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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Traumatic optic neuropathy (TON) is an uncommon cause of visual loss following blunt or penetrating head trauma, but the consequences can be devastating, especially in cases with bilateral optic nerve involvement. Although the majority of patients are young adult males, about 20% of cases occur during childhood. A diagnosis of TON is usually straightforward based on the clinical history and examination findings indicative of an optic neuropathy. However, the assessment can be difficult when the patient’s mental status is impaired owing to severe trauma. TON frequently results in profound loss of central vision, and the final visual outcome is largely dictated by the patient’s baseline visual acuities. Other poor prognostic factors include loss of consciousness, no improvement in vision after 48 hours, the absence of visual evoked responses, and evidence of optic canal fractures on neuroimaging. The management of TON remains controversial. Some clinicians favor observation alone, whereas others opt to intervene with systemic steroids, surgical decompression of the optic canal, or both. The evidence base for these various treatment options is weak, and the routine use of high-dose steroids or surgery in TON is not without any attendant risks. There is a relatively high rate of spontaneous visual recovery among patients managed conservatively, and the possible adverse effects of intervention therefore need to be even more carefully considered in the balance.

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Swept source optical coherence tomography (SS-OCT) was introduced in clinical practice in 2012. Because of its deeper penetration and faster acquisition time, SS-OCT has the ability to visualize choroid, vitreous, and retinal structures behind dense preretinal hemorrhages. Swept source optical coherence tomography has positively influenced and hugely contributed to the research of the vitreous body. It is the first ophthalmic diagnostic technology to demonstrate the entire structure of the posterior pre-cortical vitreous pocket (PPVP) in vivo. The roles of the PPVP in physiological posterior vitreous detachment and vitreoretinal interface disorders have now been elucidated. The presence of a connecting channel between the PPVP and Cloquet’s canal suggests that the aqueous humor drains into the premacular space. Deeper penetration of SS-OCT has made it possible to view the choroid. It also has an important role in central serous chorioretinopathy and uveitis. We have also been able to treat Harada disease by monitoring the choroidal thickness by SS-OCT.

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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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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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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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Rhino-orbital-cerebral mucormycosis (ROCM) is a rare but devastating fungal infection caused by filamentous fungi of the family Mucoraceae. We report a rare case of unilateral ROCM in a diabetic patient where central retinal artery occlusion (CRAO) was the first manifestation of the disease. Magnetic resonance imaging scan revealed orbital and intracranial spread of the disease. Definitive diagnosis was established by culture of the biopsy specimen which showed Rhizopus oryzae on Sabouraud's dextrose agar. The patient was successfully treated with extensive debridement of sinuses and intravenous liposomal amphotericin B; however, the left eye remained blind following the CRAO.

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Dermal soft-tissue augmentation using a filler is a technique widely used for facial cosmetic enhancement. However, potential complications following facial cosmetic injections have heightened the possibility of iatrogenic visual loss. We report two cases of severe ocular complications after nasal cosmetic enhancement. Both cases had poor visual outcomes in spite of emergency management. The second patient is a rare case with bilateral anterior ischemic optic neuropathy after dermal soft-tissue augmentation. The visual outcome was correlated with the location and the extent of the arterial embolization. Unfortunately, there is still no standard treatment protocol for vision-threatening complications. Clinicians should always keep in mind that embolic arterial occlusion may occur after augmentation.

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Reductions of intraocular pressure during trabeculotomy result from relieving the resistance to aqueous flow by cleavage of the trabecular meshwork and inner walls of Schlemm's canal at the point of outflow resistance of the aqueous humor. Since trabeculotomy does not result in bleb formation, development of late-onset sight-threatening complications, such as hypotony maculopathy and endophthalmitis, that are associated occasionally with trabeculectomy develop less frequently after trabeculotomy. The ab-interno approach is used in several new procedures, including the Trabectome, Kahook Dual Blade, microhook abinterno trabeculotomy, and 360° suture trabeculotomy, instead of the ab-externo approach as in conventional trabeculotomy. These newly developed novel, minimally invasive, and trabeculotomy-related glaucoma surgeries can be good options for certain glaucoma cases, including early-stage open-angle glaucoma, developmental glaucoma, and glaucoma in elderly patients.

