April 29, 2022 Conference
AM Session 1: What Not to Miss in Ocular Inflammation (with Foster Lecture)
- To explore the range of non-autoimmune intraocular inflammatory disease with signs and symptoms similar to autoimmune uveitis
- To identify strategies for the accurate diagnosis of patients presenting with finding suggestive of uveitis
- To review medical legal issues around accurate diagnosis of patients presenting with finding suggestive of uveitis
In adults presenting with presumed ocular inflammation (uveitis) unresponsive to conventional treatment protocols, one must always consider the possibility of Vitreoretinal Lymphoma (VRL). VLR is a subset or variant of primary central nervous system lymphoma (PCNSL), a rare and often fatal malignancy. It is imperative to be able to recognize the clinical signs of VRL. We must be familiar with the diagnostic approach and the management options for these patients. Cytopathology and histopathology identification of malignant cells is crucial to making accurate diagnoses. Patients with confirmed PVRL have several treatment options, including local radiation and intravitreal chemotherapy with either methotrexate or rituximab. If confirmed concomitant CNS disease, systemic chemotherapy, radiotherapy, and stem cell transplant may be necessary to treat patients with overall good results.
Uveitis is characterized by intraocular inflammation involving the uveal tract. A classic feature of uveitis is the presence of inflammatory white blood cells in the anterior chamber and/or vitreous. Various conditions may appear to be uveitis at first but upon closer examination actually involve pigmented cells or particles in the anterior chamber and/or vitreous, as opposed to white blood cells, and thus cannot be categorized or treated as uveitis. This talk serves as a review of such masquerade conditions, to include hyphema/microhyphema, pigment dispersion syndrome, iris chafe, ghost cell glaucoma, bilateral acute iris transillumination (BAIT), Schwartz-Matsuo syndrome, retinal detachment, and vitreous hemorrhage.
Purpose: To review the common and rare ocular side effects of checkpoint inhibitors. To discuss the optimal management of these complications. Methods: Review of the literature Results: The checkpoint inhibitors are novel treatment modalities that have changed the prognosis and treatment paradigm of certain malignancies. Unfortunately, T-cell activation comes with some adverse reactions in the body, including the eye. Some of the ocular effects that have been previously reported include complications in the orbit, the cornea, the retina, the choroid and the optic nerve. Even though most of these are benign and self-limited, there are a few that, if left untreated, can be vision- threatening. Conclusions: The ophthalmologists should be prepared to promptly diagnose and manage the ocular side effects of checkpoint inhibitors. Co-management with the oncologist and cessation of the medication is sometimes indicated.
Robert Janigian Jr.
We all know and learn about endophthalmitis in its various forms but why do we sometimes misdiagnose it? One reason is endophthalmitis and immune mediated uveitis have overlapping features. Both have similar ocular findings but there are some key facets in the presentation of this vision, and potentially life-threatening condition, that we will review to help diagnose it promptly.
Uveitis is estimated to be the 5th or 6th leading cause of blindness in the U.S. Accurate and prompt diagnosis of uveitis is essential to the institution of early, appropriate therapy to avoid the structural ocular complications that cause visual loss in patients with uveitis. Uveitic masquerade syndromes (UMS) include ocular or systemic disease that manifest as infiltrating cells inside the eye that are not autoimmune or infectious in nature. UMS are typically categorized as neoplastic or non-neoplastic. The clinical similarities between UMS and autoimmune uveitis may result in a delay in diagnosis with severe implications for the patient’s vision and in cases of malignancy, health. The objective of this lecture is to review both neoplastic and non-neoplastic UMS to increase awareness of their prevalence and highlight their characteristics to assist in earlier diagnosis and improved outcomes.
Uveitis poses a risk for permanent loss of visual acuity and visual field through its attendant structural ocular complications such as glaucoma and macular edema. A delay in diagnosis or an inaccurate diagnosis may lead to poor clinical outcomes. Additionally, local and regional treatments of uveitis with corticosteroids may lead to ocular complications. Among patients with severe uveitis, use of systemic corticosteroids and immunosuppressive drug may be associated with systemic side effects. Therefore, uveitis treatment requires the ophthalmologist to be prompt in establishing the diagnosis and instituting appropriate therapy as well as determining appropriate follow up and monitoring to avoid complications of the disease and its treatment. The purpose of this talk is to review the medical legal concerns associated with the accurate diagnosis of patients presenting with uveitis, with a focus of diseases not to miss and appropriate monitoring for ocular and systemic side effects of treatment.
