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Purpose: To study patient follow-up after they engage in a teleretinal screening program and to understand potential barriers to care. Methods: This was a retrospective analysis and a prospective study of telephone-based patient interviews of outpatients screened for diabetic retinopathy (DR) through a teleretinal referral system. Results: Of 2761 patients screened through a teleretinal referral program, 123 (4.5%) had moderate nonproliferative DR (NPDR), 83 (3.0%) had severe NPDR, and 31 (1.1%) had proliferative DR. Of the 114 patients with severe NPDR or worse, 67 (58.8%) saw an ophthalmologist within 3 months of referral. Eighty percent of interviewed patients reported they were not aware of the need for follow-up eye appointments. Conclusions: Of patients with severe retinopathy or worse, 58.8% presented for in-person evaluation and treatment within 3 months of screening. Although this result was negatively affected by factors related to the COVID-19 pandemic, key elements of patient education and improved referral strategies to facilitate in-person treatment are essential to improving follow-up after patients engage in telescreening.

Belantamab mafodotin (belamaf) demonstrated deep and durable responses in patients with heavily pretreated relapsed or refractory multiple myeloma (RRMM) in DREAMM-2 (NCT03525678). Corneal events, specifically keratopathy (including superficial punctate keratopathy and/or microcyst-like epithelial changes (MECs), eye examination findings with/without symptoms), were common, consistent with reports from other antibody–drug conjugates. Given the novel nature of corneal events in RRMM management, guidelines are required for their prompt identification and appropriate management. Eye examination findings from DREAMM-2 and insights from hematology/oncology investigators and ophthalmologists, including corneal specialists, were collated and used to develop corneal event management guidelines. The following recommendations were formulated: close collaboration among hematologist/oncologists and eye care professionals is needed, in part, to provide optimal care in relation to the belamaf benefit–risk profile. Patients receiving belamaf should undergo eye examinations before and during every treatment cycle and promptly upon worsening of symptoms. Severity of corneal events should be determined based on corneal examination findings and changes in best-corrected visual acuity. Treatment decisions, including dose modifications, should be based on the most severe finding present. These guidelines are recommended for the assessment and management of belamaf-associated ocular events to help mitigate ocular risk and enable patients to continue to experience a clinical benefit with belamaf.

Fuchs endothelial corneal dystrophy (FECD) is the most common posterior corneal dystrophy and the leading indication for corneal transplantation in the United States. FECD is slowly progressive, and patients develop gradual corneal endothelial decompensation, eventually resulting in failure of the endothelium to maintain corneal deturgescence. Medical management consists of topical hyperosmotic agents to facilitate dehydration of the cornea, but surgical intervention is often required to regain corneal clarity. The surgical management of FECD has evolved over the past two decades as corneal transplantation techniques have allowed for more selective keratoplasty and replacement of only the diseased layers of the cornea. Prior surgical management consisted of penetrating keratoplasty (PK) that carried significant intraoperative risks associated with “open sky” as well as postoperative risks of graft rejection, wound dehiscence, postoperative astigmatism, and prolonged visual rehabilitation. In the past 15 years, endothelial keratoplasty (EK) has become the treatment of choice for endothelial disease, significantly reducing the risks associated with the surgical treatment of FECD. Here we discuss the current surgical management of FECD, including the introduction of Descemet stripping only (DSO), and highlight future investigative efforts.

PURPOSE To quantitatively analyze the angle anatomy in eyes with a Boston type 1 keratoprosthesis (KPro) using anterior segment optical coherence tomography (AS-OCT) and to assess the diagnostic ability of AS-OCT in KPro-associated glaucoma. METHODS AS-OCT (RTVue) images from KPro eyes with and without glaucoma were reviewed. The angle opening distance at 500 μm from the scleral spur (AOD500), trabecular-iris angle at 500 μm from the scleral spur (TIA500), and trabecular-iris surface area at 500 μm from the scleral spur (TISA500) were measured by 2 observers masked to the diagnosis. The measurements for each visible quadrant were compared between KPro eyes with and without glaucoma. RESULTS Twenty-two eyes with glaucoma and 17 eyes without glaucoma from 39 patients with KPro were included. Of the 4 quadrants imaged, the temporal angle was the most visible (79.5%) and angle measurements of the temporal quadrant were the only ones that differentiated the 2 groups: the mean AOD500, TIA500, and TISA500 were significantly lower in KPro eyes with glaucoma than without glaucoma (388.2 ± 234.4 μm vs. 624.5 ± 310.5 μm, P = 0.02; 26.1 ± 14.0 degrees vs. 39.1 ± 17.1 degrees, P = 0.03; and 0.15 ± 0.09 mm vs. 0.23 ± 0.12 mm, P = 0.03; respectively). The highest area under the receiver operating characteristic curve for detecting glaucoma was 0.75 for temporal TIA500 (95% confidence interval 0.57-0.94, P = 0.02) with 50% specificity at 80% of sensitivity and a cutoff value of 37 degrees. CONCLUSIONS The temporal angle was the most visible on AS-OCT in eyes with a KPro. Significant narrowing of the temporal angle detected on AS-OCT was associated with glaucoma in these eyes.

