frequency of orbital space-occupying lesions in our population of patients.

Material and methods: A retrospective, descriptive, chart review was performed. All biopsied/surgically removed orbital lesions treated in our department between 2010 and 2014 were identified by surgical CPT codes. In each case gender and age of the patients, pathological diagnosis, number of reinterventions and laterality were registered.

Results: We identified 157 procedures performed on 133 patients by a single surgeon over 4 year period. There was a slight predominance of male patients. Average age at the time of the procedure was 54 (the youngest patient was 6 mo. and the oldest 98).

Out of 157 procedures 46 (30%) were orbitotomy with bone flap, 16 (10.4%) were orbital exenteration, 12 (7.8%) were orbitotomy with drainage, 51 (32.5%) were orbitotomy with removal of the lesion, 32 (20.4%) were orbitotomy without bone flap and 2 (1.3%) were exploratory orbitotomy.

Pathological characteristic of the lesions showed: • 24 patients (18%) had invasive carcinomas including squamous cell, basal cell, metastatic adenocarcinoma, lacrimal gland carcinoma and other malign tumors including sarcoma, spindle cell, solitary fibrous tumor • 20 patients (15%) had benign lesions including lipoma, papilloma and granuloma • 16 patients (12%) had infectious orbital cellulites, 6 of which (37.5%) were mucormycosis and 1 (6.3%) • 16 patients (12%) had inflammatory disease including sarcoidosis and IgG4 related disease • 13 patients (9.8%) had lymphomas • 11 patients (8.3%) had cystic lesions including dermoid • 8 patients (6%) had vascular malformations including cavernous hemagioma, AVM and lymphangioma

Dumitrescu, A Four Year Retrospective Review of Space Occupying Lesions of the Orbit, continued

• 6 patients (4.5%) had trauma related complications • 5 patients (3.8%) had melanoma • 5 patients (3.8%) had meningioma • 9 patients (6.8%) had no abnormal findings on pathology exam

There were 16 reinterventions on 13 patients representing 10.2% of the procedures and 9.8% of the patients, respectively. A larger number of the procedures involved the right orbit.

Conclusions: Orbital space-occupying lesions represent an important part in our practice. They carry a significant morbidity and mortality. Despite the use of MRI and CT scanning, the histological examination remains necessary for final diagnosis.

Notes

The Practice of Medicine

‘Something new under the sun’ • Dates back to Prehistoric times: 8000 BC Advances in pediatric ophthalmology – Spirit healers performed ceremonies and cast spells – Primitive surgery of trepaning • Removal of a piece of bone from the skull

Timothy P. Lindquist, M.D. • Ancient Greeks set foundation for modern Children’s Mercy Hospital & Clinics Assistant Professor, University of Missouri at Kansas City diagnostic techniques: 400 BC–300 AD Clinical Assistant Professor, University of Kansas Department of Ophthalmology

Acknowledgement: Laura Plummer, MD, who generously shared slides used in this presentation

Financial Disclosure Leeching

• Nothing to disclose • Practice of applying leeches to the body to draw blood for therapeutic purposes – Began around 2000BC – Utilized throughout history – Regaining favor in modern use

Ecclesiastes 1:9 “. . . there is nothing new under the sun.” Bloodletting

• Release of evil or bad ‘humors,’ practiced into the mid-19th century Couching Advances in treatment of disease • One of the oldest surgical procedures Hemangioma – Dates back to 2000BC • • Retinoblastoma • Retinopathy of Prematurity

Couching Infantile Hemangioma

• Surgical procedure to displace the lens to the Most common soft tissue back of the eye using a • sharp instrument of childhood • “Surgeon” would move from town to town, so • Experience a growth was nowhere around phase, plateau phase, then when involution phase arose

Infantile Hemangioma

• May classified by: – Depth of skin involvement • Superficial – bright red • Deep – bluish hue • Compound – both elements – Type of orbital involvement • Preseptal • Intraorbital • compound – Based upon morphology – greatest prognostic value • Localized • Segmental • Indeterminate Infantile Hemangioma Infantile Hemangioma • Adverse effects of systemic • Majority do not require intervention – Cushingoid facies – GI upset • Potential complications requiring treatment – Irritability – Sleep disturbances – Ulceration – Immunosuppression – Life or function threatening complications – Adrenal suppression Disfigurement – – HTN – Psychosocial distress – Bone demineralization – Growth retardation – Cardiomyopathy

Infantile Hemangioma Infantile Hemangioma

• Visual compromise – Major reason for intervention • Potential complication of intralesional – Amblyopia: – Central retinal artery occlusion • - induced astigmatism – Skin atrophy, necrosis, and/or calcification • and/or obstruction of visual axis – Depigmentation of skin • Strabismus – Additional ocular complications: • Proptosis • • NLDO • Compressive optic neuropathy