Paul A. Gaudio
Ocular ischemia exacerbates ocular inflammation. This talk includes a presentation of an illustrative case in which a patient’s ocular inflammation proved very recalcitrant and angiography demonstrated broad areas of retinal ischemia. Additional examples from the literature will be discussed briefly. The pathobiological mechanism for this exacerbation is not clear but appears to involved damage associated molecular patterns (DAMP). Practitioners do well to consider ischemia in cases of ocular inflammation that are poorly responsive to therapy.
Postsurgical uveitis (PSU), though variably defined, generally refers to patients for whom typical postoperative (cataract surgery primarily, but other intraocular surgery as well) inflammation persists beyond routine postoperative topical steroid taper. Inciting factors, such as complicated surgery, retained lens fragments, infection, mechanical (IOL-related), among others must be absent. The incidence of PSU ranges from 1.6 to 6.6% and it is the most frequent postoperative complication. Incidence increases with the addition of other intraocular procedures, such as endocyclophotocoagulation (24%). It tends to have a chronic course and virtually all patients have anterior uveitis only. Fellow eyes, after PSU associated with first eye surgery, are at increased risk for developing this complication. More than half of PSU patients will have problems with intraocular pressure and up to one-quarter develop cystoid macular edema (CME). The single most important risk factor for developing PSU is race, with patients of the African diaspora having up to 11-fold heightened risk, possibly because of intraocular melanin and oxidative stress. Regarding risk factors related to surgery, iris manipulation and pupil expansion also increase risk. Disruption of the blood-ocular barrier, from more invasive surgery, likely leads to exposure of occult ocular antigens and subsequent autoimmune inflammation, due to a lack of immunotolerance. Nearly half of patients will require only topical corticosteroid for management, while the remainder (~50%) may need systemic therapy. In general, despite the risk for ocular hypertension/glaucoma and CME, visual acuity outcomes are favorable. Several diagnoses must not be missed, when considering a diagnosis of PSU, including endophthalmitis (acute or chronic), toxic anterior segment syndrome (TASS), retained lens fragment, mechanical/lens-induced, and sympathetic ophthalmia.
While drug-induced uveitis is a rare cause of uveitis, occurring in less than 0.5% of cases in tertiary uveitis clinic, it is an important entity which should be recognized and treated promptly to avoid sight-threatening complications. Topical and systemic medications can be responsible for unilateral or bilateral, anterior, intermediate, posterior, or panuveitis. Typical symptoms of drug-induced uveitis include redness, photophobia, pain, and/or blurred vision. In order to establish causality, several criteria should be met (not all must be fulfilled: - Other potential etiologies have been excluded - Reaction must be have been previously documented - Symptoms resolve with discontinuation of drug - Symptoms worsen when drug is increased or with re-challenge Most common offenders of drug-induced uveitis are: - Bisphosphonates - Rifabutin - Cidofovir - Sulfonamides - Fluoroquinolones - Immune checkpoint Inhibitors - Topicals: Moxifloxacin Prostaglandin analogues - Brimonidine Typically, drug-induced uveitis is responsive to steroids and the offending drug may not need to be stopped, particularly if it is life-saving medication. Drug-induced tubulointerstitial uveitis and nephritis (TINU) syndrome is an important distinct entity that may occur after antibiotic or NSAID use that may be associated with laboratory abnormalities including kidney function abnormalities and elevated urine b2 microglobulin. The uveitis may precede renal dysfunction and require steroid-sparing immunosuppression for inflammatory control.
Janine R. Eagle
Masquerade syndromes in children are often elusive and devastating, and thus warrant serious consideration when evaluating any child with atypical ocular inflammation. A detailed pediatric eye exam is of paramount importance, but can be inherently challenging, so EUA may be indicated and ancillary testing (such as B-scan, UBM, OCT and ERG) can play an important role in making the correct diagnosis and management decisions. We will review the differential diagnoses including malignancies like retinoblastoma, leukemia, lymphoma and medulloepithelioma; and other more benign entities such as juvenile xanthogranuloma, occult intraocular foreign body, and retinal conditions. This talk will highlight the importance of practicing with a questioning attitude, and looking out for clinical clues and red flags on history, exam and imaging to aid in making a more targeted and timely diagnosis that can be vision and life saving for the child.
