The visual system has high metabolic requirements and is therefore particularly vulnerable to mitochondrial dysfunction. The most commonly affected tissues include the extraocular muscles, photoreceptors, retinal pigment epithelium, optic nerve and visual cortex. Hence, the most common manifestations of mitochondrial disorders are progressive external ophthalmoplegia, macular pattern dystrophy, pigmentary retinopathy, optic neuropathy and retrochiasmal visual field loss. With the exception of Leber hereditary optic neuropathy and stroke-like episodes seen in mitochondrial encephalopathy, lactic acidosis and stroke-like episodes, the majority of neuro-ophthalmic manifestations have an insidious onset. As such, some patients may not recognize subtle progressive visual symptoms. When mitochondrial disorders are highly suspected, meticulous examination performed by an ophthalmologist with targeted ancillary testing can help confirm the diagnosis. Similarly, neuro-ophthalmic symptoms and signs may be the first indication of mitochondrial disease and should prompt systemic investigations for potentially life-threatening associations, such as cardiac conduction defects. Finally, the ophthalmologist can offer symptomatic treatments for some of the most disabling manifestations of these disorders.

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Idiopathic intracranial hypertension (IIH) is a disorder of unknown etiology that results in isolated raised intracranial pressure. Classic symptoms and signs of IIH include headache, papilledema, diplopia from sixth nerve palsy and divergence insufficiency, and pulsatile tinnitus. Atypical presentations include: (1) highly asymmetric or even unilateral papilledema, and IIH without papilledema; (2) ocular motor disturbances from third nerve palsy, fourth nerve palsy, internuclear ophthalmoplegia, diffuse ophthalmoplegia, and skew deviation; (3) olfactory dysfunction; (4) trigeminal nerve dysfunction; (5) facial nerve dysfunction; (6) hearing loss and vestibular dysfunction; (7) lower cranial nerve dysfunction including deviated uvula, torticollis, and tongue weakness; (8) spontaneous skull base cerebrospinal fluid leak; and (9) seizures. Although atypical findings should raise a red flag and prompt further investigation for an alternative etiology, clinicians should be familiar with these unusual presentations.

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The multifocal intraocular lenses (IOLs) available are often able to restore visual function and allow spectacle independence after their implantation with great levels of patient satisfaction. The factors associated with the postoperatory success include the careful selection of the patient, the knowledge about the IOLs' design, and their visual performance added to the proper surgical technique and management of possible complications as demonstrated by the evidence available.

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PURPOSE: The purpose is to evaluate functional changes after ocriplasmin injection to treat vitreomacular traction (VMT) by microperimetry. MATERIALS AND METHODS: Prospective interventional study on patients underwent an intravitreal ocriplasmin injection. Optical coherence tomography, best-corrected visual acuity (BCVA) test, and microperimetry were performed at baseline, 1 week, 1 and 3 months. Microperimeter recorded retinal sensitivity (RS) and central retinal sensitivity (CRS) at central 12° and 4°, respectively, and fixation as bivariate contour ellipse area (BCEA) at 68%, 95%, and 99% of fixation points. Functional parameters were analyzed in patients who had (Group A) or not (Group B) VMT release. RESULTS: Twenty-one patients including 18 with VMT and 3 with VMT plus macular hole (MH) were treated. Eleven patients achieved VMT resolution including all cases with MH that achieved hole closure. An impairment of BCVA, RS and CRS (P < 0.01; P < 0.001; P = 0.001, respectively) was reported at 1 week followed by a significant improvement (BCVA, P = 0.001; RS, CRS, P = 0.02) at 3 months. The early impairment of visual acuity and sensitivity significantly occurred in Group B (P ≤ 0.01) while their recovery significantly occurred in Group A (P < 0.01). BCEA significantly increased in dimension (68%, P = 0.01; 95%, P = 0.03) at 1 week, subsequently returning to near baseline values over follow-up. Only in Group A, fixation stability significantly improved at 3 months. CONCLUSION: Microperimetry confirms an early and reversible functional impairment after ocriplasmin injection regardless VMT resolution. If a greater decrease in function could occur in the eyes without VMT resolution, a better functional recovery could occur in the event of VMT resolution.

