The Asia-Pacific Journal of Ophthalmology

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Original Study - Clinical

Clinical Course and Treatment Response of Neuromyelitis Optica Spectrum Disease: An 8-Year Experience Lin, Chao-Wen; Lin, I-Hung; Chen, Ta-Ching; Jou, Jieh-Ren; Woung, Lin-Chung
Purpose: To report the clinical course and treatment response in a cohort of neuromyelitis optica spectrum disorder (NMOSD) patients from a single referral center.

Design: Case series.

Methods: Ninety-six NMOSD patients who received treatment and follow-up checks at National Taiwan University Hospital for at least 2 years between 2008 and 2016 were recruited. Detailed characteristics of clinical course including acute episodes, maintenance therapies, and visual outcome were collected. Patients were examined every 6 months and during each episode of relapse. The study was approved by the institutional review board and informed consent was obtained.


Results: Among all patients, 68 (70.8%) had optic neuritis. Optic neuritis was the initial presentation of NMOSD in 44 patients. Among patients with optic neuritis, 32.4% had recurrent optic neuritis within 1 year from the first episode. Compared with the group without optic neuritis, the group with optic neuritis had a younger age of presentation (34.4 ± 15.9 versus 42.4 ± 14.7 years, P = 0.02) and a higher 1-year recurrence rate (64.7% versus 17.9%, P < 0.001). Among the patients with optic neuritis, 51.5% of patients had a final visual acuity of worse than 20/200. Multiple regression analysis showed that the presence of aquaporin-4-IgG antibodies and poor initial visual acuity were risk factors of worse visual outcome.

Conclusions: In this study, patients with optic neuritis were younger and had a higher 1-year recurrence rate. Despite using steroid therapy during the acute phase and immunosuppressive agents as long-term treatment, visual prognosis was poor. Aquaporin-4-IgG antibodies and initial visual acuity were associated with final visual outcome.
Plaque Radiotherapy for Medulloepithelioma in 6 Cases From a Single Center Ang, Su Mae; Dalvin, Lauren A.; Emrich, Jacqueline; Komarnicky, Lydia; Shields, Jerry A.; Shields, Carol L.
Purpose: To describe outcomes of 6 patients treated with plaque radiotherapy for medulloepithelioma.

Design: Retrospective review.

Methods: Computerized medical records were reviewed for cytopathologically confirmed medulloepithelioma treated with plaque radiotherapy from 1970 to 2017. Clinical and radiotherapy parameters along with outcomes of tumor regression, globe salvage, and metastatic disease were recorded.


Results: Of 6 patients with medulloepithelioma, plaque radiotherapy was primary (n = 5) or secondary (n = 1) treatment. The median patient age at diagnosis was 41 months (mean, 145; range, 10–624 months). Patient demographics included white race (n = 6) and female sex (n = 5). Patients presented with strabismus (n = 1), decreased vision (n = 1), ocular pain (n = 1), hyphema (n = 1), corectopia (n = 1), and visible iris lesion (n = 1). Mean tumor basal diameter was 11.2 mm (median, 10.0; range, 7.0–16.0 mm), and mean tumor thickness was 6.8 mm (median, 6.4; range, 3.1–11.0 mm). Related findings included cataract (n = 4), iris neovascularization (n = 5), secondary glaucoma (n = 2), and ectropion uveae (n = 2). Mean radiation dose to tumor apex was 44 Gy (median, 38; range, 35–70 Gy). At mean follow-up of 59 months, tumor control was achieved in 5 (83%) eyes with globe salvage in 4 (67%) eyes. No patient had evidence of metastases or death at last follow-up visit (mean, 59 months; range, 12–210 months).