AM Session 2: Hot Topics in Oculoplastics
- Identify new medicines that have entered the field.
- Review new surgical techniques in the field.
- Recognize newer patient interfaces (i.e., social media).
The treatment of pediatric lymphatic-venous malformations and plexiform neurofibromas has historically been with surgical intervention. Sirolimus has recently been shown to be effective as a non-surgical treatment option for pediatric lymphatic-venous malformations. Selumetinib has recently been shown to be effective as a non-surgical treatment option for pediatric plexiform neurofibromas.
Cases were selected to describe patient outcomes after treat with targeted and immunotherapy for periocular disease. Targeted therapy can be used in select cases of periocular malignancy when specific gene mutations are present. Immunotherapy can be used to treat periocular and orbital malignancy with high tumor mutational burden. Both targeted therapy and immunotherapy can be globe sparing options to treat periocular and orbital malignancy. Treatment with targeted or immunotherapy should be considered for the treatment of periocular and orbital malignancy; it is important to consider these agents especially when orbital exenteration is the alternative.
Suzanne K. Freitag
Thyroid eye disease (TED) occurs in approximately 30-50% of individuals with Graves’ disease. TED can result in disfigurement, discomfort, decreased quality of life, double vision and in about 5% of cases visual loss from compressive optic neuropathy. Traditionally patients with mild disease were treated with comfort measures and those with more severe disease with any of the following or combinations of corticosteroid, biologics such as rituximab, external beam radiation therapy or surgery. In January 2020, the US FDA approved teprotumumab broadly for the treatment of moderate to severe TED. Teprotumumab is a fully human monoclonal antibody that blocks the insulin-like growth factor-1 receptor. It is delivered as an intravenous infusion and the currently recommended dosing regimen is every 3 weeks for a total of 8 doses. Logistical issues include the very high cost of the drug requiring insurance approval, as well as arrangement of infusion center or home infusions. The drug is contraindicated in pregnancy, breastfeeding and in children under the age of 18 years. Care must be used in prescribing to patients with inflammatory bowel disease, hearing loss and diabetes mellitus. Phase 2 and 3 clinical trial results were published in the New England Journal of Medicine in 2017 and 2020 respectively. The drug was effective versus placebo in reducing proptosis, improving double vision, decreasing clinical activity score, and improving quality of life, among other study endpoints. In 2021, the OPTIC-X extension trial data was published, showing that patients with longer periods of time from disease onset to treatment still had successful results. Medical literature now demonstrates efficacy of teprotumumab in cases of chronic TED and in patients with compressive optic neuropathy, which were not situations included in the clinical trials. Adverse reactions include muscle spasms, fatigue, nausea, dysgeusia, diarrhea, amenorrhea, body hair loss, hyperglycemia, and hearing changes. Case presentations will be shown.
Neurotrophic keratopathy is a devastating corneal condition that can lead to ocular morbidity and blindness. Current medical and surgical treatments poorly tackle the essential problem of corneal aesthesia and therefore fail to provide a permanent cure. Recent advances in corneal neurotization techniques have shown promise to restore corneal nerves in neurotrophic keratopathy. This talk aims at reviewing the current surgical advances, along with the current thoughts and evidence available for corneal nerve regeneration.
Social media has become an integral part of building a successful plastic surgery practice. I will discuss the key aspects of creating a business profile, content and growth strategies.
Dermal filler injections are an increasingly common procedure in aesthetic medicine. Inadvertent injection of fillers into facial arteries can result in serious complications such as retinal artery occlusions and skin necrosis. The location of major blood vessels can vary significantly between individual patients. This talk will demonstrate the use of ultrasound to visualize individual patient vascular anatomy prior to injection to minimize the risk of injection complications.
