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Eye (London, England) May 2018The first descriptions of ageing macula disorder (AMD), be it under other names, appeared in 1855 and 1868. The earliest accounts of AMD linked the presence of drusen... (Review)
Review
The first descriptions of ageing macula disorder (AMD), be it under other names, appeared in 1855 and 1868. The earliest accounts of AMD linked the presence of drusen with visual loss. It took a century before these connections between drusen and AMD were generally accepted by medical science and in clinical articles. The first signs of AMD appear in the region of the choriocapillaris, Bruch's membrane and the retinal pigment epithelium. The pathogenesis of drusen and of AMD is still uncertain. This is reflected in the wide variation in nomenclature of both, since the first publications.
Topics: History, 19th Century; History, 20th Century; History, 21st Century; Humans; Macular Degeneration; Retinal Drusen; Terminology as Topic
PubMed: 29424832
DOI: 10.1038/eye.2017.298 -
Survey of Ophthalmology 2002Optic disk drusen occur in 3.4 to 24 per 1,000 population and are bilateral in approximately 75%. Disturbance in the axonal metabolism in the presence of a small scleral... (Review)
Review
Optic disk drusen occur in 3.4 to 24 per 1,000 population and are bilateral in approximately 75%. Disturbance in the axonal metabolism in the presence of a small scleral canal--regardless of eyelength--is considered responsible for the development. The drusen increase in size, becoming more visible with age due to continuing calcium apposition, and they are associated with visual field defects in a considerable number of patients. Patients do not usually notice these defects, despite their progressive nature over the years, and this indicates an insidious course. A correct diagnosis of optic disk drusen is mandatory, although effective treatment is not yet available. It is most important to differentiate optic disk drusen from papilledema in order to avoid unnecessary neurological examinations, but also to avoid overlooking genuine neurologic disorders. Because optic disk drusen can cause severe visual field defects, patients require individual consultation regarding work issues and whether or not to drive. Optic disk drusen can be accompanied by vascular complications as well. In some cases these vascular changes--for example, choroidal neovascularization--are treatable. Patients with optic disk drusen should undergo regular visual field, IOP, and nerve fiber layer examinations. In patients with deteriorating visual field and borderline IOP, we recommend antiglaucomatous therapy.
Topics: Diagnosis, Differential; Diagnostic Techniques, Ophthalmological; Fluorescein Angiography; Fundus Oculi; Humans; Optic Disk Drusen; Tomography; Visual Acuity; Visual Fields
PubMed: 12504737
DOI: 10.1016/s0039-6257(02)00357-0 -
Survey of Ophthalmology 2016Optic disk drusen occur in 0.4% of children and consist of acellular intracellular and extracellular deposits that often become calcified over time. They are typically... (Review)
Review
Optic disk drusen occur in 0.4% of children and consist of acellular intracellular and extracellular deposits that often become calcified over time. They are typically buried early in life and generally become superficial, and therefore visible, later in childhood, at the average age of 12 years. Their main clinical significance lies in the ability of optic disk drusen, particularly when buried, to simulate true optic disk edema. Misdiagnosing drusen as true disk edema may lead to an invasive and unnecessary workup for elevated intracranial pressure. Ancillary testing, including ultrasonography, fluorescein angiography, fundus autofluorescence, and optical coherence tomography, may aid in the correct diagnosis of optic disk drusen. Complications of optic disk drusen in children include visual field defects, hemorrhages, choroidal neovascular membrane, nonarteritic anterior ischemic optic neuropathy, and retinal vascular occlusions. Treatment options for these complications include ocular hypotensive agents for visual field defects and intravitreal anti-vascular endothelial growth factor agents for choroidal neovascular membranes. In most cases, however, children with optic disk drusen can be managed by observation with serial examinations and visual field testing once true optic disk edema has been excluded.
Topics: Child; Fluorescein Angiography; Fundus Oculi; Global Health; Humans; Incidence; Optic Disk; Optic Disk Drusen; Tomography, Optical Coherence; Visual Acuity; Visual Fields
PubMed: 27033945
DOI: 10.1016/j.survophthal.2016.03.007 -
Japanese Journal of Ophthalmology Jan 2023Drusen are extracellular material considered a precursor lesion to advanced age-related macular degeneration (AMD), located either on the retinal pigment epithelium... (Review)
Review
Drusen are extracellular material considered a precursor lesion to advanced age-related macular degeneration (AMD), located either on the retinal pigment epithelium (RPE) or the sub-RPE; they contain various proteins associated with inflammation and lipids. Previous studies suggest that the lifecycle of drusen varies depending on drusen type and size. In general, conventional drusen grow and aggregate/coalesce in the first stage, and in the second stage, they regress with or without showing RPE atrophy. The risk of advanced AMD also varies depending on the drusen and drusenoid deposit types' along with their size and RPE abnormalities. In eyes with macular neovascularization (MNV), specific drusen/drusenoid deposits are closely associated with the MNV subtype. Recently, pachychoroid-associated drusen (pachydrusen) were proposed and clinical findings regarding this entity have been accumulating, as more attention is focused on drusen as well as pachychoroid diseases. With the advance in imaging modalities, various modalities can show specific characteristics depending on drusen types. To assess the risk of advanced AMD, it is essential for physicians to have accurate clinical knowledge about each druse/drusenoid lesion and correctly evaluate its imaging characteristics using multimodal imaging. This review summarizes the latest clinical knowledge about each druse/drusenoid lesions and documents their imaging characteristics on multimodal imaging, allowing clinicians to better manage patients and stratify the risk of developing advanced AMD. The most representative cases are illustrated, which can be helpful in the differential diagnosis of drusen and drusenoid deposits.