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Gauging stromal dissection depth is crucial to successfully perform deep anterior lamellar keratoplasty (DALK) surgery. Intraoperative optical coherence tomography (iOCT) offers a promising tool to aid DALK surgery but visualization of surgical maneuvers is impaired due to artifacts from metallic instruments. We describe a novel surgical technique utilizing suture-assisted iOCT guidance that facilitates clear visualization of corneal dissection planes during DALK. A stromal dissection tunnel is performed with a Fogla probe and its depth is subsequently identified by threading a 1 cm segment of 8-0 nylon into the tunnel. In contrast to the Fogla probe, the 8-0 nylon is conspicuously highlighted on iOCT. If the tunnel is too superficial, a separate, deeper stromal tunnel can be created and visualized again with the 8-0 nylon suture and iOCT. This iterative process facilitates a deep stromal dissection, increasing the probability of successful big-bubble formation and Descemet baring DALK surgery. This technique was utilized for a successful big-bubble DALK in a patient with severe keratoconus.

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Trimethoprim-sulfamethoxazole (TMP-SMX) is a commonly used antimicrobial agent because of its low cost, diverse antimicrobial profile, and minimal severe adverse effects. A rare side effect is psychosis, a complication that has not been published in the ophthalmology literature. A 53-year-old female presented to the ophthalmology office with left upper eyelid erythema, focal tenderness, and discharge. She was diagnosed with preseptal cellulitis of the left upper lid and started on TMP-SMX. The next day, the patient's condition improved with reduced swelling and no discharge. However, 2 days later, she experienced visual hallucinations whereby worms were growing out of her left eye accompanied by theme-congruent tactile hallucinations. TMP-SMX was discontinued and substituted for clindamycin, and she reported resolution of her symptoms 8 h later. TMP-SMX has extensive cerebrospinal fluid penetration and causes a folic acid deficiency, which may explain the rare occurrence of neuropsychiatric side effects. This patient had a substance-induced psychosis, in which visual and tactile hallucinations began 3 days after taking TMP-SMX and resolved 8 h after discontinuation, a timeline consistent with the literature. Central nervous system toxicity is rare in nonelderly immunocompetent patients, with only three such cases reported in the literature. While visual and auditory hallucinations have been described previously, this is the first reported case of TMP-SMX-induced tactile hallucinations and unilateral visual hallucinations. Moreover, because TMP-SMX is a first-line agent commonly used to treat orbital and preseptal cellulitis, it is important for ophthalmologists to be aware of this atypical side effect, as it can be life threatening.

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Polypoidal choroidal vasculopathy (PCV) is a subtype of neovascular age-related macular degeneration (nAMD), commonly seen in the Asian population. It is dissimilar in epidemiology, genetic heterogeneity, pathogenesis, natural history, and response to treatment in comparison to nAMD. Confocal scanning laser ophthalmoscopy-based simultaneous fluorescein angiography and indocyanine green angiography, spectral-domain optical coherence tomography (OCT) with enhanced depth imaging, swept-source OCT, and OCT angiography have improved the ability to detect PCV, understand its pathology, and monitor treatment response. A plethora of literature has discussed the efficacy of photodynamic therapy, anti-vascular endothelial growth factor (VEGF) monotherapy, and combination of both, but only a few studies with higher level of evidence and limited follow-up duration are available. This review discusses the understanding of PCV with respect to epidemiology, pathogenesis, clinical features, natural history, imaging techniques, and various treatment options. Recent clinical trials (EVEREST-II and PLANET study) have emphasized that either anti-VEGF monotherapy or combination treatment is equally capable to strike a balance between polyp regression and stabilization of visual acuity. The recurrent nature of the disease, the development of macular atrophy, and the long-term poor visual prognosis despite treatment are concerns that open avenues for further research.

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Pseudopapilledema is a fairly common finding in ophthalmic practice, and in many cases, the diagnosis is straightforward. However, an accurate diagnosis can challenge the most seasoned clinicians, and missing true papilledema can result in life-threatening or vision-threatening consequences. In this review, we describe the clinical findings and a diagnostic algorithm to distinguish pseudopapilledema and papilledema in the pediatric patients. We also describe the clinical evaluation once a diagnosis of papilledema has been established.