Conclusions: Plaque radiotherapy is a reasonable alternative treatment for localized, small to medium-size medulloepithelioma. Tumors larger than 11.0 mm, with retrolental cyclitic membrane or extrascelral extension, might still require enucleation.
Bilateral Implantation of a Single-Piece Bifocal Diffractive Intraocular Lens in Presbyopic Patients: A Prospective Case Series Chang, John S.M.
Purpose: To report visual outcomes and quality after bilateral implantation of a single-piece diffractive multifocal intraocular lens (MIOL) with a bifocal design.Design: Prospective, observational case series.Methods: All presbyopic subjects were implanted with the ZMB00 MIOL (Abbott Medical Optics) at the Hong Kong Sanatorium and Hospital. We evaluated the distance, intermediate, and near visual acuity (VA) under photopic and mesopic conditions; contrast sensitivity with and without glare under photopic and mesopic conditions; quality of vision; and rate of spectacle independence. The current study was conducted in accordance with the Declaration of Helsinki and the ethics committee of our hospital approved the study.Results: Thirty-six subjects were included. No intraoperative complications developed. At the 6-month visit, the mean monocular uncorrected VAs at distance, intermediate, and near were 0.01 ± 0.12 (standard deviation), 0.26 ± 0.17, and 0.09 ± 0.08, respectively. The mean binocular uncorrected VAs were −0.05 ± 0.09, 0.12 ± 0.13, and 0.04 ± 0.06, respectively. No eyes lost any corrected distance VA. The binocular contrast sensitivity was significantly better without glare than with glare under photopic and mesopic conditions (P < 0.05 for all spatial frequencies under both conditions). Twenty-one (58%) and 21 subjects (58%) reported halo and glare, respectively. Thirty subjects (83%) reported complete spectacle independence.Conclusions: Bilateral implantation of the bifocal MIOL in presbyopic patients with or without cataract was safe and resulted in satisfactory vision and a high rate of spectacle independence. Halos and glare were common.
Asia-Pacific Technology and Trend Survey 2016–2017 Chhablani, Jay; Shaikh, Adnan; Goud, Abhilash; Kawasaki, Ryo; Kwon, Oh W.; Chang, Andrew; Lam, Dennis; Das, Taraprasad
Purpose: To report the results of a survey conducted among retina specialists in the Asia-Pacific region on real-life practice patterns in the management of vitreoretinal diseases.Design: Prospective study.Methods: In 2016 and 2017, a link was sent to 1400 retinal specialists across the Asia-Pacific region by e-mail, which directed to a web-based questionnaire (Google forms or Survey Monkey) with secure confidential access. The study had institutional review board approval. Answers to some of the common questions were compared with the 2016 American Society of Retina Specialists domestic and global trends.Results: The surveys of 2016 and 2017 received 539 (38.5%) and 200 (14.3%) responses, respectively, across the Asia-Pacific region. Of the respondents, 85% practiced combined medical and surgical retina. The survey indicated that ranibizumab was the drug of choice (41% of respondents) in the management of wet age-related macular degeneration. In the management of polypoidal choroidal vasculopathy, both combination of verteporfin photodynamic therapy (vPDT ) and anti–vascular endothelial growth factor (VEGF) (n = 59%) and intravitreal aflibercept monotherapy (n = 53%) were preferred. Anti-VEGF treatment remained the first choice for center-involving diabetic macular edema (DME) (n = 78%) and switch to dexamethasone implant in nonresponding DME was preferred after 2–3 anti-VEGF injections (n = 53%).Conclusions: The survey revealed information that may be close to real-world practices and could be of help to understand the transformation of global trends and practices due to evolving evidence and technologies.
Concept and Uptake of Just A Minute Clinical Pearl: A Novel Tele-Ophthalmology Teaching Tool Dave, Vivek Pravin; Pathengay, Avinash; Keeffe, Jill; Green, Catherine
Purpose: To describe the concept and report the uptake of Just-A-Minute (JAM) clinical pearls, a novel tele-ophthalmology educational tool.Design: Analysis of a survey conducted among the recipients of JAM clinical pearls.

Design: Analysis of a survey conducted among the recipients of JAM clinical pearls.

Methods: One clinical pearl was disseminated daily to all ophthalmologists in the email database of L V Prasad Eye Institute from August 2016 to September 2017. A survey was circulated to the recipients and their responses were recorded and analyzed to evaluate the JAM pearls regarding qualification of the recipients, type of practice, regularity of accessing the pearls, type of pearl most sought, clarity of content, applicability to the clinic, relevance to practice, satisfaction with the format, and likelihood of recommendation.

Results: There were a total of 14,311 recipients. The majority were in India (66.5%) followed by the United States (26.4%). There were 719 survey respondents (5%). Among these, 52.9% were postgraduate ophthalmologists in general practice, 38.3% were post-fellowship subspecialty practitioners, 5.8% were fellows, and 2.9% were residents. Private practitioners constituted 57.3% of all respondents. The pearls were accessed “always” by 53.7%, “very regularly” by 34.6%, and “intermittently” by 11.8% of respondents. The subspecialty most desired was cornea and anterior segment (48.7%). The JAM pearls were scored on a scale of 1 to 10 for clarity of content (9/10), applicability to the clinic (9/10), satisfaction with the format (9/10), and recommendation to a colleague (10/10).

Conclusions: JAM clinical pearls are a unique, beneficial mode of tele-education with easily understandable and clinically applicable concepts.
Anesthesia Considerations in Modified Osteo-Odonto-Keratoprosthesis Raman, Sonali; Singh, Sumeer; Jagdish, V.
Purpose: The purpose of this study was to elucidate anesthesia-related challenges in 74 cases who underwent modified osteo-odonto-keratoprosthesis (MOOKP).