Infantile Hemangioma Infantile Hemangioma

• No current FDA approved agents • Newer treatment option: β-Blockers • Past mainstay of medical treatment: • Systemic Propranolol • Topical Timolol Drops – Corticosteroids • Systemic • Topical • Intralesional injection • Additional treatment options: – Surgical excision – Laser Oral Propranolol Topical Timolol • Guo et al. JAMA 2010 • Haider et al, Indiana University – 0.5 % timolol maleate BID x 5 weeks • Report of 17 pts treated w/ oral propranolol – Results reported as 10 excellent, 6 good (decrease of >50%, <50%, respectively) • Dose up to 2 mg/kg daily, treated starting at age varying 3 wks to 12 months • Treatment continued until resolution or age 9- • Xue et al , JAMA 2013 11 months, at discretion of physician – 2 cases of response of deep lesions to topical 0.5% timolol

Infantile Hemangioma

• Currently, systemic propranolol widely used – Perceived lower side-effect profile – Impressive and relatively quick results

• Improves color, softens, causes growth arrest and regression of lesions

Haider KM, Plager DA, Neely DE, Eikenberry J, Haggstrom A. Outpatient treatment of periocular infantile hemangiomas with oral Effects extend beyond proliferative phase propranolol. J AAPOS. 2010 Jun;14(3):251-6. •

Oral Propranolol Infantile Hemangioma

• Vassallo et al • Potential adverse effects of Propranolol • 4 mo of treatment in pts under 1 results in – Hypotension resolution of lesions – Bradycardia – Hypoglycemia – Respiratory distress • Review of published literature in Eye in 2011 Sleep disturbance summarized results of 19 articles, mostly small – case series, recommending need for RCT – GI problems – Hyperkalemia Infantile Hemangioma Retinoblastoma

• No real consensus on work up before • Most common presenting sign in US: propranolol initiation

• Most recommend EKG, cardiology exam

• Propranolol is contraindicated in heart disease or asthma/reactive airway disease

Retinoblastoma • Typical presentation resulting in detection differs among worldwide locations* • United States – Leukocoria – 56% – Strabismus – 24% – Poor vision – 8% • Africa – Proptosis – 55% – Leukocoria – 38% – Strabismus – 6% – Buphthalmos – 2% • Sudan – Buphthalmos – 56% – Leukocoria – 32%

*Shields CL, et al. Retinoblastoma frontiers with intravenous, intra-arterial, periocular, and intravitreal chemotherapy. Eye. 2013;27:253-264. http://www.stephenoachs.com/

Retinoblastoma Retinoblastoma

• Most common malignant ocular tumor of • Survival rates vary among regions childhood • According to Shields et al, survival rates parallel economic development* • One of most common pediatric solid tumors – Africa 30% – Asia 60% • Typically diagnosed – Latin America 80% – Europe, Japan and USA 95-97% – 1st year of life in familial – Between 1 and 3 years old in sporadic *Shields CL, et al. Retinoblastoma frontiers with intravenous, intra-arterial, periocular, and intravitreal chemotherapy. Eye. 2013;27:253-264. Retinoblastoma Retinoblastoma

• Management is complex process • External Beam Radiotherapy (EBRT) – Accurate diagnosis Treatment option popular in 1980’s – Therapy based upon Stage of disease, laterality, tumor location and – size, associated vitreous or subretinal seeding, patient health and age – Improved “globe salvage” and vision preservation – Now avoided when possible due to later discovery of radiation- • Management has changed over past four decades, evolving related second cancers due to outcomes, late complications, new discoveries

.

Retinoblastoma Retinoblastoma

• Summary of management practices* • Systemic Intravenous Chemotherapy (IVC) – 1970’s Enucleation important for life prognosis – Introduced in the 1990’s as frontline treatment – 1980’s External Beam Radiotherapy (EBRT) became popular – “Chemoreduction” – 1990’s Systemic Intravenous Chemotherapy (IVC) began – Remains mainstay of treatment in developed countries, – 2000’s Intra-arterial Chemotherapy (IAC) being utilized especially for germline mutation cases – Utilizes agents: vincristine, etoposide, and carboplatin for 6-9 consecutive months – Efficacious in control of intraocular Rb as well as prevention of metastasis, pinealblastoma, and second cancers – Minimal systemic toxicities *Shields CL, et al. Retinoblastoma frontiers with intravenous, intra-arterial, periocular, – No ophthalmic toxicities. and intravitreal chemotherapy. Eye. 2013;27:253-264.