Topics: Humans; Macular Degeneration; Retinal Drusen; Tomography, Optical Coherence; Retinal Pigment Epithelium; Multimodal Imaging; Fluorescein Angiography
PubMed: 36477878
DOI: 10.1007/s10384-022-00943-y -
Acta Ophthalmologica Aug 2016Age-related macular degeneration (AMD), the most common cause of visual loss after the age of 65, displays a degeneration of the retinal pigment epithelial (RPE) cells... (Review)
Review
Age-related macular degeneration (AMD), the most common cause of visual loss after the age of 65, displays a degeneration of the retinal pigment epithelial (RPE) cells and photoreceptors in the retinal centre (macula). The central macula (fovea) that contains mostly cone photoreceptors mediates the high visual acuity. Drusen maculopathy may lead to visual deterioration. Drusen are extracellular deposits of debris that accumulate on Bruch's membrane. Drusen attract inflammatory, immunological and vasoactive stimuli. RPE and photoreceptor cells overlying drusen exhibit biochemical and morphological signs of degeneration. Strong and intermittent light exposure (photons) induces the formation of free radicals in the very high oxygen tension milieu of the retina. The negative effects of irradiation stimulate accumulation of lipofuscin in RPE and photoreceptor cells leading to mitochondrial dysfunction and apoptotic cell death. A hydrophobic barrier is built up in Bruch's membrane reducing diffusion to the choroid. Hereditary and inflammatory factors modify the risk for AMD. There is a genetic dysregulation of the complement system leading to inappropriate complement activation. The genetic polymorphism of complement factor H (CFH) and age-related maculopathy susceptibilty 2 (ARMS2) increase the risk of progression to advanced AMD. The photoelectric effect creates free radicals, resulting in a continuous increase of lipofuscin formation and impairing mitochondrial activity. In addition, inflammation and complement dysregulation contribute to the formation of drusen and vasoproliferative reactions with neovascularization. Antioxidants neutralize reactive oxygen species and reduce lipofuscin accumulation in RPE and photoreceptor cells. For prophylactic treatment of drusen maculopathy, high doses of antioxidants such as vitamins C and E, lutein, zeaxanthine and zinc are used according to the Age-Related Eye Disease Study 2 (AREDS 2). The risk of developing advanced AMD was reduced by 27% at 10 years follow-up. No adverse events were noted.
Topics: Antioxidants; Humans; Macular Degeneration; Photoreceptor Cells, Vertebrate; Retinal Drusen; Retinal Pigment Epithelium; Risk Factors; Vision Disorders
PubMed: 27009526
DOI: 10.1111/aos.13011 -
Current Neurology and Neuroscience... Sep 2008Optic disc drusen are acellular calcific deposits occurring in small, crowded optic discs with abnormal vasculature. Evidence suggests axoplasmic transport alteration... (Review)
Review
Optic disc drusen are acellular calcific deposits occurring in small, crowded optic discs with abnormal vasculature. Evidence suggests axoplasmic transport alteration and axonal degeneration are involved in disc drusen formation. In affected patients, the number and size of disc drusen are highly variable, and the drusen may be visible near the disc surface or buried within the disc, causing them to appear as pseudopapilledema. B-scan echography is the most sensitive method for detecting disc drusen. Most patients with disc drusen are asymptomatic, but progressive visual field loss and vascular complications, including anterior ischemic optic neuropathy and choroidal neovascularization, may occur. Optic disc drusen have no established effective treatment. Diagnosing disc drusen correctly is important to avoid unnecessary work-up and to avoid overlooking potential serious conditions such as true papilledema. Disc drusen patients with more-than-expected visual field defects or progressive visual loss should have work-up to exclude other causes.
