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In order to evaluate the accuracy of commercially available handheld fundus cameras for a variety of ophthalmic diagnoses, we conducted a systematic review, searching PubMed and PubMed Central and performing a bivariate analysis to determine the pooled sensitivity and specificity of handheld fundus cameras. Eleven studies validating handheld fundus cameras against a gold-standard method for disease diagnosis were included. For nonmydriatic images, pooled sensitivity was 83% (95% confidence interval (CI): 77-88%) and specificity was 92% (95% CI: 79-97%). For mydriatic images, pooled sensitivity was 87% (95% CI: 79-92%) and specificity was 90% (95% CI: 78-96%). Overall pooled sensitivity was 85% (95% CI: 80-89%) and specificity was 91% (95% CI: 83-95%). Of the 11 studies included, 5 assessed the diagnosis of diabetic retinopathy, for which sensitivity was 87% (95% CI: 80-92%) and specificity was 95% (95% CI: 85-98%). For all other diagnoses combined, sensitivity was 81% (95% CI: 74-87%) and specificity was 83% (95% CI: 76-89%). These findings suggest that handheld fundus cameras are capable of achieving acceptable sensitivity and specificity values for eye disease, with mydriatic images being more sensitive for disease. Diabetic retinopathy was the single diagnosis with the strongest data to support the use of handheld fundus cameras for disease screening.

Thyroid eye disease (TED) is an autoimmune disorder that manifests in the orbit. In TED, the connective tissue behind the eye becomes inflamed and remodels with increased fat accumulation and/or increased muscle and scar tissue. As orbital tissue expands, patients develop edema, exophthalmos, diplopia, and optic neuropathy. In severe cases, vision loss may occur secondary to corneal scarring from exposure or optic nerve compression. Currently there is no cure for TED, and treatments are limited. A major breakthrough in TED therapy occurred with the FDA approval of teprotumumab, a monoclonal insulin-like growth factor 1 receptor (IGF1R) blocking antibody. Yet, teprotumumab therapy has limitations, including cost, infusion method of drug delivery, variable response, and relapse. We describe approaches to target orbital fibroblasts and the complex pathophysiology that underlies tissue remodeling and inflammation driving TED. Further advances in the elucidation of the mechanisms of TED may lead to prophylaxis based upon early biomarkers as well as lead to more convenient, less expensive therapies.

Primary central nervous system lymphoma (PCNSL) may manifest initially in the eye (termed vitreoretinal lymphoma or VRL) or in non-ocular CNS compartments, or in both. The nature of the onset of PCNSL implies two clinical specialists - ophthalmologists and neuro-oncologists - independently may assess the primary presentation of this rare malignancy. Clinically relevant perspectives on expectations of PCNSL manifestation in both ocular and non-ocular CNS compartments would help inform management practices in each specialty, which should impact clinical outcomes. A recent increase in the number of published PCNSL cohort studies provides new opportunity to review the current prevalence rates of ocular involvement, and the timing of this involvement over the course of disease. In PCNSL cohorts defined by non-ocular CNS compartment involvement, with or without ocular involvement (termed "PCNSL ± ocular involvement" cohorts), mean rates of concomitant VRL at diagnosis, or at any time during the course, are 10% and 16%, respectively. Only a few individuals within this cohort group present with exclusive eye disease (

The literature regarding orbital decompression for thyroid eye disease is vast, spanning multiple specialty areas including neurosurgery, head and neck, maxillofacial, and ophthalmic plastic surgery. Although techniques have advanced considerably over the more than 100 years during which this procedure has been performed, the 4 major approaches remain: transorbital, transcranial, transantral, and transnasal. The explosion in literature related to orbital decompression has mostly involved minor technical variations on broader surgical themes. The purpose of this review is to organize the major approaches in terms of bony anatomy and to contextualize variation in transdisciplinary techniques within a common conceptualization.

A 73-year-old woman presented with vision loss and recurrent proptosis with conjunctival chemosis 4 weeks after an uncomplicated fat-only orbital decompression for thyroid eye disease. Her Graves disease was biochemically “burnt out,” and she had diabetes mellitus. Initial neuro-imaging showed a straightened optic nerve and orbital apex fat streaking so a bilateral bony decompression of the orbital apex was performed for presumed compressive optic neuropathy. Vision failed to improve, and she experienced cognitive decline. She described metamorphopsia and insomnia. Examination showed a hemifield loss of red sensitivity, difficulties with higher visual processing, and memory problems. Occipital cortical ribboning was seen on diffusion weighted magnetic resonance imaging, cerebrospinal fluid was positive for 14-3-3, and her electroencephalogram showed periodic complexes. A diagnosis of the Heidenhain variant of Creutzfeldt-Jakob disease was made. She became akinetic and mute, dying a few months later.

Canaliculitis is an uncommon inflammation of the proximal lacrimal drainage system that is frequently misdiagnosed. It classically presents with symptoms of unilateral conjunctivitis, mucopurulent discharge, medial canthal inflammation, epiphora, and a red, pouting punctum. We summarize the literature on canaliculitis published from antiquity to the modern era and explore therapeutic options.