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Optical coherence tomography angiography (OCTA) is a functional extension of OCT that provides information on retinal and choroidal circulations without the need for dye injections. With the recent development of high-speed OCT systems and efficient algorithms, OCTA has become clinically feasible. In this review article, we discuss the technical principles of OCTA, including image processing and artifacts, and its clinical applications in ophthalmology. We summarize recent studies which qualitatively or quantitatively assess disease presentation, progression, and/or response to treatment.

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Glaucoma suspects are controversial clinical dilemmas. These individuals harbor certain risk factors or demonstrate some clinical features suggestive of an increased probability to develop glaucomatous optic atrophy in the future. These characteristics range from high intraocular pressure; optic disc, visual field, or retinal nerve fiber layer abnormalities; or abnormal angles to a positive family history of glaucoma and other risk factors. Individuals having these characteristics should be assessed diligently before a diagnosis of glaucoma is made. Glaucoma is a chronic, lifelong condition, having a negative impact on the quality of life, with an increased risk of medication-related side-effects, adverse economic impacts, and the need for lifestyle changes in the patient. Overdiagnosis and unnecessary treatment of such individuals is bereft of any advantage. This review aims to provide a practical blueprint for the proper diagnosis and management of such glaucoma suspects.

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Foveal hypoplasia is a retinal disorder in which there is a lack of full development of the morphology of the fovea. The optical coherence tomography (OCT) and functional findings are presented in relation to the underlying genetic and developmental conditions. Recent advancements of high-resolution OCT imaging have unveiled characteristics of foveal hypoplasia that were not detected by conventional imaging methods. An absence of a foveal pit does not necessarily imply poor visual acuity, and the maturation of the cone photoreceptors is important for the visual acuity. Regardless of the degree of the development of the inner retinal layers, the visual acuity can be preserved as in diseases such as Stickler syndrome that is a newly identified retinal disorder associated with foveal hypoplasia.

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Presbyopia results from loss or insufficiency of the eye's accommodative ability, and clinically manifests as the inability to focus near objects on the retina. It is one of the most common causes of visual impairment worldwide especially in adults of productive or working age. Various means of compensating for the loss of accommodative ability have been devised from optical tools such as spectacles and contact lenses, to topical medications and to surgical procedures. A comprehensive search on journal articles about topical and surgical correction of presbyopia was undertaken. The various techniques for presbyopia correction, as enumerated in these articles, are discussed in this paper with the addition of our personal experience and perspective on the future of these techniques.

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The continued development of intraocular lens (IOL) technology has led to a dramatic improvement in refractive outcomes. New and innovative ways of achieving the desired postoperative refractive goals continue to be developed. This article aims to review the currently available IOL modalities for correction of presbyopia at the time of cataract surgery, including reference to high-quality comparative studies, where available, and discussion of strengths as well as limitations of the currently available IOL technologies. It has been shown that multifocal compared to monofocal IOL was associated with higher rates of spectacle independence, but higher rates and severity of symptomatic glare as well as reduced contrast sensitivity. Within multifocal IOLs, diffractive compared to refractive IOLs tended to have better near vision and a lower rate of symptomatic glare. Extended depth-of-focus IOLs compared to diffractive multifocal IOL demonstrated equal or superior intermediate visual acuity, with less than or equal rates of glare. Accommodative IOLs represent a broad range of technologies that continue to develop, and new technologies offering opportunities for postoperative adjustment of refractive outcome are emerging.

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Unilateral congenital ptosis with poor levator function of ≤4 mm continues to be a difficult challenge for the oculoplastic surgeon. Surgical correction can be accomplished with unilateral frontalis suspension, maximal levator resection, or bilateral frontalis suspension with or without levator muscle excision of the normal eyelid. Bilateral frontalis suspension was proposed by Beard and Callahan to overcome the challenge of postoperative asymmetry, allowing symmetrical lagophthalmos on downgaze, postoperatively. However, most surgeons and patients prefer unilateral correction on the abnormal eyelid either with a frontalis suspension or maximal levator resection. Frontalis suspension may be performed through the various surgical techniques using different autogenous or exogenous materials. Autogenous fascia lata is considered the material of choice with low recurrence rates but carries the drawbacks of the difficulty of harvesting and postoperative morbidity from the second surgical site. Recent reports have suggested that maximal levator resection provides improved cosmesis, a more natural contour, and avoids brow scars. Although both treatments have shown to have similar success rates, there is much debate about what the most favorable method for treating severe unilateral ptosis. We review the literature on the various surgical treatments for unilateral severe congenital ptosis, including the rationale, advantages and disadvantages of each technique.