Purpose To assess outcomes and glaucoma management in eyes with aniridia following Boston type 1 Keratoprosthesis (KPro) implantation. Design Retrospective, interventional comparative case series. Methods The population included patients with aniridia and patients with other preoperative diagnoses (excluding Stevens-Johnson syndrome, mucous membrane pemphigoid, and congenital disorders) who underwent KPro implantation at Massachusetts Eye and Ear with at least 2 years of follow-up. One eye per patient was selected based on the longer follow-up time. The main outcome was intermediate and long-term outcomes related to glaucoma. Results The aniridia (n = 22) and comparison (n = 61) groups had similar preoperative visual acuity (VA, mean ± standard deviation, 1.86 ± 0.52 logMAR, P = .33) and follow-up time (65.6 ± 26.3 months, P = .25). Before KPro implantation, eyes with aniridia had more glaucoma (76.2%) and glaucoma surgery (57.1%) than comparison eyes (51.8%, P = .053; 23.2%, P = .005, respectively). More Ahmed valves were co-implanted with KPro in aniridia (47.6%) vs comparison eyes (17.9%, P = .008). At final follow-up, more aniridia eyes had glaucoma (90.5%) than comparison eyes (64.3%, P = .02), but the 2 groups had similar percentages of eyes with cup-to-disc ratio (CDR) >0.8 (23.8% vs. 30.4%, P = .57) or CDR progression of ≥0.2 (42.9% vs 44.6%, P = .89, respectively). None of the eyes with prophylactic tube implantation developed glaucoma. Eyes with and without aniridia did not differ in post-KPro VA improvement (72.7%, 72.1%, P = .96), and final VA (1.28 ± 0.79 logMAR, 1.23 ± 0.98 logMAR, P = .51). Conclusion Despite a higher glaucoma prevalence, eyes with aniridia achieved similar VA as comparison eyes with more than 5 years of mean follow-up time. Boston KPro offers satisfactory visual rehabilitation in aniridia when glaucoma is managed aggressively.

This article reviews our current understanding of the regenerative capacity of the corneal endothelium and critically evaluates new and important research in the treatment of endothelial dysfunction. The development and application of new surgical (Descemet’s membrane stripping only), pharmacologic (rho kinase inhibitors, such as ripasudil), and injectable cell culture modalities for the treatment of endothelial dysfunction have sparked debate in the cornea world and may revolutionize future clinical practice. The exact mechanisms involved in corneal endothelial regeneration remain elusive. The latest treatment strategies for endothelial dysfunction have the potential to transform our previous perceptions of endothelial physiology by eliciting new and exciting research questions for future investigations.

Fuchs endothelial corneal dystrophy (FECD) is the most common indication for corneal transplantation in the United States, accounting 36% of the almost 47,000 transplants performed in 2016. Although the surgical management of FECD has undergone a revolution over the past 20 years, its pathogenesis remains elusive, with multiple putative disease pathways and an ever increasing number of candidate genes thought to play a role. This review will summarize the recent advancements in our understanding of the biology of FECD, including potential parallels with neurodegenerative disease like amyotrophic lateral sclerosis and will highlight prospects for future treatment advances.

Descemet stripping only (DSO) has become a viable treatment option for certain cases of Fuchs Endothelial Corneal Dystrophy (FECD). This article describes the history of the development of DSO, its current status, and what successful DSO tells us about the pathophysiology of FECD. Remaining questions and future directions are also highlighted.

The current understanding on the clinical efficacy of Rho-associated protein kinase (ROCK) inhibitor for treating Fuchs endothelial corneal dystrophy is summarized to clarify whether the “off-label” ROCK-inhibitor eye-drop application are appropriate. ROCK-inhibitor eye drops may eventually be deemed a cutting-edge therapy for Fuchs endothelial corneal dystrophy patients with acute corneal endothelial defect.

Corneal blindness is the third leading cause of blindness worldwide.1 Corneal transplantation remains the only method of restoring vision in eyes with end-stage corneal decompensation and is the most frequently performed type of transplant worldwide.2 Due to the immunological and lymphogenic privilege of the avascular cornea, keratoplasty is the most successful tissue transplantation procedure currently performed in humans.3 However, as with most solid-organ transplants, the risk of transmitting infectious diseases and viruses is an important aspect of corneal transplantation that can affect recipients or those handling donor tissue.4 Thus, the rapid emergence of a novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) since December 2019 has led to a devastating impact on corneal transplantation worldwide. To date, the SARS-CoV-2 has infected close to 13 million people and led to more than 560000 deaths worldwide in a short space of 6 months (WHO COVID-2019 situation report as of July 13, 2020). Since its emergence in Wuhan China, the SARS-CoV-2 has been reported to cause conjunctivitis and detected in tears or conjunctival secretions.5 The potential for SARS-CoV-2 to affect the ocular surface had immediate implications for eye banking around the world. Furthermore, travel restrictions and curtailed elective procedures have led to the reduction in the supply, retrieval and demand for corneal transplantation.6 Thus, the future of corneal transplantation faces an unprecedented threat, as access to this sight-restoring surgery is affected on a global scale.7 Beyond the pandemic, COVID-19 may have lasting effects on corneal transplantation, as healthcare services resume to face long waiting lists and a potential shortage of donors. Therefore, there is an urgent need for a consensus on recommendations for restoring corneal transplantation services, even as the outbreak evolves and we learn more about the SARS-CoV-2. In order to do this, there are several …