Glaucoma, primarily a disease of the older population, may affect women of childbearing age. Pregnancy affects the intraocular pressure (IOP) of women with pre-existing glaucoma. Both elevations and reductions of IOP have been reported during pregnancy. Additionally, visual field test results may fluctuate during pregnancy. In managing the pregnant glaucoma patient with medical therapy, one must consider not only the systemic side effects on the mother, but also any potentially harmful effects on the developing fetus. All anti-glaucoma medications are categorized as class C by the Food and Drug Administration, except brimonidine and nonspecific adrenergic agonists, which belong to class B. In general, the lowest effective dosage of medication should be used. Systemic absorption can be reduced by punctal occlusion, eyelid closure, and blotting the excess drops away during administration. In those patients who need surgery, most local anesthetics may be used safely because they have not been shown to be teratogenic in humans. Antifibrotic agents commonly used adjunctively in trabeculectomy, however, should be avoided. Glaucoma laser procedures, such as laser peripheral iridotomy and laser trabeculoplasty, have been employed without identifiable teratogenic effects or increased risk of side effects for pregnant women.

Age-related macular degeneration (AMD) is the leading cause of blindness in people over 60 in western countries. Inflammatory markers have been implicated in the development and progression of AMD. C-reactive protein (CRP) is an inflammatory marker known to be associated with cardiovascular disease, and a link between AMD and CRP has been suggested. In this systematic review we summarize the currently available evidence from clinic-based and population-based studies investigating this association. A meta-analysis of evidence from eleven studies (41,690 study participants) shows that high serum levels (>3 mg/L) of CRP are associated with a two-fold likelihood of late onset AMD, compared to low levels (

Acute zonal occult outer retinopathy (AZOOR) is a rare unilateral or bilateral disease of unknown etiology characterized by focal degeneration of photoreceptors. A total of 131 cases of AZOOR (205 eyes), including the variant known as acute annular outer retinopathy, have been reported in the English language literature. In this group of predominantly white individuals, average age at presentation was 36.7 years, and the male:female ratio was 1:3.2. The majority of patients complained of the acute onset of a scotoma, which was associated with photopsia. Visual acuity was 20/40 or better in 74% of tested eyes, and fundus examination was unremarkable in 76% of eyes. Blind spot enlargement, with or without other field defects, was observed in 75% of the visual fields examined, and electroretinographic abnormalities were recorded in 99% of patients tested. Typically patients retained good visual acuity, although retinal pigment epithelial disturbances commonly developed over time. It was unusual for visual field loss to continue beyond six months. Various treatments have been attempted in patients with AZOOR--including systemic corticosteroids, other systemic immunosuppressive agents, and different antimicrobials--but none have been proven effective.

Although glaucoma is a multifactorial disease, elevated intraocular pressure (IOP) remains the most important known risk factor. Different systemic and local factors are thought to influence an individual's IOP. There can be a clinically significant rise in IOP when going from upright to horizontal or inverted body positions. Although there is a significant interindividual variability, the magnitude of the IOP change is greater in glaucomatous eyes. As patients usually spend a significant portion of their lives in the horizontal position, mainly during sleep, this is highly relevant. In this review we discuss the relationship between postural changes and IOP fluctuation, including changes in both body and head position. The possible mechanisms involved and the main implications for glaucomatous eyes are discussed. Finally, considerations with regard to sleep position in glaucoma patients are made based on evidence in the literature.

Graves disease represents a systemic autoimmune process targeting the thyroid, orbit, and pretibial skin. The thyroid dysfunction is treatable, but no consistently effective medical therapy has yet been described for the orbital manifestations of Graves disease, also known as thyroid-associated ophthalmopathy or thyroid eye disease. Several autoantigens are potentially relevant to the pathogenesis of thyroid eye disease. Activating antibodies generated against the thyrotropin receptor can be detected in a majority of patients, and these drive hyperthyroidism. However, stimulating antibodies against the insulin-like growth factor-1 receptor (IGF-1R) may also play a role in the extra-thyroid manifestations of Graves disease. IGF-1R is overexpressed by orbital fibroblasts derived from patients with thyroid eye disease, whereas IGF-1R(+) T and IGF-1R(+) B cells are considerably more frequent in Graves disease. Actions of several cytokines and the molecular interplay peculiar to the orbit appear to provoke the inflammation, fat expansion, and deposition of excessive extracellular matrix molecules in thyroid eye disease. Based upon these new insights, several therapeutic strategies can now be proposed that, for the first time, might specifically interrupt its pathogenesis.

The growth of the eye results in a myopic shift in aphakic and pseudophakic eyes during childhood. Cataract surgery after the age of 6 months, with or without lens implantation, appears to have little effect on the rate of refractive growth. Most children with pseudophakia have a large amount of myopic shift. This myopic shift is greatest in children with surgery at younger ages. It is also greater in eyes with high-power intraocular lenses due to an optical phenomenon analogous to the effect of vertex distance. The amount of myopic shift and the variance in rate of refractive growth can be predicted using an empiric, logarithmic model. We describe a revision of this logarithmic model to extend it patients with surgery before 3 months of age. We also analyze the variance in the rate of refractive growth, based on data from pseudophakic children with the longest follow-up in proportion to age.