Topics: Adolescent; Adult; Aged; Axonal Transport; Calcinosis; Diagnosis, Differential; Fluorescein Angiography; Humans; Middle Aged; Mitochondria; Optic Disk Drusen; Optic Neuropathy, Ischemic; Papilledema; Prevalence; Retinal Diseases; Retinal Vessels; Vision Disorders; Visual Fields
PubMed: 18713576
DOI: 10.1007/s11910-008-0062-6 -
Journal of Clinical Medicine Apr 2024Drusen are one of the most characteristic pathologies of precursor lesion of age-related macular degeneration (AMD). Drusen comprise a yellowish white substance that... (Review)
Review
Drusen are one of the most characteristic pathologies of precursor lesion of age-related macular degeneration (AMD). Drusen comprise a yellowish white substance that accumulates typically under the retinal pigment epithelium (RPE), and their constituents are lipids, complement, amyloid, crystallin, and others. In the past, many researchers have focused on drusen and tried to elucidate the pathophysiology of AMD because they believed that disease progression from early AMD to advanced AMD might be based on drusen or drusen might cause AMD. In fact, it is well established that drusen are the hallmark of precursor lesion of AMD and a major risk factor for AMD progression mainly based on their size and number. However, the existence of advanced AMD without drusen has long been recognized. For example, polypoidal choroidal vasculopathy (PCV), which comprises the majority of AMD cases in Asians, often lacks drusen. Thus, there is the possibility that drusen might be no more than a biomarker of AMD and not a cause of AMD. Now is the time to reconsider the relationship between AMD and drusen. In this review, we focus on early AMD pathogenesis based on basic research from the perspective of cholesterol metabolism and hypoxic response in the retina, and we discuss the role of drusen.
PubMed: 38731137
DOI: 10.3390/jcm13092608 -
Journal Francais D'ophtalmologie 1990
Review
Topics: Humans; Prognosis; Retinal Drusen; Risk Factors; Time Factors
PubMed: 2081842
DOI: No ID Found -
Survey of Ophthalmology 1999Drusen are subretinal pigment epithelial deposits that are characteristic of but not uniquely associated with age-related macular degeneration (AMD). Age-related macular... (Review)
Review
Drusen are subretinal pigment epithelial deposits that are characteristic of but not uniquely associated with age-related macular degeneration (AMD). Age-related macular degeneration is associated with two types of drusen that have different clinical appearances and different prognoses. Hard drusen appear as small, punctate, yellow nodules and can precede the development of atrophic AMD. Areolar atrophy of the retinal pigment epithelium (RPE), choriocapillaris, and outer retina develop as the drusen disappear, but drusen can regress without evidence of atrophy. Soft drusen appear as large (usually larger than 63 microm in diameter), pale yellow or grayish-white, dome-shaped elevations that can resemble localized serous RPE detachments. They tend to precede the development of clinically evident RPE detachments and choroidal neovascularization. Drusen characteristics correlated with progression to exudative maculopathy include drusen number (five or more), drusen size (larger than 63 microm in diameter), and confluence of drusen. Focal hyperpigmentation in the macula and systemic hypertension also are associated with an increased risk of developing choroidal new vessels (CNVs). Large drusen are usually a sign of diffuse thickening of Bruch's membrane with basal linear deposit, a vesicular material that probably arises from the RPE, constitutes a diffusion barrier to water-soluble constituents in the plasma, results in lipidization of Bruch's membrane, and creates a potential cleavage plane between the RPE basement membrane and the inner collagenous layer of Bruch's membrane through which CNVs can grow. Disappearance of drusen spontaneously and in areas adjacent to laser photocoagulation scars was first noted by Gass (Gass JD: Arch Ophthalmol 90:206-217, 1973; Trans Am Acad Ophthalmol Otolaryngol 75:580-608, 1971). Subsequent reports have confirmed these observations. Photocoagulation-induced drusen regression might prevent patients with drusen from developing exudative maculopathy. The mechanism for spontaneous drusen regression probably involves RPE atrophy. The mechanism for photocoagulation-induced drusen regression is unknown. If photocoagulation-induced drusen regression is anatomically similar to atrophy-associated drusen regression, then the former will be associated with dissolution of basal linear deposit and a residuum of basal laminar deposit. Sarks and coworkers (Sarks JP, Sarks SH, Killingsworth MC: Eye 11:515-522, 1997) proposed that this in turn will eliminate the potential cleavage plane between the RPE basement membrane and inner collagenous layer of Bruch's membrane through which CNVs grow, thus retarding the growth of CNVs.
Topics: Bruch Membrane; Choroidal Neovascularization; Disease Progression; Exudates and Transudates; Fluorescein Angiography; Fundus Oculi; Humans; Laser Coagulation; Macular Degeneration; Pigment Epithelium of Eye; Retinal Drusen
PubMed: 10466585
DOI: 10.1016/s0039-6257(99)00072-7