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Myasthenia gravis (MG) is an autoimmune disease involving the neuromuscular junction. Autoantibodies to the acetylcholine receptor or, less frequently, to muscle-specific kinase, attack against the postsynaptic junctional proteins, resulting in fluctuating and variable weakness of muscles. Extraocular, levator palpebrae superioris, and orbicularis oculi muscles are particularly susceptible. The majority of patients with MG present with purely ocular symptoms including ptosis and diplopia initially. About half of these patients progress to generalized disease within 2 years. The prevalence of MG in Taiwan is 140 per million with male to female ratio of 0.7. The incidence rate is higher in the elderly. Several immune-related diseases such as lymphoid malignancy, diabetes, and thyroid diseases are associated with MG in the national population-based studies in Taiwan. Ice pack test, rest test, Tensilon/neostigmine test, circulating antibody measurement, and electrophysiological studies are useful diagnostic tools with variable sensitivity and specificity. For the patients with ocular MG, acetylcholinesterase inhibitors are usually the first-line treatment. Corticosteroids and immunosuppressant could provide better disease control and may reduce the risk of conversion to generalized form although there is still some controversy. A thymectomy is also beneficial for ocular MG, especially in refractory cases. The correction of ptosis and strabismus surgery could improve the visual outcome but should be performed only in stable disease.

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The tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disorder that is probably underdiagnosed in clinical practice. Ocular involvement in TINU syndrome not only presents with the nongranulomatous anterior uveitis in 80% of patients but also manifests as intermediate, posterior, or panuveitis. This case report mentions an adult male patient who presented with granulomatous iridocyclitis with panuveitis and mild renal insufficiency. Workup for connective tissue and infectious diseases was negative for the patient. He was diagnosed with TINU syndrome based on the findings of renal biopsy. Both the uveitis and nephritis promptly responded well to steroid treatment, and there was no recurrence during the follow-up of 24 months.

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Duane syndrome (DS) is a common form of congenital cranial dysinnervation disorders. The ocular motility pattern lies in a wide clinical spectrum, and the choice of treatment must be individualized depending on the severity of the clinical findings. There is no perfect method of treatment and no real “cure” in DS. In this paper, the aim is to give some guidelines to the reader for selection of the most appropriate treatment method for the patient.

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In recent years, there has been increasing scientific interest in the use of tear film biomarkers in the diagnosis and management of dry eye disease (DED), owing to their potential important roles in the pathogenesis of ocular surface damage, as well as the technical feasibility of tear sample collection techniques. An Entrez PubMed search was conducted on March 2, 2019, to include papers investigating the use of tear film biomarkers in DED, and the results were classified according to whether the DED is associated with systemic inflammatory disease or not and further classified within each section according to the molecular nature of the biomarker for further discussion. A total of 58 relevant articles were reviewed. Certain cytokines, including interleukin-6 (IL-6), tumor necrosis factor-alpha, IL-17, and IL-8, were found by a number of studies to consistently reflect disease severity well and had strong correlations with tear film metrics and tests for ocular surface damage in dry eye without systemic inflammatory disease. For dry eye with systemic inflammatory disease, IL-17, IL-8, and IL-1 receptor antagonists were shown to be consistently higher in affected eyes and correlated well with ocular surface disease severity in more than one type of inflammatory disease. With the advancement in technology and lowered costs in the future, tear film biomarker counts would allow better diagnosis and monitoring of DED, as well as facilitate personalized treatment strategies.