Ocular ischemic syndrome encompasses a spectrum of clinical findings that result from chronic ocular hypoperfusion. It is relatively uncommon, and the diagnosis may be difficult to make because of its variable presentations. The presence of an ocular ischemic syndrome always implies underlying severe carotid occlusive disease and may be its sole clinical manifestation. It may also result from other causes of reduced blood flow to the eye and the orbit such as systemic vasculitis. Besides visual loss and ocular/orbital pain, affected patients are also at risk for developing cerebral and myocardial infarction. Establishing the diagnosis is therefore essential with respect not only to visual prognosis but also to patient survival. Ophthalmologists have an important role in early diagnosis and in coordinating the systemic evaluation of patients. Referral to the neuroradiologist and the neurovascular specialist is warranted. We present the current knowledge on the ocular ischemic syndrome.

Glaucoma is one of the leading causes of visual impairment and blindness. Lowering intraocular pressure (IOP) is the only proven means to slow or halt disease progression among those at higher risk of developing glaucoma and those with early to moderate or more advanced glaucoma. Recent publications have highlighted the potential for increased rates or likelihood of worsening glaucoma among those with larger IOP swings within defined time periods. The purpose of this systematic, comprehensive review and analysis of the literature was to assess the state of knowledge in the area of IOP changes over time and the potential impact of such changes on treatment. Current literature indicates that a random IOP measurement is a poor surrogate for IOP levels throughout the day and across visits. We address several key questions: 1) What is the best way to measure IOP? 2) Should multiple IOP measurements be performed in a day in the office (short-term IOP fluctuation)? 3) Is measurement at night required? 4) Should clinicians begin to assess long-term IOP fluctuation in patients under stable treatment (across days or visits)? and 5) Should therapy choices be influenced by properties of different treatment options relative to short- or long-term IOP fluctuation?

Galileo Galilei became blind. Before this happened he revealed that his left eye had always had less than perfect vision. A study of his written works, his handwriting, and the originals of the portraits undertaken during his lifetime indicate that this probably was the case. These portraits suggest that his left eye tended to lose fixation and that, at the age of 60, he suffered from a mucocoele of the right frontal sinus; but these conditions would not have caused blindness. Considering the systemic diseases from which he suffered over his lifetime, he could possibly have had a long standing uveitis with secondary pupillary block glaucoma, common in those with the group of conditions classified as sero-negative arthropathies. Posterior scleritis with secondary glaucoma is less likely. If either of these were the cause, then the disease was probably triggered by a well-documented, severe acute illness as a young adult, the inflammation being localized to the eye as a result of severe recurrent conjunctival infections in his youth. The intermittent nature of the visual loss, the normal appearance of the cornea and pupils in his portraits, the absence of any evidence of inflammatory joint disease, the presence of halos, and the severe nature of the pain-combined with the high level of visual acuity in between attacks and its persistence until the last few weeks of vision means that angle-closure glaucoma must also be considered. These suggestions might be confirmed or refuted by studying his remains. Application has been made for this to be done.

Intraocular pressure change has been found concurrent with many orbital pathologies, particularly those involving proptosis. The objective of this review is to offer an inclusive classification of orbital disease-related intraocular pressure change, not only for oculoplastics and glaucoma specialists, but also for general ophthalmologists. Various orbital conditions associated with increased intraocular pressure and glaucoma are comprehensively summarized, and pathophysiology, clinical manifestations, and treatment options of these diseases are discussed. Graves disease, arterio-venous shunts, trauma, and orbital neoplasia, and other common conditions are discussed in detail; less frequent syndromes such as orbitocraniofacial deformities, phakomatoses, and mucopolysaccharidoses are included for the sake of comprehensiveness, but discussed less extensively.

Juvenile neuronal ceroid lipofuscinoses, or Batten disease, is the most common type of NCL in the United States and Europe. This devastating disorder presents with vision failure and progresses to include seizures, motor dysfunction, and dementia. Death usually occurs in the third decade, but some patients die before age twenty. Though the mechanism of visual failure remains poorly understood, recent advances in molecular genetics have improved diagnostic testing and suggested possible therapeutic strategies. The ophthalmologist plays a crucial role in both early diagnosis and documentation of progression of juvenile neuronal ceroid lipofuscinoses. We update Batten disease research, particularly as it relates to the eye, and present various theories on the pathophysiology of retinal degeneration.