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Background/Purpose: To assess the prevalence of nonstrabismic accommodative and vergence dysfunc tions among primary schoolchildren in Hampyeong, a rural area of South Korea. Methods: Five hundred and eighty-nine primary schoolchildren, 8–13 years old, were each given a thorough eye examination, including binocular-vision testing, near point of convergence, horizontal phoria measurement by von Graefe, and negative and positive vergence amplitudes with prism bar, to determine any form of accommodative or vergence dysfunctions. Results: Of the 589 participants examined, 168 (28.5%) primary schoolchildren presented some form of nonstrabismic accommodative or vergence dysfunctions. The prevalence of accommodative dysfunctions and vergence dysfunctions was 13.2% and 9%, respectively. Convergence insufficiency (10.3%) was more prevalent than convergence excess (1.9%), and accommodative insufficiency (5.3%) was more prevalent than accommodative excess (1.2%). Conclusion: This study suggests that nonstrabismic accommodative and vergence dysfunctions are prevalent in the rural area of South Korean primary schoolchildren, and convergence insufficiency was the most prevalent.

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Background/Purpose: Orbital fractures are a common facial fracture managed by multiple surgical specialties. Methods: A retrospective review of the electronic medical records of patients (age, 18–85 years) presenting to Northwestern Memorial Hospital and Northwestern Medical Faculty Foundation in Chicago, IL, USA with International Classification of Diseases, Ninth Revision codes for facial fractures or CPT (Current Procedural Terminology) codes for orbital fracture repair. Results: A review of the electronic medical records identified 504 individual incidents of orbital fractures with available imaging for review. The most common location for an orbital fracture was a floor fracture (48.0%) followed by a medial wall fracture (25.2%). Left-sided orbital fractures were statistically significantly more common than right-sided orbital fractures (99% confidence interval). Orbital fractures were more prevalent in younger age groups. The mean patient age was 39.3 years. The most common cause of all orbital fractures was assault followed by falls. However, falls were the most common cause of orbital fractures in women and in patients aged 50 years and older. Evaluation by an ophthalmologist occurred in 62.8% of orbital fracture patients, and evaluation by a team comprising the facial trauma service (Otolar-yngology, Plastic Surgery, and Oral and Maxillofacial Surgery) occurred in 81.9% of orbital fracture patients. Conclusion: Assault was the largest cause of all orbital fractures, and occurred most commonly in young males. Assaulted patients were more likely to have left-sided fractures compared to nonassaulted patients. In patients aged 50 years and older, falls were the most common cause of orbital fractures.

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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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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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Ocular inflammation is a significant cause of ocular morbidity and visual impairment. Topical, periocular, intraocular, and systemic corticosteroids are highly effective for treating appropriate forms of ocular inflammation. However, their use may be constrained by local and/or systemic side effects, especially if long-term therapy is required. As a result, immunosuppressive agents increasingly have been used to manage ocular inflammation alongside or in place of corticosteroids. The four categories of agents used today are antimetabolites [primarily methotrexate, mycophenolate mofetil (MMF), and azathioprine]; T-cell inhibitors (usually cyclosporine, less often tacrolimus or sirolimus); alkylating agents (cyclophos-phamide and chlorambucil); and biologic agents [tumor necrosis factor (TNF) inhibitors, lymphocyte inhibitors, and interleukin inhibitors]. The primary goals of immunosuppressive therapy are (1) to control inflammation when corticosteroids fail to do so; (2) to prevent corticosteroid-induced toxicity when the necessary corticosteroid dosage exceeds the desired or safe level (corticosteroid sparing); and (3) to treat specific high-risk uveitis syndromes known to respond poorly to corticosteroids alone. Growing evidence shows the effectiveness of immunosuppressive drugs in achieving these goals, as well as improved visual function, prevention of ocular complications, and in some cases even disease remission. However, these agents also have side effects, which must be considered in each patient's management. In this report, we summarize the effectiveness and safety of immunosuppressive drug therapy utilized in the treatment of ocular inflammatory diseases.