Retinoblastoma Retinoblastoma

• Intra-arterial Chemotherapy (IAC) • Enucleation – Newest treatment option with reports published 2011 – Main treatment in 1970’s – Delivers focal chemotherapy utilizing melphalan & – Remains critical treatment option for advanced unilateral cases occasionally carboplatin by catheterization of ophthalmic – Remains significant treatment option in Asia and Africa artery – Powerful treatment option for high risk retinoblastoma – Utilized less over the past two decades – Unilateral cases, initial or secondary therapy – Excellent intraocular tumor control – Few systemic complications – Concern over ocular toxicities due to vascular compromise of ophthalmic artery, retinal artery or choroidal vessels leading to poor visual outcome – Technique being modified in attempt to reduce disruption of . blood flow during procedure Retinoblastoma

• Periocular Chemotherapy – Utilized in conjunction with systemic chemotherapy – Carboplatin placed either subconjunctival or subtenon’s space – Later recurrences noted – Local complications include orbital or edema, orbital fat atrophy, muscle fibrosis with subsequent strabismus and optic atrophy.

Retinoblastoma Retinopathy of Prematurity • Vasoproliferative • Regression patterns include no visible remnant, calcified remnant, non-calcified remnant, partially calcified retinopathy of remnant and flat scar premature and low- birth-weight infants • Complex, multi- factorial disease • A leading cause of blindness in children worldwide

Retinoblastoma Retinopathy of Prematurity

• What hasn’t changed: – Close monitoring is critical • Estimated: – Hereditary Rb: patient and siblings examined q 4 months – ROP is cause of some visual loss in1300 children until 3 or 4 yo, then q 6 months until 6 yo born in US each year – Causes severe vision impairment in 250-300 of those children*

– *Basic and Clinical Science Course. Section 12. 2005-2006: 124. Retinopathy of Prematurity Retinopathy of Prematurity

• Classified according to the “International • Infants found to be at greatest risk: Classification of ROP” published 1984, revised – Birth weight ≤ 1500gm 2005 Gestational age of 30 weeks or less – – Describes disease: – Selected infants with birth weight 1500-2000gm or • Stages- 0 (Immature) through 5 (total RD) gest age > 30 weeks with unstable clinical course • Zones- I (posterior pole) through III temporal crescent • Extent – utilized in CRYO-ROP Study • Plus disease – vascular shunting signifies severe disease and essential in decision to treat

Retinopathy of Prematurity Retinopathy of Prematurity

• According to the Early Treatment for Retinopathy of Prematurity Study (ETROP)* – 68% of infants weighing less than 1251gm developed ROP of some degree • 44% ROP with BW 1000-1250gm • 76% ROP with BW 751-999gm • 93% ROP with BW 750gm or less

• *Basic and Clinical Science Course. Section 6. 2011-2012: 280-287.

Retinopathy of Prematurity Retinopathy of Prematurity Complications and sequelae: • ETROP study further classified in 2003: – Retinal detachment resulting in functional or complete • blindness – Type 1 ROP – Retinal folds • Zone I, any stage with plus disease – Dragging of the macula • Zone I, stage 3 without plus disease Myopia – both treated and untreated ROP – • Zone II, stage 2 or 3 with plus disease – Amblyopia Type 2 ROP – Strabismus – – Pseudostrabismus with large positive angle kappa • Zone I, stage 1 or 2 without plus disease – Late changes include late detachments in treated eyes, • Zone II, stage 3 without plus disease microphthalmia, cataract, glaucoma and Retinopathy of Prematurity Retinopathy of Prematurity

• Treatment guidelines based upon several multicenter • Early Treatment for Retinopathy of Prematurity trials (ETROP) Trial – Published results 2003 • Cryotherapy for Retinopathy of Prematurity (CRYO- – Revised the indications for treatment with cryo and ROP): efficacy of peripheral retinal cryotherapy in laser reducing unfavorable outcomes for Threshold ROP – – Recommended treatment of Pre-threshold Type 1 1988 ROP to reduce unfavorable outcomes even more

• Both cryo and laser induce regression of NV

Retinopathy of Prematurity Retinopathy of Prematurity

• Potential complications from laser treatment • Diode Laser Photocoagulation of immature peripheral retina utilizing same guidelines as CRYO-ROP found – Intense inflammatory response to be as efficacious as cryotherapy in treatment – outcome – Cataract – Glaucoma