Histopathology of young individuals affected by choroideremia is rarely available to allow correlation with the clinical presentation. A 30-year-old man with choroideremia died in a motor vehicle accident and one eye was subjected to histopathological examination. Immunoblot analysis of protein derived from white blood cells of a living brother, also affected with choroideremia, confirmed the absence of Rab escort protein-1, the normal CHM gene product. Direct sequencing of the coding region and adjacent splice sites of the CHM gene was undertaken on genomic DNA from the living brother and revealed a transition mutation, C to T, in exon 6 (R253X) which resulted in a stop codon and was predicted to truncate the protein product. Histopathological examination of the eye of the deceased brother showed relative independent degeneration of choriocapillaris, retinal pigment epithelium, and retina, similar to observations in the mouse model of choroideremia. In addition, mild T-lymphocytic infiltration was found within the choroid. The ophthalmic features and the pathology of choroideremia are discussed in light of new findings in the current case.

Regular exercise is a healthy lifestyle choice with numerous benefits to general health. Ophthalmologists may face questions of the benefits or risks of exercise to eyes. Here the effects of acute exertion and regular physical activity on ocular physiology and disease are reviewed. Intraocular pressure is transiently reduced by dynamic exercise. For the great majority of patients exercise is beneficial to the eyes by reducing risk of central retinal vein occlusion and neovascular age-related macular degeneration, and by improving control of systemic hypertension and diabetes. Ophthalmologists should be advocates of regular exercise with appropriate eye protection.

Eyelid involvement is common in facial burns. Ocular sequelae, including corneal ulceration, are usually preventable and secondary to the development of eyelid deformities, exposure keratopathy, and rarely, orbital compartment syndrome. Early ophthalmic review and prophylactic ocular lubrication is mandatory in burns involving the eyelids. Early surgical intervention, often requiring repeat procedures, is indicated if eyelid retraction causing corneal exposure occurs. Permanent visual impairment is rare with such prompt management. No binding aphorisms exist regarding the tissue used for eyelid reconstruction, with each case requiring an individual approach based on available skin. This review article covers the principles of ophthalmic management in addition to intermediate and long-term management of eyelid burns.

Primary angle-closure glaucoma is a major cause of blindness worldwide. It is a disease of ocular anatomy that is related to pupillary-block and angle-crowding mechanisms of filtration angle closure. Eyes at increased risk for primary angle-closure are small with decreased axial length, anterior chamber depth, and filtration angle width, associated with a proportionately large lens. Angle-closure glaucoma afflicts Asian and Eskimo eyes more frequently than eyes in other races with similar predisposing dimensions. The treatment of primary angle closure addresses its causal mechanisms. Laser peripheral iridotomy equalizes the anterior and posterior pressures and widens the filtration angle by reducing the effect of pupillary block. Argon laser peripheral iridoplasty contracts the iris stroma to reduce angle crowding and is helpful for some affected eyes. Lensectomy dramatically widens the angle and eliminates pupillary block. Clinical reports of lensectomy with posterior chamber intraocular lens implantation in the treatment of acute, chronic, and secondary angle-closure glaucoma describe very favorable results. The appropriate role for lensectomy in the management of primary angle closure, however, remains unproven. Prospective, randomized clinical trials are ongoing to determine the value and comparative risks and efficacy of lensectomy versus medical therapy, laser peripheral iridotomy, laser iridoplasty, and filtration procedures for the treatment of acute and chronic primary angle closure and for the prevention of chronic angle-closure glaucoma, both after and in place of laser peripheral iridotomy.

The definition of glaucoma has changed over the decades from a simple ocular pressure disease to a systemic disorder of multivariate etiology. Glaucoma may be defined for the individual eye as a chronic ocular disease with various underlying pathophysiologic disorders. However, elevated intraocular pressure (IOP) is still the most important risk factor for an untreated glaucomatous eye to progress to a more severe stage of the disease. As the main risk factor within therapeutic reach, IOP and its appropriate measurement deserve our ongoing interest. Not only has our understanding of glaucoma changed but also our approach to the measurement of the IOP. In this article we focus our attention on the various developments in tonometry from the simple force-tonometers of the late 19th century to the high-technology pressure tonometers that were recently introduced for clinical use.

Intraocular pressure, a major risk factor for glaucoma, is known to vary throughout the day, yet glaucoma continues to progress in some patients despite it being well controlled. It is important to understand how other glaucomatous risk factors are affected by circadian variations. The purpose of this review is to analyze the literature concerning circadian variations in systemic blood pressure, ocular perfusion pressure, and ocular blood flow and to identify consensus findings regarding their impact on glaucoma. This review suggests that nonphysiologic nocturnal blood pressure dipping and wider circadian fluctuations in ocular perfusion pressure are linked with the development and progression of glaucoma. No consensus concerning circadian variations in ocular blood flow exists in the current literature, and future investigations of nocturnal changes in blood flow and glaucoma progression are required.