Purpose This article evaluates whether a targeted, stepwise curriculum for microbypass stent placement leads to successful insertion of the iStent (Glaukos, Laguna Hills, CA) by resident surgeons. Participants Senior (postgraduate year 4) ophthalmology residents (9 residents) from three classes during the 2015 to 2016, 2016 to 2017, and 2017 to 2018 academic years. Design Residents participated in a three-stage surgical curriculum. First, a wet laboratory was held for residents to gain familiarity with the device and develop bimanual surgical proficiency. The wet laboratory involved several stations, with each station requiring increased dexterity for successful completion. Next, residents practiced bimanual intraoperative gonioscopy after routine phacoemulsification procedures. Finally, residents performed combined phacoemulsification and iStent insertion under the supervision of an experienced attending surgeon. Primary success was determined by correct anatomical placement of the device confirmed via subsequent intraoperative gonioscopy by the supervising surgeon. Secondary success was measured by change in intraocular pressure (IOP) and number of topical hypotensive medications used after surgery. Residents provided feedback about the effectiveness of the curriculum via an online survey. Results There were a total of 43 cases. There were no significant intraoperative complications, including hyphema. The iStent location was confirmed during both intraoperative and postoperative gonioscopy and was noted to be in appropriate position for the duration of the follow-up period (6–12 months) for all patients. Average preoperative IOP in our subjects was 17.5 ± 4.0 mm Hg and mean number of preoperative medications was 1.4 ± 1.0. At 6 months' postoperative, average IOP was 14.5 ± 2.2 mm Hg (13% reduction from baseline [±15%]). At 12 months' postoperative, average IOP was 14.4 ± 3.5 mm Hg, with a reduction of 14% (±18%). The mean number of medications at follow-up was 1.2 ± 1.1. Mean postoperative IOP at 6 and 12 months was significantly lower compared with baseline (p = 0.0002, p = 0.0001). Residents agreed that the curriculum prepared them to perform iStent insertion during residency and most residents felt prepared after residency. Conclusion This stepwise surgical curriculum for trabecular microbypass stent placement leads to successful performance of the procedure by residents and is a useful model for other residency programs.

Purpose To quantitatively evaluate the angle anatomy in eyes with the Boston type I keratoprosthesis (B-KPro) differing in the back plate (BP) material and size using anterior segment optical coherence tomography. Methods B-KPro eyes with poly(methyl methacrylate) (PMMA) (7.0 and 8.5 mm) and titanium (7.0, 8.5, and 9.5 mm) BPs were imaged with anterior segment optical coherence tomography. The angle opening distance at 500 μm from the scleral spur (AOD500), trabecular iris surface area at 500 μm from the scleral spur (TISA500), and trabecular iris angle at 500 μm from the scleral spur (TIA500) were measured. Among the visible quadrants, the average, the temporal, the widest, and the narrowest angle of each eye were included in the analysis. Average time between B-KPro implantation and imaging was 7.5 ± 1.4 years for a PMMA BP and 2.4 ± 2.3 years for a titanium BP (P Results We analyzed 17 B-KPro eyes with PMMA BPs and 24 B-KPro eyes with titanium BPs. The average AOD500 (394.1 ± 226.9 vs. 454.5 ± 255.6 μm, P = 0.44), average TIA500 (26.2 ± 14.2 vs. 29.8 ± 13.9 degrees, P = 0.43), and average TISA500 (0.15 ± 0.08 vs. 0.17 ± 0.10 μm, P = 0.52) were not statistically different between eyes with PMMA and titanium BPs, nor were the temporal, the narrowest, and the widest angle measurements of each eye (all P > 0.05). Similarly, no significant differences were found between the angle measurements of B-KPro eyes with a titanium BP diameter of 8.5 or 9.5 mm (all P > 0.05). Conclusions We successfully visualized the angle anatomy in 66.1% of the imaged eyes, including all BPs studied. Neither the material nor the size of the B-KPro BP had a significant impact on the angle anatomy.

An unprecedented pigmented caruncular apocrine hidrocystoma with the additional feature of an oncocytic transformation of the cyst's lining cells is reported. Over a year, a 79-year-old woman developed a centrally pigmented lesion of her right caruncle with translucent borders. Because of concern about a melanoma, a carunculectomy with adjunctive cryotherapy and placement of an amniotic membrane graft were performed, and the excised specimen was evaluated microscopically. A large cyst dominated the caruncle and was lined by an inner layer of columnar eosinophilic and granular cells with an outer, interrupted layer of flattened myoepithelial cells. Phosphotungstic acid hematoxylin staining disclosed myriad cytoplasmic, dot-like mitochondria signifying an oncocytic change. Immunohistochemistry revealed gross cystic fluid disease protein-15 and cytokeratin 7-positivity indicative of apocrine differentiation. Oncocytic change is characteristically encountered in lacrimal ductal cysts and tumors.

