The Asia-Pacific Journal of Ophthalmology

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Original Study - Clinical

Blindness and Visual Impairment Profile and Rapid Assessment of Avoidable Blindness in South East Asia: Analysis of New Data. 2017 APAO Holmes Lecture. Das, Taraprasad
Purpose: The International Agency for Prevention of Blindness (IAPB) South East Asia region (SEAR) that consists of 11 countries contains 26% of the world’s population (1,761,000,000). In this region 12 million are blind and 78.5 million are visually impaired. This amounts to 30% of global blindness and 32% of global visual impairment.
Design: Rapid assessment of avoidable blindness (RAAB) survey analysis.
Methods: RAAB, either a repeat or a first time survey, was completed in 8 countries in this decade (2010 onwards). These include Bangladesh, Bhutan, India, Indonesia, Maldives, Sri Lanka, Thailand, and Timor Leste.
Results: Cataract is the principal cause of blindness and severe visual impairment in all countries. Refractive error is the principal cause of moderate visual impairment in 4 countries: Bangladesh, India, Maldives, and Sri Lanka; cataract continues to be the principal cause of moderate visual impairment in 4 other countries: Bhutan, Indonesia, Thailand, and Timor Leste. Outcome of cataract surgery is suboptimal in the Maldives and Timor Leste.
Conclusions: Rigorous focus is necessary to improve cataract surgery outcomes and correction of refractive error without neglecting the quality of care. At the same time allowances must be made for care of the emerging causes of visual impairment and blindness such as glaucoma and posterior segment disorders, particularly diabetic retinopathy.
First Rapid Assessment of Avoidable Blindness Survey in the Maldives: Prevalence and Causes of Blindness and Cataract Surgery Thoufeeq, Ubeydulla; Das, Taraprasad; Limburg, Hans; Maitra; Maharshi; Panda, Lapam; Sil, Asim; Trevelyan John; Sapkota, Yuddha
Purpose: A nationwide rapid assessment of avoidable blindness survey was undertaken in the Maldives among people aged 50 years or more to assess the prevalence and causes of blindness and visual impairment, cataract surgical coverage, cataract surgery outcome, and barriers to uptake of cataract surgical services.

Design: Prospective population-based study.

Methods: In the cluster sampling probability proportionate to size method, 3100 participants in 62 clusters across all 20 atolls were enrolled through house-to-house visits. They were examined in clusters by an ophthalmologist-led team. Data was recorded in mRAAB version 1.25 software on a smartphone.

Results: The age-sex standardized prevalence of blindness was 2.0% [95% confidence interval (CI), 1.5-2.6]. Cataract was the leading cause of blindness (51.4%) and uncorrected refractive error was the leading cause of visual impairment (50.9%). Blindness was more prevalent in higher age groups and women (16.3%). Cataract surgical coverage was 86% in cataract blind eyes and 93.5% in cataract blind persons. Good visual outcome in cataract operated eyes was 67.9% (presenting) and 76.6% (best corrected visual acuity). In this study, 48.1% of people had received cataract surgery in neighboring countries. Important barriers for not using the services were “did not feel the need” (29.7%) and “treatment deferred” (33.3%).

Conclusions: Cataract surgical coverage is good, though nearly half the people received surgery outside the Maldives. Cataract surgery outcomes are below World Health Organization standards. Some barriers could be overcome with additional human resources and training to improve cataract surgical outcomes, which could encourage greater uptake of services within the country.
Predictive Value of Baseline Biochemical Parameters for Clinical Response of Macular Edema to Bevacizumab in Eyes With Central Retinal Vein Occlusion: A Retrospective Analysis Madanagopalan, V.G.; Kumari, Bibha

Purpose: To evaluate the influence of baseline biochemical parameters on the improvement in best corrected visual acuity (BCVA) and reduction in central foveal thickness (CFT) in patients treated with intravitreal bevacizumab (IVB; Avastin, Genentech, Inc) for macular edema (ME) secondary to central retinal vein occlusion (CRVO).

Design: A retrospective study.

Methods: Seventy eyes of 70 participants with CRVO who underwent IVB for ME and had at least 1 month of follow-up after the last injection were studied. Demographic variables, systolic and diastolic blood pressure (SBP and DBP), BCVA, CFT measured by optical coherence tomography (OCT), and biochemical investigations [hemoglobin, fasting and postprandial blood sugar (FBS and PPBS), lipid profile, blood urea (BU), serum creatinine (SC), glycosylated hemoglobin (HbA1c)] at baseline were noted. IVB need at every visit was based on clinical and OCT parameters. Changes in BCVA (ΔBCVA) and CFT (ΔCFT) from baseline to last injection were estimated.