Pneumatic retinopexy has become an important surgical technique in the modern era of retinal surgical management for retinal detachments. It is primarily indicated for uncomplicated retinal detachments with retinal breaks involving the superior 8 clock hours of the fundus, although more complex retinal detachments may be successfully managed with this technique on a selected basis. Qualified candidates must be willing to maintain a specific head posture for five or more days for optimal outcome with pneumatic retinopexy. Basic surgical steps of pneumatic retinopexy include retinopexy of retinal breaks with cryotherapy or laser, intraocular gas injection before or after retinopexy, and maintenance of proper head posture by the patient for the required time period after surgery. Phakic eyes fared better than nonphakic eyes for pneumatic retinopexy, with the single-operation successes of 71-84% for the former and 41-67% for the latter. Despite lower single-operation successes with pneumatic retinopexy in comparison to sclera buckling, the multicenter pneumatic retinopexy trial and other published reports have shown that the final anatomical and visual outcomes are not disadvantaged by the initial pneumatic retinopexy. An extensive discussion of complications associated with pneumatic retinopexy is presented. In addition, a key feature of this review is a comprehensive update in the outcome of pneumatic retinopexy in published reports from 1986 to the present in chronological order not available in the current literature. This comprehensive summary shows updated average surgical outcomes for the 4,138 eyes in the 21-year period to be similar to previous reports: single-operation successes (74.4%), final operation successes (96.1%), new retinal breaks (11.7%), and proliferative vitreoretinopathy (5.2%).

Intrauterine infections are important causes of childhood blindness in both developed and developing countries. Chorioretinal scars are the most characteristic eye manifestation of a congenital or prenatal infection. The various ocular manifestations of congenital infections, summarized by the mnemonic TORCH, and recent additions to the "other" category (lymphocytic choriomeningitis virus and West Nile virus) are discussed.

Choroidal neovascularization is a common cause of vision loss in patients with pathologic myopia, often resulting in irreversible central vision loss. This is particularly important because choroidal neovascularization secondary to pathologic myopia affects many people of working age. Patients may be limited in the types of tasks they can perform effectively or may even have to give up work; thus, in addition to the emotional strain, the vision loss caused by choroidal neovascularization can have a severe impact on career expectations and financial status. This is an important issue for younger patients who may be supporting themselves and their families. In this article, the epidemiology and risk factors of pathologic myopia are reviewed, as well as the pathologic mechanisms, clinical features, and diagnostic tests for choroidal neovascularization secondary to pathologic myopia. The focus of the article is on treatment options, which until recently were limited. The evidence for the beneficial effects of laser photocoagulation, photodynamic therapy with verteporfin, surgery, and other techniques in the treatment of choroidal neovascularization secondary to pathologic myopia will be evaluated.

It has been nearly a century since Jules Gonin performed the first intervention for rhegmatogenous retinal detachment, trans-scleral cautery, achieving successful outcomes in close to 50% of his cases. With the introduction of alternative surgical approaches in the last half-century, including Charles Schepens' scleral buckle technique and Robert Machemer's pars plana vitrectomy, the surgical success rates have risen to close to 90%. Nonetheless, despite dramatic progress in the success of reattachment surgeries, reasonable disagreement exists as to which approach (or combination of approaches) is the best form of surgical intervention for patients with rhegmatogenous retinal detachments. In this review, the authors summarize the current knowledge of retinal detachment, and examine emerging results from the first large scale, prospective, randomized, controlled clinical trials addressing the efficacy of these surgical approaches for retinal detachment, with the hope of identifying the most appropriate (evidence-based) therapeutic intervention for the treatment of rhegmatogenous retinal detachment.

A 38-year-old woman presented with bilateral optic neuropathy, leg weakness, and urinary symptoms. Despite her spinal MR being normal, her serum was positive for NMO-IgG. This indicated that she likely had Devic disease.

The article attempts to provide useful information on psychophysical tests in glaucoma management. This includes the suitability of various glaucoma screening methods, optimum screening parameters for glaucoma detection, and methods for glaucoma follow-up. There is clear evidence that perimetry methods using temporal modulation show a larger area under the curve than standard perimetry methods for the detection of glaucoma. Once the disease is established, there is no significant advantage for flickering stimulus targets. In most cases pattern standard deviation (sLV), or the number of points with a total deviation at the 5% probability level, are best for the identification of glaucoma suspects. If early diagnosis and glaucoma screening is requested, perimetry methods with temporal modulation show a larger area under the curve than standard perimetry methods. In the course of the disease, standard automated perimetry is still the method of choice for functional follow-up.

A case of progressive optic neuropathy in a woman with a history of breast cancer is presented. Differential diagnoses including optic neuritis, infiltrative optic neuropathy, carcinomatous meningitis, and toxic optic neuropathies are discussed. Risk factors for metastatic brain lesions are also discussed.

Infectious keratitis following corneal transplantation is one of the leading causes of failure of a corneal graft. The incidence of graft infection is variable, with developing countries having a higher incidence. The majority of the graft infections occur within 1 year of the corneal transplantation. Suture-related problems and persistent epithelial defect are the most common risk factors predisposing to graft infection. Pneumococcus species and Staphylococcus aureus have been found to be the commonest microorganisms in the developed world, whereas Staphylococcus epidermidis is the most often detected microorganism in corneal graft infection in the developing world. The early identification of predisposing risk factors in patients and their appropriate management at the earliest may prevent the occurrence of graft infection and might improve graft survival. Visual prognosis in eyes with post-keratoplasty graft infection is poor even after optimal therapy and there is a high rate of graft decompensation.