Retinopathy of Prematurity Retinopathy of Prematurity • “Efficacy of Intravitreal Bevacizumab for Stage 3+ • Diode laser is presently the preferred treatment Retinopathy of Prematurity” (BEAT-ROP) modality between the two methods published in 2011 – Easier access to zone I and II – More retinal sparing, less traumatic – Less painful and faster recovery • Utilizes an Anti- Vascular Endothelial Growth – Appears to improve chances for better visual outcome Factor (VEGF) agent via intravitreal injection to treat ROP

• Off label use of Bevacizumab Retinopathy of Prematurity Retinopathy of Prematurity

• BEAT-ROP concluded:* • Intravitreal Bevacizumab vs laser photocoagulation – Benefit for Stage 3 with Plus disease in Zone I but – Additional considerations: not Zone II • Side effects of injection balanced against side effects of – Development of peripheral retinal vessels continued sedation, stress on neonate during long laser procedure after treatment compared to ablation following laser • Cost of injection vs laser, considering all resources Access to drug vs laser equipment in developing world – Admitted trial was too small to assess systemic safety • • Long term vision outcomes of patient’s treated with either modality (data to come) *Mintz-Hittner et al. Efficacy of Intravitreal Bevacizumab for Stage 3+ Retinopathy of Prematurity. New England Journal of Med. 2011; 364(7): 603-615.

Retinopathy of Prematurity

• BEAT-ROP refraction data at age two years – Spherical equivalent:* • Zone 1 – IVB: -2.56 ± 3.29 – CLT: -12.63 ± 6.91 • Zone 2 posterior – IVB: -0.69 ± 2.51 – CLT: -5.72 ± 6.40

(*79 patients, 154 eyes, data presented at AAPOS 2013 annual meeting) IVB= intravitreal bevacizumab CTL= conventional laser therapy

Retinopathy of Prematurity

• Intravitreal Bevacizumab – Unanswered questions: • Effect on organ development elsewhere in body • Function of vascularized retina beyond treated disease • Optimal dosage • Role of receptor specific drugs • Follow up – Recurrences of ROP post injection reported to occur much later than post-laser treatment – longer follow up required References 1. Holland K, Drolet B. Approach to the Patient with an Infantile Hemangioma. Dermatol Clin. 31 (2013): 289-301. 2. Lauren C, Garzon M. Treatment of Infantile Hemangiomas. Pediatric Annals. 2012; 41(8): 1-7. 3. Xue K. Deep Periocular Infantile Capillary Hemangiomas Responding to Topical Application of Timolol Maleate, 0.5%, Drops. JAMA Ophthalmology. 2013;131(9):1246-1248. 4. Vassallo P, Forte R, Di Mezza A, Magli A. Treatment of infantile capillary hemangioma of the eyelid with systemic propranolol. Am J Ophthalmol. 2013 Jan;155(1):165-170.e2. 5. Haider KM, Plager DA, Neely DE, Eikenberry J, Haggstrom A. Outpatient treatment of periocular infantile hemangiomas with oral propranolol. J AAPOS. 2010 Jun;14(3):251-6. 6. Guo S, Ni N. Topical treatment for capillary hemangioma of the eyelid using beta-blocker solution. Arch Ophthalmol. 2010 Feb;128(2):255-6. 7. Léauté-Labrèze C, Dumas de la Roque E, Hubiche T, Boralevi F, Thambo JB, Taïeb A. Propranolol for severe hemangiomas of infancy. N Engl J Med. 2008 Jun 12;358(24):2649-51. 8. Spiteri Cornish K, Reddy AR. The use of propranolol in the management of periocular capillary haemangioma--a systematic review. Eye (Lond). 2011 Oct;25(10):1277-83. 9. Basic and Clinical Science Course. Section 6. 2011-2012: 338-341. 10. Shields CL, et al. Retinoblastoma frontiers with intravenous, intra-arterial, periocular, and intravitreal chemotherapy. Eye. 2013;27:253-264.

References 11. Shields CL, et al. Intravenous and intra-arterial chemotherapy for retinoblastoma: what have we learned? Current Opinion in Ophthalmology. 2012; 23(3): 202-209. 12. Bracco S, et al. Intra-arterial chemotherapy with melphalan for intraocular retinoblastoma. Br J Ophthalmol. 2013; 97(9): 1219-1221. 13. Basic and Clinical Science Course. Section 6. 2011-2012:354-361. 14. Basic and Clinical Science Course. Section 12. 2005-2006:124. 15. Basic and Clinical Science Course. Section 6. 2011-2012: 280-287. 16. Mintz-Hittner et al. Efficacy of Intravitreal Bevacizumab for Stage 3+ Retinopathy of Prematurity. New England Journal of Med. 2011; 364(7): 603-615. 17. Shalev B, et al. Randomized Comparison of Diode Laser Photocoagulation Versus Cryotherapy for Threshold Retinopathy of Prematurity: Seven-year Outcome. Am J Ophthalmol. 2001; 132(1): 76-80. 18. Good W, et al. Revised Indications for the Treatment of Retinopathy of Prematurity. Arch Ophthalmol. 2003; 121:1684-1696. 19. Darlow B, et al. Are we there yet? Bevacizumab therapy for retinopathy of prematurity. Child Fetal Neonatal Ed. 2013; 98:F170-F174. 20. Policy Statement. Screening Examination of Premature Infants for Retinopathy of Prematurity. Pediatrics. 2013; 131(1):189-195. 21. Matinez Castellanos MA, et al. Short-term outcome after intravitreal ranibizumab injections for the treatment of retinopathy of prematurity. Br J Ophthalmol. 2013; 97: 816-819.