Surgical reconstruction of the orbit has experienced substantial advances since the earliest days of autologous bone grafts for fracture repair or bony replacement. Currently, there are several methods to model orbital abnormalities to plan and perform surgery, as well as to create patient specific implants. These technologies employ varying methods of planning, materials, and intraoperative approaches. The indications, advantages, disadvantages, and options for these surgical implants will be reviewed and discussed.
PM Session 1: Ancillary Testing in Neuro ophthalmology: Innovations and Pitfalls
- To examine how specific ancillary tests may be utilized for the diagnostic work-up in neuro-ophthalmic diseases.
- To become familiar with common misinterpretations in optical coherence tomography (OCT) and to recognize the advantages and limitations of OCT as a tool to manage neuro-ophthalmic diseases.
- To gain exposure to innovative developments in ancillary testing in neuro-ophthalmology Improving the outcome in diagnostic testing.
Visual field testing is often a crucial piece of the neuro-ophthalmologic evaluation. Widely implemented technologies offer automation and standardization, with limitations. Neuro-ophthalmology frequently borrows technologies that where primarily developed and fine-tuned for other purposes. Developing technological advances may provide a better fit for specific neuro-ophthalmologic applications and greater assistance in the diagnosis of neuro-ophthalmic diseases.
This focused review will illustrate VEP techniques, challenges, and testing types. It will then provide examples of the diagnostic use of VEP testing. It will conclude by discussing newer, specialized VEP techniques such as sweep VEP and multifocal VEP.
A diverse set of neuro-ophthalmic disorders arise from pathogenic variants in autosomal and mitochondrial DNA. The approach to genetic testing for these disorders will be discussed including when to obtain genetic testing, what options are available for genetic testing, and how to interpret genetic results including variants of uncertain significance.
Optical coherence tomography (OCT) has become an important tool for diagnosing optic nerve and retinal disease. The structural details and reproducibility of OCT continues to improve with further advances in technology. However, artifacts and misinterpretation of OCT can lead to clinical misdiagnosis of diseases if they go unrecognized. I will present cases of important applications of OCT for the diagnosis of retinal and optic nerve disorders and describe the most common artifacts and errors in interpretation encountered on OCT reports. I will provide examples of the artifacts, discuss the causes, and provide methods of detecting them. In addition, I will present a systematic approach to OCT analysis and report interpretation to facilitate the recognition of artifacts and to avoid clinical misinterpretation. While OCT is invaluable in diagnosing retinal and optic nerve disease, we need to be cognizant of the artifacts that can occur with OCT. Failure to recognize some of these artifacts can lead to misdiagnoses and inappropriate investigations.
The differentiation of pseudopapilledema or optic nerve head drusen and papilledema can be very challenging. There are multipleancillary imaging modalities that can be employed to confirm the diagnosis of pseudopapilledema. They are often used in concert. These imaging modalities include fundus photography, fundus autofluoresence, B-scan ultrasonography, optical coherence tomography and fluorescein angiography. The goal of this talk is to review these different modalities, the strengths and weaknesses of each and to explain how they can all be used to help determine a final diagnosis.
Pupil diameter and pupillary movement under different lighting conditions represent important parameters used for clinical assessment of patients. The pupil is clinically important because it is an objective indicator of afferent light input and can be used to assess the integrity of the retina and optic nerve. The pupil is also a sensitive indicator of the innervational status of the iris muscles. The presence of pupil inequality, or anisocoria, is a reflection of autonomic nerve output to each iris and ability of the iris sphincter and dilator muscles to respond changes in lighting conditions. This presentation will use a case-based approach to the evaluation and management of pupillary inequality.
Obtaining a detailed history and a careful stepwise approach to the examination are crucial. By answering the following questions, clinicians should be able to narrow down the potential cause(s) of a patient’s anisocoria and then confirm with pharmacologic testing when indicated:
- Is the anisocoria greater in dim light or bright light?
- Is the pupillary reaction to light normal in each eye?
- Is there a light–near dissociation?
- Is there ptosis or abnormal ocular motility?
- Are iris abnormalities such as transillumination defects or segmental sphincter palsies noted at the slit lamp?