An 18-year-old girl presented with an acute acquired comitant esotropia. Investigation showed a brainstem glioma. Discussion of types of acute acquired comitant esotropia, differential diagnosis and neurologic work-up is given. Review of the literature involving acute acquired comitant esotropia is provided.

The anterior segment dysgeneses are an ill-defined group of ocular developmental abnormalities that share some common features and have a high prevalence of glaucoma. Current classification of what are and what are not anterior segment dysgeneses seems to vary and our knowledge of them is incomplete. As the limits of classical clinical medicine based on evaluation of signs and symptoms are reached, further advancements increasingly will come from molecular medicine and genetics. In this article we review the normal and abnormal development of the anterior segment (concentrating primarily upon neural crest derived dysgeneses), describe the various clinical entities produced and their diagnosis, and discuss the current knowledge of the genetics of these disorders. We also suggest a new approach to the classification of anterior segment dysgeneses, based upon the embryological contribution to the formation of the anterior segment of the eye.

Microphthalmos is a rare condition that is often associated with several other ocular abnormalities. Given the considerable differences between microphthalmic and anatomically normal eyes, cataract surgery is technically demanding in these patients, and special attention must be given to adequate preoperative planning of these procedures. Furthermore, the unique nature of these surgeries creates a particular subset of intraoperative and postoperative complications. However, with the advent of piggyback intraocular lens placement, the visual outcomes of cataract surgery in small adult eyes have improved considerably over the past 20 years. This review discusses the nature of the microphthalmic eye, and addresses proper pre-, intra-, and postoperative care of the microphthalmic patient.

Corneal deposits in association with paraproteinemias have been well described in the ophthalmic literature. Recent reports in the renal literature have described immunotactoid deposition associated with glomerulopathy—organized microtubular deposits of IgGκ that measured 32–50 nm in diameter on renal biopsies. We present a case of corneal immunotactoid deposition in the setting of chronic lymphocytic leukemia and review previous reports of corneal deposition in the setting of systemic paraproteinemia, highlighting the etiology, differential diagnosis, prognosis, and treatment of corneal involvement. We propose the use of the term immunotactoid keratopathy to describe corneal IgGκ deposits appearing as tubular, electron-dense, crystalloid deposits with a central lucent core on electron microscopy and suggest that these patients undergo directed systemic workup to evaluate for potential etiologies of their systemic paraproteinemia. To illustrate the spectrum of paraprotein deposition in the cornea, and to emphasize the importance of ophthalmic evaluation in the setting of systemic paraproteinemias, we include a case of a 44-year-old man with immunoprotein corneal deposition who was subsequently diagnosed with multiple myleoma.

Laser photocoagulation and various radiation therapy methods have been used in the treatment of circumscribed choroidal hemangioma that produce visual loss. Recently, transpupillary thermotherapy has been employed in the management of choroidal hemangioma. Thirty-six cases of choroidal hemangioma treated with transpupillary thermotherapy are reviewed. Twenty-eight cases were reported in the literature and 10 cases were managed by the author. Transpupillary thermotherapy has been used as a primary treatment (36 cases) or secondary treatment (2 cases) for choroidal hemangioma. The goal of transpupillary thermotherapy is to achieve resolution of exudative detachment of the fovea and improvement of vision. In all eyes the foveal detachment subsided. In 26 eyes having pretreatment visual acuityor=0.05 (20/400), the visual acuity increased in 20 (77%) eyes and remained unchanged in 6 (23%) eyes. After transpupillary thermotherapy, 16 (42%) cases showed complete regression, 20 (53%) demonstrated partial regression, and in 2 (5%) there was no change in tumor thickness at follow-ups ranging from 2 to 44 months. Complications of transpupillary thermotherapy of choroidal hemangioma included cystoid macular edema (3 eyes), preretinal fibrosis (2 eyes), focal iris atrophy (3 eyes), and retinal vascular occlusion (1 eye). Although the follow-up in many studies is limited, transpupillary thermotherapy has not been associated with any significant complications.

Purpose To evaluate the IOP-lowering efficacy of bimatoprost and travoprost for the treatment of glaucoma and ocular hypertension. Methods Randomized, investigator-masked, parallel-group clinical trial. After completing a washout from all glaucoma medications, patients (n=26) were randomized to bimatoprost or travoprost for 6 months. Visits were at baseline, week 1, and months 1, 3, and 6. IOP was measured at 9 am at each visit and also at 1 and 4 pm at baseline and months 3 and 6. Results At the baseline visit, there were no significant between-group differences in IOP at 9 am, 1 pm, or 4 pm ( P ≥.776). After 6 months of therapy, both medications provided significant mean reductions from baseline IOP at every time point ( P ≤.007). Mean IOP reductions ranged from 7.4 mm Hg to 8.8 mm Hg (34% to 36%) with bimatoprost and from 4.6 mm Hg to 7.2 mm Hg (19% to 29%) with travoprost ( P ≥.057) after 6 months of medication. At the final study visit, more patients achieved low target pressures with bimatoprost than with travoprost at each time point. Both study medications were well tolerated and ocular redness was the most commonly reported adverse event in both treatment groups. Conclusion Although both bimatoprost and travoprost effectively lowered IOP in patients with glaucoma or ocular hypertension, bimatoprost provided larger mean IOP reductions than travoprost. More patients achieved low target pressures with bimatoprost than with travoprost. The between-group differences were not statistically significant due to the small sample size. These findings are being further evaluated in an ongoing multicenter clinical trial.