The Incidence of Scleral Lens Associated Infections in the Setting of Ocular Graft vs. Host Disease

Michelle Boyce, MD, Resident Class of 2016 Primary Supervisor: Miranda Bishara, MD

Graft versus host disease develops in 25-70% of bone marrow or stem cell transplant recipients. Chronic graft versus host disease (cGVHD) typically develops after 100 days and involves the eye in 60-90% of patients.1 Treatment of severe dry eye associated with cGVHD is fraught with perils for patients and physicians alike. These patients are particularly challenging because their disease is multifactorial involving tear deficiency as well as ocular surface inflammation. Classically, treatments included topical lubricants, topical steroids, topical immunomodulators, punctal occlusion, and autologous serum eye drops as well as systemic steroids and immunomodulators.2 Despite these therapies, patients with the most severe disease continue to experience symptoms of ocular pain, photophobia, decreased vision, non- healing corneal epithelial defects, and corneal perforation. For the most severely affected patients who are refractory to conventional therapies, surgical intervention in the form of tarsorrhaphy or amniotic membrane transplantation has been the next available treatment option. More recently, the use of scleral contact lenses has become an alternative to spare or prolong the need for surgical intervention.

The use of scleral lenses for the management of severe ocular surface disease was approved by the FDA in 1994 for the Boston Scleral lens (Prosthetic Replacement of Ocular Surface Ecosystem [PROSE] device). Studies have shown this lens to be useful for the treatment of the ocular complications of cGVHD.3 This lens requires custom-design and fitting that is only available at a small number of centers in the United States. As a result, commercially available scleral lenses are being utilized for the same purpose. Complications associated with the use of the commercially available scleral lenses have not been widely documented in the literature for this patient population in the United States.

The literature on the PROSE device has not shown a strong association with ocular infections. A cohort study of 33 patients using the PROSE lens in the treatment of chronic graft versus host disease over a period of 1 week to 2 years showed no reports of infectious .2 A study by Rosenthal et al. reported an incidence of infectious keratitis in 4 of 14 eyes using the PROSE lens for persistent epithelial defects.4 A second study of the PROSE lens for persistent corneal epithelial defect in 9 eyes reported 1 episode of in a patient with Stevens-Johnson Syndrome.5 Lastly, a study on the commercially available Jupiter lens for the management of cGVHD in 10 eyes over a period of 4 to 14 months reported no infectious complications.6

The Department of Ophthalmology at the University of Kansas Medical Center treats a small cohort of ocular GvHD patients that have severe enough disease to require the use of scleral lenses. In this small cohort, we have observed 1 case of infectious keratitis associated with the PROSE lens and 2 cases associated with commercially available scleral lenses. It is our goal to report the incidence of these infections in our experience as well as to describe any identifiable associated risk factors.

1. Anderson NG, Regillo C. Ocular Manifestations of Graft versus Host Disease. Curr Opin Ophthalmol 2004;15:503–507. 2. Jacobs DS, Rosenthal P. Boston Scleral Lens Prosthetic Device for Treatment of Severe dry eye in Chronic Graft-versus-host Disease. Cornea 2007;26:1195–1199. 3. Jacobs DS. Update on Scleral Lenses. Curr Opin Ophthalmol 2008;19:298–301. 4. Rosenthal P, Cotter JM, Baum J. Treatment of Persistent Corneal Epithelial Defect with Extended Wear of a Fluid-ventilated Gas-permeable Scleral . Am J Ophthalmol 2000;130:33–41. 5. Ling JD, Gire A, Pflugfelder. PROSE Therapy used to Minimize Corneal Trauma in Patient with Corneal Epithelial Defects. Am J Ophthalmol 2013;155(4):615-619. 6. Schornack M, Baratz KH, Patel SV, Maguire LJ. Jupiter Scleral Lenses in the Management of Chronic Graft versus Host Disease. Eye Contact Lens 2008;34:302-5. Outcomes of Treatment: Retinopathy of Prematurity and Bevacizumab

Mary Champion, MD, Resident, Class of 2015 Primary Supervisors: Timothy Lindquist, MD and Laura Plummer, MD

Retinopathy of prematurity (ROP) is a developmental disease that can lead to blindness. It is estimated that the incidence is 0.17% overall and 15.58%1 in premature infants in the United States. The treatment standard for ROP has been laser photocoagulation to stop neovascularization and to prevent retinal detachment. This treatment has limitations, including permanent reduction of the visual field and induction of high myopic refractive error.