Results: After IVB, there was a statistically significant reduction in mean CFT (P < 0.01). The group of patients with normal BU and SC had more than 2 lines of improvement compared with those with elevated values (P = 0.043 and 0.009, respectively). Other parameters like FBS, PPBS, hemoglobin, HbA1c, and serum lipids were not associated with improvement of BCVA and reduction of CFT.

Conclusions: Normal baseline renal parameters (BU and SC) predict better visual outcome after treatment for ME in CRVO and offer additional benefit over and above that obtained with ME reduction.

Review Article

Neuromyelitis Optica: Review and Utility of Testing Aquaporin-4 Antibody in Typical Optic Neuritis Seay, Meagan; Rucker, Janet C.
Neuromyelitis optica (NMO) is an autoimmune, inflammatory demyelinating disorder often leading to severe vision impairment and disability. The discovery of a diagnostic biomarker, the aquaporin-4 antibody (AQP4-IgG), transformed the clinical diagnosis and treatment of NMO and broadened the spectrum of disease [NMO spectrum disorders (NMOSD)]. Though the antibody is highly sensitive and specific to NMOSD, routine testing in patients with typical optic neuritis is considered controversial. This article will provide a brief review of NMOSD and highlight the pros and cons of routine testing in typical optic neuritis.
Retinoblastoma for Pediatric Ophthalmologists AlAli, Alaa; Kletke, Stephanie; Gallie, Brenda; Lam, Wai-Ching
Retinoblastoma can present in 1 or both eyes and is the most common intraocular malignancy in childhood. It is typically initiated by biallelic mutation of the RB1 tumor suppressor gene, leading to malignant transformation of primitive retinal cells. The most common presentation is leukocoria, followed by strabismus. Heritable retinoblastoma accounts for 45% of all cases, with 80% being bilateral. Treatment and prognosis of retinoblastoma is dictated by the disease stage at initial presentation. The 8th Edition American Joint Committee on Cancer (AJCC) TNMH (tumor, node, metastasis, heritable trait) staging system defines evidence-based clinical and pathological staging for overall prognosis for eye(s) and child. Multiple treatment options are available in 2018 for retinoblastoma management with a multidisciplinary team, including pediatric ocular oncology, medical oncology, radiation oncology, genetics, nursing, and social work. Survival exceeds 95% when disease is diagnosed early and treated in centers specializing in retinoblastoma. However, survival rates are less than 50% with extraocular tumor dissemination. We summarize the epidemiology, genetics, prenatal screening, diagnosis, classification, investigations, and current therapeutic options in the management of retinoblastoma.
Treatment of Nonarteritic Acute Central Retinal Artery Occlusion Sharma, Rahul A.; Dattilo, Michael; Newman, Nancy J.; Biousse, Valerie
Central retinal artery occlusion (CRAO) is an event most often caused by an embolus originating in the ipsilateral carotid artery, aortic arch, or heart. CRAO may result from partial or complete occlusion of the central retinal artery (CRA), which acts as the primary blood supply to the inner neurosensory retina, and typically results in profound vision loss and permanent visual disability. No consensus has emerged regarding the optimal treatment of CRAO. All proposed treatments are of questionable efficacy and many have uncertain risk profiles. In certain circumstances, thrombolysis may be attempted as a treatment option; however, the evidence to support broad use of thrombolytics in the treatment of acute CRAO remains elusive. It is known that the risk factors that predispose to other cardiovascular and cerebrovascular events are often present in CRAO. Accordingly, identification of patients at highest risk of stroke and secondary prevention of ischemic events remains the primary focus of management. This review offers a summary of the clinical presentation, diagnosis, and prognosis of CRAO, with an emphasis on treatment options.
Pharmacotherapy and ROP: Going Back to the Basics Shulman, Julia P.; Hartnett, M. Elizabeth
Retinopathy of prematurity (ROP) is a leading cause of blindness in preterm infants around the world. Through the development of animal models and clinical trials our understanding of the pathophysiology of this disease and approach to therapy has evolved significantly since ROP was first described in the 1940s in the United States. The mainstay of treatment in ROP remains ablative laser therapy to the avascular retina but pharmacologic agents are being more and more commonly used with new targets for pharmacotherapy emerging. This paper summarizes our current understanding of the pathophysiology of ROP based on the data gleaned from animal models and discusses current approaches to pharmacotherapy
Pediatric Uveitis Chan, Nicole Shu-Wen; Choi, Jessy; Cheung, Chui Ming Gemmy