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An understanding of the pathogenesis of glaucoma is one of the foundations in glaucoma management. A number of theories have been presented to explain glaucomatous neural degeneration. The vascular theory attempts to explain the causation of glaucoma on the basis of vasogenic factors and altered he-modynamics in the body; however, this theory remains controversial. There are proponents for and against the role played by vascular factors in the development of glaucomatous optic nerve degeneration. This review aims to analyze the various studies performed to provide evidence for and against the vascular theory of glaucoma. It also affirms the need to undertake further studies regarding the path-ogenesis of glaucoma and integrate them into our management strategies. The literature search for this systemic analysis was performed using search engines, such as PubMed, The Virtual Library of the Ministry of Health Malaysia, Google Scholar, and ClinicalKey.

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Central retinal vein occlusion (CRVO) can cause vision loss. The pathogenesis of CRVO involves a thrombus formation leading to increased retinal capillary pressure, increased vascular permeability, and possibly retinal neovascularization. Vision loss due to CRVO is commonly caused by macular edema. Multiple treatment modalities have been used to treat macular edema. Currently, the most common therapy used is intravitreal inhibition of vascular endothelial growth factor (VEGF). The three most widely used agents are aflibercept, bevacizumab, and ranibizumab and they are effective at blocking VEGF. In addition, intraocular steroids can be used to treat macular edema. This review will briefly cover the treatment options and discuss in greater detail the efficacy and safety of aflibercept.

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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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Neurotrophic keratitis (NK), a degenerative disease caused by damage to the trigeminal nerve, abolishes both tearing and blinking reflexes, thus causing the most severe forms of dry eye disease (DED). Conversely, the increasing severity of DED also leads to progressive loss of corneal nerve density, potentially resulting in NK. Both diseases manifest the same spectrum of corneal pathologies including inflammation and corneal epithelial keratitis, which can progress into vision-threatening epithelial defect and stromal ulceration. This review summarizes the current literature regarding outcomes following sutured and sutureless cryopreserved amniotic membrane (AM) in treating DED as well as epithelial defects and corneal ulcers due to underlying NK. These studies collectively support the safety and effectiveness of cryopreserved AM in restoring corneal epithelial health, improving visual acuity in eyes with NK and DED, and alleviating symptomatic DED. Future randomized controlled trials are warranted to validate the above findings and determine whether such clinical efficacy lies in promoting corneal nerve regeneration.

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PURPOSE: Autologous serum eye drops are considered safe and efficient for the treatment of various ocular surface disorders, including dry eye diseases (DED) caused by the primary and secondary Sjogren syndrome (SS). However, the serum components in patients of SS may be different from those of normal patients and can thus lead to unpredictable therapeutic effects. This study divided the SS patients into active and inactive types based on the erythrocyte sedimentation rate and the presence or absence of active rheumatoid arthritis. METHODS: We compared the serum components of these two groups with standard and multiplex enzyme linked immunosorbent assay arrays and predicted the therapeutic effects of topical autologous serum for the treatment of DED with ocular surface disease index (OSDI) and Oxford Schema scale (OSS). RESULTS: Hyaluronic acid and transforming growth factor b1 levels were significantly higher in the active SS group compared to the inactive SS group (P < 0.01), whereas epidermal growth factors, insulin growth factor 1, and fibroblast growth factor b had no significant differences between these two groups. Active SS group had significantly higher expressions of interleukin (IL) 1 beta, IL 6, and tumor necrosis factor alpha compared to inactive SS patients (P < 0.05). There were no statistical differences in therapeutic effects between these two groups, as measured with the OSDI or OSS. CONCLUSION: Dividing the Sjogren dry eye patients into active and inactive groups may appear as a reasonable method to predict the quality of autologous serum eye drops, but there seems to be no significant predictability to the therapeutic effects.