Intravitreal bevacizumab has been studied as an alternative to laser for ROP and has many advantages, including sparing of retinal tissue, ability to be performed without the general anesthesia, and reduction of refractive error. The Bevacizumab Eliminates the Angiogenic Threat of Retinopathy of Prematurity (BEAT-ROP) study concluded that there was a benefit to using bevacizumab for Stage 3+ disease in Zone 1.2 Since the BEAT-ROP study, concerns have been raised about the delayed recurrence of ROP following bevacizumab,3-6 systemic escape and effect of the medication on fellow eyes,7,8 and delayed time to complete retinal vascularization4 resulting in a need for prolonged follow-up.

This is a retrospective chart review of all the patients with ROP treated with bevacizumab at one institution between 2011 and 2014. Our research objectives are to assess the outcomes of all the cases of ROP treated with bevacizumab at a single institution and to compare the results with previously reported data.

Most long-term follow-up data in the literature is from small series and retrospective chart reviews.9,10 Large clinical trials to evaluate for long term effects of bevacizumab for ROP are still lacking.

REFERENCES 1. Lad EM, Hernandez-Boussard T, Morton JM, Moshfeghi DM. Incidence of Retinopathy of Prematurity in the United States: 1997 Through 2005. American Journal of Ophthalmology. Sep 2009;148(3):451-458. 2. Mintz-Hittner HA, Kennedy KA, Chuang AZ, Group B-RC. Efficacy of Intravitreal Bevacizumab for Stage 3+ Retinopathy of Prematurity. The New England Journal of Medicine. Feb 17 2011;364(7):603-615. 3. Hu J, Blair MP, Shapiro MJ, Lichtenstein SJ, Galasso JM, Kapur R. Reactivation of Retinopathy of Prematurity After Bevacizumab Injection. Archives of Ophthalmology. Aug 2012;130(8):1000-1006. 4. de Klerk TA, Park DY, Biswas S. Prolonged Follow-up Period Following Intravitreal Bevacizumab Injection for Stage 3+ Retinopathy of Prematurity. Eye. Oct 2013;27(10):1218. 5. Ittiara S, Blair MP, Shapiro MJ, Lichtenstein SJ. Exudative Retinopathy and Detachment: A Late Reactivation of Retinopathy of Prematurity After Intravitreal bevacizumab. Journal of AAPOS: the official publication of the American Association for Pediatric Ophthalmology and Strabismus /American Association for Pediatric Ophthalmology and Strabismus. Jun 2013;17(3):323-325. 6. Moshfeghi DM, Berrocal AM. Retinopathy of Prematurity in the Time of Bevacizumab: Incorporating the BEAT-ROP Results Into Clinical Practice. Ophthalmology. Jul 2011;118(7):1227-1228. 7. Karaca C, Oner AO, Mirza E, Polat OA, Sahiner M. Bilateral Effect of Unilateral Bevacizumab Injection in Retinopathy of Prematurity. JAMA Ophthalmology. Aug 2013;131(8):1099-1101. 8. Sato T, Wada K, Arahori H, et al. Serum Concentrations of Bevacizumab (Avastin) and Vascular Endothelial Growth Factor in Infants with Retinopathy of Prematurity. American Journal of Ophthalmology. Feb 2012;153(2):327-333 e321. 9. Wu WC, Kuo HK, Yeh PT, Yang CM, Lai CC, Chen SN. An Updated Study of the use of Bevacizumab in the Treatment of Patients with Prethreshold Retinopathy of Prematurity in Taiwan. American Journal of Ophthalmology. Jan 2013;155(1):150-158 e151. 10. Martinez-Castellanos MA, Schwartz S, Hernandez-Rojas ML, et al. Long-term Effect of Antiangiogenic Therapy for retinopathy of Prematurity up to 5 Years of Follow-up. Retina. Feb 2013;33(2):329-338.