PURPOSE To evaluate the feasibility of Descemet stripping endothelial keratoplasty using grafts preloaded by an eye bank in a commercially available insertion device. METHODS In this retrospective case series, a series of 35 eyes in 34 consecutive patients who underwent Descemet stripping endothelial keratoplasty for Fuchs endothelial dystrophy or previously failed full-thickness grafts at a single tertiary care center from March 2013 to March 2014 was included. The donor tissue had undergone pre-lamellar dissection, trephination, and loading into EndoGlide Ultrathin inserters at the Lions Eye Institute for Transplant and Research (Tampa, FL) and was shipped overnight in Optisol GS to the surgeon (K.C.). Surgery was performed within 24 hours from tissue preparation and loading by the eye bank. Donor and recipient characteristics, endothelial cell density (ECD), best-corrected visual acuity, and central corneal thickness were recorded. The main outcome measures were intraoperative and postoperative complications and ECD loss at 3, 6, and 12 months. RESULTS One primary graft failure (2.8%), 2 rebubblings (5.7%), and 1 graft rejection (2.8%) occurred. Mean preoperative donor ECD was 2821 ± 199 cells/mm. Six months postoperatively, the mean endothelial cell loss was 25.3% ± 17.2% (n = 32), which remained stable at 1 year (31.5% ± 17.9%, n = 32). Mean best-corrected visual acuity improved from 20/100 preoperatively to 20/25 at a mean follow-up of 1 year (n = 32). Mean central corneal thickness was reduced from 711 ± 110 μm to 638 ± 66 μm at the last follow-up visit. CONCLUSIONS Donor graft tissue preloaded by an eye bank can be used successfully for endothelial keratoplasty. Preloading reduces intraoperative tissue manipulation.

Claes Henrik Dohlman, MD, PhD (CHD), has devoted his entire career to advancing our understanding of the biology of the cornea and the pathophysiology and management of corneal diseases. He is truly a renaissance scholar of the cornea, whose influence has defined and shaped the field. His basic science work, which spans all structures of the cornea, is eloquently reviewed in this volume by his long-term colleague Ilene Gipson (Chap. 17). While these studies are noteworthy, and for many would constitute a lifetime of contribution, Claes’ most significant impact on the field of cornea falls into two major areas – development of the most widely used keratoprosthesis (Boston KPro) in the world (reviewed in Chap. 15 by Mona Harissi-Dagher, one of the leaders of the current generation of KPro surgeons) and training multiple generations of corneal specialists. It is estimated that Dr. Dohlman has trained over 200 corneal specialists in his career, many of whom have held or currently hold leadership positions in ophthalmology around the world. Dr. Dohlman’s true impact is much greater, however, when one considers all of the corneal specialists trained by the Dohlman fellows. It is rare to find corneal specialist in the United States today who cannot be traced to have some connection back to Claes Dohlman. As I write this chapter, I am truly grateful for the many lessons I have learned from Dr. Dohlman, some of which I will attempt to convey in the following pages.

Fuchs endothelial corneal dystrophy (FECD), estimated to affect 4% of the US population [1], is the leading cause of endogenous corneal endothelial degeneration and the primary indication for keratoplasty worldwide [2]. FECD is a slowly progressive bilateral corneal disease that is characterized by abnormal corneal endothelial morphology, such as pleomorphism and polymegethism, decreased cell density, thickening of the Descemet membrane (DM), and formation of extracellular matrix (ECM) excrescences called guttae [3], which lead to derangements in endothelial cell function. Quality of vision is slowly compromised by corneal edema secondary to gradual failure of corneal endothelial pump action and guttae-induced visual disturbances such as decreased contrast sensitivity [4] and higher order aberrations [5]. Its pathogenesis is proposed to be an interaction between endogenous and exogenous factors [6]. The disease manifests in the early third to late fifth decade of life and has a female preponderance [7–9]. Since the first description of the disease by Ernest Fuchs more than 100 years ago [10], major advances in understanding the nature and pathogenesis of FECD have been made and are described in this chapter.

Among the group of diseases comprising corneal dystrophies, there are a number for which contact lenses may be considered. For example, soft contact lenses may be beneficial in the management of recurrent corneal erosions and advanced bullous keratopathy and may lead to disease attenuation in some conditions. They also may be used in conjunction with surgical procedures to promote tissue healing and patient comfort in the postoperative period. Rigid contact lenses may be beneficial in the setting of irregular astigmatism. The present review of the literature will discuss the use of contact lenses in the management of corneal dystrophies.Bei den unter dem Begriff „Hornhautdystrophien“ zusammengefassten Krankheiten kann bei einer ganzen Reihe der Einsatz von Kontaktlinsen erwogen werden. So können sich etwa weiche Kontaktlinsen als vorteilhaft erweisen für die Behandlung wiederkehrender Hornhauterosionen und fortgeschrittener bullöser Keratopathie und unter Umständen zu einer Abschwächung der Krankheit führen. Sie können auch in Kombination mit chirurgischen Verfahren eingesetzt werden, um die Gewebeheilung und das Wohlbefinden des Patienten in der postoperativen Phase zu fördern. Starre Kontaktlinsen können sich als vorteilhaft bei der Korrektur von unregelmäßigem Astigmatismus erweisen. Die vorliegende Literaturübersicht beschäftigt sich mit der Verwendung von Kontaktlinsen bei der Behandlung von Hornhautdystrophien.