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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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Stickler Syndrome (SS) is a significant cause of retinal blindness in children. The immediate cause of blindness is retina detachment from giant retinal tear (GRT). It is frequently diagnosed late and the giant retinal tear (GRT) may be complicated by high-grade proliferative vitreoretinopathy (PVR). The surgery for the combined GRT with PVR has limited structural results and the vision mainly remains impaired. In order to improve the visual outcomes, we propose an organized program oriented toward early diagnosis and surveillance. Adding an effective prophylaxis may maintain normal vision in a high percent of patients. The critical diagnostic moments for this program are prenatal and at birth. The tools include a directed history, general physical exam and advanced ophthalmologic exam looking for the particular features of SS. Some features may need advanced skills transfer, because they are not reliably taught in retina fellowships. Much of this program requires a partnership with obstetricians, pediatricians, neonatologists and geneticists. Finally, we review the evidence regarding prophylaxis and discuss our approach in the absence of guidance from a randomized clinical trial.

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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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Glaucoma is the most common optic neuropathy characterized by normal to raised intraocular pressure (IOP), visual field defects, loss of retinal nerve fiber layer, thinning of the neuroretinal rim, and cupping of the optic disc. IOP reduction by medical, laser, or surgical therapies remains the only clinically proven treatment of glaucoma. The challenges in glaucoma management are diverse. They include early detection and diagnosis, setting of appropriate target IOP, choice of treatment, monitoring of quality of life and sight, and compliance with the treatment. Early diagnosis can be made by assessing optic nerve structure using imaging devices and optic nerve function through perimetry. Reducing IOP and controlling its fluctuations are considered to be the most important factors in limiting progression of glaucoma. Selection of the best suitable therapy out of medical, surgical, or laser treatment options is yet another management challenge. Patients suffering from glaucoma experience poor quality of life owing to the diagnosis itself, functional visual loss, inconvenience and cost of treatment, and side effects of treatment. All these factors lead to poor compliance, adherence, and persistence to treatment, and further progression of the disease. It is, therefore, important that ophthalmologists keep all the aforementioned factors in mind when managing patients with glaucoma.

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We present the clinical course, management, and final outcome of spontaneous suprachoroidal hemorrhage (SSCH) in an age-related macular degeneration (AMD) patient—a 64-year-old male receiving antiplatelet therapy who developed SSCH during the Valsalva maneuver. In addition to our case study, we discuss the results of a systemic review of the literature and reference lists of retrieved studies published from January 2001 to December 2013. Among a total of 31 patients (32 eyes), acute secondary glaucoma was a complication in 87.5% of the cases, and over half of the cases (20 eyes, 62.5%) received surgery. Twenty cases (64.5%) were characterized by systemic hypertension (HTN), followed by cardiovascular or cerebral vascular disease in 17 cases (54.8%). The Valsalva maneuver was performed in five cases (16.1%) prior to the episode. Twenty-three cases (74.2%) had abnormal hemostasis, including use of anticoagulants or thrombolytic agents (18 cases), chronic renal failure (CRF, 5 cases), and blood dyscrasia (3 cases). AMD was the most common (17 eyes of 16 patients, 53.1%) ocular disease. Visual acuity was classified as hand motion (HM) or worse in 20 eyes (of 28 eyes, 71.4%) at initial presentation and in 24 eyes (of 30 eyes, 80%) upon final examination. Anticoagulated patients with AMD should be informed of the risk of intraocular hemorrhage. Medical therapy usually fails in the treatment of glaucoma. Surgical intervention provides an option for the purpose of pain relief. Even so, the final visual prognosis is usually poor.

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Familial exudative vitreoretinopathy (FEVR) is a hereditary vitreoretinal disorder that can cause various types of retinal detachments. The abnormalities in eyes with FEVR are caused by poor vascularization in the peripheral retina. The genetics of FEVR is highly heterogeneous, and mutations in the genes for Wnt signaling and a transcription factor have been reported to be responsible for FEVR. These factors have been shown to be the regulators of the pathophysiological pathways of retinal vascular development. Studies conducted to identify the causative genes of FEVR have uncovered a diverse and complex relationship between FEVR and other diseases; for example, Norrie disease, a Mendelian-inherited disease; retinopathy of prematurity, a multifactorial genetic disease; and Coats disease, a nongenetic disease, associated with pediatric retinal detachments.

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