Retrospective Review of Interventional Thrombolysis for Central Retinal Artery Occlusion at the University of Kansas Medical Center

Lillian Yang, MD, Resident, Class of 2016 Primary Supervisors: Ajay Singh, MD and Thomas Whittaker, MD

Central retinal artery occlusion (CRAO) is an ophthalmological emergency with an incidence of

about 8.5 in 100,000 in the United States. CRAO presents as monocular vision loss, leaving most

affected patients with final visual acuity of counting fingers or worse. It is most commonly caused

by an embolic occlusion of the central retinal artery. Intra-arterial thrombolysis with tPA has been

postulated for use in acute CRAO. This treatment has been attempted in patients at the University

of Kansas Medical Center with varying results.

This presentation will discuss methodology and outcomes compared to the natural history of

central retinal artery occlusion.

Small Incision Lenticule Extraction (SMILE) for Simple Myopia

John Doane, MD, F.A.C.S.

Purpose: Present preliminary data from an FDA approved clinical trial to evaluate the safety and effectiveness of a femtosecond laser lenticule removal procedure for the reduction or elimination - - -10.25 D.

Methodsof spherical: In myopia this prospective from ≥ 1.0 multi D to-center ≤ 10.0 clinical D with trial, MRSE subjects ≤ 22 years and older are treated for spherical myopia and followed over a 12-month period. The investigative procedure uses the VisuMaxTM Femtosecond Laser to cut a refractive corneal lenticule, after which the lenticule is removed through a small side incision. Effectiveness, stability, and safety parameters are evaluated in accordance with the ANSI standard on Laser Systems for Corneal Reshaping. Preoperative and postoperative clinical assessments include manifest/cycloplegic refraction, UCVA and BSCVA, slit-lamp exam, fundus exam, topography, pachymetry, mesopic pupil size, WaveFrontTM analysis, mesopic contrast sensitivity, IOP, and patient satisfaction questionnaire.

Results: Results are available from 103 subjects with follow-up from Day 1 - 12-Month postoperatively. Mean age was 35 years, with 62 females/41 males. At 3, 6, and 9-month visits, 95% (91/96), 94% (74/79), and 98% (46/47) of eyes were within ± 0.50 D of intended and 91% (88/97), 95% (76/80), and 91% (43/47) had 20/20 or better UCVA. Mean preoperative MRSPH was -4.48 D ± 1.78 D; postoperative MRSPH at 3, 6, and 9 months was 0.01 D ± 0.25 D, 0.02 D ± 0.37 D, and 0.03 D ± -day visit. Operative events included 2 suction losses, 1 radial cap tear and 1 case of partial lenticule removal. Visual outcomes were excellent;0.20 D. subject No eyes satisfaction lost ≥ 2 lines is high. BSVCA beyond the 7

Conclusion: Results are promising from this study of a femtosecond laser refractive lenticule extraction procedure for the correction of myopia. Data from the first 103 eyes treated in the study shows a positive safety and effectiveness profile. FDA has granted approval to continue enrollment up to 360 subjects.

Trabectome Efficacy and Safety: Early Experience

Anita Campbell, MD, Resident, Class of 2014 Primary Supervisor: Michael Stiles, MD

Purpose: To evaluate safety and efficacy of Trabectome procedure by single surgeon.

Method: A total of 200 cases were included in the study. Patients without pre-operative IOP or who have less than 3 months of follow-up were excluded. All surgeries were performed by a single surgeon (MCS). Outcome measures include IOP, number of medications and secondary glaucoma surgery, if any. Kaplan- 21mmHg, IOP reduced by 20% or more from baseline on any two consecutive visits after 3 months and no secondaryMeier glaucoma was used surgery for survival analysis and success was defined as IOP ≤

Results: Mean age of the study group was 71 years old. Majority were Caucasians (82%) diagnosed with primary open angle glaucoma (68%). Average baseline IOP was 22.1±6.3 mmHg with 2.4±1.2 glaucoma medications. At 12 months, the IOP was reduced to 15.8±3.5mmHg (p<0.01) and number of medications was 1.7±1.0 (p<0.01). At 24 months, the average IOP was 15.7±3.5 mmHg (p<0.01) and average number of medications was 1.7±1.0 (p<0.01). Survival at 24 months was 77%. 23 cases (12%) required additional glaucoma surgery. One case of hypotony was noted on day one, but it was quickly resolved. For POAG cases (n=138), IOP was reduced from 21.1±5.3 to 17.3±4.2mmHg (p<0.01) at 24 months, while number of medications was reduced from 2.4±1.1 to 1.6±1.1. For pseudoexfoliative glaucoma cases (PEX, n=31), IOP was reduced from 23.1±6.3 to 15.3±2.9mmHg, while number of medications remained about the same (1.9±1.4 to 2.0±0.7 at 24 months). The survival rate at 24 months was 79% and 84% for POAG and PEX cases, respectively.