A 16-year-old boy developed bilateral visual loss 24 hours after a sprint car accident. Bilateral central scotomas were demonstrated but were unexplained by a thorough eye examination and neuro-imaging. A diagnosis of commotio retinae was established by multi-focal ERG testing. This relatively new technique is discussed, including comparison to full-field ERG and clinical applications.

The intimate anatomical relationship between the orbit and the paranasal sinuses places the orbit and its contents at risk of harm from primary pathologic processes of the sinuses. In the absence of ophthalmic signs or symptoms, ophthalmologists are not routinely involved in the management of patients with sinus disease. Occasionally, some patients may develop ophthalmic complaints after surgical intervention. The orbit, optic nerve, extraocular muscles, and lacrimal drainage system are susceptible to injury during endoscopic sinus surgery. The risk of injury is related to the skill of the sinus surgeon, history of previous surgery, extent and severity of disease, and anatomic variation. Furthermore, recent advances in endoscopic sinus surgery, in particular the use of powered cutting instruments, has resulted in a novel mechanism of injury to the ocular structures.

The management of uveal melanomas always has been a challenge to the clinicians and has evolved from the era of eye-removing surgeries to elimination of the tumor by the body's own immune system through vaccines. Evaluating the outcome of each strategy improves our understanding of the disease process and helps us to improvise on the existing modalities of treatment. Internal resection of choroidal melanomas has been described as one of the treatment modalities for this malignant tumor. Tumor recurrences reported following this surgical procedure have been uncommon. We report a rare case of a 61-year-old Caucasian woman who presented with a 2-month history of progressive, painful proptosis in her right eye. Thirteen years ago she underwent internal resection of an intraocular choroidal melanoma in the same eye and had no evidence of metastatic disease. Magnetic resonance imaging demonstrated a mass occupying the superotemporal portion of the right orbit adjacent to the globe and behind the area of prior internal resection. Biopsy of the lesion and the subsequent A right orbital exenteration confirmed the diagnosis of recurrent malignant melanoma. Twenty-four months following exenteration the patient continues to be free of metastatic disease. Since internal surgical resection was described in 1984, this is the latest known recurrence of a posterior choroidal melanoma. In this review, we highlight the clinical features of this rare case and discuss in brief the rationale of various treatment modalities for choroidal melanoma.

Acute macular neuroretinopathy (AMNR) is a rare condition that produces transient or permanent visual impairment. Typical cases have acute onset multifocal scotomas that correspond rather precisely with reddish, flat, or depressed circumscribed lesions in the macula. These lesions are wedge-shaped and generally point toward the fovea. The pathophysiology of AMNR is unclear, the causes are uncertain, and there is no specific treatment for this condition. This review summarizes the presentation, possible risk factors, and prognosis of the 41 cases of AMNR reported in the published, English-language literature from 1975 through April 2002. Possible areas for future research into the etiology of this rare condition are discussed.

Central serous chorioretinopathy is a relatively common retinal disease characterized by the accumulation of subretinal fluid at the posterior pole of the fundus, creating a circumscribed area of serous retinal detachment. It typically affects young and middle-aged men with no previous medical and family history, and no systemic symptoms or signs. However, it has been noted that central serous chorioretinopathy is associated with different conditions, characterized by exposure to increased levels of endogenous or exogenous glucocorticoids. In fact, central serous chorioretinopathy has been described in patients with endogenous Cushing's syndrome. It is also prevalent in patients with type-A behavior, and following stressful events, and pregnancy probably represents a risk factor for central serous chorioretinopathy; these conditions are characterized by endogenous hypercortisolism. In addition, many cases of central serous chorioretinopathy have been described during or following treatment with glucocorticoids, administrated by any route, for various systemic or ocular conditions. Central serous chorioretinopathy, when related to the exposure to exogenous glucocorticoids, has a less prominent male predilection, presents more often with a chronic or atypical form, and is frequently bilateral. Furthermore, treatment of central serous chorioretinopathy with glucocorticoids was found to exacerbate the clinical picture. Based on these observations it could be suggested that glucocorticoids may be involved in the development of central serous chorioretinopathy, even though the exact pathogenic mechanism remains unclear. Glucocorticoids should not be used in the treatment of central serous chorioretinopathy and central serous chorioretinopathy should be added to the list of ocular complications of glucocorticoids.