Pediatric uveitis differs from adult-onset uveitis and is a topic of special interest because of its diagnostic and therapeutic challenges. Children with uveitis are often asymptomatic and the uveitis is often chronic, persistent, recurrent, and resistant to conventional treatment. Anterior uveitis is the most common type of uveitis in children; the prevalence of intermediate, posterior, and panuveitis varies geographically and among ethnic groups. Regarding etiology, most cases of pediatric uveitis are idiopathic but can be due to systemic inflammatory disorders, infections, or a manifestation of masquerade syndrome. Ocular complications include cataracts, hypotony or glaucoma, band keratopathy, synechiae formation, macular edema, optic disc edema, choroidal neovascular membranes, and retinal detachment. These complications are often severe, leading to irreversible structural damage and significant visual disability due to delayed presentation and diagnosis, persistent chronic inflammation from suboptimal treatment, topical and systemic corticosteroid dependence, and delayed initiation of systemic disease‒modifying agents. Treatment for noninfectious uveitis is a stepwise approach starting with corticosteroids. Immunomodulatory therapy should be initiated in cases where quiescence cannot be achieved without steroid dependence. Patients should be monitored regularly for complications of uveitis along with systemic and ocular adverse effects from treatments. The goals are to achieve steroid-free durable remission, to reduce the risk of sight-threatening complications from the uncontrolled ocular inflammation, and to avoid the impact of lifelong burden of visual loss on the child and their family. Multidisciplinary management will ensure holistic care of affected children and improve the support for their families.
Updates and Controversies in the Management of Acute Optic Neuritis Meltzer, Ethan; Prasad, Sashank
Optic neuritis remains a common diagnosis with controversial management. Although typical optic neuritis is often associated with “good” recovery of visual acuity, patients are often left with persistent impairments of contrast sensitivity, color vision, and visual field. These permanent visual deficits correlate with structural injury to the anterior visual pathway and are closely linked to visual quality of life. High dose corticosteroids are commonly used for patients with acute optic neuritis. However, even several decades after the initial clinical trials, there remains significant controversy regarding the efficacy and utility of this treatment. There is a need for more effective treatments, and many new immunomodulatory and neuroprotective agents have been investigated recently. Atypical optic neuritis, such as that seen with neuromyelitis optica spectrum disorder, often requires more aggressive initial treatment. Thus, it is important for clinicians to have a framework for rapid diagnosis and triage of patients who present with typical or atypical optic neuritis. Lastly, optic neuritis is associated with an elevated long-term risk of developing multiple sclerosis. Some patients may benefit from initiation of medications targeting multiple sclerosis at the time of initial presentation of optic neuritis. Appropriate identification and treatment of patients at highest risk of developing multiple sclerosis may help impact their disease course, while limiting exposure to potential adverse effects in patients who are at lower risk and do not require disease-modifying treatment.
Vascular Anomalies of the Orbit—A Reappraisal Sullivan, Timothy John
The recent International Society for the Study of Vascular Anomalies (ISSVA) classification of vascular anomalies can be applied to orbital lesions, dividing them into vascular tumors and vascular malformations. Orbital cavernous hemangiomas are probably best considered cavernous venous malformations under this classification. Management of symptomatic lesions can be with surgical excision or stereotactic fractionated radiotherapy in selected cases. Beta-blockers including propranolol and topical timolol maleate represent first-line therapy for infantile hemangiomas, although surgery has a role in selected cases. Orbital venous-lymphatic malformations are problematic but with improved imaging, neuroradiological intervention, and a multidisciplinary approach to management, outcomes are improving.
Visual Evoked Potentials and Glaucoma Tai, Tak Yee Tania
Visual evoked potentials (VEP) can be used to assess the function of the visual system objectively. Research on VEP testing as a method of glaucoma evaluation has been performed for many years. Pattern VEP has shown good specificity and sensitivity in the detection of glaucoma in some studies, but other studies have not shown similar efficacy. Multifocal VEP can produce a topographical measure of glaucomatous damage and has been shown to be able to detect a similar number of defects in patients with glaucoma or ocular hypertension as compared with the visual field test. Despite promising data on these VEP test modalities in the assessment of glaucoma, multiple aspects of test administration make their routine use impractical in a clinical setting. New VEP testing modalities, such as short-duration transient VEP and isolated-check VEP, allow the test to be performed more quickly and easily. Further research on these more recent technologies may allow us to use VEP effectively in the diagnosis and management of glaucoma.
Congenital X-Linked Retinoschisis: An Updated Clinical Review Rao, Prethy; Dedania, Vaidehi S.; Drenser, Kimberly A.