Conclusion: Patients showed statistically significant reduction in IOP and number of glaucoma medications. No serious complication was observed. Trabectome appears to be safe and effective for glaucoma patients.

International Volunteering for Ophthalmologists: Challenges Service Trips and Opportunities • Benefits – Able to reach patients not served by MOH/local infrastructure – Can augment the skills of local providers by offering subspecialty care • Challenges W. Abraham White, MD – If performing surgery, need to arrange for Clinical Assistant Professor, KU Eye follow up care (short term trips) University of Kansas Medical Center – Extent of your reach is limited to the duration of the trip

Overview Skills Transfer

• Types of • Benefits Opportunities – Form partnerships with local providers • Where to begin – Allows your work to continue beyond your • How to prepare departure • Pitfalls and pointers • Challenges – Need to have contacts “in-country” – Limited time and resources may influence your impact – Works best with teaching one or a few simple techniques

Types of Opportunities Choosing a Team

Disaster Relief • Working through an established organization is • highly encouraged • Service Trips – More resources, help with trip planning and organization – “Cataract Camps” – Can partner you with experienced leaders – Glaucoma Screenings – NGOs, Medical Societies, Church/Religious Organizations • Skills Transfer • Consider local, national, and international – Partnership with teaching institution opportunities – Working with local providers • Know the organization –History – Philosophy Learn the Culture Know What to Expect

• Invaluable for providing high • Same things you see here quality care in the U.S., just in greater • Local providers can be very helpful severity (HTN, DM, etc) • Careless attitude can hurt the • Learn endemic diseases credibility of the organization (malaria, etc) you are working with • Preserve the dignity of those • Talk with those who have you are there to serve been before • State Department Website - travel.state.gov • Expect the unexpected, • CDC website - immunizations be flexible

Prepare the Battlefield Notable Organizations

• Know what resources will be available before you go • ORBIS (equipment, medication, etc) • Plan to obtain needed supplies – Ophthalmology Specific well in advance – Pharmaceutical Companies – Long and short term opportunities – Equipment Reps • Sometimes it is beneficial to make a preparatory trip to the service • Mercy Ships site – More important with first trip or if – Primarily focused on cataract and some no one has been in a while – If it is your first trip to a new subspecialty work area, count on not having anything that you don’t bring with you – Short term opportunities – Remember that water and electricity are not in ready supply everywhere

Partner With Local Health Care Notable Organizations Team

• Skills transfer • If doing surgery, make • CMDA arrangements for follow up – Global Health Outreach care • Partnerships can extend the – Medical Education International reach of your mission beyond the confines of your trip • Local/Regional Eye Hospitals • Keep an open mind – HCP in developing world adapt to austere and hostile environments – Find creative ways to deal with adversity Parting Thoughts

• You won’t fix everyone – Resist the urge to practice outside your training – For patients, sometimes knowing nothing more can be done can be helpful • Starfish analogy

Soemmering’s Ring Subluxation with Encapsulated Lens Implant

John D. Hunkeler, M.D.

This is a report of experience in the diagnosis, surgical management and outcomes for a series of thirteen eyes with spontaneous subluxation of lens implant surrounded by Soemmering’s Ring. Soemmering’s Ring was first described in the early 19th century associated with trauma or extracapsular cataract surgery. More recently, several series of subluxation of Soemmering’s Ring with a well centered posterior chamber lens implant have been published. The observation of pseudo-phacodensis at the slit lamp is followed chronologically by migration of the capsule cortex and lens implant complex toward six o’clock. Once the downward subluxation is identified, surgical intervention is planned. The surgical approach is to elevate the implant, cortex and lens capsule complex into the anterior chamber under viscoelastic protection. A six millimeter posterior limbal dissected incision was previously prepared for intracapsular delivery of the lens complex. An appropriately powered and sized anterior chamber lens is inserted and rotated 45° allowing access to the superotemporal iris for vitrectomy instrument iridectomy. The incision is closed with 10-0 polypropylene sutures after adequate removal of the viscoelastic. Visual recovery to the pre subluxation acuity is expected and achieved. Transient usually spontaneously resolves. The most frequent concomitant pre-existing condition for subluxation of Soemmering’s Ring is pseudoexfoliation of the lens capsule.

May 9 & 10, 2014 KU Edwards Campus BEST Conference Center 12604 Quivira Overland Park, KS 66213

Sponsored by the University of Kansas Department of Ophthalmology and the Lemoine Alumni Society and in association with the Kansas Society of Eye Physicians and Surgeons (KSEPS)