Design: Retrospective cohort study.

Methods: Medical records of 74 consecutive patients who underwent MOOKP surgery between January 2007 and January 2015 were studied. Data such as demographic details, systemic comorbidities, indications for MOOKP, anesthesia techniques, and postoperative events in various stages of MOOKP were collected and analyzed.

Results: The mean age of the patients was 29.7 ± 9.13 years. Of the 74 patients, 47 were men. The most common indication for MOOKP was found to be Stevens–Johnson syndrome (53%) followed by chemical injury (38%). In all stages of the MOOKP procedure, 94.6% of cases were found to have American Society of Anesthesiologists (ASA) score grade II, whereas 4.6% had grade III. Mallampati score of 1 was found in 79.7% of cases in stage 1, 82.4% of cases in stage 1B + 1C, and 81% of cases in stage 2. Recovery grade of 10 was noted in all the cases at all stages of the MOOKP procedure. Intubation in the second attempt was carried out in 8 cases and oral intubation in stage 1B + 1C was performed in 4 cases. Difficulty in intubation was noted in 3 cases in stage 1A, 4 cases in stage 1B + 1C, and 1 case in stage 2.

Conclusions: In patients undergoing MOOKP, a good preanesthetic evaluation and intraoperative anesthesia plan before each stage could help in successful administration of anesthesia in these patients.