Pediatric Horner syndrome which presents as a triad of ptosis, miosis of the affected pupil, and anhidrosis is a common cause for referral to pediatric ophthalmology and neuro-ophthalmology. In children, Horner syndrome may be associated with life threatening disease, and therefore a standardized protocol for evaluation is needed. Currently, consensus guidelines regarding which imaging modalities to pursue and the value of urine catecholamine testing have not been developed. The purpose of this talk is to describe a single center, standardized protocol for the diagnostic work up of pediatric Horner syndrome in an effort to provide recommendations to practitioners and families for best practices for the evaluation of these patients.
The two main neuroimaging modalities used in evaluating neuro-ophthalmologic conditions are computed tomography (CT) and magnetic resonance imaging (MRI). Specific protocols and appropriate locations of the imaging must be considered in order to obtain useful information. CT is preferred when there are patient-related situations such as claustrophobia, embedded metallic foreign bodies, or the presence of non-MRI compatible medical devices. Because of the imaging quality, MRI is often preferred for most clinical indications. However, CT is better for evaluating orbital diseases involving bony structures and the sinuses. CT and MRI can be comparable in evaluating thyroid eye disease and orbital tumors. CTA or MRA preference is often institute dependent. By knowing which imaging protocol is best for each suspected condition, we will be able to avoid common pitfalls and ensure timely diagnosis and treatment.
PM Session 2: Procedure Selection in Subspecialty Glaucoma
- Describe glaucoma angle procedures, including indications and technique.
- Identify new perspectives on traditional glaucoma filtration, glaucoma drainage device, and cyclodestructive procedures.
- Review approaches to procedure selection when choosing between glaucoma surgical techniques to employ in specific clinical situation.
Peter T. Zacharia
The glaucoma surgery session will be divided into 3 sections. The first section will contain lectures discussing angle stenting or goniotomy based procedures. The second section will discuss external filtration procedures and devices. The third section will discuss other topics in glaucoma surgery. Prior to each of the first two sections, the moderator will present clinical cases and poll the audience regarding surgical management for each case. Each case will be discussed further including surgical procedure undertaken and outcome during each speaker's talk.
Canal-based stents have been available in the United States for almost 10 years as a surgical option for glaucoma to be performed in conjunction with cataract surgery. Their use has increased significantly over the past decade, and studies have repeatedly demonstrated their efficacy in comparison to cataract surgery alone. The efficacy and safety of these procedures will be discussed, as well as keys for patient selection and comparison to other available angle-based procedures.
David Solá-Del Valle
KDB and ECP are two microinvasive glaucoma surgery plus (MIGS Plus) methods that have been shown to effectively lower intraocular eye pressure (IOP) and reduce medication burden.? ECP can be used as a stand-alone procedure or in combination with phacoemulsification (phaco-ECP). IOP reduction reports range from 2.7-11.5 mmHg with absolute reductions between 14.9%-46.9% based on patients who underwent ECP or phaco-ECP. It has also been shown to reduce glaucoma medication use by 0.4–1.7 based on patients who underwent ECP or phaco-ECP. KDB can also be used as a stand-alone procedure or in combination with phacoemulsification (phaco-KDB). IOP reduction reports range from 2.5-12.7 mmHg with absolute reductions between 14.5% and 36.0% based on patients who underwent KDB or phaco-KDB.? It has also been shown to reduce glaucoma medication use by 0–2.1 based on patients who underwent KDB or phaco-KDB. When KDB is combined with Phaco-ECP (PEcK), this procedure reduces aqueous humor (AH) production and increases AH outflow. It is an example of combination MIGS (cMIGS), which attempts to maximize the IOP-lowering effects of individual MIGS with phacoemulsification. Two studies have looked at the efficacy of the PEcK procedure showing it can reduce IOP by about 5 mmHg, and glaucoma medication use by 1.2 to 1.6. A case will be presented that displays the effects of the PEcK procedure.  Literature presented on KDB, ECP, and cMIGS should be interpreted carefully since much of the current literature is retrospective, nonrandomized, and unmasked. A paper from 2019 showed that only 45.6% of MIGS research studies adhered to the strict World Glaucoma Association (WGA) guidelines that were proposed to help standardize the reporting of glaucoma clinical studies.
The rise of microinvasive glaucoma surgery has triggered renewed interest in aqueous outflow pathophysiology. Previous studies primarily focused on structural imaging of aqueous outflow anatomy, but new technologies allow investigation of functional dynamics of the angle and downstream pathways. Lessons learned from these imaging techniques may help guide surgeons in making pre-operative and intra-operative decisions to optimize surgical outcomes.