Purpose: Boston keratoprosthesis (KPro) patients are prone to glaucoma even with well-controlled intraocular pressure (IOP). Recent experimental data have shown that soluble tumor necrosis factor a...

Purpose To compare the outcomes of Boston keratoprosthesis type 1 implantation after failed keratoplasty in patients who are blind or sighted in the contralateral eye. Methods Retrospective comparative case series of Boston keratoprosthesis type I recipients performed for failed keratoplasty, between January 1, 2008, and June 30, 2016, at a single center. Patients were divided based on the best-corrected visual acuity in the contralateral eye at the time of surgery: group I, ≤20/200, and Group II, >20/200. Preoperative diagnoses, postoperative visual acuity, device retention, and postoperative complications were compared. Results Group I (37 eyes) and group II (36 eyes) had similar demographics, median preoperative best-corrected visual acuity (count fingers) in the operated eye, and median duration of postoperative follow-up (37.4 vs. 45.2 months, respectively). Keratoprosthesis retention after the first year postimplantation was significantly better in group I versus group II (P = 0.038). Sterile vitritis and sterile keratolysis occurred more frequently in group II compared with group I (P = 0.013 and P = 0.056, respectively). At final examination, visual outcomes were not significantly different between the 2 groups. Conclusions Most patients with failed keratoplasty who were implanted with a Boston keratoprosthesis type I experienced improved vision, and visual acuity of the contralateral eye did not seem to influence the visual outcome of surgery. However, patients with good vision in the contralateral eye were more likely to experience complications, possibly because of reduced vigilance when the other eye has ambulatory vision.

Background Approximately 2.4 million eye injuries per year occur in the United States. Because of the complexity of these injuries, many of these cases present to academic institutions. Ophthalmology residency programs have a wide range of resources available for eye injuries requiring after-hours surgical intervention. Purpose The purpose of this study was to determine availability of resources for after-hours eye surgery and their effect on clinician satisfaction. Methods Association of University Professors of Ophthalmology (AUPO) program directors were surveyed to assess the availability of eye-trained operating room (OR) nurses, functional operating microscopes, and suitable surgical supplies. The primary outcome measure was the availability of resources for after-hours eye surgery. Secondary outcome measures included the impact of resources on clinician satisfaction. Results Fifty-seven program directors completed the survey. Most programs operated at level 1 trauma centers (95%), had access to a functional microscope (95%), and had frequent adequate surgical supplies (88%). On the other hand, only 28 (49%) programs reported consistent access to OR nurses with ophthalmologic training. Clinician satisfaction scores were three times higher for programs with frequent access to trained surgical OR staff than for programs without (p Conclusion Approximately half of programs report consistent access to ophthalmologic-trained OR staff after hours. In contrast, most programs report access to a functional microscope and surgical supplies. The results of this survey suggest that directing resources toward improving access to after-hours staffing with specialty OR staff may improve clinician satisfaction.

Objectives To examine clinical outcomes of oversized titanium back plates in type I Boston keratoprosthesis (KPro) implantation. Methods Retrospective study of 22 sequential eyes (20 patients) undergoing type I KPro implantation with an oversized titanium back plate (larger than trephined wound diameter by 1.0 mm or more), performed by a single surgeon (K.A.C.) from June 2010 to November 2014. Data were collected regarding preoperative eye characteristics, surgical details, and postoperative clinical outcomes. Results Mean follow-up time per eye was 24.1±14.9 months. All eyes had improved vision after surgery; 13 eyes (59.1%) maintained visual acuity improvement at last follow-up. Initial KPro's were retained in 19 eyes (86.4%); one eye required KPro replacement. Primary retroprosthetic membrane (RPM) developed in three eyes (13.6%), with similar occurrence in aniridic (14.3%) and nonaniridic eyes (13.3%). Secondary RPM's developed in two eyes (9.1%) after vitritis (one eye) and retinal and choroidal detachment (one eye). Glaucoma was a common comorbidity: 2 of 14 eyes (14.3%) with preoperative glaucoma had glaucoma progression, and 4 of 8 eyes (50.0%) without preoperative glaucoma developed glaucoma postoperatively. Other postoperative complications included retinal detachment (5 eyes, 22.7%) and idiopathic vitritis (3 eyes, 13.6%). Conclusions Oversized titanium KPro back plates are associated with a low rate of primary RPM formation and may have particular utility in reducing primary RPM formation in aniridic eyes. Glaucoma remains a challenge in postoperative KPro management. Complex eyes, at increased risk of postoperative complications, require careful management.