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PURPOSE: Although Taiwan was one of the first countries to develop coronavirus disease 2019 (COVID-19), with effective antiepidemic measures, Taiwan has effectively controlled the spread of the disease. The purpose of this article is to provide useful safety strategies for ophthalmologists in daily practice during the COVID-19 pandemic. MATERIALS AND METHODS: Infection control strategies in the hospital and Department of Ophthalmology, Chang Gung Memorial Hospital, Linkou, are discussed. RESULTS: Ophthalmologists are at high risk of contracting COVID-19 infection, as they have close contact with patients during ocular examinations, and are also facing high patient volume in outpatient clinics as well as emergency consultations. Furthermore, ocular symptoms, such as conjunctivitis, may be the presenting signs of COVID-19 infection. We provide our strategies, which include hospital's gate control with triage station, patient volume control, proper personal protective equipment, and consultation with telemedicine technology, to decrease the risk of cross-infection between medical staffs and patients. CONCLUSION: To achieve the goal of preventing viral spread and maximizing patient and medical staffs' safety, besides providing proper protective equipment, it is also crucial for staffs and patients to strictly follow antiepidemic measures. We hope that our experience can help ophthalmologists and health-care workers to have a safer working environment when facing COVID-19 pandemic.

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The coronavirus disease 2019 (COVID 19) pandemic has presented major challenges to ophthalmologists. Reports have shown that ocular manifestations can be the first presenting symptoms of COVID 19 infection and conjunctiva may be a portal of entry for the severe acute respiratory syndrome (SARS) associated coronavirus 2 (SARS CoV 2). The purpose of this article is to provide general guidance for ophthalmologists to understand the prevalence of ocular presentation in COVID 19 patients and to reduce the risk of transmission during practice. Relevant studies published in the period of November 1, 2019, and July 15, 2020, regarding ocular manifestations of COVID 19 and detection of SARS CoV 2 in the eye were included in this systematic review and meta analysis. The pooled prevalence of the ocular manifestations has been estimated at 7% (95% confidence interval [CI]: 0.03–0.10) among COVID 19 patients. The pooled detection rate of SARS CoV 2 from conjunctiva was low (1%, 95% CI: 0.00–0.03). Conjunctival symptoms were the most common ocular manifestations in COVID 19, but the positive detection rate of the SARS CoV 2 virus by reverse transcription–polymerase chain reaction of conjunctival tears or secretions remained low. No study has shown a definite transmission of COVID 19 through ocular mucosa or secretions. In summary, ocular manifestations in COVID 19 patients commonly comprise ocular surface symptoms. Although a low prevalence of ocular symptoms was encountered among patients infected by SARS CoV 2, it is imperative for all ophthalmologists to understand the full spectrum of COVID 19 symptoms or signs including those of the eyes as well as to adopt appropriate protective measures during clinical practice.

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Understanding of the molecular pathology of ocular surface disease (OSD) is poor, and treatment is highly unsatisfactory. To facilitate treatment of OSD, a relatively noninvasive procedure, i.e. impression cytology (IC) has been shown to be useful. Recently, the technologies employed in research studies using IC in OSD have vastly improved, and standardized IC has even been used in clinical trials of dry eye. Here, this review aims to describe the advances of IC in the last 10 years, which serves as an update on the progress in this field since the last major review of IC. OSD that has been recently evaluated include meibomian gland dysfunction, Sjogren's syndrome, Steven–Johnson syndrome, and postmenopausal dry eye. The recent studies (4 longitudinal, 18 cross-sectional analyses) which utilized IC analyzed DNA, RNA, proteins, and ocular surface cells, including memory T-lymphocytes, dendritic cells (DCs), neutrophils, conjunctival epithelial cells, and goblet cells. These studies employed quantification of transcripts associated with inflammation, proteins involved in oxidative stress, enzymes such as matrix metalloproteinases, and cell surface proteins by flow cytometry, such as HLA-DR, cytokine and chemokine receptors, markers for T cell differentiation, and DC activation, in addition to the more traditional morphological evaluation of squamous metaplasia and staining for goblet cells. Some challenges in the clinical use of IC have also been described, including issues related to storage and normalization of data. In summary, advances in IC have permitted a more robust evaluation of the ocular surface and will facilitate progress in the understanding and treatment of OSD.