Trabeculectomy remains the gold standard in glaucoma surgery. It is the only surgical intervention that can be titrated in the post-operative period, and it has the most longitudinal data. Several studies will be reviewed that evaluate how trabeculectomy performs compared to other glaucoma surgeries. The Tube Versus Trab (TVT) and Primary Tube Versus Trab (PTVT) studies were randomized trials comparing trabeculectomy to Baerveldt tube shunts. The latter study, in which participants had no prior incisional surgery, showed that the trabeculectomy group had lower intraocular pressure (IOP) and fewer glaucoma medications at three years, but there was no statistically significant difference in failure between the two surgeries. Another randomized trial compared trabeculectomy to Ahmed tube shunts, and it found no significant difference in IOP, glaucoma medications, success rate, or complications. There are no prospective, randomized studies comparing trabeculectomy to Xen, though retrospective studies have generally shown that trabeculectomy achieves lower IOP with fewer medications, and Xen has fewer complications. Overall, the choice of surgery type should likely be individualized and based on many factors, including glaucoma severity, target IOP, patient and surgeon risk tolerance, and surgeon experience.
POAG is one of the leading causes of irreversible blindness. Elevated IOP is considered the only modifiable risk factor as of today. Mainstay of treatment is IOP reduction, which is typically achieved with topical or oral drugs, laser treatments and surgery. First line treatment typically involves- topical eyedrops- with its side effects, local as well as systemic. Laser treatment has been proven to reduce IOP but effect may be short-lived and has to be repeated with waning effect. Traditional surgeries like trabeculectomies and tube shunts have proven to reduce IOP but are riddled with long term significant complications including infections, hemorrhage and hypotony. In recent years we have seen advances in MIGS procedures (Minimally Invasive Glaucoma Surgeries), now an essential part of treatment armamentarium. Aim of MIGS is to decrease IOP with reduced risk of complications as well as rapid recovery. Three main types of MIGS procedures are: 1. Trabecular- usually combined with cataract surgery with modest IOP lowering 2. Supra-choroidal- none in market now, awaiting ongoing trials 3. Subconjunctival- XEN gel stent now widely available since its FDA approval in 2016 The device of interest that we are discussing is Xen Gel Stent( Allergan, an Abbvie company, Irvine, CA, USA). it is a 6 mm long hydrophilic tube with an inetrnal diameter of 45 microns that creates a channel through the sclera, allowing for aqueous humor from the anteior chamber to pass into the subconjunctival space. this new outflow route, lowers the IOP. We are going to disucs the Indications of Xen Gel Stent, various techniques, efficacy, complications and need for addiitonal procedures.
Although less common than POAG, PACG is more likely to cause blindness. Twenty five percent of PACG patients progress to blindness. 5.3 million people will be blinded by PACG in 2040. The goal of PACG treatment is to protect the optic nerve from damage by lowering eye pressure via restoration of the angle anatomy and prevention of PAS formation. Cataract surgery has been shown to widen the angle and lower intraocular pressure. Current evidence supports cataract surgery and clear lens extraction as effective treatments for PAC/PACG.
Even in experienced hands 15 to 20% of all primary trabeculectomies will sooner or later fail due to scarring of the scleral flap. As surgeons we can move to another site for a second drainage procedure or attempt to restore aqueous drainage from the original sclerostomy and reform the bleb. The latter strategy is often appropriate to spare conjunctiva for future needs and to avoid the risks of an implanted device. In this talk we will review the pros and cons of various approaches including bleb needling and open bleb revision and a description of my preferred technique.
M. Lisa McHam
Today, both comprehensive ophthalmologists and glaucoma specialists are caring for older and sicker patients than ever before. Many patients have been exposed to topical therapy for decades and many have had prior ocular surgeries. When glaucoma becomes uncontrolled despite conventional medical therapy, laser trabeculoplasty, and cataract MIGs procedures, many of our patients have risk factors for poor outcomes with traditional glaucoma surgeries . Over the past few years, there has been renewed interest in the use of cyclophotocoagulation in managing different types of patients. We will review current the current state of knowledge in this area.