PURPOSE Idiopathic vitritis is a poorly understood complication after Boston keratoprosthesis surgery with unclear etiology. We sought to determine whether an association exists between periprosthetic corneal tissue loss and the development of idiopathic vitritis in keratoprosthesis recipients. METHODS Thirteen Boston type I keratoprosthesis recipient eyes with a history of idiopathic vitritis and 34 type I keratoprosthesis recipient eyes with no history of idiopathic vitritis underwent anterior segment optical coherence tomography (AS-OCT) at a median time postoperatively of 2.4 years versus 1.9 years (range, 0.5-14.2 vs. 0.1-13.6 years), respectively. Areas of corneal graft tissue loss ("gaps") around the keratoprosthesis stem were identified and analyzed by 2 masked observers. The difference in the presence, number, and size of gaps was compared between cases and controls. RESULTS A periprosthetic gap was identified more commonly in idiopathic vitritis cases than in controls on AS-OCT (11/13, 86% vs. 11/34, 33.3%, P < 0.001). The number of gaps between cases and controls was also significantly different (2.6 ± 1.6 vs. 0.5 ± 0.8, P < 0.001), but not the estimated gap area (0.056 ± 0.049 mm² vs. 0.039 ± 0.025 mm², P = 0.22). CONCLUSIONS A significantly higher proportion of keratoprosthesis recipient eyes with idiopathic vitritis had corneal tissue loss around the keratoprosthesis stem than did controls. Tissue loss could serve as an entry point for debris or bacterial components, triggering idiopathic vitritis. Our study underscores the utility of AS-OCT imaging in the postoperative management of keratoprosthesis patients.

The aim of this study was to revisit the clinical paradigm attributed to Boston keratoprosthesis recipients presenting with idiopathic vitreous inflammation.A retrospective chart review was performed of keratoprosthesis recipients at Massachusetts Eye and Ear Infirmary, from January 2000 to August 2013, for demographic data, indication(s) for surgery, timing and presentation of vitreous inflammation, and best-corrected visual acuity at baseline, on presentation, and after resolution of vitritis.Twenty-three (23 eyes) of 346 patients developed idiopathic vitreous inflammation after keratoprosthesis implantation. Six of 23 patients presented with signs and symptoms similar to infectious endophthalmitis but were culture negative. The proportion of patients who fit the previous paradigm of sudden painless loss of vision without external signs of infection ("sterile vitritis") at their first presentation with vitritis was only 4 of 23. Vision decline was variable (median, 9 lines on Snellen chart; range, 0-24), as was time to recovery of best vision (median, 8.9 weeks; range, 0.9-36.7). Nine eyes had repeat bouts (43 episodes in 23 patients). Ten of 43 episodes did not recover to baseline vision. Seventeen of 23 eyes with idiopathic vitritis after keratoprosthesis later developed other complications.The current paradigm for idiopathic vitritis after keratoprosthesis implantation includes sudden painless loss of vision with full recovery of vision on treatment with periocular corticosteroids. However, idiopathic vitritis after keratoprosthesis can also mimic infectious endophthalmitis with pain and external signs of inflammation. Visual loss can be gradual. Vision may not recover to baseline despite treatment. Vitritis may be a part of a common pathway of chronic inflammation after keratoprosthesis.

Purpose To propose a new treatment paradigm for chemical burns to the eye - in the acute and chronic phases. Methods Recent laboratory and clinical data on the biology and treatment of chemical burns are analyzed. Results Corneal blindness from chemical burns can now be successfully treated with a keratoprosthesis, on immediate and intermediate bases. Long term outcomes, however, are hampered by early retinal damage causing glaucoma. New data suggest that rapid diffusion of inflammatory cytokines posteriorly (TNF-α, etc) can severely damage the ganglion cells. Prompt anti-TNF-α treatment is markedly neuroprotective. Long term profound reduction of the intraocular pressure is also vital. Conclusion A new regimen, in addition to standard treatment, for severe chemical burns is proposed. This involves tumor necrosis factor alpha (TNF-α) inhibition promptly after the accident (primarily for retinal neuroprotection), prophylactic maximal lowering of the intraocular pressure (starting immediately), and keratoprosthesis implantation in a later quiet state.

PURPOSE To describe the clinical course, surgical experience, and postoperative outcomes of 3 patients with Fuchs endothelial dystrophy who underwent Descemet membrane endothelial keratoplasty (DMEK) after failed Descemet stripping without endothelial keratoplasty. METHODS Three patients who underwent DMEK for management of persistent corneal edema after deliberate Descemet stripping in the setting of Fuchs endothelial dystrophy were identified. Patients were examined at day 1, week 1, and months 1, 3, and 6 after DMEK. Visual acuity, central corneal thickness (CCT), and evaluation of central corneal endothelial cell counts were recorded. RESULTS Two women and one man, aged 56, 72, and 68 years, were included. The time interval between primary Descemet stripping and DMEK ranged from 3.5 to 8 months. Preoperative visual acuities were 20/200, 20/300, and 20/80. Immediately before DMEK, no patients had countable central endothelial cells, and CCTs were 825, 1034, and 878 μm. After DMEK, all patients had improvement in visual acuity to 20/70, 20/20, and 20/20 with CCTs of 529, 504, and 528. The postoperative period in the first case was notable for the immediate development of a pigmented pupillary membrane with posterior synechiae, as well as cystoid macular edema, of uncertain chronicity, noted 1 month postoperatively. The second case also developed posterior synechiae. Two cases completed 6-month endothelial cell counts totaling 2200 and 3114 cells per square millimeter (endothelial cell loss of 13% and 5.3%). CONCLUSIONS DMEK is a reliable procedure to facilitate corneal rehabilitation and visual recovery in the event of poor corneal clearance after Descemet stripping without endothelial keratoplasty.