The presence of a circadian variation of both intraocular pressure (IOP) and aqueous humor flow has been demonstrated in several studies. It must therefore be considered important to monitor IOP and evaluate the efficacy of ocular hypotensive drugs over the 24 hours of the day. The efficacy of latanoprost on IOP during both day and night has been evaluated and the most important results from four such studies are reviewed. The studies reviewed here clearly demonstrate that topical administration of latanoprost 0.005% once daily provided a steady reduction of the IOP during both day and night. Given as a single dose to healthy volunteers, latanoprost resulted in a sustained effect with a significant IOP reduction over 24 hours, and the reduction was still present, however less pronounced, even after 48 hours. Latanoprost administered once daily for 4 weeks to patients with glaucoma or ocular hypertension was more effective in reducing the IOP over 24 hours than timolol gel solution 0.5% once daily, timolol aqueous solution 0.5% twice daily, or dorzolamide 2% three times daily. Latanoprost applied once daily thus provided a better effect on the IOP together with a stable and sustained IOP reduction during both day and night.

Objective: To study the intraocular pressure (IOP) reduction of latanoprost or timolol in a heterogeneous global population. Methods: A total of 1,389 glaucoma or ocular hypertensive patients treated with 0.005% latanoprost once daily (n = 737) or 0.5% timolol twice daily (n = 652) from eight clinical trials were included. After 3–6 months of treatment, the IOP was analyzed with use of analysis of covariance. Results: Latanoprost or timolol gave statistically significant mean diurnal IOP reduction in the African-American, Asian, Caucasian, and Mexican patients, latanoprost with 7.9 mm Hg and timolol with 6.4 mm Hg. The Asian and Mexican patients showed a larger difference in mean diurnal IOP reduction with use of the two drugs (range 1.8–3.1 mm Hg) than the European and U.S. patients (range 0.6–1.7 mm Hg, p = 0.030). Latanoprost produced similar mean diurnal IOP reduction in patients with and without previous glaucoma treatment other than prostaglandins. Conclusion: Latanoprost or timolol statistically significantly reduced the mean diurnal IOP in a heterogenous global population in eight clinical trials. The degree of reduction appeared to be clinically useful. The greatest difference in the mean diurnal IOP-lowering effect of latanoprost or timolol was observed in Mexican and Asian clinical trials.

Two independent, prospective trials were recently conducted to assess the efficacy of latanoprost in reducing intraocular pressure (IOP) in patients with primary angle-closure glaucoma (PACG). The first study was a 2-week, randomized, double-masked comparison of latanoprost treatment and timolol treatment in patients with PACG. Patients were randomized to one of two parallel treatment groups, receiving either placebo in the morning and latanoprost 0.005% in the evening, or timolol 0.5% twice daily. The mean IOP reduction in latanoprost group was 8.8 +/- 1.1 mm Hg (mean +/- SEM, p0.001; 34.2%) from a mean baseline IOP of 25.7 +/- 0.9 mm Hg, and the corresponding figures for the timolol group were 5.7 +/- 0.9 mm Hg (p0.001; 22.6%) from a mean baseline IOP of 25.2 +/- 1.1 mm Hg. A significantly greater IOP reduction of 3.1 +/- 1.5 mm Hg (95% confidence interval: 0.1 to 6.0) was achieved in the latanoprost group compared to the timolol treatment group (p = 0.04). In the second study, latanoprost 0.005% once a day was added adjunctively to PACG patients with persistently elevated IOP after iridectomy, despite treatment with beta-blockers and pilocarpine. The IOP decreased by about 21% during the first 3 months, and showed a reduction of about 36% at the end of 1 year. At the 1-year follow-up, IOP was20 mm Hg in all eyes. In both studies, latanoprost was well tolerated with few adverse events. These results demonstrate that latanoprost is effective in reducing IOP in patients with PACG.

Latanoprost therapy can lead to iris darkening in susceptible individuals, particularly those with hazel eyes. Concerns have been raised about whether latanoprost, and for that matter other prostanoids, may have a harmful effect on the iris. In addition, it is unknown whether latanoprost causes increased pigmentation of the outflow pathways that might eventually lead to blockage and a type of pigmentary glaucoma. The present study summarizes findings from the authors' own laboratories on the effects of latanoprost as seen by light and electron microscopy of the iris and outflow tissues and reviews the as yet limited, relevant literature. The findings support the proposal that latanoprost-induced eye color change is likely to be due to an increased amount of melanin within iris stromal melanocytes rather than any increase in melanocyte numbers, although many aspects of the darkening process remain obscure. No marked pathological changes were found in the latanoprost-treated iris; however, the numbers of specimens examined by us to date are still small (40 specimens in all) and those with latanoprost-induced darkening are even fewer (18). In the authors' experience, pigmentation of the outflow system in latanoprost-treated eyes was no greater than in eyes with primary open-angle glaucoma without prostanoid treatment, but there have been only limited numbers of trabeculectomy specimens examined. Concerns about latanoprost producing meshwork hyperpigmentation and pigmentary glaucoma are discussed.