Review Article

The History of Exfoliation Syndrome Grzybowski, Andrzej; Kanclerz, Piotr; Ritch, Robert
The first description of exfoliation syndrome (XFS) was presented by John Gustaf Lindberg in his doctoral thesis 100 years ago. The syndrome is an age-related disorder in which abnormal fibrillar extracellular material is produced and accumulates in several ocular tissues. Once thought to be peculiar to Scandinavia, XFS is found in almost every race and ethnic group. Subsequent studies provided evidence for the systemic nature of XFS, which involves an aberrant connective tissue metabolism throughout the body. There is a prominent association with zonular instability. This study presents the developments in the understanding of the pathomechanism and clinical significance of XFS and explains the problem of terminology in the description of XFS and pseudoexfoliation.
Neurofibromatosis Type 1: Review and Update on Emerging Therapies Karaconji, Tanya; Whist, Eline; Jamieson, Robyn V.; Flaherty, Maree P.; Grigg, John R.B.
Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disorder affecting 1:3000 births. This familial tumor predisposition syndrome is diagnosed clinically and affects the skin, bones, and nervous system. Malignant tumors can arise in childhood or adulthood and are the commonest cause of mortality in this population. Early diagnosis and management led by a multidisciplinary team remains the standard of care, particularly in the management of optic pathway glioma. Emerging concepts in the genetic patterns of this condition have led to the introduction of new treatment modalities that target the mitogen activated protein kinase (MAPK) and the mammalian target of rapamycin (mTOR) pathways. The role of the ophthalmologist and approach to screening for optic pathway glioma is outlined based on previous recommendations. Updates on choroidal involvement, as a diagnostic criterion, will also be discussed, further highlighting the pivotal role of the ophthalmologist in the diagnosis and management of this complex condition.
Persistent Fetal Vasculature Chen, Chonglin; Xiao, Hu; Ding, Xiaoyan
During development, the fetal vasculature nourishes the developing lens and retina, subsequently regressing after the formation of the retinal vessels. Persistent fetal vasculature (PFV) occurs as a result of a failure of fetal ocular vasculature to undergo normal programmed involution, which leads to blindness or serious loss of vision. Persistent fetal vasculature is responsible for as much as 5% of childhood blindness in Western countries. The regulatory mechanisms responsible for fetal vascular regress remain obscure, as do the underlying causes of the failure of regression. Because of recent advancements in microinvasive surgical techniques, the early treatment of PFV has become safer and more effective, thus paving the way for the development of a future new treatment strategy. In this review, clinical and imaging manifestations of PFV and the progress in the treatment of PFV are highlighted.
New Technologies for Glaucoma Detection Rodriguez-Una, Ignacio; Azuara-Blanco, Augusto
This review describes some of the most relevant recent advances in the development and application of new technologies for detecting and managing glaucoma, including imaging, visual function testing, and tonometry. The widespread availability of mobile technology in the developing world is improving health care delivery, for example, with smartphones and mobile applications that allow patient data to be assessed remotely by health care providers.
Controlling Progression of Myopia: Optical and Pharmaceutical Strategies Sankaridurg, Padmaja; Conrad, Fabian; Tran, Huy; Zhu, Jianfeng
The burden associated with the rising prevalence of myopia and high myopia, and the associated vision impairment and sight-threatening complications, has triggered the need to evaluate strategies to control the progression of myopia. We provide an overview of the literature on the use of optical (spectacles, contact lenses, and orthokeratology) and pharmaceutical approaches to slow progress of myopia. The evidence indicates that myopia progression can be slowed by varying degrees using
these strategies. All approaches play a role in the management of myopia as needs and requirements of an individual vary based on age, suitability, affordability, safety of the approach, subjective needs of the individual, and rate of progression. This review also identifies and discusses the lack of long-term efficacy data and rebound on discontinuation of myopia control products.
Diagnosis and Treatment of Myopic Maculopathy Yokoi, Tae; Ohno-Matsui, Kyoko
Visual impairment resulting from pathological myopia is a serious issue worldwide. This is mainly due to the development of different types of myopic maculopathy. Despite being a major cause of visual impairment worldwide, myopic maculopathy was not consistently defined until 2015. To overcome this problem, the Meta-Analysis for Pathologic Myopia study group proposed a simplified, uniform classification system for myopic maculopathy. Among several lesions of myopic maculopathy, myopic choroidal neovascularization (CNV) is 1 of the most common and severe vision-threatening complications. Recent large clinical trials have reported a good initial efficacy for visual acuity improvement by application of anti–vascular endothelial growth factor therapy for myopic CNVs. However, long-term clinical studies demonstrate a gradual decrease in the visual acuity gain and return to the baseline visual acuity due to a development of myopic CNV-related macular atrophy. Regarding visual impairment caused by advanced myopic chorioretinal atrophy, the only way to prevent blindness is to prevent myopia from developing or progressing at a young age before the axial length elongates extremely. As peripapillary diffuse atrophy in childhood may be an indicator of more advanced myopic chorioretinal atrophy in later life, some preventive measures should be considered in such children.
Simulation in Ophthalmic Training Flanagan, Judith L.; De Souza, Neilsen
Vision impairment and blindness arise both as a cause, and a consequence, of poverty. Achievement of the United Nations Sustainable Development Goals in providing universal access and equity in eye care, both within and between among countries, remains challenging. A severe shortage of eye care providers is creating unnecessary blindness and vision impairment in developing communities worldwide. Education
and training develops and strengthens the capacity of emerging nations to contribute to global eye health and the World Health Organization Development Goals in an effective and sustainable way. Although relative to other medical professions, adoption of simulation in ophthalmic training has been relatively slow, simulation potentially offers reduced training costs, increased accessibility, objective measurement of training
outcomes, and improvements in patient safety during and after clinician training, all of which can help address the global burden of vision impairment and blindness. Simulation training offers advantages over apprenticeship models, the traditional mode of transferring knowledge and skills in medicine and health, which suffers from imperfect transference due to inherent biases, heuristic and idiosyncratic expectations of experts, and subjective measures of outcomes. Simulation does not completely do away with these confounders because it is made to fit into established curricula, making it difficult to measure effectiveness of the simulation in isolation. The power of simulation training for resource-limited regions and countries is immense in offering cost-effective training in-country; however, it is important that any such tools are developed within the context of the limitations in situ.
Fenofibrate for Diabetic Retinopathy Stewart, Stephen; Lois, Noemi
Fenofibrate is a safe and inexpensive orally administered fibric acid derivative conventionally used to treat dyslipidemia. Two large randomized clinical trials, the Fenofibrate Intervention and Event Lowering in Diabetes (FIELD) and the Action to Control Cardiovascular Risk in Diabetes (ACCORD) studies, demonstrated the benefit of oral fenofibrate in the treatment of people with type 2 diabetes and diabetic retinopathy (DR), including reduced disease progression and need for laser treatment for diabetic macular edema (DME) and proliferative diabetic retinopathy (PDR). These findings are supported by results of experimental studies, which have demonstrated beneficial effects of fenofibrate ameliorating retinal vascular leakage and leukostasis, downregulating vascular endothelial growth factor (VEGF), and reducing endothelial cell and pericyte loss, among others—all characteristic features of DR. Fenofibrate is not prescribed routinely for the treatment of patients with diabetes and DR, despite this evidence. In FIELD and ACCORD, retinopathy was not the primary outcome and this may explain, at least partly, its lack of use for this indication. New trials are now underway to specifically address the effects of fenofibrate in DR; these trials will provide additional and robust data that may support current evidence favoring the use of fenofibrate in this common microvascular complication of diabetes.
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