This chapter will discuss corneal surgery in children, focusing on how to modify surgical techniques for pediatric patients. Indications for surgery, surgical techniques, and postoperative care will be discussed for surgeries including variations of keratoplasty, (penetrating, anterior lamellar, and endothelial), keratoprosthesis surgery, and ocular surface reconstruction. Ophthalmologists who diagnose, surgically manage, and care for children with corneal eye disease are rare and published treatment guidelines for pediatric corneal surgery are even rarer.

Infectious keratitis is a serious, potentially vision-threatening condition that represents one of the most common causes of ocular morbidity worldwide. While it is considered an uncommon event among pediatric populations, estimated to be between 9 and 13% of all cases, the timely diagnosis and treatment of these infections is particularly important as younger patients are susceptible to developing not only blindness from corneal disease, but also secondary amblyopia. Thus, any suspicion of an infectious etiology should be thoroughly investigated, with special attention to the history of infectious exposure, epidemiological trends, and clinical clues—including ocular findings and patterns of corneal inflammation and systemic signs and symptoms that may be specific to a certain pathogen. While corneal scraping and laboratory culture may aid in making the correct diagnosis and in guiding the therapeutic approach, it should be noted that culture positivity has been shown to range anywhere between 34 and 87% in children with presumed microbial keratitis; therefore, knowledge of the epidemiological and clinical features of the most likely pathogens is crucial in prompt diagnosis, treatment, and, ultimately, the prevention of poor outcomes.

The ocular surface consists of the conjunctival mucosa, corneoscleral limbus, corneal epithelium, and tear film. Its proper functioning is dependent on the integrity of the adjacent structures: the eyelids, meibomian glands, and lacrimal system. A healthy ocular surface maintains the clarity of the cornea, the major refracting power of the eye. It also protects the underlying globe from infection and trauma. The ocular surface must maintain a delicate homeostasis, both in physical and chemical composition. Dysregulation can result in impaired vision, corneal scarring, and impaired ocular immunity. The ocular surface can be damaged as a result of toxic, traumatic, infectious, congenital, autoimmune, iatrogenic, and nutritional insult. This chapter reviews the etiologies of ocular surface disease in children. These diverse diseases can be broadly categorized as diseases of tear film dysfunction, neurotrophic keratitis, exposure keratitis, and limbal stem cell deficiency.

Allergies occur frequently in all pediatric age groups, affecting up to 40% of children, with a peak age in late childhood and young adulthood. It affects the health and overall quality of life of children and their families. Patients frequently have a history of other atopic diseases, such as eczema, asthma, or, most commonly, rhinitis. Allergic conjunctivitis is the most common ocular allergy syndrome among children, with atopic keratoconjunctivitis and vernal keratoconjunctivitis being less common but potentially more severe forms of ocular allergy. Symptoms include bilateral involvement, itching, tearing, mucoid discharge, redness, eyelid edema, and chemosis. IgE and non-IgE-mediated mechanisms are involved in the development of ocular allergic eye disease. The multiple mediators and pathways are differently expressed in the different allergic disorders, inducing the distinctive clinical aspects, diagnostic features, and response to treatment. There are a variety of pharmacological interventions that are available such as vasoconstrictors/histamine (H1) receptor blockers, mast cell stabilizers, multi-action agents, topical ophthalmic corticosteroids, topical nonsteroidal anti-inflammatory drugs (NSAIDs), topical cyclosporine, and tacrolimus as well as systemic drugs. Immunotherapy is also considered in the treatment of persistent severe cases. Despite all the available therapeutic agents, there continues to be a constant need to discover more effective ways to treat the different allergic eye disorders. In this chapter, we review the clinical features, diagnosis, potential complications, and treatment of these ocular allergic pediatric conditions. Early detection is necessary to prevent potentially serious consequences that could be sight threatening. Involvement of pediatric ophthalmologists and cornea specialists may be necessary to avoid preventable vision loss in severe cases.

Strategies for examining children who are suspected of having a corneal condition vary according to the age of the child. Distraction, familiarity with various things of interest to children, and casual conversation will help put most children at ease. Developing a therapeutic relationship with the child will increase the likelihood of achieving an adequate exam in the office. In the setting of severe symptoms, an exam under anesthesia may be needed if an adequate office exam is not possible. There are multiple causes of a red eye in children, most of which are self-limited. Concerning signs include severe loss of vision, severe pain, or unremitting or recurrent symptoms. Special care must be taken to rule out a vision-threatening condition in the setting of eye trauma or contact lens use.