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Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized inflammatory disease of unknown etiology. It characterized by distinctive histopathological appearance of dense IgG4-positive lymphoplasmacytic infiltrates, storiform fibrosis, and obliterative phlebitis in one or more organs, simultaneously or subsequently. In cases of ocular adnexal involvement, unique clinicohistopathological features were delineated by recent studies, and IgG4-related ophthalmic disease (IgG4-ROD) is generally recognized as the disease name. A significant proportion of previous labeled idiopathic orbital inflammations and Mikulicz's disease are now consistent with a diagnosis of IgG4-ROD. Increasing studies have accumulated regarding its epidemiology, diagnosis, clinical features, treatment, and the association between lymphoma. In this review, we summarize our present understanding of IgG4-ROD.

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Neovascular glaucoma (NVG), which is refractory to both medical management and surgical intervention, is a disastrous ocular disease for it always ends up with intolerable pain and extinguishing patients' residual visual function. Since insufficient acknowledge of the pathophysiological and molecular mechanisms of NVG, it has been laying a challenging dilemma of managing NVG in clinical practice for a long time. Along with the progression on some new agents and surgical options targeting certain possible roles in the NVG process, there seems having been some new sights but still much unknown and to be revealed. This review discusses the underlying etiologic diseases of NVG, molecular findings and characteristics of its pathogenic process, as well as the management of NVG in detail. In addition, here represents some of our hypothesis regarding the interesting findings about NVG in clinical practice, aiming to provide some new enlightenment for future research.

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Diabetic macular edema (DME) is a chronic condition with a multifactorial pathogenesis. Vascular endothelial growth factor (VEGF) and several inflammatory mediators are upregulated in eyes with DME. VEGF inhibitors and corticosteroids have all been used successfully in the management of DME. Currently available corticosteroids include triamcinolone acetonide (TA), the dexamethasone (DEX) intravitreal implant, and the fluocinolone acetonide (FA) intravitreal implant. The response to treatment can vary substantially with each treatment modality. Some cases of DME are VEGF driven, and in others, inflammation plays a key role. Chronicity appears to favor corticosteroid treatment. There are no clear guidelines to guide switching from an anti-VEGF to a corticosteroid. Combination therapy of an anti-VEGF drug and a corticosteroid does not appear to provide additional benefit over monotherapy with either drug. The main advantage of corticosteroids over VEGF inhibitors is their longer duration of action. Vitrectomy does not affect the pharmacokinetics of the corticosteroid implants. Common adverse events of corticosteroids include cataract formation, cataract progression, and ocular hypertension. TA may cause a sterile endophthalmitis and pseudoendophthalmitis. Migration of the intravitreal DEX and FA implants into the anterior chamber can be problematic. Because of their less favorable safety profile, corticosteroids are generally used as a second-line treatment for DME. Advantages of using an intravitreal corticosteroid implant include the reduction of treatment burden and predictable pharmacokinetics even in vitrectomized eyes. Pseudophakic eyes, previously vitrectomized eyes and eyes with long-standing DME, particularly of patients who have difficulty in maintaining a monthly appointment, may benefit from primary treatment with a corticosteroid intravitreal implant.

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Myopia has become epidemic in the world. Without effective control, the progression may lead to excessive myopia with severe complications affecting vision and ocular alignment. The genetic factors and environmental factors of myopia are closely interrelated to each other. Asian ethnicity and parental myopia, among other genetic factors, influence the refractive outcome dramatically when environmental risk factors such as hours of near work and reading distance are analyzed. Outdoor activities are protective measures that retard myopia progression. Total time under the sun and not the specific outdoor activities are contributing factors. Current effective treatments for myopia include atropine of high, moderate, and low doses, relative peripheral myopia-inducing devices, and bifocal spectacles including prism bifocal spectacle lenses. Although atropine is considered highly effective in randomized controlled trials, it is not well tolerated in a clinical setting, especially in high dosage. Since the severity of rebound effect of atropine after cessation of usage and the side effects are directly related to the concentration of the medication, it is recommended that low-dose atropine is used in the initial attempt. Higher concentration for better control can be considered when compliance is observed. Devices that induce relative peripheral myopia such as orthokeratology are moderately effective interventions that are well accepted by children who wish to be spectacle free. Bifocal spectacles generally have low effect in myopia control. Prism bifocal spectacle lenses may have a special niche in myopia retardation for patients with low lags of accommodation.

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