We present an updated clinical review of the pathophysiology, progression, and current treatments in pediatric patients with congenital X-linked retinoschisis (CXLRS). CXLRS is an X-linked inherited retinal degeneration characterized by splitting of the superficial layers of the retina. Most recent classification divides CXLRS into 4 distinct clinical phenotypes: type 1, foveal; type 2, foveolamellar; type 3, complex; and type 4, foveoperipheral. The majority of retinoschisis cavities remain stable throughout life and may spontaneously collapse. However, a select number of patients progress to macula-involving peripheral retinoschisis, rhegmatogenous, and combined tractional-rhegmatogenous detachments that require further intervention. Although several advances have been made over the past several decades, medical therapy remains limited to case series‒based carbonic anhydrase therapy and prophylactic laser retinopexy. Recent advances in genetic-based clinical trials with the retinoschisis gene are promising. Vitreoretinal surgical approaches remain complex, case-based, and require careful planning depending on the configuration and location of the retinoschisis cavity.
Familial Exudative Vitreoretinopathy: Pathophysiology, Diagnosis, and Management Tauqeer, Zujaja; Yonekawa, Yoshihiro
Familial exudative vitreoretinopathy (FEVR) is a heritable vitreoretinopathy characterized by anomalous retinal vascular development. The principal feature of the disease is an avascular peripheral retina. This in turn can cause further pathological changes including neovascularization, exudation, hemorrhage, and retinal detachment. The biological basis of the disease is thought to be from defects in the Wnt signaling pathway. Many gene mutations have been implicated, and these can be inherited in an autosomal dominant (most common), autosomal recessive, and X-linked recessive fashion. Examination with wide-field fluorescein angiography is essential and can identify the disease in its earlier stages, enabling timely treatment, in addition to helping identify asymptomatic family members. The current treatment paradigm involves laser photocoagulation of the avascular peripheral retina for neovascular sequelae and vitreoretinal surgery for progressive retinal detachment. Further studies are underway to better characterize this complex vitreoretinopathy.
Anti-VEGF for ROP and Pediatric Retinal Diseases Wu, An-Lun; Wu, Wei-Chi
Vascular endothelial growth factor (VEGF) is an important mediator of the pathological neovascularization and vascular permeability of the eye. The increasing use of intravitreal therapies targeting VEGF has revolutionized the treatment of pediatric vitreoretinal diseases. In retinopathy of prematurity (ROP), the role of VEGF in pathogenesis has been well recognized and the use of anti-VEGF treatment in phase 2 ROP has demonstrated promising results, particularly in severe cases of posterior disease. This has made VEGF an established target in the treatment of pediatric retinal diseases known to have abnormal vascular activity. However, questions remain about late systemic and neurodevelopmental effects after anti-VEGF therapy for children because intravitreal injection of anti-VEGF may result in systemic circulation of anti-VEGF agent and a corresponding suppression of systemic VEGF. We do not currently know whether the short-term suppression of systemic VEGF affects long-term neurodevelopmental outcome because VEGF is a vital supporting factor during neurodevelopment. This review article focuses on the evidence for the use of anti-VEGF treatment in certain pediatric ocular diseases, including ROP, Coats' disease, and retinoblastoma (RB). More extensive and prospective studies are warranted to further elucidate the role of anti-VEGF therapy in these diseases and illustrate how we can optimally use these agents in pediatric patients.
Surgical Management of Coats Disease Kusaka, Shunji
Coats disease is a sporadic, chronic retinal vascular disorder with telangiectatic and aneurysmal retinal vessels, with retinal exudation and retinal detachment in severe cases. Various treatment modalities have been used, including laser therapy, cryotherapy, anti-vascular endothelial growth factor (VEGF) therapy, and surgery. Anti-VEGF therapy seems to be effective in reducing exudative changes and is often combined with other treatment modalities. For severe cases with exudative retinal detachment, external drainage of subretinal fluid with or without vitrectomy is commonly performed, combined with laser therapy and/or cryotherapy. Relatively good anatomical outcomes have been reported; however, postoperative functional results, particularly in young patients and/or severe cases, seem to be limited.
Caring for Hereditary Childhood Retinal Blindness Jauregui, Ruben; Cho, Galaxy Y.; Takahashi, Vitor K.L.; Takiuti, Julia T.; Bassuk, Alexander G.; Mahajan, Vinit B.; Tsang, Stephen H.
Inherited retinal diseases (IRDs) are a major cause of incurable familial blindness in the Western world. In the pediatric population, IRDs are a major contributor to the 19 million children worldwide with visual impairment. Unfortunately, the road to the correct diagnosis is often complicated in the pediatric population, as typical diagnostic tools such as fundus examination, electrodiagnostic studies, and other imaging modalities may be difficult to perform in the pediatric patient. In this review, we describe the most significant IRDs with onset during the pediatric years (ie, before the age of 18). We describe the pathogenesis, clinical presentation, and potential treatment of these diseases. In addition, we advocate the use of a pedigree (family medical history), electroretinography, and genetic testing as the 3 most crucial tools for the correct diagnosis of IRDs in the pediatric population.
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