For patients with keratoconus, visual acuity with contact lenses often surpasses the acuity achieved with spectacles. There are multiple contact lens modalities that can be used. These lenses include corneal rigid gas permeable, piggyback, hybrid, scleral, and soft toric lenses, as well as PROSE devices. Corneal rigid gas permeable lenses, the mainstay for the improvement of visual acuity in patients with keratoconus since the 1960s, remain the most popular choice for this patient population. However, hybrid and scleral lenses have grown tremendously in popularity over the past decade. Every patient that presents with keratoconus is a unique fitting situation and a lens must be chosen based on the particular needs of the patient. In this chapter, lens modalities along with advantages and disadvantages of each will be described.

PURPOSE To describe the clinical and histopathologic features distinguishing an extensive complex choristoma of the epibulbar surface and to address the management of such lesions. METHODS Clinical history, diagnostic imaging studies, and histopathologic sections stained with hematoxylin and eosin were reviewed from a 2-year-old girl with a congenital conjunctival lesion of the right eye that was surgically excised. RESULTS The patient clinically displayed an extensive, vascularized amelanotic conjunctival lesion located superotemporally with extension onto the cornea. Her visual acuity was reduced to 20/670. The clinical diagnosis was a large lacrimal gland choristoma with corneal involvement and resulting deprivation amblyopia. The patient underwent an excision of the lesion including the corneal portion, and the ocular surface was reconstructed with amniotic membrane. Histopathologic evaluation disclosed lobules of lacrimal tissue and cartilage plaques, smooth muscle, and nerves consistent with a complex choristoma. Six weeks postoperatively, the visual acuity had improved to 20/180. The patient returned to her local ophthalmologist for amblyopia management. CONCLUSIONS We emphasize the importance of recognizing lesion-induced amblyopia and the timely performance of appropriate surgery for complex epibulbar choristomas. A differential diagnosis of other congenital epibulbar lesions is provided.

To evaluate the effect of deliberate removal of the central Descemet membrane on endothelial function and morphology in patients with Fuchs endothelial dystrophy (FED) and cataract undergoing phacoemulsification.In this retrospective case series, patients with FED and visually significant cataract underwent phacoemulsification in an academic cornea practice in Boston, MA. Four millimeters of the central Descemet membrane was stripped and removed after intraocular lens insertion. Vision, corneal pachymetry, and confocal imaging of the endothelial anatomy were performed before surgery and at 1, 3, 6, and 12 months after surgery. Patients were classified as fast responders, responders, slow responders, and nonresponders on the basis of postoperative time to resolution of corneal edema with visible central endothelial mosaic.Eleven patients (13 eyes) aged 51 to 91 years were included in the study. No eyes had countable central endothelial cells by confocal imaging before surgery. Preoperative visual acuity ranged from 20/25 to 20/400. All corneas showed stromal and microcystic edema in the area of Descemet stripping at days 1 and 7 after surgery. Four eyes demonstrated resolution of corneal edema with visible central endothelial cell mosaic (range: 410-864 cells/mm) by postoperative month 1 with visual acuity ranging between 20/25 and 20/40. Four additional eyes demonstrated a similar response by postoperative month 3 and an additional 2 eyes had resolution of corneal edema with an intact central endothelial mosaic at postoperative month 6 or later. Cell counts (range: 428-864 cells/mm) were maintained in all 10 responders at the last follow-up visit (range: postoperative months 6-24). Final vision ranged from 20/15 to 20/20 in these 10 eyes with the exception of 2 eyes with retinal pathology. Three eyes required endothelial keratoplasty.Repopulation of the central corneal endothelium with corneal deturgescence can occur after deliberate central Descemet stripping in patients with FED who underwent cataract removal. This may offer a novel treatment for patients with FED that could reduce the need for endothelial transplantation. Further studies are needed to delineate the optimal patient population for Descemet stripping because not all patients will respond to this intervention.

To study corneal reinnervation and sensation recovery in Herpes zoster ophthalmicus (HZO).Two patients with HZO were studied over time with serial corneal esthesiometry and laser in vivo confocal microscopy (IVCM). A Boston keratoprosthesis type 1 was implanted, and the explanted corneal tissues were examined by immunofluorescence histochemistry for βIII-tubulin to stain for corneal nerves.The initial central corneal IVCM performed in each patient showed a complete lack of the subbasal nerve plexus, which was in accordance with severe loss of sensation (0 of 6 cm) measured by esthesiometry. When IVCM was repeated 2 years later before undergoing surgery, case 1 showed a persistent lack of central subbasal nerves and sensation (0 of 6). In contrast, case 2 showed regeneration of the central subbasal nerves (4786 μm/mm) with partial recovery of corneal sensation (2.5 of 6 cm). Immunostaining of the explanted corneal button in case 1 showed no corneal nerves, whereas case 2 showed central and peripheral corneal nerves. Eight months after surgery, IVCM was again repeated in the donor tissue around the Boston keratoprosthesis in both patients to study innervation of the corneal transplant. Case 1 showed no nerves, whereas case 2 showed new nerves growing from the periphery into the corneal graft.We demonstrate that regaining corneal innervation and corneal function are possible in patients with HZO as shown by corneal sensation, IVCM, and ex vivo immunostaining, indicating zoster neural damage is not always permanent and it may recover over an extended period of time.